[ { "text": "对儿童SARST细胞亚群的研究表明,与成人SARS相比,儿童细胞下降不明显,证明上述推测成立。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "bod", "entity": "SARST细胞亚群" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "成人SARS" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "细胞下降" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "细胞" } ] }, { "text": "研究证实,细胞减少与肺内病变程度及肺内炎性病变吸收程度密切相关。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "细胞减少" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "细胞" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "肺内病变" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "肺内炎性病变" } ] }, { "text": "因而儿童肺部病变较轻,肺内炎性病变吸收较快。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "肺部病变" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "肺内炎性病变" } ] }, { "text": "一般有明确SARS接触史。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "SARS" } ] }, { "text": "(二)临床症状、体征与成年人类似,起病急,主要表现为发热和咳嗽,热峰高,腋温多在39℃以上,发热持续时间平均超过一周。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "咳嗽" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "热峰高" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "腋温多在39℃以上" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "腋温" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "发热" } ] }, { "text": "可为不规则发热、稽留热或弛张热,但以不规则发热为多,可能与患儿应用退热药物导致热型不规律有关。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "不规则发热" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "稽留热" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "弛张热" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "不规则发热" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "退热药物" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "热型不规律" } ] }, { "text": "多数患儿咳嗽与发热同时出现,少数发热数天后始出现咳嗽。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "咳嗽" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "发热" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "咳嗽" } ] }, { "text": "咳嗽多为有痰咳嗽,少数为干咳,亦有少部分患儿整个病程中均无咳嗽。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "咳嗽" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "有痰咳嗽" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "痰" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "干咳" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "咳嗽" } ] }, { "text": "大多数患儿无咽痛、鼻塞或流涕等典型上呼吸道病毒感染症状。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "咽痛" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "咽" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "鼻塞" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "鼻" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "流涕" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "上呼吸道病毒感染" } ] }, { "text": "除个别年长患儿诉胸痛和头痛外,大多无肌肉酸痛、寒战、头痛、胸痛等,与成年人报道不同。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "胸痛" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "胸" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "头痛" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "头" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "肌肉酸痛" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肌肉" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "寒战" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "头痛" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "头" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "胸痛" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "胸" } ] }, { "text": "阳性体征主要表现在呼吸系统,多数患儿肺部可闻及啰音,啰音可出现在疾病早期或中期,多为细湿啰音,啰音出现部位多与胸部X线片显示的肺实变部位一致,可以双侧或单侧,随着疾病好转而消失,少数患儿始终无啰音、呼吸音减弱或管状呼吸音等病理性呼吸音出现。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺部" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "肺部可闻及啰音" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "啰音" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "细湿啰音" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "啰音" }, { "start_idx": 55, "end_idx": 59, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "肺实变" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "啰音" }, { "start_idx": 99, "end_idx": 103, "type": "sym", "entity": "呼吸音减弱" }, { "start_idx": 105, "end_idx": 109, "type": "sym", "entity": "管状呼吸音" }, { "start_idx": 111, "end_idx": 116, "type": "sym", "entity": "病理性呼吸音" } ] }, { "text": "【实验室检查】儿童SARS的诊治要注意实验室检查资料的收集和分析。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "儿童SARS" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "实验室检查" } ] }, { "text": "(一)全国儿童严重呼吸综合征诊疗方案(试行)检查包括:1.三大常规及结核菌素实验(血常规须在入院后连续3天动态检测)。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "儿童严重呼吸综合征" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "三大常规" }, { "start_idx": 34, "end_idx": 39, "type": "pro", "entity": "结核菌素实验" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "血常规" }, { "start_idx": 46, "end_idx": 56, "type": "pro", "entity": "入院后连续3天动态检测" } ] }, { "text": "2.正、侧位胸部X线片检查(早期每天1次,连续3~4次)。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "正、侧位胸部X线片检查" } ] }, { "text": "3.心电图检查,阳性结果定期复查。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "心电图检查" } ] }, { "text": "4.支原体抗体、衣原体抗体检测,有关细菌和病毒学检测。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "pro", "entity": "支原体抗体、衣原体抗体检测" }, { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "细菌和病毒学检测" } ] }, { "text": "5.血沉、C反应蛋白、心肌酶、肝功能、肾功能、血电解质检测。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "血沉" }, { "start_idx": 5, "end_idx": 9, "type": "ite", "entity": "C反应蛋白" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "心肌酶" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "肝功能" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "肾功能" }, { "start_idx": 23, "end_idx": 28, "type": "ite", "entity": "血电解质检测" } ] }, { "text": "6.对重症者进行血气分析。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "血气分析" } ] }, { "text": "(二)临床常规进行的实验室检查1.血常规应每1~2天一次,必要时每天一次,恢复期适当延长间隔时间。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "实验室检查" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "血常规" } ] }, { "text": "血常规的动态变化是本病的特点之一,是重要的诊断依据,典型病例其外周血白细胞在病情进展期呈进行性下降。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "血常规" }, { "start_idx": 31, "end_idx": 48, "type": "sym", "entity": "外周血白细胞在病情进展期呈进行性下降" }, { "start_idx": 31, "end_idx": 36, "type": "ite", "entity": "外周血白细胞" } ] }, { "text": "2.在没有准确的病原学检查依据之前,定期检查胸部X线片具有重要的诊断学意义。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "病原学检查" }, { "start_idx": 20, "end_idx": 26, "type": "pro", "entity": "检查胸部X线片" } ] }, { "text": "3.心电图应常规查EKG。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "心电图" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "查EKG" } ] }, { "text": "SARS患儿有部分出现心脏损害,对有心脏损害者应进行床边动态心电监护。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "SARS" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "心脏" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "心脏损害" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "心脏" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心脏损害" }, { "start_idx": 26, "end_idx": 33, "type": "pro", "entity": "床边动态心电监护" } ] }, { "text": "4.咽拭子冠状病毒抗原测定及血冠状病毒抗原测定建立RT-PCR检测冠状病毒抗原。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "咽拭子冠状病毒抗原测定" }, { "start_idx": 14, "end_idx": 22, "type": "pro", "entity": "血冠状病毒抗原测定" }, { "start_idx": 25, "end_idx": 38, "type": "pro", "entity": "RT-PCR检测冠状病毒抗原" } ] }, { "text": "注意在发热早期,病毒血症期留标本。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "病毒血症" } ] }, { "text": "咽拭子病毒分离查呼吸道合胞病毒、A型流感病毒、B型流感病毒、肠道病毒、拉萨热病毒、汉坦病毒、腺病毒,抽血查支原体抗体(MP-IgM)、衣原体抗体(CP-IgM)对排除其他病原肺炎是必要的。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "咽拭子病毒分离" }, { "start_idx": 8, "end_idx": 14, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 16, "end_idx": 21, "type": "mic", "entity": "A型流感病毒" }, { "start_idx": 23, "end_idx": 28, "type": "mic", "entity": "B型流感病毒" }, { "start_idx": 30, "end_idx": 33, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "拉萨热病毒" }, { "start_idx": 41, "end_idx": 44, "type": "mic", "entity": "汉坦病毒" }, { "start_idx": 46, "end_idx": 48, "type": "mic", "entity": "腺病毒" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "抽血" }, { "start_idx": 53, "end_idx": 57, "type": "mic", "entity": "支原体抗体" }, { "start_idx": 59, "end_idx": 64, "type": "mic", "entity": "MP-IgM" }, { "start_idx": 67, "end_idx": 71, "type": "mic", "entity": "衣原体抗体" }, { "start_idx": 73, "end_idx": 78, "type": "bod", "entity": "CP-IgM" }, { "start_idx": 87, "end_idx": 88, "type": "dis", "entity": "肺炎" } ] }, { "text": "5.痰细菌培养加药敏、血培养致病性球杆菌加药敏有助于排除或诊断细菌感染。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "痰细菌培养加药敏" }, { "start_idx": 11, "end_idx": 22, "type": "pro", "entity": "血培养致病性球杆菌加药敏" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "细菌感染" } ] }, { "text": "6.腹泻患者加做大便冠状病毒、轮状病毒、腺病毒抗原测定。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "腹泻" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "腹" }, { "start_idx": 8, "end_idx": 26, "type": "pro", "entity": "大便冠状病毒、轮状病毒、腺病毒抗原测定" } ] }, { "text": "7.PPD皮试有助于排除或诊断肺部结核感染。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "PPD皮试" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "肺部结核感染" } ] }, { "text": "8.查血沉、C反应蛋白、冷凝集试验,发热超过一周者加做肥达试验、外斐试验、抗溶血素链球菌“O”、类风湿因子(RF),有助于鉴别诊断。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "血沉" }, { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "C反应蛋白" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "冷凝集试验" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "发热" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "肥达试验" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "外斐试验" }, { "start_idx": 37, "end_idx": 46, "type": "ite", "entity": "抗溶血素链球菌“O”" }, { "start_idx": 48, "end_idx": 52, "type": "ite", "entity": "类风湿因子" }, { "start_idx": 54, "end_idx": 55, "type": "ite", "entity": "RF" } ] }, { "text": "9.查T细胞功能及免疫球蛋白水平有助于了解患儿免疫功能的变化。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "T细胞功能" }, { "start_idx": 9, "end_idx": 15, "type": "ite", "entity": "免疫球蛋白水平" } ] }, { "text": "10.起病初期查脏器功能指标并定期复查,包括谷丙转氨酶、谷草转氨酶、碱性磷酸酶、γ-谷氨酰转肽酶、总蛋白、清蛋白、球蛋白、清蛋白/球蛋白比值、总胆红素、间接胆红素、直接胆红素、葡萄糖、尿素氮、尿酸、肌酐、肌酸激酶同工酶(CK-MB)、乳酸脱氢酶、淀粉酶。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "ite", "entity": "脏器功能指标" }, { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "谷丙转氨酶" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "谷草转氨酶" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "碱性磷酸酶" }, { "start_idx": 40, "end_idx": 47, "type": "ite", "entity": "γ-谷氨酰转肽酶" }, { "start_idx": 49, "end_idx": 51, "type": "ite", "entity": "总蛋白" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "清蛋白" }, { "start_idx": 57, "end_idx": 59, "type": "ite", "entity": "球蛋白" }, { "start_idx": 61, "end_idx": 69, "type": "ite", "entity": "清蛋白/球蛋白比值" }, { "start_idx": 71, "end_idx": 74, "type": "ite", "entity": "总胆红素" }, { "start_idx": 76, "end_idx": 80, "type": "ite", "entity": "间接胆红素" }, { "start_idx": 82, "end_idx": 86, "type": "ite", "entity": "直接胆红素" }, { "start_idx": 88, "end_idx": 90, "type": "ite", "entity": "葡萄糖" }, { "start_idx": 92, "end_idx": 94, "type": "ite", "entity": "尿素氮" }, { "start_idx": 96, "end_idx": 97, "type": "ite", "entity": "尿酸" }, { "start_idx": 99, "end_idx": 100, "type": "ite", "entity": "肌酐" }, { "start_idx": 102, "end_idx": 108, "type": "ite", "entity": "肌酸激酶同工酶" }, { "start_idx": 110, "end_idx": 114, "type": "ite", "entity": "CK-MB" }, { "start_idx": 117, "end_idx": 121, "type": "ite", "entity": "乳酸脱氢酶" }, { "start_idx": 123, "end_idx": 125, "type": "ite", "entity": "淀粉酶" } ] }, { "text": "11.查血气分析及电解质,有助于判断呼吸衰竭及电解质紊乱。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "血气分析" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "电解质" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "电解质紊乱" } ] }, { "text": "注意留急性期及恢复期双份血清,备冠状病毒抗体检测。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血清" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "冠状病毒抗体检测" } ] }, { "text": "【诊断】中华医学会儿科分会呼吸学组制定的儿童严重呼吸综合征诊断标准(试行)如下:(一)诊断条例1.流行病学史(1)与发病者有密切接触史或来自发病区域者;属群体发病者之一;有明确传染他人的证据者(密切接触者指2周内与SARS患者共同生活、学习、玩耍,或接触过SARS患者的呼吸道分泌物或体液者)。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dep", "entity": "儿科" }, { "start_idx": 20, "end_idx": 28, "type": "dis", "entity": "儿童严重呼吸综合征" }, { "start_idx": 107, "end_idx": 110, "type": "dis", "entity": "SARS" }, { "start_idx": 128, "end_idx": 131, "type": "dis", "entity": "SARS" }, { "start_idx": 135, "end_idx": 140, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "体液" } ] }, { "text": "(2)发病前4周内曾到过或居住于SARS流行地区者。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "SARS" } ] }, { "text": "2.症状与体征起病急,以发热为首发症状,体温一般高于38.5℃,偶有畏寒;常无上呼吸道卡他症状;有咳嗽,多为干咳、少痰;可有胸闷气促,肺部听诊可闻干湿性啰音。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "发热" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "体温一般高于38.5℃" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "体温" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "偶有畏寒" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "上呼吸道卡他症状" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "咳嗽" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "干咳" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "少痰" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "痰" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "胸闷" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "胸" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "气促" }, { "start_idx": 67, "end_idx": 77, "type": "sym", "entity": "肺部听诊可闻干湿性啰音" }, { "start_idx": 67, "end_idx": 70, "type": "pro", "entity": "肺部听诊" } ] }, { "text": "年长儿可诉头痛、关节和肌肉酸痛、乏力等,可有腹泻。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "头痛" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "头" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "关节" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "关节和肌肉酸痛" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肌肉" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "乏力" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "腹泻" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "腹" } ] }, { "text": "3.实验室检查外周血白细胞计数一般不高,或降低;常有淋巴细胞计数减少;C反应蛋白<8mg/L。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "实验室检查" }, { "start_idx": 7, "end_idx": 22, "type": "sym", "entity": "外周血白细胞计数一般不高,或降低" }, { "start_idx": 7, "end_idx": 14, "type": "ite", "entity": "外周血白细胞计数" }, { "start_idx": 26, "end_idx": 31, "type": "ite", "entity": "淋巴细胞计数" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "淋巴细胞计数减少" }, { "start_idx": 35, "end_idx": 39, "type": "ite", "entity": "C反应蛋白" } ] }, { "text": "4.胸部X线检查归纳儿童SARS胸部X线片特点为:①单侧或双侧的局灶性非对称性浸润影,为斑片状、絮状或类圆形阴影,以肺下叶、肺门和肺野外带多见,应特别注意脊柱旁、心影后斑片状阴影;②胸部X线片改变在病程早期即可出现,进展较一般肺炎为快,阴影范围迅速扩大或从单侧变为双侧;③肺部阴影吸收较慢,肺纤维化、胸腔积液、肺门淋巴结肿大在儿童SARS患儿中较少见。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "儿童SARS" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "胸部X线" }, { "start_idx": 26, "end_idx": 70, "type": "sym", "entity": "单侧或双侧的局灶性非对称性浸润影,为斑片状、絮状或类圆形阴影,以肺下叶、肺门和肺野外带多见" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "肺下叶" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "肺门" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "肺野" }, { "start_idx": 77, "end_idx": 88, "type": "sym", "entity": "脊柱旁、心影后斑片状阴影" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "脊柱旁" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "心" }, { "start_idx": 91, "end_idx": 95, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 113, "end_idx": 114, "type": "dis", "entity": "肺炎" }, { "start_idx": 118, "end_idx": 133, "type": "sym", "entity": "阴影范围迅速扩大或从单侧变为双侧" }, { "start_idx": 136, "end_idx": 137, "type": "bod", "entity": "肺部" }, { "start_idx": 136, "end_idx": 143, "type": "sym", "entity": "肺部阴影吸收较慢" }, { "start_idx": 145, "end_idx": 148, "type": "dis", "entity": "肺纤维化" }, { "start_idx": 150, "end_idx": 153, "type": "dis", "entity": "胸腔积液" }, { "start_idx": 155, "end_idx": 161, "type": "dis", "entity": "肺门淋巴结肿大" }, { "start_idx": 163, "end_idx": 168, "type": "dis", "entity": "儿童SARS" } ] }, { "text": "5.抗生素药物治疗无明显效果。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "抗生素药物治疗" } ] }, { "text": "6.有条件的单位,可作SARS病毒抗体和(或)RT-PCR检测,以助诊断。", "entities": [ { "start_idx": 11, "end_idx": 30, "type": "pro", "entity": "SARS病毒抗体和(或)RT-PCR检测" } ] }, { "text": "(二)SARS诊断标准1.临床确立诊断标准患儿符合以下两条中的1条可确立诊断:(1)诊断条例1.(1)+2、+3、+4。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "SARS" } ] }, { "text": "(三)重症SARS诊断标准符合下列任何一项条件者可作出诊断:1.呼吸困难,发绀。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "SARS" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "发绀" } ] }, { "text": "2.低氧血症,吸氧3~5升/分条件下,动脉血氧分压<70mmHg,或脉搏容积血氧饱和度(SpO2)<0.93;或已可以诊断为急性肺损伤(ALI),氧合指数≤300,或急性呼吸窘迫综合征(ARDS)≤200。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "低氧血症" }, { "start_idx": 19, "end_idx": 24, "type": "ite", "entity": "动脉血氧分压" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "动脉血氧分压<70mmHg" }, { "start_idx": 34, "end_idx": 42, "type": "ite", "entity": "脉搏容积血氧饱和度" }, { "start_idx": 34, "end_idx": 59, "type": "sym", "entity": "脉搏容积血氧饱和度(SpO2)<0.93" }, { "start_idx": 44, "end_idx": 53, "type": "ite", "entity": "SpO2" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "ALI" }, { "start_idx": 79, "end_idx": 82, "type": "ite", "entity": "氧合指数" }, { "start_idx": 79, "end_idx": 86, "type": "sym", "entity": "氧合指数≤300" }, { "start_idx": 89, "end_idx": 97, "type": "dis", "entity": "急性呼吸窘迫综合征" }, { "start_idx": 99, "end_idx": 102, "type": "dis", "entity": "ARDS" } ] }, { "text": "3.肺部有多叶病变或X线胸片显示24~48小时病灶面积进展>50%者。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "肺部有多叶病变" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肺部" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "多叶" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "X线胸片" }, { "start_idx": 10, "end_idx": 32, "type": "sym", "entity": "X线胸片显示24~48小时病灶面积进展>50%" } ] }, { "text": "4.有休克表现者。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "休克" } ] }, { "text": "5.有多器官功能障碍综合征(MODS)表现者。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "多器官功能障碍综合征" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "MODS" } ] }, { "text": "【鉴别诊断】临床上要注意排除其他病毒性、支原体、衣原体、细菌性、真菌性肺炎,以及肺结核、流行性出血热、肺嗜酸性粒细胞浸润症等临床表现类似的呼吸系统疾患。", "entities": [ { "start_idx": 16, "end_idx": 36, "type": "dis", "entity": "病毒性、支原体、衣原体、细菌性、真菌性肺炎" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "肺结核" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "流行性出血热" }, { "start_idx": 51, "end_idx": 60, "type": "dis", "entity": "肺嗜酸性粒细胞浸润症" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "呼吸系统疾患" } ] }, { "text": "对临床疑似病例,应连续3天动态观察外周血象和胸部正、侧位X线片。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "外周血象" }, { "start_idx": 22, "end_idx": 30, "type": "pro", "entity": "胸部正、侧位X线片" } ] }, { "text": "儿童SARS应与以下的呼吸道感染疾病鉴别:(一)上呼吸道感染(感冒、扁桃体炎、咽炎、喉炎等)上呼吸道感染一般有喷嚏、流涕、鼻塞、咽喉疼痛、扁桃体肿大、声音嘶哑等,也可以发热、咳嗽,部分病例周围血白细胞也可减少,但肺部听诊无啰音,胸部X线片没有肺炎表现,并且数日后病情好转痊愈。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "儿童SARS" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "感冒" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "咽炎" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "喉炎" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "喷嚏" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "流涕" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "鼻塞" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "鼻" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "咽喉" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "咽喉疼痛" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "扁桃体" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "扁桃体肿大" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "发热" }, { "start_idx": 87, "end_idx": 88, "type": "sym", "entity": "咳嗽" }, { "start_idx": 94, "end_idx": 99, "type": "ite", "entity": "周围血白细胞" }, { "start_idx": 94, "end_idx": 103, "type": "sym", "entity": "周围血白细胞也可减少" }, { "start_idx": 106, "end_idx": 109, "type": "pro", "entity": "肺部听诊" }, { "start_idx": 111, "end_idx": 112, "type": "sym", "entity": "啰音" }, { "start_idx": 114, "end_idx": 118, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 121, "end_idx": 122, "type": "dis", "entity": "肺炎" } ] }, { "text": "(二)细菌性肺炎细菌性肺炎可有或无原发感染灶,咳嗽多伴有白痰、脓痰或小婴儿表现喉鸣,有发热,周围血白细胞数升高,核左移。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "原发感染灶" }, { "start_idx": 23, "end_idx": 32, "type": "sym", "entity": "咳嗽多伴有白痰、脓痰" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "痰" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "痰" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "喉" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "喉鸣" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "发热" }, { "start_idx": 46, "end_idx": 52, "type": "ite", "entity": "周围血白细胞数" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "周围血白细胞数升高" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "核" }, { "start_idx": 56, "end_idx": 58, "type": "sym", "entity": "核左移" } ] }, { "text": "两肺听诊可闻及干湿性啰音,胸部X线片两肺有斑片状模糊阴影,尤以双下肺明显,严重病例可合并胸腔积液或脓胸。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "两肺听诊可闻及干湿性啰音" }, { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "两肺听诊" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 13, "end_idx": 35, "type": "sym", "entity": "胸部X线片两肺有斑片状模糊阴影,尤以双下肺明显" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "两肺" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "双下肺" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "胸腔积液" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "脓胸" } ] }, { "text": "抗生素治疗有效,没有强的传染性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "(三)肺炎支原体肺炎肺炎支原体肺炎年长儿多见,也可有流行。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "肺炎支原体肺炎" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肺炎支原体肺炎" } ] }, { "text": "初为干咳,后转为顽固性剧咳,可有发热、头痛,肺部体征常不明显,胸部X线片改变却与体征不相符,常以肺门周围阴影增浓为突出的间质性肺炎改变,或均一的实变影。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "干咳" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "顽固性剧咳" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "发热" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "头痛" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "头" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肺部" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "肺部体征常不明显" }, { "start_idx": 31, "end_idx": 74, "type": "sym", "entity": "胸部X线片改变却与体征不相符,常以肺门周围阴影增浓为突出的间质性肺炎改变,或均一的实变影" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "肺门周围" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "间质性肺炎" } ] }, { "text": "大环内酯类抗生素有效,但近年出现耐药情况。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "大环内酯类抗生素" } ] }, { "text": "(四)呼吸道合胞病毒肺炎呼吸道合胞病毒肺炎多见于2岁以内,尤其以2~6个月婴儿多见。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "呼吸道合胞病毒肺炎" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "呼吸道合胞病毒肺炎" } ] }, { "text": "起病急,可不发热或有中、低热,咳嗽、喘息明显,呼吸困难,肺部体征出现早,可听到满肺喘鸣音和肺底部细湿啰音,胸部X线片显示小片状阴影,多伴有明显肺气肿征。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "中、低热" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "咳嗽、喘息明显" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肺部" }, { "start_idx": 28, "end_idx": 51, "type": "sym", "entity": "肺部体征出现早,可听到满肺喘鸣音和肺底部细湿啰音" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "满肺" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "肺底部" }, { "start_idx": 53, "end_idx": 57, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 53, "end_idx": 74, "type": "sym", "entity": "胸部X线片显示小片状阴影,多伴有明显肺气肿征" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "肺气肿" } ] }, { "text": "抗生素治疗无效,经对症支持疗法3~5天,喘息停止,病情逐渐好转,预后良好。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "对症支持疗法" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "喘息" } ] }, { "text": "(五)腺病毒肺炎腺病毒肺炎多见于6个月至2岁的小儿,常有稽留高热,全身中毒症状重,精神委靡,面色苍白,咳嗽,呼吸困难,发绀。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "腺病毒肺炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "腺病毒肺炎" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "稽留高热" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "全身中毒" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "精神委靡" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "面色苍白" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "面" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "咳嗽" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "发绀" } ] }, { "text": "肺部体征出现较晚,高热4~5天后方闻及两肺细湿啰音,肺部病变融合则有肺实变体征,胸部X线片改变出现较早,可见大小不等的片状阴影,或融合成大病灶,多见肺气肿。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "高热" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "两肺细湿啰音" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "两肺" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "肺部病变融合" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "肺实变" }, { "start_idx": 40, "end_idx": 70, "type": "sym", "entity": "胸部X线片改变出现较早,可见大小不等的片状阴影,或融合成大病灶" }, { "start_idx": 40, "end_idx": 44, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "肺气肿" } ] }, { "text": "常遗留下不可逆肺损伤,后遗症有肺不张、肺纤维化、支气管扩张、反复发作性肺炎等。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "不可逆肺损伤" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肺不张" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "肺纤维化" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "反复发作性肺炎" } ] }, { "text": "(六)真菌性肺炎真菌性肺炎常在许多全身性疾病基础上发生,如血液病、营养不良、结核病、免疫功能缺陷等,或继发于婴幼儿肺炎、支气管扩张症等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "真菌性肺炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "真菌性肺炎" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "血液病" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "营养不良" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "结核病" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "免疫功能缺陷" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "婴幼儿肺炎" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "支气管扩张症" } ] }, { "text": "起病较缓慢,病程迁延,更换多种抗生素无效,病情日益加重,常合并鹅口疮,皮肤或消化道等部位的真菌感染。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "抗生素" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 35, "end_idx": 48, "type": "dis", "entity": "皮肤或消化道等部位的真菌感染" } ] }, { "text": "可有发热、咳嗽、气促、发绀、精神委靡,年长儿可有无色胶冻样痰咳出,肺部听诊呼吸音减弱,可闻及管状呼吸音和中小水泡音,胸部X线片有点状阴影,可似粟粒性肺结核改变,亦可有棉絮状阴影或大片实变灶。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "发热" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "咳嗽" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "气促" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "发绀" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "精神委靡" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "无色胶冻样痰咳出" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "痰" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "肺部听诊" }, { "start_idx": 33, "end_idx": 56, "type": "sym", "entity": "肺部听诊呼吸音减弱,可闻及管状呼吸音和中小水泡音" }, { "start_idx": 58, "end_idx": 62, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 58, "end_idx": 93, "type": "sym", "entity": "胸部X线片有点状阴影,可似粟粒性肺结核改变,亦可有棉絮状阴影或大片实变灶" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "粟粒性肺结核" } ] }, { "text": "抗真菌药物治疗有效,如氟康唑、两性霉素B、伏立康唑等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "抗真菌药物治疗" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "氟康唑" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "伏立康唑" } ] }, { "text": "(七)衣原体肺炎根据抗原结构、对磺胺敏感性和包涵体性质等分为四种:沙眼衣原体(C.trachomatis,CT),肺炎衣原体(C.pneumonial,Cpn),鹦鹉热衣原体(C.pecorum),家畜衣原体(C.pecorum)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "衣原体肺炎" }, { "start_idx": 16, "end_idx": 17, "type": "dru", "entity": "磺胺" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "包涵体" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 39, "end_idx": 51, "type": "mic", "entity": "C.trachomatis" }, { "start_idx": 53, "end_idx": 54, "type": "mic", "entity": "CT" }, { "start_idx": 57, "end_idx": 61, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 63, "end_idx": 74, "type": "mic", "entity": "C.pneumonial" }, { "start_idx": 76, "end_idx": 78, "type": "mic", "entity": "Cpn" }, { "start_idx": 81, "end_idx": 86, "type": "mic", "entity": "鹦鹉热衣原体" }, { "start_idx": 88, "end_idx": 96, "type": "mic", "entity": "C.pecorum" }, { "start_idx": 99, "end_idx": 103, "type": "mic", "entity": "家畜衣原体" }, { "start_idx": 105, "end_idx": 113, "type": "mic", "entity": "C.pecorum" } ] }, { "text": "1.沙眼衣原体肺炎占婴儿肺炎的18.4%,多见于1~4月龄幼婴,可由沙眼衣原体感染的母亲垂直传染给婴儿。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "沙眼衣原体肺炎" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "婴儿肺炎" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "沙眼衣原体感染" } ] }, { "text": "起病较缓慢,先有上呼吸道感染症状,鼻塞、流涕,多数无发热或仅有低热,以后出现呼吸增快及明显咳嗽。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "鼻塞" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "鼻" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "流涕" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "低热" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "呼吸增快" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "明显咳嗽" } ] }, { "text": "50%以上病例伴有耳鼓膜外观异常,眼结膜炎,体格检查可闻及两肺细湿啰音或喘鸣音,胸部X线片呈广泛肺间质及肺泡浸润,常见过度充气。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "耳鼓膜" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "耳鼓膜外观异常" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "眼结膜炎" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "体格检查" }, { "start_idx": 22, "end_idx": 38, "type": "sym", "entity": "体格检查可闻及两肺细湿啰音或喘鸣音" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "两肺" }, { "start_idx": 40, "end_idx": 55, "type": "sym", "entity": "胸部X线片呈广泛肺间质及肺泡浸润" }, { "start_idx": 40, "end_idx": 44, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 46, "end_idx": 53, "type": "bod", "entity": "广泛肺间质及肺泡" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "过度充气" } ] }, { "text": "2.肺炎衣原体肺炎是1989年被正式命名的新衣原体种,现公认是5岁以上小儿及成人重要的肺炎病原之一。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "肺炎衣原体肺炎" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "衣原体" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "肺炎" } ] }, { "text": "肺炎衣原体肺炎起病缓慢,一般症状较轻。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肺炎衣原体肺炎" } ] }, { "text": "常伴咽炎、喉炎及鼻窦炎,多见咳嗽,并且持续时间长,可达3周,胸部听诊可闻及干湿啰音,胸片无特异性改变,多见单侧下叶浸润,可并发胸腔积液。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "咽炎" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "喉炎" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "咳嗽" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "胸部听诊可闻及干湿啰音" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "胸部听诊" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "胸片" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "单侧下叶浸润" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "单侧下叶" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "胸腔积液" } ] }, { "text": "3.鹦鹉热衣原体肺炎是人畜共患性疾病,人受感染主要是吸入含有鹦鹉热衣原体的鸟粪、粉尘或与病鸟接触而致病。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "鹦鹉热衣原体肺炎" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "人畜共患性疾病" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "感染" }, { "start_idx": 30, "end_idx": 35, "type": "mic", "entity": "鹦鹉热衣原体" } ] }, { "text": "多见于年长儿及成人,起病急,全身中毒症状明显,常有寒战、高热、头痛、肌痛、关节痛。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "全身中毒" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "寒战" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "高热" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "头痛" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "头" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "肌痛" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肌" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "关节痛" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "关节" } ] }, { "text": "咳嗽明显,初为干咳,以后频繁剧咳,咳脓性痰,偶有血丝痰,呕吐,畏光,肝脾肿大,贫血,两肺可闻及啰音,胸部X线片早期有弥漫性肺间质浸润,从肺门向周边特别向下肺野伸展。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "咳嗽明显" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "干咳" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "频繁剧咳" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "咳脓性痰" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "痰" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "偶有血丝痰" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "痰" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "呕吐" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "畏光" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肝" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "脾" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "贫血" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "两肺可闻及啰音" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "两肺" }, { "start_idx": 50, "end_idx": 80, "type": "sym", "entity": "胸部X线片早期有弥漫性肺间质浸润,从肺门向周边特别向下肺野伸展" }, { "start_idx": 50, "end_idx": 54, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "肺间质" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "肺门" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "下肺野" } ] }, { "text": "实验室检查:可进行咽分泌物、痰等涂片直接镜检,或通过衣原体分离、血清学检查、PCR技术等方法检测。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "实验室检查" }, { "start_idx": 9, "end_idx": 21, "type": "pro", "entity": "咽分泌物、痰等涂片直接镜检" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "衣原体分离" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "血清学检查" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "PCR技术" } ] }, { "text": "治疗以选用大环内酯类抗生素,沙眼衣原体肺炎也可用磺胺二甲基异唑,年长儿和成人用氟喹诺酮类效果也很好。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dru", "entity": "大环内酯类抗生素" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "沙眼衣原体肺炎" }, { "start_idx": 24, "end_idx": 30, "type": "dru", "entity": "磺胺二甲基异唑" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "氟喹诺酮类" } ] }, { "text": "(八)小儿军团菌病我国1982年首次报告了军团菌病,此病是由军团杆菌引起的感染性疾病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "小儿军团菌病" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "军团菌病" }, { "start_idx": 30, "end_idx": 33, "type": "mic", "entity": "军团杆菌" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "小儿受军团菌感染后容易发生肺炎。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "军团菌感染" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肺炎" } ] }, { "text": "临床以肺炎表现为主的多系统、多器官受损,从而具有多种多样的临床表现。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肺炎" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "多系统、多器官受损" } ] }, { "text": "病初期酷似感冒或重感冒,表现全身不适、头痛、肌痛、疲乏、发热、咳嗽、流涕,若能很快自限则好转痊愈,若病程不自限,且进一步恶化,患儿就有寒战、高热、呼吸困难、咳脓痰至咯血,肺部可出现干湿啰音和实变体征。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "感冒" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "重感冒" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "头痛" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "头" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "肌痛" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肌" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "疲乏" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "发热" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "咳嗽" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "流涕" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "寒战" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "高热" }, { "start_idx": 73, "end_idx": 76, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 78, "end_idx": 80, "type": "sym", "entity": "咳脓痰" }, { "start_idx": 80, "end_idx": 80, "type": "bod", "entity": "痰" }, { "start_idx": 82, "end_idx": 83, "type": "sym", "entity": "咯血" }, { "start_idx": 83, "end_idx": 83, "type": "bod", "entity": "血" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "肺部" }, { "start_idx": 85, "end_idx": 98, "type": "sym", "entity": "肺部可出现干湿啰音和实变体征" } ] }, { "text": "实验室检查周围血白细胞明显升高,痰等分泌物培养有军团菌生长,血清特异性抗体IgM和IgG阳性,PCR检测敏感性可高达100%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "实验室检查" }, { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "周围血白细胞" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "周围血白细胞明显升高" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "痰等分泌物培养" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "军团菌" }, { "start_idx": 30, "end_idx": 45, "type": "sym", "entity": "血清特异性抗体IgM和IgG阳性" }, { "start_idx": 30, "end_idx": 43, "type": "ite", "entity": "血清特异性抗体IgM和IgG" }, { "start_idx": 47, "end_idx": 51, "type": "pro", "entity": "PCR检测" } ] }, { "text": "治疗以红霉素为首选,疗程3周。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "红霉素" } ] }, { "text": "【治疗】2003年5月全国儿童严重呼吸综合征诊疗方案(试行)如下。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "儿童严重呼吸综合征" } ] }, { "text": "1.一般治疗环境通风、休息、多饮水、加强营养。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "环境通风" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "休息" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "多饮水" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "加强营养" } ] }, { "text": "2.高热发热超过38.5℃者,全身酸痛明显者,可使用物理降温措施或给予解热镇痛药,如布洛芬(低于38.5℃者给半量)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "高热" }, { "start_idx": 4, "end_idx": 12, "type": "sym", "entity": "发热超过38.5℃" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "全身酸痛明显" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "全身" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "物理降温措施" }, { "start_idx": 35, "end_idx": 39, "type": "dru", "entity": "解热镇痛药" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "布洛芬" } ] }, { "text": "禁用阿司匹林。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "3.止咳化痰。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "止咳化痰" } ] }, { "text": "4.抗病毒可用利巴韦林10~15mg/(kg•d),静脉滴注或口服7~10天。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "病毒" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "利巴韦林" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "口服" } ] }, { "text": "5.抗生素大环内酯类抗生素如阿奇霉素10mg/(kg•d),可静脉滴注5天,停药3天,为一个疗程;根据病情可考虑再用1~2个疗程。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "抗生素" }, { "start_idx": 5, "end_idx": 12, "type": "dru", "entity": "大环内酯类抗生素" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "若出现其他细菌感染,视情况选用第二代或第三代头孢菌素。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "细菌感染" }, { "start_idx": 15, "end_idx": 25, "type": "dru", "entity": "第二代或第三代头孢菌素" } ] }, { "text": "6.免疫调节剂丙种球蛋白400mg/(kg•d),静脉滴注3~5天。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "免疫调节剂" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "重症患儿可考虑用血浆,10~20ml/(kg•d),连用3~5天。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dru", "entity": "血浆" } ] }, { "text": "7.肾上腺皮质激素严格除外禁忌证后,重症患儿(高热、咳重、气促、胸部X线片改变明显且进展快,或伴有其他脏器损害)在加强支持治疗的前提下可选用。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "高热" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "咳重" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "气促" }, { "start_idx": 32, "end_idx": 44, "type": "sym", "entity": "胸部X线片改变明显且进展快" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "其他脏器损害" } ] }, { "text": "如甲泼尼龙2mg/(kg•d),用2~3天;或选用地塞米松每次0.1~0.2mg/kg。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "甲泼尼龙" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "地塞米松" } ] }, { "text": "8.其他营养心肌药物,如能量合剂和维生素C;以及护肝药物等。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "营养心肌药物" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "能量合剂" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "维生素C" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "护肝药物" } ] }, { "text": "9.及时早期给氧及保持呼吸道通畅。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "及时早期给氧" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "保持呼吸道通畅" } ] }, { "text": "10.中医中药治疗,应辨证施治。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "中医中药治疗" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "辨证施治" } ] }, { "text": "11.呼吸衰竭及其他并发症请ICU及有关科室会诊,尤应注意呼吸机的使用指征和方法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 14, "end_idx": 16, "type": "dep", "entity": "ICU" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "呼吸机" } ] }, { "text": "SARS病理生理过程的关键是全身炎症反应综合征(SIRS)的不断放大,发生级联反应(cascade),导致“细胞因子风暴”和“炎症介质瀑布”;也可能发生“肠道细菌移位”和“肠源性内毒素血症”,进而发生感染性休克和组织器官损伤,导致MODS和MOF。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "SARS" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "全身炎症反应综合征" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "SIRS" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "细胞因子风暴" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "细胞因子" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "炎症介质" }, { "start_idx": 63, "end_idx": 68, "type": "sym", "entity": "炎症介质瀑布" }, { "start_idx": 77, "end_idx": 82, "type": "sym", "entity": "肠道细菌移位" }, { "start_idx": 77, "end_idx": 80, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 86, "end_idx": 93, "type": "dis", "entity": "肠源性内毒素血症" }, { "start_idx": 100, "end_idx": 104, "type": "dis", "entity": "感染性休克" }, { "start_idx": 106, "end_idx": 111, "type": "dis", "entity": "组织器官损伤" }, { "start_idx": 115, "end_idx": 118, "type": "dis", "entity": "MODS" }, { "start_idx": 120, "end_idx": 122, "type": "dis", "entity": "MOF" } ] }, { "text": "所以治疗的关键就是想办法阻断级联反应及预防“肠道细菌移位”和“肠源性内毒素血症”,因此强调抑制炎症因子的药物和肠道抗生素的应用。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "阻断级联反应" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "肠道细菌移位" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "肠源性内毒素血症" }, { "start_idx": 45, "end_idx": 53, "type": "dru", "entity": "抑制炎症因子的药物" }, { "start_idx": 55, "end_idx": 59, "type": "dru", "entity": "肠道抗生素" } ] }, { "text": "另外,CT及成人SARS患者尸解表明,SARS患者的肺纤维化不是间质的纤维化,而是肺内炎症及超敏反应产生纤维黏液性渗出所致的气管内的堵塞,肾上腺皮质激素可轻度减轻堵塞,但意义不大,关键是早期的强有力的呼吸道护理和支持治疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "CT" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "成人SARS" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "尸解" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "SARS" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "肺纤维化" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肺" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "间质的纤维化" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "间质" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "肺内炎症" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "超敏反应" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "纤维黏液性渗出" }, { "start_idx": 62, "end_idx": 67, "type": "sym", "entity": "气管内的堵塞" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "气管内" }, { "start_idx": 69, "end_idx": 75, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 81, "end_idx": 82, "type": "sym", "entity": "堵塞" }, { "start_idx": 93, "end_idx": 109, "type": "pro", "entity": "早期的强有力的呼吸道护理和支持治疗" } ] }, { "text": "大量给予肾上腺皮质激素还可能加重机体的应激状态,也会造成严重的继发性感染使病情加重。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "继发性感染" } ] }, { "text": "而且早期使用肾上腺皮质激素无预防多器官损伤的作用,故根据儿科病例的情况,不宜常规使用激素。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "多器官损伤" }, { "start_idx": 28, "end_idx": 29, "type": "dep", "entity": "儿科" }, { "start_idx": 42, "end_idx": 43, "type": "dru", "entity": "激素" } ] }, { "text": "建议应用肾上腺皮质激素的指征为:①有严重中毒症状;②达到重症病例标准者。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "中毒" } ] }, { "text": "如果其他治疗措施有效,尽量不用糖皮质激素。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "适当补充液体及维生素,鼓励患儿多吃水果及富含蛋白质的食物。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "适当补充液体及维生素" }, { "start_idx": 15, "end_idx": 27, "type": "pro", "entity": "多吃水果及富含蛋白质的食物" } ] }, { "text": "如果患儿营养状态较差,应给予静脉营养支持治疗,尤其对于婴幼儿喂养困难者,要特别注意静脉营养支持治疗,适当补充维生素、氨基酸及脂肪乳。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "营养状态较差" }, { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "静脉营养支持治疗" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "喂养困难" }, { "start_idx": 41, "end_idx": 48, "type": "pro", "entity": "静脉营养支持治疗" }, { "start_idx": 50, "end_idx": 64, "type": "pro", "entity": "适当补充维生素、氨基酸及脂肪乳" } ] }, { "text": "脂肪乳的运用,要注意患儿的肝功能,尤其是小婴儿,因为部分患儿可并发肝损害。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脂肪乳" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肝功能" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "肝损害" } ] }, { "text": "在肺实变期,避免用力和剧烈咳嗽。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "肺实变" }, { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "避免用力和剧烈咳嗽" } ] }, { "text": "一般情况下,应鼓励患儿咳嗽,注意翻身拍背,尽力促使呼吸道分泌物排出。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "鼓励患儿咳嗽" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "翻身拍背" }, { "start_idx": 21, "end_idx": 32, "type": "pro", "entity": "尽力促使呼吸道分泌物排出" } ] }, { "text": "定期复查胸部X线片及心、肝、肾功能等。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 10, "end_idx": 16, "type": "ite", "entity": "心、肝、肾功能" } ] }, { "text": "每天监测经皮血氧饱和度。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "监测经皮血氧饱和度" } ] }, { "text": "强调早期足量全程给氧。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "早期足量全程给氧" } ] }, { "text": "多数患者在发病后14天内都可能属于进展期,尤其是在病程进入第2周,即使患儿的发热、咳嗽症状不重,肺部实变仍可继续进展,并有发生突然变化的可能。", "entities": [ { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "发热" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "咳嗽症状不重" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "肺部实变" } ] }, { "text": "一般给予鼻导管给氧(1L),小婴儿给予头罩给氧(3~5L),保证氧饱和度>97%。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "鼻导管给氧(1L)" }, { "start_idx": 19, "end_idx": 39, "type": "pro", "entity": "头罩给氧(3~5L),保证氧饱和度>97%" } ] }, { "text": "气促明显、轻度低氧血症者应及早给予持续鼻导管吸氧。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "气促明显" }, { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "轻度低氧血症" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "持续鼻导管吸氧" } ] }, { "text": "给氧应注意患儿的依从性,根据病情可间断给氧。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "给氧" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "间断给氧" } ] }, { "text": "间断给氧持续时间应到疾病恢复期。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "pro", "entity": "间断给氧持续时间应到疾病恢复期" } ] }, { "text": "有发热超过38.5℃者,可使用解热镇痛药、非甾体类抗炎药如布洛芬。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "sym", "entity": "发热超过38.5℃" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "解热镇痛药" }, { "start_idx": 21, "end_idx": 27, "type": "dru", "entity": "非甾体类抗炎药" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "布洛芬" } ] }, { "text": "儿童忌用阿司匹林,因该药有可能引起Reye综合征。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "Reye综合征" } ] }, { "text": "高热者给予冰敷、乙醇擦浴等物理降温措施。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "高热" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "冰敷" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "乙醇擦浴" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "物理降温措施" } ] }, { "text": "咳嗽、咳痰者给予异丙嗪等镇咳、祛痰药。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "咳嗽" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "咳痰" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "异丙嗪" }, { "start_idx": 12, "end_idx": 17, "type": "dru", "entity": "镇咳、祛痰药" } ] }, { "text": "有心、肝、肾等器官功能损害,应作相应的处理。", "entities": [ { "start_idx": 1, "end_idx": 12, "type": "dis", "entity": "心、肝、肾等器官功能损害" } ] }, { "text": "对于顽固性高热、肺部病变进展迅速、呼吸困难、低氧血症及有进展到休克、ARDS或MODS趋势的患儿,应及时给予大剂量静脉注射丙种球蛋白(IVIG),400mg/(kg•d),连用3天。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "顽固性高热" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "肺部病变" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "低氧血症" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "休克" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "ARDS" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "MODS" }, { "start_idx": 50, "end_idx": 71, "type": "pro", "entity": "及时给予大剂量静脉注射丙种球蛋白(IVIG)" } ] }, { "text": "高热的患儿多在应用IVIG的第2天体温下降,少数患儿在应用IVIG的第3天体温下降,个别患儿在应用IVIG的第4天体温下降。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "高热" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "IVIG" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "体温下降" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "体温" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "IVIG" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "体温下降" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "体温" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "IVIG" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "体温下降" } ] }, { "text": "一般患儿在应用IVIG后4天内体温下降至正常。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "IVIG" } ] }, { "text": "IVIG的明显治疗效果可能与以下因素有关:①IVIG具有吞噬调理作用,可增加吞噬细胞的功能;②IVIG具有特异性抗体作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "IVIG" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "IVIG" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "IVIG" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "特异性抗体" } ] }, { "text": "由于我国人群以往冠状病毒中和抗体阳性率为30%~60%,如果本次的病原为冠状病毒变异株,IVIG依然会含有针对冠状病毒共同抗原决定簇的抗体;③IVIG具有封闭细胞因子、阻断细胞因子瀑布的作用。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "冠状病毒中和抗体" }, { "start_idx": 36, "end_idx": 42, "type": "mic", "entity": "冠状病毒变异株" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "IVIG" }, { "start_idx": 53, "end_idx": 68, "type": "bod", "entity": "针对冠状病毒共同抗原决定簇的抗体" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "IVIG" }, { "start_idx": 77, "end_idx": 82, "type": "sym", "entity": "封闭细胞因子" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "细胞因子" }, { "start_idx": 84, "end_idx": 91, "type": "sym", "entity": "阻断细胞因子瀑布" }, { "start_idx": 86, "end_idx": 89, "type": "bod", "entity": "细胞因子" } ] }, { "text": "没有证据表明抗生素治疗有效,但也没有证据表明抗生素治疗无效。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "抗生素治疗早期选用大环内酯类、头孢类抗生素,如果痰培养或临床上提示有耐药球菌感染,可选用(去甲)万古霉素等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "大环内酯类" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "头孢类抗生素" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "痰培养" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "耐药球菌感染" }, { "start_idx": 44, "end_idx": 51, "type": "dru", "entity": "(去甲)万古霉素" } ] }, { "text": "抗病毒药可试用利巴韦林。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "抗病毒药" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "利巴韦林" } ] }, { "text": "也可选用中药辅助治疗,治疗原则为:温病,卫气、营血和三焦辨证论治。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "中药辅助治疗" }, { "start_idx": 20, "end_idx": 31, "type": "pro", "entity": "卫气、营血和三焦辨证论治" } ] }, { "text": "可用静脉用复方毛冬青,口服金银花露等清热解毒药。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "静脉" }, { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "复方毛冬青" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "口服" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "金银花露" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "清热解毒药" } ] }, { "text": "可选用增强免疫功能的药物,包括转移因子、干扰素等。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dru", "entity": "增强免疫功能的药物" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "转移因子" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "干扰素" } ] }, { "text": "个体化治疗原则主要指糖皮质激素及IVIG的应用要掌握好适应证。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "IVIG" } ] }, { "text": "在儿童SARS的治疗过程中,尤其应体现由传统单纯的生物医学模式向社会-心理-生物医学模式转变。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "儿童SARS" } ] }, { "text": "对患儿尤其是年长儿应进行心理辅导,消除恐惧、失望心理。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "心理辅导" }, { "start_idx": 17, "end_idx": 25, "type": "pro", "entity": "消除恐惧、失望心理" } ] }, { "text": "【预后】经过积极正确的治疗,儿童SARS的预后良好,尚未见死亡病例报道。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "儿童SARS" } ] }, { "text": "【消毒隔离】本病的发病时间是在冬春季,这个季度也是呼吸道传染病的高发期。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "消毒隔离" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "呼吸道传染病" } ] }, { "text": "流行病学的表现主要是以近距离呼吸道飞沫、接触患者分泌物传播。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "呼吸道飞沫" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "分泌物" } ] }, { "text": "因此,制止传染病的扩散,除早期诊断,早期隔离患者外,对患者的排泄物、分泌物及患者接触的环境、物品进行消毒隔离,也是必不可少的措施。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "早期诊断" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "早期隔离患者" }, { "start_idx": 26, "end_idx": 53, "type": "pro", "entity": "对患者的排泄物、分泌物及患者接触的环境、物品进行消毒隔离" } ] }, { "text": "要建立收治该类患者的隔离病房和专科门诊。", "entities": [] }, { "text": "专用病区设在病区的一端,病区内分污染区、半污染区、清洁区、危重抢救室等。", "entities": [ { "start_idx": 29, "end_idx": 33, "type": "dep", "entity": "危重抢救室" } ] }, { "text": "但门口设有专门的隔离衣、体温计、血压计、听诊器等用具,另外备有消毒液供医护人员洗手消毒,水龙头是电动感应开关;疑似患者和确诊患者分别收入不同的病房。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "equ", "entity": "体温计" }, { "start_idx": 16, "end_idx": 18, "type": "equ", "entity": "血压计" }, { "start_idx": 20, "end_idx": 22, "type": "equ", "entity": "听诊器" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "消毒液" } ] }, { "text": "进入病区均要求戴12层棉纱口罩,帽子,换好隔离衣、隔离裤、一次性袜子、鞋套。", "entities": [] }, { "text": "病区出入口设脚垫(经含氯消毒液浸湿,有效氯2000mg/L,以供出入时消毒鞋底),并不定时补充喷晒消毒液以保持湿润。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "含氯消毒液" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "消毒液" } ] }, { "text": "住院患儿均戴口罩,严格隔离和管理,不得离开病房。", "entities": [] }, { "text": "严格探视制度,原则上不设陪护,尽量不探视,如果患儿病情危重等情况,探视者必须戴口罩、帽子,穿隔离衣、鞋套。", "entities": [] }, { "text": "(一)病区的消毒隔离1.空气消毒病房每天定期进行空气消毒,每4小时一次;采用紫外线照射,每次不少于1小时(无患者病房),含氯消毒剂熏蒸,喷雾消毒(用量:有效氯20~30ml/m3,1500mg/L含氯消毒剂,作用30分钟)。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "消毒隔离" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "空气消毒" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "空气消毒" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "紫外线照射" }, { "start_idx": 60, "end_idx": 66, "type": "pro", "entity": "含氯消毒剂熏蒸" }, { "start_idx": 68, "end_idx": 71, "type": "pro", "entity": "喷雾消毒" }, { "start_idx": 76, "end_idx": 78, "type": "dru", "entity": "有效氯" }, { "start_idx": 104, "end_idx": 108, "type": "dru", "entity": "含氯消毒剂" } ] }, { "text": "2.地面和物体表面消毒每天用含氯消毒剂(有效氯1500mg/2L)拖地2次,24小时保洁。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "地面和物体表面消毒" }, { "start_idx": 14, "end_idx": 36, "type": "pro", "entity": "含氯消毒剂(有效氯1500mg/2L)拖地2次" } ] }, { "text": "桌椅、床头柜、门把手、病历夹等物品,用含氯消毒液擦拭。", "entities": [ { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "含氯消毒液擦拭" } ] }, { "text": "专用病区每个患者的化验单、病历纸用臭氧发生器消毒0.5~1小时后,方可送院病案室归档。", "entities": [ { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "用臭氧发生器消毒" }, { "start_idx": 37, "end_idx": 39, "type": "dep", "entity": "病案室" } ] }, { "text": "3.患者使用的物品消毒①患者使用的加盖容器内的分泌物、排泄物及时消毒处理。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "患者使用的物品消毒" }, { "start_idx": 12, "end_idx": 35, "type": "pro", "entity": "患者使用的加盖容器内的分泌物、排泄物及时消毒处理" } ] }, { "text": "每天喝清热解毒中药,并口服预防量抗病毒药及漱口,具体措施:1.医护人员进入病区进行诊疗护理时,需戴12层棉纱口罩及N95口罩,佩戴时间不超过4小时,潮湿或污染随时立即更换。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "清热解毒中药" }, { "start_idx": 11, "end_idx": 22, "type": "pro", "entity": "口服预防量抗病毒药及漱口" }, { "start_idx": 74, "end_idx": 84, "type": "pro", "entity": "潮湿或污染随时立即更换" } ] }, { "text": "进行近距离操作时,佩戴防护眼镜。", "entities": [] }, { "text": "2.进入病室者,均需穿三层棉质隔离衣、二层棉质隔离裤、工作帽。", "entities": [] }, { "text": "戴一次性胶手套,穿一次性袜子、鞋套。", "entities": [] }, { "text": "3.医护人员每次接触患者后,必须立即进行手的消毒和清洗,或使用快速手消毒剂。", "entities": [ { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "手" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "消毒" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "清洗" }, { "start_idx": 31, "end_idx": 36, "type": "dru", "entity": "快速手消毒剂" } ] }, { "text": "5.医护人员接触患者后及下班前可用醋酸氯乙定含漱液或复方氯乙定含漱液漱口。", "entities": [ { "start_idx": 17, "end_idx": 35, "type": "pro", "entity": "醋酸氯乙定含漱液或复方氯乙定含漱液漱口" } ] }, { "text": "下班前认真做好个人清洗(洗手、洗脸、清洁鼻腔),洗澡更衣后才离开病区。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "个人清洗" }, { "start_idx": 12, "end_idx": 21, "type": "pro", "entity": "洗手、洗脸、清洁鼻腔" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "洗澡更衣" } ] }, { "text": "【预防】一般来讲,非典型肺炎的预防要做到以下几点:①保持生活、工作环境的空气流通。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "非典型肺炎" }, { "start_idx": 26, "end_idx": 39, "type": "pro", "entity": "保持生活、工作环境的空气流通" } ] }, { "text": "根据季节变化,尽可能开窗通风换气。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "开窗通风换气" } ] }, { "text": "③避免与患者密切接触,接触时需戴口罩。注意手的清洁消毒,尽量不要用脏手揉眼睛。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "接触时需戴口罩" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "注意手的清洁消毒" }, { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "不要用脏手揉眼睛" } ] }, { "text": "④注意休息和饮食卫生,增强体质,注意防寒保暖,提高抵抗疾病的能力。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "pro", "entity": "注意休息和饮食卫生" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "增强体质" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "注意防寒保暖" }, { "start_idx": 23, "end_idx": 31, "type": "pro", "entity": "提高抵抗疾病的能力" } ] }, { "text": "四、染色体畸变的原因现已知道,多种因素可造成染色体畸变,即大多数致突变的因素都可以引起染色体畸变。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "染色体畸变" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "染色体畸变" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "染色体畸变" } ] }, { "text": "1.物理因素在物理致畸因素中,电离辐射因导致染色体不分离而引人注目。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "致畸" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "染色体不分离" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "染色体" } ] }, { "text": "有实验证明,将受照射小鼠处于分裂近中期的卵细胞和未受照射的同期卵细胞比较,发现染色体不分离在受照射组中明显增高,这一现象在年龄较大的小鼠中尤为明显。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "分裂近中期的卵细胞" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "卵细胞" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "染色体不分离" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "染色体" } ] }, { "text": "人的淋巴细胞受照射或在受照射的血清内生长,发现实验组的三体型频率较对照组高,引起易位、缺失等染色体畸变。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血清" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "易位" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "缺失" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "染色体畸变" } ] }, { "text": "也有报道,受电离辐射的母亲,生育21-三体病孩的风险明显增高。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "21-三体病" } ] }, { "text": "2.化学因素许多种化学药物、毒物和抗代谢药均可引起染色体畸变,例如抗代谢药物、抗癫痫药物、农药以及毒物(如苯、甲苯和砷)等。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "抗代谢药" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "染色体畸变" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "抗代谢药物" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "抗癫痫药物" }, { "start_idx": 45, "end_idx": 46, "type": "dru", "entity": "农药" } ] }, { "text": "3.生物因素一些病毒感染,如风疹病毒、巨细胞包涵体病毒、麻疹病毒以及腮腺炎病毒的感染可引起染色体断裂。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "病毒感染" }, { "start_idx": 14, "end_idx": 17, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 19, "end_idx": 26, "type": "mic", "entity": "巨细胞包涵体病毒" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 34, "end_idx": 38, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "感染" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "染色体断裂" } ] }, { "text": "在实验中,以病毒处理培养细胞,也会引起各种类型的染色体畸变。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "细胞" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "染色体畸变" } ] }, { "text": "4.孕妇年龄孕妇年龄大是引起21-三体综合征和其他三体型的主要原因之一。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "其他三体型" } ] }, { "text": "母亲娩出21-三体综合征的风险因孕妇年龄增加而增加,这可能与生殖细胞的老化有关。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "生殖细胞的老化" } ] }, { "text": "女婴在出生时,所有卵细胞已经形成,卵细胞的进一步发育是在青春期,在月经间期卵细胞将发育成熟,直到妇女进入绝经期。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "卵细胞" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "卵细胞" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "卵细胞" } ] }, { "text": "在妇女的生命中,一个卵细胞受精越迟,这细胞已经存在的时间越长。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "卵细胞" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "细胞" } ] }, { "text": "因此,一个40岁妇女的受精卵比一个20岁妇女的受精卵要大20年,暴露在可引起细胞损害的有害环境的风险率也越高。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "受精卵" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "受精卵" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "细胞损害" } ] }, { "text": "此外,越是接近绝经期,女性控制卵细胞成熟的激素效应也更差,容易影响卵细胞的发育和成熟。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "卵细胞" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "卵细胞" } ] }, { "text": "而在男性,其精子的产生从青春期开始贯穿生命过程,因此,一个40岁男性的精子,并不比一个20岁男性的精子老,这可解释父方年龄和染色体异常之间缺乏相关依据。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "精子" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "精子" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "精子" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "染色体异常" } ] }, { "text": "5.遗传因素染色体异常可表现有家族性倾向,这提示染色体畸变与遗传有关。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "染色体异常" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "染色体畸变" } ] }, { "text": "据报道,同一家系中,同时有相同的或不同种类的非整倍体患者存在。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "整倍体" } ] }, { "text": "此外,染色体异常的父母可以不同方式传给下一代,最明显的例子是一些平衡易位的携带者,可引起染色体异常或正常的后代出现,其中又以涉及D组和G组染色体比较常见,因为它们是近端着丝粒染色体,在有丝分裂过程中形成随体联合,这可能是造成染色体不分离的原因之一。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "染色体异常" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "染色体异常" }, { "start_idx": 64, "end_idx": 71, "type": "bod", "entity": "D组和G组染色体" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "染色体" }, { "start_idx": 112, "end_idx": 117, "type": "sym", "entity": "染色体不分离" }, { "start_idx": 112, "end_idx": 114, "type": "bod", "entity": "染色体" } ] }, { "text": "一般感染与严重感染、中枢感染与其他感染用药剂量常不同;肝肾功能不全时药物剂量常需减少。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "感染" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "严重感染" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "中枢感染" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "感染" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "肝肾功能不全" } ] }, { "text": "病儿体重应以实际测得值为准,年长儿按体重计算如已超过成人量则以成人量为上限。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "体重" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "体重" } ] }, { "text": "三、气溶胶吸入疗法的选择吸入疗法是呼吸系统疾病治疗的重要手段之一,它的效能取决于药物能否到达药物作用的部位。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "气溶胶吸入疗法" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "吸入疗法" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "呼吸系统疾病" } ] }, { "text": "参考文献1.中华医学会儿科学会呼吸学组.儿童支气管哮喘诊断和防治指南,2008,46(10):745-7452.BacharierLB,BonerA,CarlsenKH,etal.Diagnosisandtreatmentofasthmainchildhood:aPRACTALLconsensusreport.Allergy,2008,63(1):5-53.BrandPLP,BaraldiE,BisgaardH,etal.Definition,assessmentandtreatmentofwheezingdisordersinpreschoolchildren:anevidence-basedapproach.EurRespirJ,2008,32(4):1096-10964.Expertpanelreport3:guidelinesforthediagnosisandmanagementofasthma.Bethesda,Maryland:NationalInstitutesofHealth,NationalAsthmaEducationandPreventionProgram;2007.NIHPublicationNo.0840515.GlobalInitiativeforAsthma(GINA).Globalstrategyforasthmamanagementandprevention;2008.Http://www.ginasthma.org", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "支气管哮喘" } ] }, { "text": "第七节脆性X综合征【概述】脆性X综合征(fragileX综合征,FX)是一种不完全外显的X染色体连锁显性遗传性疾病,因患者X染色体的短臂Xq27.3带有一脆性断裂点脆性断裂点而得名。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 20, "end_idx": 30, "type": "dis", "entity": "fragileX综合征" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "FX" }, { "start_idx": 44, "end_idx": 56, "type": "dis", "entity": "X染色体连锁显性遗传性疾病" }, { "start_idx": 61, "end_idx": 81, "type": "sym", "entity": "X染色体的短臂Xq27.3带有一脆性断裂点" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "X染色体" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "脆性断裂" } ] }, { "text": "FX是一种家族性智力障碍疾病,临床以智力低下、特殊面容、巨睾症、大耳、语言和行为异常为其典型表现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "FX" }, { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "家族性智力障碍疾病" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "智力低下" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "特殊面容" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "巨睾症" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "睾" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "大耳" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "耳" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "语言和行为异常" } ] }, { "text": "本病是第一个被鉴定的人类动态突变性遗传病,已成为动态突变遗传病研究的范例。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "动态突变性遗传病" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "动态突变遗传病" } ] }, { "text": "【流行病学】FX发病率仅次于21-三体综合征。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "FX" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "FX在弱智者中占6.0%~10.4%,在学习困难儿童中占2.6%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "FX" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "弱智" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "学习困难" } ] }, { "text": "河南医科大学对130例先天性智力低下儿童进行脆性X综合征研究,检出5例脆性X综合征,检出率为3.77%。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "先天性智力低下" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "脆性X综合征" } ] }, { "text": "【病理生理和发病机制】FX病人Xq27.3区带存在脆性位点(FRAXA)是其典型的细胞遗传学特征。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "FX" }, { "start_idx": 15, "end_idx": 28, "type": "sym", "entity": "Xq27.3区带存在脆性位点" } ] }, { "text": "1993年FX病编码基因cDNA被克隆,发现(CGG)n结构中n拷贝数由正常6~52扩增至大于或等于230时是脆性X综合征患者发病的分子基础,异常扩增的(CGG)n结构位于FMR-1基因翻译区外显子1。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "bod", "entity": "FX病编码基因cDNA" }, { "start_idx": 22, "end_idx": 29, "type": "bod", "entity": "(CGG)n结构" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 71, "end_idx": 99, "type": "sym", "entity": "异常扩增的(CGG)n结构位于FMR-1基因翻译区外显子1" }, { "start_idx": 76, "end_idx": 83, "type": "bod", "entity": "(CGG)n结构" }, { "start_idx": 86, "end_idx": 99, "type": "bod", "entity": "FMR-1基因翻译区外显子1" } ] }, { "text": "至今已知FMR-1基因的错义突变和缺失突变的错义突变和缺失突变携带者也表现出FMR-1基因动态突变相同的临床症状,从而显示出脆性X综合征临床症候的患者具有高度的遗传异质性,进而使这些患者及其家庭成员的基因诊断进一步复杂化。", "entities": [ { "start_idx": 4, "end_idx": 20, "type": "sym", "entity": "FMR-1基因的错义突变和缺失突变" }, { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "错义突变" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "缺失突变" }, { "start_idx": 38, "end_idx": 48, "type": "sym", "entity": "FMR-1基因动态突变" }, { "start_idx": 38, "end_idx": 44, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "基因" } ] }, { "text": "(一)细胞遗传学X染色体长臂27.3带上一个叶酸敏感的部位与FX有关,该部位经特殊处理后可显示脆性断裂,因而被称为脆性部位。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "细胞" }, { "start_idx": 8, "end_idx": 28, "type": "bod", "entity": "X染色体长臂27.3带上一个叶酸敏感的部位" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "FX" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "脆性断裂" } ] }, { "text": "具有脆性部位的染色体被称为脆性染色体。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "染色体" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "脆性染色体" } ] }, { "text": "迄今已有26个染色体的脆性部位被发现,其中仅X染色体X27-X28区域的脆性部位(FRAXA)与遗传性疾病有关,而其他与疾病无关的脆性部位称为普通脆性部位。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "bod", "entity": "26个染色体的脆性部位" }, { "start_idx": 22, "end_idx": 39, "type": "bod", "entity": "X染色体X27-X28区域的脆性部位" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "FRAXA" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "脆性部位" }, { "start_idx": 71, "end_idx": 76, "type": "bod", "entity": "普通脆性部位" } ] }, { "text": "X脆性部位产生的机制尚不完全清楚,目前认为与DNA的合成代谢过程有关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "X脆性部位" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "DNA" } ] }, { "text": "已发现在缺乏叶酸或用较大剂量的5-氟尿嘧啶(5-FU)等条件下处理,可致使胸腺核苷合成部分受到抑制,染色体结构就可能在某些特定的部位上产生裂隙或断裂。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "缺乏叶酸" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "叶酸" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "5-氟尿嘧啶" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "5-FU" }, { "start_idx": 37, "end_idx": 48, "type": "sym", "entity": "胸腺核苷合成部分受到抑制" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "胸腺核苷" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "染色体" }, { "start_idx": 50, "end_idx": 73, "type": "sym", "entity": "染色体结构就可能在某些特定的部位上产生裂隙或断裂" } ] }, { "text": "(二)FMR-1基因的结构、转录和翻译FX的基因称为脆性X智能落后1基因(fragileXmentalretardation-1,FMR-1),定位于Xq27.3区带,在基因组中跨越38kb,由17个外显子和16个内含子组成。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "FX的基因" }, { "start_idx": 26, "end_idx": 35, "type": "bod", "entity": "脆性X智能落后1基因" }, { "start_idx": 37, "end_idx": 63, "type": "bod", "entity": "fragileXmentalretardation-1" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "FMR-1" }, { "start_idx": 75, "end_idx": 82, "type": "bod", "entity": "Xq27.3区带" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "基因组" } ] }, { "text": "FMR-1基因的mRNA的mRNA为4.4kb,编码一个分子量约为69~70kD,由596个氨基酸组成的脆性智能落后蛋白(FMRP),这是一种RNA结合蛋白,在体内多种组织中都表达。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "bod", "entity": "FMR-1基因的mRNA" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "mRNA" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "596个氨基酸" }, { "start_idx": 52, "end_idx": 59, "type": "bod", "entity": "脆性智能落后蛋白" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "FMRP" }, { "start_idx": 71, "end_idx": 77, "type": "bod", "entity": "RNA结合蛋白" } ] }, { "text": "FMR-1基因的各外显子较小(51~196bp),但内含子较大,平均大小为2.2kb,其中内含子1约为9.9kb。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "FMR-1基因" } ] }, { "text": "基因中存在多种累及FMR-1基因3'端外显子10、12、14、15和17的多种转录拼接形式,其中涉及外显子12和14通常导致整个外显子的丢失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 9, "end_idx": 18, "type": "bod", "entity": "FMR-1基因3'端" }, { "start_idx": 62, "end_idx": 69, "type": "sym", "entity": "整个外显子的丢失" } ] }, { "text": "但累及外显子10、15和17的则只丢失这3个外显子5'端的部分序列,这是因为这3个外显子的5'一端序列上分别存在一个拼接保守信号,转录后拼接3个外显子5'端的部分序列丢失3个外显子5'端的部分序列丢失。", "entities": [ { "start_idx": 17, "end_idx": 32, "type": "sym", "entity": "丢失这3个外显子5'端的部分序列" }, { "start_idx": 70, "end_idx": 84, "type": "sym", "entity": "3个外显子5'端的部分序列丢失" }, { "start_idx": 85, "end_idx": 99, "type": "sym", "entity": "3个外显子5'端的部分序列丢失" } ] }, { "text": "(三)FMR-1基因的动态突变FMR-1基因的5'端有一个CGG三核苷酸重复区域,在正常个体中CGG结构的重复次数具有多态性,介于6~52次之间,平均为30次,中国人群中以(CGG)28最多见。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "FMR-1基因的动态突变" }, { "start_idx": 15, "end_idx": 25, "type": "bod", "entity": "FMR-1基因的5'端" } ] }, { "text": "在FX患者中,CGG拷贝数一般>200次,多则可达1000次以上。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "FX" }, { "start_idx": 7, "end_idx": 31, "type": "sym", "entity": "CGG拷贝数一般>200次,多则可达1000次以上" }, { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "CGG拷贝数" } ] }, { "text": "脆性X综合征发生的根本原因是FMR-1基因的突变所致。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 14, "end_idx": 23, "type": "sym", "entity": "FMR-1基因的突变" } ] }, { "text": "动态突变是指FMR-1基因在传递过程中CGG拷贝数不稳定,会发生扩增,这是95%以上的FX患者发病的分子遗传学基础。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "动态突变" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 6, "end_idx": 27, "type": "sym", "entity": "FMR-1基因在传递过程中CGG拷贝数不稳定" }, { "start_idx": 19, "end_idx": 24, "type": "ite", "entity": "CGG拷贝数" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "发生扩增" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "FX" } ] }, { "text": "动态突变包括3种类型:1.FMR-1基因的前突变(premutation)FMR-1基因(CGG)n结构中n拷贝数扩增至53~230后代的CGG重复数大为增加,并有异常表型出现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "动态突变" }, { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "FMR-1基因的前突变" }, { "start_idx": 25, "end_idx": 35, "type": "sym", "entity": "premutation" }, { "start_idx": 37, "end_idx": 65, "type": "sym", "entity": "FMR-1基因(CGG)n结构中n拷贝数扩增至53~230" }, { "start_idx": 37, "end_idx": 43, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 66, "end_idx": 78, "type": "sym", "entity": "后代的CGG重复数大为增加" }, { "start_idx": 82, "end_idx": 87, "type": "sym", "entity": "异常表型出现" } ] }, { "text": "FMR-1基因的这种突变称为前突变,男性或者女性FMR-1前突变基因携带者智力水平与正常人并没有区别前突变FMR-1基因(CGG)n结构中,n拷贝数没有区别,但随着女性携有的前突变FMR-1基因(CGG)n结构传代过程中扩增至前突变概率也逐渐增加。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "FMR-1基因的这种突变" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "前突变" }, { "start_idx": 24, "end_idx": 33, "type": "bod", "entity": "FMR-1前突变基因" }, { "start_idx": 50, "end_idx": 59, "type": "bod", "entity": "前突变FMR-1基因" }, { "start_idx": 70, "end_idx": 73, "type": "ite", "entity": "n拷贝数" }, { "start_idx": 87, "end_idx": 96, "type": "bod", "entity": "前突变FMR-1基因" }, { "start_idx": 113, "end_idx": 115, "type": "sym", "entity": "前突变" } ] }, { "text": "而38%前突变型FMR-1基因经父源性传递至女儿时其(CGG)结构中n拷贝数发生了缩减母源性前突变型FMR-1基因传递至女儿时,从而提示前突变型FMR-1基因在母女传递的过程中其(CGG)n结构中n拷贝数具有扩增的倾向,但父女传递时则存在缩减的趋势。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "bod", "entity": "前突变型FMR-1基因" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "n拷贝数发生了缩减" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "n拷贝数" }, { "start_idx": 43, "end_idx": 56, "type": "bod", "entity": "母源性前突变型FMR-1基因" }, { "start_idx": 68, "end_idx": 78, "type": "bod", "entity": "前突变型FMR-1基因" }, { "start_idx": 98, "end_idx": 123, "type": "sym", "entity": "n拷贝数具有扩增的倾向,但父女传递时则存在缩减的趋势" }, { "start_idx": 98, "end_idx": 101, "type": "ite", "entity": "n拷贝数" } ] }, { "text": "2.FMR-1基因的全突变(fullmutation)FMR-1基因由前突变状态(CGG)53~230扩增至>230次脆性X综合征,53%的女性携带者表现出轻重程度不等的智力低下,此时称为全突变。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 2, "end_idx": 12, "type": "sym", "entity": "FMR-1基因的全突变" }, { "start_idx": 14, "end_idx": 25, "type": "sym", "entity": "fullmutation" }, { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 27, "end_idx": 58, "type": "sym", "entity": "FMR-1基因由前突变状态(CGG)53~230扩增至>230次" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 78, "end_idx": 88, "type": "sym", "entity": "轻重程度不等的智力低下" }, { "start_idx": 94, "end_idx": 96, "type": "sym", "entity": "全突变" } ] }, { "text": "全突变与智力低下临床表现的出现直接相关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "全突变" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "智力低下" } ] }, { "text": "经研究发现,当CGG结构的重复数达230次以上次以上时,FMR-1基因5'端的CpG岛开始非正常地甲基化,这种甲基化延伸至启动子区,致使转录不能启动,mRNA不能转录,基因编码的蛋白产物也因之缺乏,导致临床症状产生。", "entities": [ { "start_idx": 7, "end_idx": 22, "type": "sym", "entity": "CGG结构的重复数达230次以上" }, { "start_idx": 7, "end_idx": 15, "type": "ite", "entity": "CGG结构的重复数" }, { "start_idx": 28, "end_idx": 51, "type": "sym", "entity": "FMR-1基因5'端的CpG岛开始非正常地甲基化" }, { "start_idx": 28, "end_idx": 37, "type": "bod", "entity": "FMR-1基因5'端" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "甲基化延伸至启动子区" }, { "start_idx": 68, "end_idx": 73, "type": "sym", "entity": "转录不能启动" }, { "start_idx": 75, "end_idx": 82, "type": "sym", "entity": "mRNA不能转录" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "mRNA" }, { "start_idx": 84, "end_idx": 97, "type": "sym", "entity": "基因编码的蛋白产物也因之缺乏" }, { "start_idx": 84, "end_idx": 92, "type": "bod", "entity": "基因编码的蛋白产物" } ] }, { "text": "值得指出的是极少数男性全突变型FMR-I基因携带者缺乏应有的FRAXA位点脆性现象,其分子遗传学的基础有待于进一步加以研究。", "entities": [ { "start_idx": 11, "end_idx": 21, "type": "bod", "entity": "全突变型FMR-I基因" }, { "start_idx": 25, "end_idx": 40, "type": "sym", "entity": "缺乏应有的FRAXA位点脆性现象" }, { "start_idx": 30, "end_idx": 36, "type": "bod", "entity": "FRAXA位点" } ] }, { "text": "就智力低下临床表现而言,几乎100%男性FMR-I全突变基因携带者存在智力低下,其中约89%(245/274)为中度智力低下,但仅21%(36/170)女性FMR-I全突变携带者表现出中度智力低下,而且高达59%(100/170),女性FMR-1全突变型携带者并不出现智力低下。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "智力低下" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "FMR-I全突变基因" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "智力低下" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "中度智力低下" }, { "start_idx": 78, "end_idx": 85, "type": "sym", "entity": "FMR-I全突变" }, { "start_idx": 92, "end_idx": 97, "type": "sym", "entity": "中度智力低下" }, { "start_idx": 118, "end_idx": 126, "type": "bod", "entity": "FMR-1全突变型" }, { "start_idx": 134, "end_idx": 137, "type": "sym", "entity": "智力低下" } ] }, { "text": "3.FMR-1基因的回复突变处于前突变或全突变状态的FMR-1基因的CGG结构在传代过程中其拷贝数目会发生一定范围的缩减,称为FMR-1基因的回复突变(reversemutation)。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 2, "end_idx": 13, "type": "sym", "entity": "FMR-1基因的回复突变" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "前突变" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "全突变" }, { "start_idx": 26, "end_idx": 59, "type": "sym", "entity": "FMR-1基因的CGG结构在传代过程中其拷贝数目会发生一定范围的缩减" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "拷贝数目" }, { "start_idx": 63, "end_idx": 74, "type": "sym", "entity": "FMR-1基因的回复突变" }, { "start_idx": 63, "end_idx": 69, "type": "bod", "entity": "FMR-1基因" } ] }, { "text": "根据突变前后FMR-1基因所处的状态,回复突变可分为3种类型:全突变型→前突变型;全突变型→全突变或前突变嵌合型;嵌合型或前突变型→正常FMR-1基因。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "突变" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "回复突变" }, { "start_idx": 66, "end_idx": 74, "type": "bod", "entity": "正常FMR-1基因" } ] }, { "text": "这些现象可发生于父源性传递过程,也可发生于母源性传递过程中,虽然较少见,却增加了预测FMR-1基因动态突变规律的困难,导致家族内遗传咨询及产前基因诊断进一步复杂化。", "entities": [ { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 42, "end_idx": 52, "type": "sym", "entity": "FMR-1基因动态突变" }, { "start_idx": 69, "end_idx": 74, "type": "pro", "entity": "产前基因诊断" } ] }, { "text": "(四)性别因素对FMR-1基因动态突变的影响目前认为FMR-1基因(CGG)n结构的扩增是一个多途径多步骤递次扩增的过程,FX的遗传模式常有特殊的规律,即具表型正常的男性携带者可将脆性部位传递给其女儿,后者一般无智力低下智力低下或其他临床症状,但她可将受累染色体传递给后代,使家系中的第三代出现FX患者。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "FMR-1基因动态突变" }, { "start_idx": 26, "end_idx": 43, "type": "sym", "entity": "FMR-1基因(CGG)n结构的扩增" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "FX" }, { "start_idx": 90, "end_idx": 93, "type": "bod", "entity": "脆性部位" }, { "start_idx": 106, "end_idx": 109, "type": "sym", "entity": "智力低下" }, { "start_idx": 110, "end_idx": 113, "type": "sym", "entity": "智力低下" }, { "start_idx": 126, "end_idx": 130, "type": "bod", "entity": "受累染色体" }, { "start_idx": 147, "end_idx": 148, "type": "dis", "entity": "FX" } ] }, { "text": "此时第三代男孩具有较明显的智力低下,而女孩往往无明显智力异常,也即由母亲传递给子女所造成的危害较由父亲传递为大,且男孩受累程度较女孩为重,此现象称Sheman现象。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "较明显的智力低下" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "明显智力异常" }, { "start_idx": 73, "end_idx": 80, "type": "sym", "entity": "Sheman现象" } ] }, { "text": "此外,还发现男性全突变型FMR-1基因携带者精子标本中只存在前突变型FMR-1基因FMR-1基因的全突变并未累及男性全突变携带者的生殖细胞,但目前尚缺乏足够的证据表明女性卵细胞也未发生FMR-1基因的全突变。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "bod", "entity": "全突变型FMR-1基因" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "精子" }, { "start_idx": 30, "end_idx": 40, "type": "bod", "entity": "前突变型FMR-1基因" }, { "start_idx": 41, "end_idx": 47, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 41, "end_idx": 51, "type": "sym", "entity": "FMR-1基因的全突变" }, { "start_idx": 58, "end_idx": 60, "type": "sym", "entity": "全突变" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 83, "end_idx": 87, "type": "bod", "entity": "女性卵细胞" }, { "start_idx": 92, "end_idx": 98, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 92, "end_idx": 102, "type": "sym", "entity": "FMR-1基因的全突变" } ] }, { "text": "此外,女性FMR-1基因动态突变携带者在传代过程中,其(CGG)n结构的扩增和大小尚依后代的性别而发生变化,即传至男性后代时则具有进一步扩增的趋势,但传至女性后代时则扩增程度较小,且尚可见到(CGG)n结构发生缩减的现象,可能女性所携有的另外1条FMR-1基因正常的X染色体抑制了女性胚胎早期阶段全突变型FMR-1基因(CGG)n结构的进一步扩增。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "sym", "entity": "FMR-1基因动态突变" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 27, "end_idx": 37, "type": "sym", "entity": "(CGG)n结构的扩增" }, { "start_idx": 95, "end_idx": 106, "type": "sym", "entity": "(CGG)n结构发生缩减" }, { "start_idx": 119, "end_idx": 129, "type": "bod", "entity": "另外1条FMR-1基因" }, { "start_idx": 133, "end_idx": 136, "type": "bod", "entity": "X染色体" }, { "start_idx": 148, "end_idx": 172, "type": "sym", "entity": "全突变型FMR-1基因(CGG)n结构的进一步扩增" }, { "start_idx": 148, "end_idx": 158, "type": "bod", "entity": "全突变型FMR-1基因" } ] }, { "text": "总之,全突变型FMR-1基因(CGG)n结构的变化趋势(是扩增,还是缩减)以及变化适度的大小尚受亲代和子代性别的双重影响。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "bod", "entity": "全突变型FMR-1基因" } ] }, { "text": "因此,在FMR-1基因动态突变家族的FMR-1基因必须考虑FMR-1基因的这种突变特征。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 4, "end_idx": 14, "type": "sym", "entity": "FMR-1基因动态突变" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "突变" } ] }, { "text": "(五)FMR-1基因FMR-1基因除了动态突变外,少数病人碱基置换和缺失等非动态突变,目前已发现一种错义突变和8种缺失型突变。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "动态突变" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "碱基置换" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "缺失" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "非动态突变" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "错义突变" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "缺失型突变" } ] }, { "text": "这些突变所导致的临床症状和动态突变相同,但缺乏FRAXA脆性位点这一特征。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "突变" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "动态突变" }, { "start_idx": 21, "end_idx": 31, "type": "sym", "entity": "缺乏FRAXA脆性位点" }, { "start_idx": 23, "end_idx": 31, "type": "bod", "entity": "FRAXA脆性位点" } ] }, { "text": "【临床表现】男性患者典型临床症状包括:1.智力低下IQ常低于50,并呈进行性加重。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "智力低下" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "IQ常低于50" } ] }, { "text": "2.特殊面容面部瘦长前额突出头围增大眶上饱满虹膜颜色变淡耳大外翻耳大外翻、高腭弓、大嘴、厚唇以及下颌大而突出等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "特殊面容" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "面" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "面部" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "面部瘦长" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "前额" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "前额突出" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "头围" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "头围增大" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "眶上" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "眶上饱满" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "虹膜" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "虹膜颜色变淡" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "耳大外翻" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "耳" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "耳" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "耳大外翻" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "高腭弓" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "腭弓" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "大嘴" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "嘴" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "厚唇" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "唇" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "下颌" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "颌大而突出" } ] }, { "text": "3.大睾丸多在青春期后期出现,少见于年幼患者,常伴大阴茎。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "大睾丸" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "睾丸" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "大阴茎" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "阴茎" } ] }, { "text": "4.语言发育障碍较为常见,表现为会话和言语表达能力的发育严重迟缓,存在构音障碍、病理性模仿和重复言语以及语法和词汇缺乏等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "语言发育障碍" }, { "start_idx": 16, "end_idx": 31, "type": "sym", "entity": "会话和言语表达能力的发育严重迟缓" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "构音障碍" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "病理性模仿" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "重复言语" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "语法和词汇缺乏" } ] }, { "text": "5.人格行为异常包括好动、精力不集中、性情孤僻、焦虑及自残等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "人格行为异常" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "好动" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "精力不集中" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "性情孤僻" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "焦虑" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "自残" } ] }, { "text": "6.神经系统症状多较轻微,常见为四肢运动障碍。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "神经系统" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "四肢运动障碍" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "四肢" } ] }, { "text": "生殖系统生殖系统性功能低下,成年患者阴毛呈女性分布和乳房女性化,但可生育后代。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "生殖系统" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "生殖系统" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "性功能低下" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "阴毛呈女性分布" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "阴毛" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "乳房女性化" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "乳房" } ] }, { "text": "【实验室检查】(一)细胞学检测1.脆性X染色体检查脆性X染色体的检查对于了解脆性部位的表达频率以及脆性部位处染色体的结构非常重要,是确认最初先证者的基本手段。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "细胞学检测" }, { "start_idx": 17, "end_idx": 24, "type": "pro", "entity": "脆性X染色体检查" }, { "start_idx": 25, "end_idx": 33, "type": "pro", "entity": "脆性X染色体的检查" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "脆性部位" }, { "start_idx": 49, "end_idx": 56, "type": "bod", "entity": "脆性部位处染色体" } ] }, { "text": "由于在X染色体上还存在别的与智力低下无关的脆性部位(如FRQXD等),所以即使检出脆性X位点的存在也不能确诊为FX患者或携带者。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "X染色体" }, { "start_idx": 13, "end_idx": 24, "type": "bod", "entity": "与智力低下无关的脆性部位" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "FRQXD" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "脆性X位点" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "FX" } ] }, { "text": "2.荧光原位杂交技术检查对于疑为FMR-1基因大片段缺失的患者可作FISH检测。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "荧光原位杂交技术检查" }, { "start_idx": 16, "end_idx": 27, "type": "sym", "entity": "FMR-1基因大片段缺失" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "FMR-1基因" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "FISH检测" } ] }, { "text": "以荧光标记的探针,对经过秋水仙素等处理,处于中期分裂象的细胞染色体进行原位杂交,正常染色体有荧光显示,而相应部位有缺失的染色体则无荧光显示。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "equ", "entity": "探针" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "秋水仙素" }, { "start_idx": 20, "end_idx": 32, "type": "bod", "entity": "处于中期分裂象的细胞染色体" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "正常染色体" }, { "start_idx": 52, "end_idx": 68, "type": "sym", "entity": "相应部位有缺失的染色体则无荧光显示" }, { "start_idx": 52, "end_idx": 62, "type": "bod", "entity": "相应部位有缺失的染色体" } ] }, { "text": "(二)基因检测1.DNA印迹技术(Southernblotting)动态突变和大片段的缺失突变会造成FMR-1基因片段长度的显著改变,因此可用Southern印迹技术进行检测。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "基因检测" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "DNA印迹技术" }, { "start_idx": 17, "end_idx": 32, "type": "pro", "entity": "Southernblotting" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "动态突变" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "大片段的缺失突变" }, { "start_idx": 50, "end_idx": 65, "type": "sym", "entity": "FMR-1基因片段长度的显著改变" }, { "start_idx": 50, "end_idx": 57, "type": "bod", "entity": "FMR-1基因片" }, { "start_idx": 71, "end_idx": 82, "type": "pro", "entity": "Southern印迹技术" } ] }, { "text": "针对FMR-1基因不同的限制性内切酶选用不同的限制性内切酶和探针。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "bod", "entity": "FMR-1基因不同的限制性内切酶" }, { "start_idx": 23, "end_idx": 28, "type": "dru", "entity": "限制性内切酶" }, { "start_idx": 30, "end_idx": 31, "type": "equ", "entity": "探针" } ] }, { "text": "EcoRI或HindⅢ限制性内切酶加pE5.1(或afaxa1)探针,可检测出全突变或大片段的前突变,因此适用于检测具有智力异常的BclI内切酶加StB12.3等探针.3等探针可检测出含CGG在内的弥散区带,能较好地检出并初步确定前突变的大小。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "dru", "entity": "EcoRI或HindⅢ限制性内切酶" }, { "start_idx": 18, "end_idx": 33, "type": "equ", "entity": "pE5.1(或afaxa1)探针" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "全突变" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "大片段的前突变" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "智力异常" }, { "start_idx": 65, "end_idx": 71, "type": "dru", "entity": "BclI内切酶" }, { "start_idx": 73, "end_idx": 82, "type": "equ", "entity": "StB12.3等探针" }, { "start_idx": 86, "end_idx": 87, "type": "equ", "entity": "探针" }, { "start_idx": 92, "end_idx": 102, "type": "bod", "entity": "含CGG在内的弥散区带" }, { "start_idx": 115, "end_idx": 117, "type": "sym", "entity": "前突变" } ] }, { "text": "PstI内切酶加OXO.55探针可检测CGG重复数较小的前突变。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "PstI内切酶" }, { "start_idx": 8, "end_idx": 15, "type": "equ", "entity": "OXO.55探针" }, { "start_idx": 19, "end_idx": 30, "type": "sym", "entity": "CGG重复数较小的前突变" } ] }, { "text": "如果使用一些对甲基化敏感的限制性内切酶,如EagI、BssHI及SacI等,则能检测出CpG岛的甲基化,可较好地检测出全突变、前突变及嵌合型。", "entities": [ { "start_idx": 7, "end_idx": 18, "type": "dru", "entity": "甲基化敏感的限制性内切酶" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "EagI" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "BssHI" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "SacI" }, { "start_idx": 59, "end_idx": 61, "type": "sym", "entity": "全突变" }, { "start_idx": 63, "end_idx": 65, "type": "sym", "entity": "前突变" }, { "start_idx": 67, "end_idx": 69, "type": "sym", "entity": "嵌合型" } ] }, { "text": "Southern印迹技术敏感、准确,是经典的检测方法,但技术繁杂,不适用于普通群体或高危群体的筛选,也无法精确测定CGG的重复数。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "Southern印迹技术" } ] }, { "text": "2.聚合酶链反应(PCR)技术选用合适的引物,对患者的FMR-1基因片段进行PCR扩增,扩增产物经变性聚丙烯酰胺凝胶电泳分离后直接观察结果,可准确判断CGG的重复数。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "pro", "entity": "聚合酶链反应(PCR)技术" }, { "start_idx": 27, "end_idx": 35, "type": "bod", "entity": "FMR-1基因片段" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "PCR扩增" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "变性" }, { "start_idx": 51, "end_idx": 61, "type": "pro", "entity": "聚丙烯酰胺凝胶电泳分离" } ] }, { "text": "此法用于发现重复数小的前突变以及观察普通群体中的(CGG)n分布,由此确定正常和前突变之间CGG重复数的分界。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "重复数小的前突变" }, { "start_idx": 40, "end_idx": 42, "type": "sym", "entity": "前突变" } ] }, { "text": "该方法简便,适于普查,缺点是CGG重复次数多的全突变顺序中含有大量GC碱基,PCR反应有一定难度。", "entities": [ { "start_idx": 14, "end_idx": 25, "type": "sym", "entity": "CGG重复次数多的全突变" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "GC碱基" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "PCR反应" } ] }, { "text": "PCR技术无法检出甲基化,故也不能检出嵌合型。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "PCR技术" } ] }, { "text": "(三)蛋白质检测由于FMRP在正常人几乎每种组织和细胞中均有表达,而在FX的患者中却不表达或异常表达,因此用抗FMRP单克隆抗体作免疫组化或免疫荧光技术可以检测该蛋白质的存在。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "蛋白质检测" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "FMRP" }, { "start_idx": 15, "end_idx": 26, "type": "bod", "entity": "正常人几乎每种组织和细胞" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "FX" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "异常表达" }, { "start_idx": 54, "end_idx": 63, "type": "bod", "entity": "抗FMRP单克隆抗体" }, { "start_idx": 65, "end_idx": 68, "type": "pro", "entity": "免疫组化" }, { "start_idx": 70, "end_idx": 75, "type": "pro", "entity": "免疫荧光技术" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "蛋白质" } ] }, { "text": "早期人们仅对可疑者的血涂片采用此法作检测,近来采用羊水中的胎儿脱落细胞观察是否存在FMRP作为FX产前诊断的指标。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "血涂片" }, { "start_idx": 25, "end_idx": 34, "type": "ite", "entity": "羊水中的胎儿脱落细胞" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "FMRP" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "FX" } ] }, { "text": "【诊断】脆性X综合征的临床表现多种多样,性格、心理及精神方面的改变也不完全相同,况且有的患者其临床症状并不十分典型,单靠临床表现很难作出诊断。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "脆性X综合征" } ] }, { "text": "以细胞学技术检测X脆性位点是一种形态学的检测方法,但准确性和敏感性不是很高。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "细胞学技术" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "X脆性位点" } ] }, { "text": "基因检测虽然是诊断FX的主要手段,但是基因检测不能完全替代染色体检测,因为随着研究的深入,发现脆性X综合征不仅只是与脆性部位FRAXA有关,而且与原来认为是普通脆性部位的FRAXE也相关,近来又发现FRAXF部位似乎也与FX有关,而目前只能检测与FRAXA相关的FMR-1基因的突变,所以只进行基因检测容易漏诊。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "基因检测" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "FX" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "基因检测" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "染色体检测" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 58, "end_idx": 66, "type": "bod", "entity": "脆性部位FRAXA" }, { "start_idx": 78, "end_idx": 89, "type": "bod", "entity": "普通脆性部位的FRAXE" }, { "start_idx": 99, "end_idx": 105, "type": "bod", "entity": "FRAXF部位" }, { "start_idx": 110, "end_idx": 111, "type": "dis", "entity": "FX" }, { "start_idx": 123, "end_idx": 137, "type": "bod", "entity": "FRAXA相关的FMR-1基因" }, { "start_idx": 123, "end_idx": 140, "type": "sym", "entity": "FRAXA相关的FMR-1基因的突变" }, { "start_idx": 147, "end_idx": 150, "type": "pro", "entity": "基因检测" } ] }, { "text": "DNA印迹技术可前突变出前突变、嵌合体、全突变以及大片段的缺失,但对较小片段的前突变和缺失则效果较差,PCR则适合于检测重复数小的前突变,但不能检测甲基化。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "DNA印迹技术" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "前突变" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "前突变" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "嵌合体" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "全突变" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "大片段的缺失" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "较小片段的前突变" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "缺失" }, { "start_idx": 51, "end_idx": 53, "type": "pro", "entity": "PCR" }, { "start_idx": 60, "end_idx": 67, "type": "sym", "entity": "重复数小的前突变" } ] }, { "text": "【治疗】本病为X连锁显性遗传病,无有效治疗方法。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "X连锁显性遗传病" } ] }, { "text": "三、数字减影血管造影(一)基本原理数字减影血管造影(digitalsubtractionangiography,DSA)基本原理是将受检部位注入造影剂和注入造影剂后(血管造影)X线荧光图像,分别经影像增强器增益后用高分辨率电视摄影管作矩阵化扫描,形成由像素组成的视频图像,进而将视频信息经过对数增幅和模/数转换为不同值的数字,即通过数字化形成数字图像并分别存储起来,然后输入电子计算器处理并使两者之数字信息相减,所获得的不同数值的差值信号,再经对比度增强和数/模转换成不同灰阶度的模拟减影图像予以显示。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "数字减影血管造影" }, { "start_idx": 17, "end_idx": 24, "type": "pro", "entity": "数字减影血管造影" }, { "start_idx": 26, "end_idx": 54, "type": "pro", "entity": "digitalsubtractionangiography" }, { "start_idx": 56, "end_idx": 58, "type": "pro", "entity": "DSA" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "造影剂" }, { "start_idx": 78, "end_idx": 80, "type": "dru", "entity": "造影剂" }, { "start_idx": 83, "end_idx": 86, "type": "pro", "entity": "血管造影" }, { "start_idx": 88, "end_idx": 93, "type": "pro", "entity": "X线荧光图像" }, { "start_idx": 98, "end_idx": 102, "type": "equ", "entity": "影像增强器" }, { "start_idx": 107, "end_idx": 115, "type": "equ", "entity": "高分辨率电视摄影管" }, { "start_idx": 117, "end_idx": 121, "type": "pro", "entity": "矩阵化扫描" }, { "start_idx": 187, "end_idx": 191, "type": "equ", "entity": "电子计算器" } ] }, { "text": "(二)造影方法DSA造影方法分为静脉法和动脉法。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "造影" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "DSA造影" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "静脉法" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "动脉法" } ] }, { "text": "凡是经浅静脉穿刺途径置入导管或套管针注射造影剂行DSA检查者,皆称之静脉DSA(IVDSA)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "浅静脉穿刺" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "导管" }, { "start_idx": 15, "end_idx": 17, "type": "equ", "entity": "套管针" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "注射造影剂" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "DSA检查" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "静脉DSA" }, { "start_idx": 40, "end_idx": 44, "type": "pro", "entity": "IVDSA" } ] }, { "text": "凡是经动脉穿刺插管注射造影剂行DSA检查者,称为动脉DSA(IADSA)。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "动脉穿刺插管" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "注射造影剂" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "DSA" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "动脉DSA" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "IADSA" } ] }, { "text": "脑血管DSA造影方法:脑的动脉血液分别由颈内动脉和锁骨下动脉的椎动脉供给,进行血管造影的路径主要为股动脉,经股动脉可行全脑DSA。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "脑血管DSA造影" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "脑的动脉血液" }, { "start_idx": 20, "end_idx": 33, "type": "bod", "entity": "颈内动脉和锁骨下动脉的椎动脉" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "血管造影" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "股动脉" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "股动脉" }, { "start_idx": 59, "end_idx": 63, "type": "pro", "entity": "全脑DSA" } ] }, { "text": "(三)临床应用1.颅内动脉瘤诊断颅内动脉瘤主要依靠脑血管DSA。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "颅内动脉瘤" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "颅内动脉瘤" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "脑血管DSA" } ] }, { "text": "造影可显示动脉位置、大小、数目、形状和脑血循环情况,还可判断手术效果。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "造影" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "动脉" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "脑血循环" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "手术" } ] }, { "text": "造影诊断的准确性可达89%~95%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "造影" } ] }, { "text": "2.脑血管畸形其中包括脑动静脉畸形(AVM)、脑海绵状血管瘤(CA)、脑静脉畸形(VM)以及Galen静脉动脉瘤样畸形(VGAM)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "脑血管畸形" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "脑动静脉畸形" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "AVM" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "脑海绵状血管瘤" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "CA" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "脑静脉畸形" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "VM" }, { "start_idx": 46, "end_idx": 58, "type": "dis", "entity": "Galen静脉动脉瘤样畸形" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "VGAM" } ] }, { "text": "DSA目的是观察畸形的部位、大小、形态、供血动脉、引流静脉、窃血现象及正常脑组织的血供情况等,为诊断、治疗及预后提供依据。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "DSA" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "畸形" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "供血动脉" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "引流静脉" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "窃血现象" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "脑组织" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "血" } ] }, { "text": "3.颈内动脉海绵窦瘘(carotid-cavernousfistula,CCF)不管何种CCF,脑DSA是诊断CCF的最可靠的检查方法。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "颈内动脉海绵窦瘘" }, { "start_idx": 11, "end_idx": 34, "type": "dis", "entity": "carotid-cavernousfistula" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "CCF" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "CCF" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "脑DSA" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "CCF" } ] }, { "text": "可了解瘘口的部位和大小、侧支循环建立情况、颈外动脉供血情况、静脉引流方向、是否自行闭塞,以及其他脑底动脉变异脑膜动静脉瘘全面的选择性脑血管DSA是目前确诊本病的唯一检查手段。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "瘘口" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "侧支循环" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "颈外动脉" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "血" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "静脉" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "闭塞" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "脑底动脉" }, { "start_idx": 48, "end_idx": 53, "type": "sym", "entity": "脑底动脉变异" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "脑膜动静脉瘘" }, { "start_idx": 63, "end_idx": 71, "type": "pro", "entity": "选择性脑血管DSA" } ] }, { "text": "检查中应注意瘘口类型、瘘口位置、供血动脉、引流静脉及动静脉分流情况。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "瘘口" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "瘘口" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "供血动脉" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "引流静脉" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "动静脉" } ] }, { "text": "5.烟雾病脑DSA是诊断本病的唯一方法。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "烟雾病脑" }, { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "DSA" } ] }, { "text": "宜行全脑血管DSA及分选颈内、外动脉,便于观察动脉狭窄、闭塞、异常血管网脑静脉血栓脑血管造影是诊断本症的重要方法,尤其是DSA技术,使脑血管影像显示得更清晰,减少漏诊和误诊。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "全脑血管DSA" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "颈内" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "外动脉" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "动脉狭窄、闭塞" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "异常血管网" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "血管网" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "脑静脉血栓" }, { "start_idx": 41, "end_idx": 45, "type": "pro", "entity": "脑血管造影" }, { "start_idx": 60, "end_idx": 64, "type": "pro", "entity": "DSA技术" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "脑血管" } ] }, { "text": "全脑血管DSA对静脉窦闭塞颅内肿瘤颅内肿瘤常引起脑血管的位置、形态、供血方式以及脑血循环的变化,颅内肿瘤可依脑血管DSA的变化,进行定位及定量诊断。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "全脑血管DSA" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "静脉窦" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "静脉窦闭塞" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑血管" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "血" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "脑血循环" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 54, "end_idx": 59, "type": "pro", "entity": "脑血管DSA" } ] }, { "text": "某些颅内肿瘤血循环较为丰富,脑血管DSA可显示其供血动脉和瘤内血管。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "血循环" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "脑血管DSA" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "供血动脉" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "瘤内血管" } ] }, { "text": "根据供血动脉形态及来源、瘤内血管形态和病理循环特点以及邻近脑血管移位情况等,可能作出病理诊断。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "供血动脉" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "瘤内血管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "脑血管" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "脑血管移位" } ] }, { "text": "8.颅脑损伤目前使用CT检查对颅脑损伤诊断的准确性已相当高,而且对患者无损伤,因此脑血管DSA已很少应用于颅脑损伤。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颅脑损伤" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "CT检查" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "颅脑损伤" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "损伤" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "脑血管DSA" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "颅脑损伤" } ] }, { "text": "第二节常见病原实验室诊断一、细菌患者标本中的细菌因受环境影响,形态、大小和染色特性均有变化,这一点在直接涂片检查时应注意。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "细菌" }, { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "细菌" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "染色" }, { "start_idx": 50, "end_idx": 55, "type": "pro", "entity": "直接涂片检查" } ] }, { "text": "(一)细菌的生理生化检查项目繁多,包括生长条件,色素观察,抵抗力的测定,糖、蛋白与氨基酸代谢,有机酸盐与铵盐利用试验,呼吸酶和毒性酶类试验,其他消化及水解试验等。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "细菌的生理生化检查" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "生长条件" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "色素观察" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "抵抗力的测定" }, { "start_idx": 36, "end_idx": 45, "type": "ite", "entity": "糖、蛋白与氨基酸代谢" }, { "start_idx": 47, "end_idx": 57, "type": "pro", "entity": "有机酸盐与铵盐利用试验" }, { "start_idx": 59, "end_idx": 68, "type": "pro", "entity": "呼吸酶和毒性酶类试验" }, { "start_idx": 72, "end_idx": 78, "type": "pro", "entity": "消化及水解试验" } ] }, { "text": "特殊项目有代谢途径试验,测定细菌本身的成分如细胞壁化学组分和分解产物,细菌的致病性与毒力测定。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "代谢途径试验" }, { "start_idx": 12, "end_idx": 33, "type": "pro", "entity": "测定细菌本身的成分如细胞壁化学组分和分解产物" }, { "start_idx": 35, "end_idx": 45, "type": "pro", "entity": "细菌的致病性与毒力测定" } ] }, { "text": "用免疫血清学方法可以分析确定细菌的种或型。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "免疫血清学方法" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "细菌" } ] }, { "text": "(二)细菌的形态学检查形态学检查是细菌检验的重要手段,并为进一步鉴定提供参考依据。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "细菌的形态学检查" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "形态学检查" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "细菌" } ] }, { "text": "分为标本直接形态学检查和培养物形态学检查。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "标本直接形态学检查" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "培养物形态学检查" } ] }, { "text": "前者可以迅速了解标本中有无细菌及大致菌量,并可依据形态、结构及染色大致决定其种属,对及时选用抗生素有一定参考价值,后者还可验证是否为纯种。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "细菌" }, { "start_idx": 18, "end_idx": 18, "type": "mic", "entity": "菌" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "染色" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "抗生素" } ] }, { "text": "有少数细菌常可根据其形态特征得出初步诊断,如痰中抗酸性杆菌和脑脊液中的脑膜炎奈瑟菌。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "痰" }, { "start_idx": 24, "end_idx": 28, "type": "mic", "entity": "抗酸性杆菌" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "脑脊液" }, { "start_idx": 35, "end_idx": 40, "type": "mic", "entity": "脑膜炎奈瑟菌" } ] }, { "text": "1.染色与不染色检查细菌不经染色直接镜检,可观察生活状态下细菌的形态及其运动情况。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "染色" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "不染色检查" }, { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "细菌" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "染色" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "镜检" }, { "start_idx": 29, "end_idx": 30, "type": "mic", "entity": "细菌" } ] }, { "text": "染色后在普通光学显微镜下即可清楚看到细菌的形态和某些结构。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "染色" }, { "start_idx": 4, "end_idx": 10, "type": "equ", "entity": "普通光学显微镜" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "细菌" } ] }, { "text": "2.常用染色法有单染色法、复染色法、特殊染色法、负染色法和荧光染色法等。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "染色法" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "单染色法" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "复染色法" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "特殊染色法" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "负染色法" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "荧光染色法" } ] }, { "text": "单染色法少用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "单染色法" } ] }, { "text": "负染色法细菌不着色,仅背景着色。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "负染色法" }, { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "细菌" } ] }, { "text": "两者均不能显示细菌内结构及染色反应的特点。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "细菌" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "染色" } ] }, { "text": "特殊染色法则能显示荚膜、芽胞、鞭毛等结构。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "特殊染色法" }, { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "荚膜" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "芽胞" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "鞭毛" } ] }, { "text": "荧光染色法可直接用荧光染料处理,或采用荧光抗体技术,后者用于快速诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "荧光染色法" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "荧光抗体技术" } ] }, { "text": "用两种以上不同颜色的染料进行复染,可将细菌染成不同颜色,有鉴别细菌种类的价值。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "复染" }, { "start_idx": 19, "end_idx": 20, "type": "mic", "entity": "细菌" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "细菌" } ] }, { "text": "常用的有革兰染色法与抗酸染色法:①革兰染色,最为常用。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "革兰染色法" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "抗酸染色法" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "革兰染色" } ] }, { "text": "此法可将细菌分为两大类:不被乙醇脱色保留紫色为革兰阳性菌,脱色而被复染成红色为革兰阴性菌。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "细菌" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "乙醇脱色" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "革兰阳性菌" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "脱色" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "复染" }, { "start_idx": 39, "end_idx": 43, "type": "mic", "entity": "革兰阴性菌" } ] }, { "text": "直接涂片革兰染色法可初步提供临床考虑使用何种抗生素,以及调整其他针对性治疗方案。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "直接涂片革兰染色法" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "抗生素" } ] }, { "text": "②抗酸染色法,用于鉴别与发现分枝杆菌属细菌,如结核杆菌与麻风杆菌。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "抗酸染色法" }, { "start_idx": 14, "end_idx": 20, "type": "mic", "entity": "分枝杆菌属细菌" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "麻风杆菌" } ] }, { "text": "(三)细菌培养与分离技术1.无菌技术细菌广泛存在于自然界。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "细菌培养与分离技术" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "无菌技术" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "细菌" } ] }, { "text": "皮肤、器皿、空气与尘埃中都有各种细菌,随时可能污染实验材料与物品,导致错误判定。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮肤" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "细菌" } ] }, { "text": "2.培养基培养基是提高细菌培养阳性率的关键。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "细菌" } ] }, { "text": "不同的细菌有不同的营养需要,当病原不明时培养基的所含成分尤其要丰富,要兼顾多种细菌的需要。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "细菌" } ] }, { "text": "营养不足是血培养致病菌阳性率低的原因之一。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "血培养" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "致病菌" } ] }, { "text": "3.接种与分离技术根据质检标本的性质、培养目的和所用培养基的种类,采用不同的接种方法分离细菌。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "接种与分离技术" }, { "start_idx": 44, "end_idx": 45, "type": "mic", "entity": "细菌" } ] }, { "text": "4.培养条件根据检验目的和细菌种类,选用需氧培养法、二氧化碳培养法和厌氧培养法。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "细菌" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "氧培养法" }, { "start_idx": 26, "end_idx": 32, "type": "pro", "entity": "二氧化碳培养法" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "厌氧培养法" } ] }, { "text": "某些细菌(如流感嗜血杆菌、脑膜炎奈瑟菌和布鲁菌等)在有5%~10%的二氧化碳的条件下才能很好生长。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "细菌" }, { "start_idx": 6, "end_idx": 11, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 13, "end_idx": 18, "type": "mic", "entity": "脑膜炎奈瑟菌" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "布鲁菌" } ] }, { "text": "5.细菌的生物化学试验与血清学试验(1)生物化学试验:各种细菌有其独特的酶系,对营养物的分解能力及其代谢产物各不相同,可借以区分和鉴定细菌。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "细菌" }, { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "生物化学试验" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "血清学试验" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "生物化学试验" }, { "start_idx": 29, "end_idx": 30, "type": "mic", "entity": "细菌" }, { "start_idx": 36, "end_idx": 37, "type": "mic", "entity": "酶系" }, { "start_idx": 67, "end_idx": 68, "type": "mic", "entity": "细菌" } ] }, { "text": "(2)血清学试验:利用抗原抗体反应来鉴定细菌。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血清学试验" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "细菌" } ] }, { "text": "6.动物实验是临床细菌学检验中不可缺少的一部分,其主要用途是分离与鉴定病原微生物,测定毒力,制备免疫血清等。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "临床细菌学检验" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "病原微生物" } ] }, { "text": "(四)抗菌药物敏感试验不同细菌或同种异株细菌,对各种抗菌药物的敏感性不尽相同。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "抗菌药物敏感试验" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "细菌" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "细菌" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "抗菌药物" } ] }, { "text": "所谓敏感与耐药,通常以抗菌药物治疗浓度(抗菌药物常用量在血清中的浓度)与该抗菌药物对细菌的最小抑菌浓度(MIC)的关系而定。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "抗菌药物" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "抗菌药物" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血清" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "抗菌药物" }, { "start_idx": 42, "end_idx": 43, "type": "mic", "entity": "细菌" }, { "start_idx": 48, "end_idx": 48, "type": "mic", "entity": "菌" } ] }, { "text": "(五)特异性抗原检测快速抗原检测诊断细菌感染广泛用于临床。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "特异性抗原检测" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "快速抗原检测" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "细菌感染" } ] }, { "text": "感染局部检出抗原对诊断价值最大,血和尿中查抗原要除外其他部位感染和近期其他感染的可能。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "感染" }, { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "抗原" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "血" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "尿" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "抗原" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "感染" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "感染" } ] }, { "text": "(六)特异性抗体检测细菌感染后,特异抗体的产生需要一段时间,除病程较长的感染性疾病、迁延不愈或慢性期有直接病原学诊断价值外,多数情况下只能是“回顾性诊断”。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "特异性抗体检测" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "细菌感染" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "特异抗体" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "要强调双份血清检测抗体水平才有较大意义。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "ite", "entity": "血清检测抗体水平" } ] }, { "text": "常用的抗体检测有直接凝集试验如肥达反应用于伤寒诊断,瑞氏反应用于布鲁菌病;间接凝集试验可诊断沙门菌感染、肉毒杆菌食物中毒、布鲁菌病;康氏试验用于诊断梅毒感染;琼脂扩散试验已广泛用于对不同抗原抗体系统进行分析研究。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "抗体检测" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "直接凝集试验" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "肥达反应" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "伤寒" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "瑞氏反应" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "布鲁菌病" }, { "start_idx": 37, "end_idx": 42, "type": "pro", "entity": "间接凝集试验" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "沙门菌感染" }, { "start_idx": 52, "end_idx": 59, "type": "dis", "entity": "肉毒杆菌食物中毒" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "布鲁菌病" }, { "start_idx": 66, "end_idx": 69, "type": "pro", "entity": "康氏试验" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "梅毒感染" }, { "start_idx": 79, "end_idx": 84, "type": "pro", "entity": "琼脂扩散试验" }, { "start_idx": 93, "end_idx": 98, "type": "ite", "entity": "抗原抗体系统" } ] }, { "text": "(七)细菌检验的自动化系统许多自动化仪器,采用光散射与发光技术、色谱技术、电气与电子技术、免疫技术与放射技术等,根据细菌的不同生物学性状和代谢产物的差异,逐步发展了微量快速培养基及微量生化反应系统,并使之系统化和标准化。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "细菌检验" }, { "start_idx": 23, "end_idx": 30, "type": "pro", "entity": "光散射与发光技术" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "色谱技术" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "电气与电子技术" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "免疫技术" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "放射技术" }, { "start_idx": 58, "end_idx": 59, "type": "mic", "entity": "细菌" } ] }, { "text": "在细菌检测与抗生素敏感试验方面取得了很大进展。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "细菌检测" }, { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "抗生素敏感试验" } ] }, { "text": "参考文献1.李正,王慧贞,吉士俊.先天畸形学.北京:人民卫生出版社,20002.王忠诚.神经外科学.武汉:湖北科学技术出版社,19983.李世绰,程学铭,王文志,等.神经系统疾病流行病学.北京:人民卫生出版社,20004.肖坤则,张芝燕,高健,等.中国神经管缺陷的流行病学.中华医学杂志,1989,69(4):189-1895.陈晓玲,戴晓瑛,刘建涛,等.叶酸与神经管畸形.河南预防医学杂志,1999,10(5):326-3266.BarberRC,LammerEJ,ShawGM,etal.Theroleoffolatetransportandmetabolisminneuraltubedefectrisk.MolGenetMetab,1999,66(1):1-17.AntonyAC,HansenDK.Hypothesis:folate-responsiveneuraltubedefectsandneurocristopathies.Teratology,2000,62(1):42-428.McCombJG.Spinalandcranialneuraltubedefects.SeminPediatrNeurol,1997,4(3):156-1569.CsabayL,SzaboI,PappC,etal.Centralnervoussystemanomalies.AnnNYAcadSci,1998,847:21-4510.CzeizelAE.Primarypreventionofneural-tubedefectsandsomeothermajorcongenitalabnormalities:recommendationsfortheappropriateuseoffolicacidduringpregnancy.PaediatrDrugs,2000,2(6):437-44911.EskesTK.Openorclosed?", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "先天畸形" }, { "start_idx": 44, "end_idx": 47, "type": "dep", "entity": "神经外科" }, { "start_idx": 83, "end_idx": 88, "type": "dis", "entity": "神经系统疾病" }, { "start_idx": 126, "end_idx": 130, "type": "dis", "entity": "神经管缺陷" }, { "start_idx": 178, "end_idx": 179, "type": "bod", "entity": "叶酸" }, { "start_idx": 181, "end_idx": 185, "type": "dis", "entity": "神经管畸形" } ] }, { "text": "Aworldofdifference:ahistoryofhomocysteineresearch.NutrRev,1998,56(8):236-23612.谭盛葵,仇小强.神经管畸形的环境致畸因素研究进展.中国公共卫生,2006,22(8):1020-102013.朱军,李胜利,等.中国出生缺陷图谱.北京:人民卫生出版社,200814.BlencoweH,CousensS,ModellB,etal.Folicacidtoreduceneonatalmortalityfromneuraltubedisorders.IntJEpidemiol,2010,39(Suppl1):1110-111015.CoppAJ,GreeneND.Geneticsanddevelopmentofneuraltubedefects.JPathol,2010,220(2):217-217", "entities": [ { "start_idx": 87, "end_idx": 91, "type": "dis", "entity": "神经管畸形" } ] }, { "text": "第四章遗传代谢病第一节遗传代谢病概述【概述】遗传代谢病(inbornerrorofmetabolism)又称先天性代谢缺陷病,是遗传性生化代谢缺陷的总称。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 28, "end_idx": 50, "type": "dis", "entity": "inbornerrorofmetabolism" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "先天性代谢缺陷病" }, { "start_idx": 64, "end_idx": 72, "type": "dis", "entity": "遗传性生化代谢缺陷" } ] }, { "text": "遗传代谢病由于基因突变,使蛋白质分子在结构和功能上发生改变酶、受体等的缺陷机体的生化反应和代谢出现异常,反应底物或者中间代谢产物在体内大量蓄积,引起一系列临床表现。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "基因突变" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "蛋白质分子" }, { "start_idx": 13, "end_idx": 28, "type": "sym", "entity": "蛋白质分子在结构和功能上发生改变" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "酶" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "酶、受体等的缺陷" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "受体" }, { "start_idx": 37, "end_idx": 50, "type": "sym", "entity": "机体的生化反应和代谢出现异常" }, { "start_idx": 52, "end_idx": 70, "type": "sym", "entity": "反应底物或者中间代谢产物在体内大量蓄积" } ] }, { "text": "大多数基因突变在临床上具有高度异质性,而且不同的突变造成的疾病,在程度上也大不一样,从轻度到致命,发病也有早有晚。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "基因突变" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "突变" } ] }, { "text": "较多先天性代谢缺陷在新生儿期或婴儿期就可有临床表现,但也有部分或者在成年期发病。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "先天性代谢缺陷" } ] }, { "text": "遗传代谢病种类繁多,目前已达数千种,常见有400~500种,单一病种患病率较低,但是总体发病率较高、危害严重,是临床的疑难杂症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "【遗传代谢病的分类】遗传代谢病种类繁多,根据累及的生化物质的特点可分为糖、氨基酸、尿素循环、有机酸、线粒体、核酸及内分泌等代谢异常(表14-8)。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "糖" }, { "start_idx": 35, "end_idx": 64, "type": "sym", "entity": "糖、氨基酸、尿素循环、有机酸、线粒体、核酸及内分泌等代谢异常" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "氨基酸" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "尿素循环" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "有机酸" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "线粒体" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "核酸" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "内分泌" } ] }, { "text": "表14-8遗传代谢病的分类【发病机制】遗传代谢病的种类繁多,但一般有以下共同特点:1.酶缺乏性疾病的发病机制遗传代谢病较多表现为酶蛋白的缺陷,通过所催化的酶促反应的改变造成疾病。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "酶缺乏性疾病" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "酶蛋白的缺陷" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "酶蛋白" }, { "start_idx": 73, "end_idx": 83, "type": "sym", "entity": "所催化的酶促反应的改变" } ] }, { "text": "在分子水平,酶的缺陷可能有两种原因:一是由于编码酶蛋白的结构基因发生突变酶蛋白结构异常或缺失合成过少的酶造成代谢率改变致病基因的克隆,越来越多的酶缺陷病因已经清楚,疾病的遗传方式多数为常染色体隐性遗传,少数为性连锁遗传或者常染色体显性遗传。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "酶" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "酶的缺陷" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "编码酶蛋白的结构基因发生突变" }, { "start_idx": 22, "end_idx": 31, "type": "bod", "entity": "编码酶蛋白的结构基因" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "酶蛋白" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "酶蛋白结构异常或缺失" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "合成过少的酶" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "酶" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "代谢率改变" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "致病基因" }, { "start_idx": 72, "end_idx": 74, "type": "sym", "entity": "酶缺陷" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "酶" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "常染色" }, { "start_idx": 111, "end_idx": 114, "type": "bod", "entity": "常染色体" } ] }, { "text": "在正常人体内,酶的数量总是大大超过维持机体新陈代谢所必需的数量,因此杂合状态下残存的50%的酶活性一般足以维持机体的正常代谢需要。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "酶" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "酶" } ] }, { "text": "事实上,5%~10%的酶活性就可以使该酶所催化的代谢反应正常进行,并维持底物和产物在适当的水平上。", "entities": [ { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "酶" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "酶" } ] }, { "text": "当然也有一些酶需要有较高活性才能使机体代谢途经正常进行。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "酶" } ] }, { "text": "由于酶的生理功能是催化底物转变为产物,因此几乎所有因酶代谢缺陷所引起的病理改变都直接或间接地与底物的堆积及产物的缺乏有关,在病理情况下堆积之底物常常循旁路代谢途径产生大量旁路代谢产物,也可造成病理性损害。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "酶" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "酶代谢缺陷" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "酶" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "底物的堆积" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "产物的缺乏" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "病理性损害" } ] }, { "text": "例如在苯丙酮尿症时,苯丙氨酸羟化酶缺乏代谢旁路开放,代谢产物苯乙酸及苯乳酸增高这些物质的毒性作用造成了神经系统的损害造成产物皮质醇及醛固酮缺乏水、电解质紊乱和休克,同时导致睾酮升高底物堆积,或产物缺乏,或旁路代谢产物产生为主,产生病理损害。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "苯丙氨酸羟化酶缺乏" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "苯丙氨酸羟化酶" }, { "start_idx": 26, "end_idx": 38, "type": "sym", "entity": "代谢产物苯乙酸及苯乳酸增高" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "苯乙酸" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "苯乳酸" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "神经系统的损害" }, { "start_idx": 60, "end_idx": 70, "type": "sym", "entity": "产物皮质醇及醛固酮缺乏" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "皮质醇" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "醛固酮" }, { "start_idx": 71, "end_idx": 77, "type": "dis", "entity": "水、电解质紊乱" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "休克" }, { "start_idx": 82, "end_idx": 89, "type": "sym", "entity": "同时导致睾酮升高" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "睾酮" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "底物堆积" }, { "start_idx": 96, "end_idx": 99, "type": "sym", "entity": "产物缺乏" }, { "start_idx": 115, "end_idx": 118, "type": "dis", "entity": "病理损害" } ] }, { "text": "2.遗传代谢病的全身性与局部性酶缺乏造成堆积之底物分子的大小与性质与病理损害的范围有关。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "全身性与局部性酶缺乏" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "酶" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "病理损害" } ] }, { "text": "【遗传代谢病的实验室诊断】遗传代谢病的临床表现与酶缺陷的关系有一定的复杂性。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "实验室诊断" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "酶缺陷" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "酶" } ] }, { "text": "在某些情况下,某一基因的突变可导致不同的酶活性改变,表现为不同的临床表型;在另一些情况下,多种不同的基因突变可产生同样的病理变化和临床特征,由此给临床医师对遗传代谢病的病理、生化改变及临床症状的分析带来一定的困难。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "某一基因的突变" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "某一基因" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "不同的酶活性改变" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "酶" }, { "start_idx": 45, "end_idx": 53, "type": "sym", "entity": "多种不同的基因突变" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "多种不同的基因" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "遗传代谢病的诊断依赖实验室检查,尿液三氯化铁试验、尿液二硝基苯肼(DNPH)试验、尿液硝普盐试验(Brand反应)及甲苯胺蓝试验可以对某些疾病进行初步筛查。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "实验室检查" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "尿液三氯化铁试验" }, { "start_idx": 25, "end_idx": 39, "type": "pro", "entity": "尿液二硝基苯肼(DNPH)试验" }, { "start_idx": 41, "end_idx": 47, "type": "pro", "entity": "尿液硝普盐试验" }, { "start_idx": 49, "end_idx": 55, "type": "pro", "entity": "Brand反应" }, { "start_idx": 58, "end_idx": 63, "type": "pro", "entity": "甲苯胺蓝试验" } ] }, { "text": "甲苯胺蓝试验主要用于尿黏多糖的定性,如为阳性,需要根据临床表现,选择黏多糖病各型酶活性检测或者基因突变检测。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "甲苯胺蓝试验" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "尿黏多糖" }, { "start_idx": 34, "end_idx": 44, "type": "pro", "entity": "黏多糖病各型酶活性检测" }, { "start_idx": 47, "end_idx": 52, "type": "pro", "entity": "基因突变检测" } ] }, { "text": "表14-9伴有异常汗味或尿味的遗传疾病表14-10尿液三氯化铁试验与代谢病表14-11尿液二硝基苯肼(DNPH)试验、乙酸试验(Acetest)与疾病遗传代谢病的确诊需进行血氨基酸分析、铜蓝蛋白、半乳糖及17-羟孕酮等特异性底物或者产物的测定。", "entities": [ { "start_idx": 5, "end_idx": 18, "type": "dis", "entity": "伴有异常汗味或尿味的遗传疾病" }, { "start_idx": 25, "end_idx": 32, "type": "pro", "entity": "尿液三氯化铁试验" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "代谢病" }, { "start_idx": 43, "end_idx": 57, "type": "pro", "entity": "尿液二硝基苯肼(DNPH)试验" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "乙酸试验" }, { "start_idx": 64, "end_idx": 70, "type": "pro", "entity": "Acetest" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 86, "end_idx": 91, "type": "pro", "entity": "血氨基酸分析" }, { "start_idx": 93, "end_idx": 120, "type": "pro", "entity": "铜蓝蛋白、半乳糖及17-羟孕酮等特异性底物或者产物的测定" } ] }, { "text": "串联质谱技术(tandemmass,MS/MS)已成为遗传代谢病的常规诊断工具,能对一个标本一次进行数十种氨基酸、有机酸及脂肪酸代谢性疾病的检测,达到了一次实验检测多种疾病的目的,目前已成为遗传代谢病的常规检测项目。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "串联质谱技术" }, { "start_idx": 7, "end_idx": 16, "type": "pro", "entity": "tandemmass" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "MS/MS" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 50, "end_idx": 55, "type": "ite", "entity": "数十种氨基酸" }, { "start_idx": 57, "end_idx": 59, "type": "ite", "entity": "有机酸" }, { "start_idx": 61, "end_idx": 68, "type": "dis", "entity": "脂肪酸代谢性疾病" }, { "start_idx": 78, "end_idx": 81, "type": "pro", "entity": "实验检测" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "串联质谱技术可检测的疾病谱(表14-14)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "串联质谱技术" } ] }, { "text": "气相色谱-质谱技术(gaschromatographymassspectrometry,GC/MS)对诊断有机酸尿症和某些疾病有重要意义。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "气相色谱-质谱技术" }, { "start_idx": 10, "end_idx": 42, "type": "pro", "entity": "gaschromatographymassspectrometry" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "GC/MS" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "有机酸尿症" } ] }, { "text": "酶学测定对酶活性降低的遗传代谢病诊断有重要价值,基因诊断对所有遗传病的最终诊断和分型越来越重要。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "酶学测定" }, { "start_idx": 5, "end_idx": 15, "type": "dis", "entity": "酶活性降低的遗传代谢病" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "基因诊断" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "遗传病" } ] }, { "text": "【临床表现】遗传代谢病可在婴幼儿期、儿童期及青少年期发病,其临床表现有急性危象期、缓解期和缓慢进展期,急性症状和检验异常包括急性代谢性脑病、高氨血症、代谢性酸中毒及低血糖等,随年龄不同有差异,全身各器官均可受累,以神经系统以及消化系统的表现较为突出,有些有容貌异常,毛发及皮肤色素改变。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "急性代谢性脑病" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "高氨血症" }, { "start_idx": 75, "end_idx": 80, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "低血糖" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "神经系统" }, { "start_idx": 113, "end_idx": 116, "type": "bod", "entity": "消化系统" }, { "start_idx": 128, "end_idx": 131, "type": "sym", "entity": "容貌异常" }, { "start_idx": 133, "end_idx": 141, "type": "sym", "entity": "毛发及皮肤色素改变" }, { "start_idx": 133, "end_idx": 134, "type": "bod", "entity": "毛发" }, { "start_idx": 136, "end_idx": 137, "type": "bod", "entity": "皮肤" } ] }, { "text": "有些患儿表现为骨骼畸形心肌肥大,皮疹,白内障,角膜混浊,视神经萎缩耳聋,大头,肝脾肿大黄疸或肝硬化。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "骨骼畸形" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "心肌肥大" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "心肌" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "皮疹" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "白内障" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "角膜混浊" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "角膜" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "视神经萎缩" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "耳聋" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "耳" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "大头" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "头" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肝" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "脾" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "黄疸" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "肝硬化" } ] }, { "text": "有些遗传代谢病在婴儿早期即可有典型表现,如在新生儿期出现喂养困难、呼吸异常、酸中毒、高氨血症、黄疸不退、脱水、电解质异常、持续呕吐、低血糖、肝大、惊厥以及尿中有持续臭味,对于不能用一般疾病解释的临床表现,应疑为遗传代谢病,并做进一步检查。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "喂养困难" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "呼吸异常" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "酸中毒" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "高氨血症" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "黄疸不退" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "脱水" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "电解质异常" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "持续呕吐" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "低血糖" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "肝大" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "肝" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "惊厥" }, { "start_idx": 77, "end_idx": 83, "type": "sym", "entity": "尿中有持续臭味" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "尿中" }, { "start_idx": 105, "end_idx": 109, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "(一)急性代谢性脑病部分遗传代谢病如有机酸中毒、尿素循环缺陷及氨基酸代谢异常以急性脑病为典型表现,临床症状的产生是由异常代谢产物对中枢神经系统的毒性作用所致。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "急性代谢性脑病" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "有机酸中毒" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "尿素循环缺陷" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "氨基酸代谢异常" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "急性脑病" }, { "start_idx": 58, "end_idx": 75, "type": "sym", "entity": "异常代谢产物对中枢神经系统的毒性作用" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "应该尽量在48~72小时内得到氨基酸和有机酸的分析结果。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "氨基酸" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "有机酸" } ] }, { "text": "在确诊前进行恰当的处理可能会挽救患儿生命,避免和减少神经系统后遗症。", "entities": [ { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "神经系统后遗症" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "神经系统" } ] }, { "text": "当怀疑患有蛋白质不耐受症时,应立即停止摄入蛋白质。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "蛋白质不耐受症" } ] }, { "text": "昏迷或依靠机械呼吸的婴儿出现脑水肿时,应立即透析,同时应提供支持治疗。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "昏迷" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑水肿" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "透析" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "支持治疗" } ] }, { "text": "当疑及尿素循环缺陷时,可静脉输入10%精氨酸盐酸6ml/kg(0.6g/kg),输注时间应超过90分钟。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "尿素循环缺陷" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "静脉输入" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "精氨酸盐酸" } ] }, { "text": "患瓜氨酸血症和精氨酸琥珀酸尿症的患儿血氨水平一般可显著下降。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "瓜氨酸血症" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "精氨酸琥珀酸尿症" }, { "start_idx": 18, "end_idx": 28, "type": "sym", "entity": "血氨水平一般可显著下降" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "血氨" } ] }, { "text": "表14-12在新生儿期表现为急性脑病的遗传代谢病实验室检查MMA:甲基丙二酸血症;PA:丙酸血症;IVA:异戊酸血症;MCD:多种羧化酶缺乏症若怀疑有机酸血症,应肌肉注射1mg维生素B12,以验证是否为维生素B12敏感性甲基丙二酸血症。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "急性脑病" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "MMA" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "甲基丙二酸血症" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "PA" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "丙酸血症" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "IVA" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "异戊酸血症" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "MCD" }, { "start_idx": 63, "end_idx": 70, "type": "dis", "entity": "多种羧化酶缺乏症" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 81, "end_idx": 84, "type": "pro", "entity": "肌肉注射" }, { "start_idx": 88, "end_idx": 93, "type": "dru", "entity": "维生素B12" }, { "start_idx": 107, "end_idx": 128, "type": "dis", "entity": "维生素B12敏感性甲基丙二酸血症" } ] }, { "text": "静滴大量葡萄糖以提供足够的热量。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "静滴" }, { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "如果临床改善,最后诊断又不明确者在完全限制蛋白质摄入2~3天后,应给予一些氨基酸,开始可口服或静滴必需氨基酸或总蛋白每日0.5g/kg,以后逐渐增加到每日1.0g/kg,并维持到明确诊断,然后制订一个长期的治疗计划。", "entities": [ { "start_idx": 17, "end_idx": 30, "type": "pro", "entity": "完全限制蛋白质摄入2~3天后" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "氨基酸" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "口服" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "静滴" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "氨基酸" }, { "start_idx": 55, "end_idx": 57, "type": "dru", "entity": "总蛋白" } ] }, { "text": "(二)高氨血症遗传代谢病导致的急性脑病最重要的实验室表现是高氨血症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "高氨血症" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "急性脑病" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "高氨血症" } ] }, { "text": "患这些疾病时血氨水平常超过1000μmol/L(正常值27~82μmol/L)。", "entities": [ { "start_idx": 6, "end_idx": 22, "type": "sym", "entity": "血氨水平常超过1000μmol/L" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "血氨" } ] }, { "text": "患儿神经系统受损及发育延迟程度取决于新生儿时高氨血症昏迷时间的长短。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "神经系统受损" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "神经系统" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "发育延迟" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "高氨血症" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "昏迷" } ] }, { "text": "新生儿一过性高氨血症幸存者不会复发,是否留有神经系统后遗症,取决于新生儿期受损的程度。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "新生儿一过性高氨血症" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "神经系统后遗症" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "受损" } ] }, { "text": "可引起先天性高氨血症的疾病(表14-14)。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "先天性高氨血症" } ] }, { "text": "表14-13引起先天性高氨血症的疾病新生儿高氨血症的鉴别(图14-14)。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "先天性高氨血症" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "新生儿高氨血症" } ] }, { "text": "首发症状出现的时间可提供重要线索,有机酸血症患者,如戊二酸血症Ⅱ型或丙酮酸羧化酶缺陷在最初24小时内可出现高氨血症表现。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "戊二酸血症Ⅱ型" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "丙酮酸羧化酶缺陷" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "高氨血症" } ] }, { "text": "24小时后出现严重高氨血症的新生儿常患有尿素循环缺陷或有机酸血症,患有机酸血症的婴儿常表现为典型的代谢性酸中毒。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "高氨血症" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "尿素循环缺陷" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "机酸血症" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "血浆氨基酸分析有助于这组疾病中各种缺陷的鉴别。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "血浆氨基酸分析" } ] }, { "text": "另外,通过尿乳清酸检测可以鉴别氨甲酰磷酸合成酶和鸟氨酸氨甲酰转移酶缺陷,因为氨甲酰磷酸合成酶缺陷时尿中乳清酸水平降低,而鸟氨酸氨甲酰转移酶缺陷时尿乳清酸升高。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "尿乳清酸检测" }, { "start_idx": 15, "end_idx": 22, "type": "bod", "entity": "氨甲酰磷酸合成酶" }, { "start_idx": 24, "end_idx": 34, "type": "dis", "entity": "鸟氨酸氨甲酰转移酶缺陷" }, { "start_idx": 38, "end_idx": 47, "type": "dis", "entity": "氨甲酰磷酸合成酶缺陷" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "尿" }, { "start_idx": 49, "end_idx": 57, "type": "sym", "entity": "尿中乳清酸水平降低" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "乳清酸" }, { "start_idx": 60, "end_idx": 70, "type": "dis", "entity": "鸟氨酸氨甲酰转移酶缺陷" }, { "start_idx": 72, "end_idx": 77, "type": "sym", "entity": "尿乳清酸升高" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "尿乳清酸" } ] }, { "text": "虽然这组疾病通常无家族史,但高氨血症男婴家庭中若有男性夭折或女性先证者,应怀疑鸟氨酸氨甲酰转移酶缺陷,这是唯一的一种尿素循环缺陷伴性连锁遗传病。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "高氨血症" }, { "start_idx": 39, "end_idx": 49, "type": "dis", "entity": "鸟氨酸氨甲酰转移酶缺陷" }, { "start_idx": 58, "end_idx": 70, "type": "dis", "entity": "尿素循环缺陷伴性连锁遗传病" } ] }, { "text": "(三)代谢性酸中毒遗传代谢病急性发作时的另一种重要的实验室依据是阴离子间隙增加的代谢性酸中毒。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "阴离子间隙增加" }, { "start_idx": 32, "end_idx": 36, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "代谢性酸中毒的婴儿,鉴别诊断(图14-4)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "有机酸血症伴血乳酸升高是由于辅酶A代谢障碍所致,且常伴有中性粒细胞减少症和血小板减少症,这些疾病的临床表现与新生儿败血症很相似。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "血乳酸升高" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "血乳酸" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "辅酶A代谢障碍" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "中性粒细胞减少症" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "血小板减少症" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "新生儿败血症" } ] }, { "text": "图14-3新生儿高氨血症鉴别诊断图ASA:精氨酸琥珀酸图14-4婴儿代谢性酸中毒鉴别诊断图L∶P为乳酸∶丙酮酸PEP,磷酸烯醇丙酮酸婴儿期丙酮酸代谢或呼吸链中的酶缺陷可引起原发性乳酸酸中毒,表现为重度代谢性酸中毒。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "新生儿高氨血症" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "ASA" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "精氨酸琥珀酸" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "L" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "P" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "乳酸" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "丙酮酸" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "PEP" }, { "start_idx": 59, "end_idx": 65, "type": "bod", "entity": "磷酸烯醇丙酮酸" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "丙酮酸" }, { "start_idx": 80, "end_idx": 82, "type": "dis", "entity": "酶缺陷" }, { "start_idx": 86, "end_idx": 93, "type": "dis", "entity": "原发性乳酸酸中毒" }, { "start_idx": 98, "end_idx": 105, "type": "dis", "entity": "重度代谢性酸中毒" } ] }, { "text": "对尿有机酸正常的乳酸酸中毒患者应考虑到这类疾病。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "尿有机酸" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "乳酸酸中毒" } ] }, { "text": "例如,新生儿期典型的非酮体性高血糖症可伴有严重和进行性的中枢神经系统功能障碍,但不伴有代谢性酸中毒,也无高氨血症。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "非酮体性高血糖症" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "中枢神经系统功能障碍" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "高氨血症" } ] }, { "text": "(四)低血糖症糖代谢紊乱在新生儿期极为常见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "低血糖症" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "糖代谢紊乱" } ] }, { "text": "由于血糖测定的方法和采血标本的差异,多数作者认为足月新生儿出生3天内全血血糖<1.67mmol/L(30mg/dl)3天后<2.2mmol/L(40mg/dl);低体重儿出生3天内血糖<1.1mmol/L(20mg/dl)1周后<2.2mmol/L(40mg/dl)为低血糖。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "血糖测定" }, { "start_idx": 26, "end_idx": 57, "type": "sym", "entity": "新生儿出生3天内全血血糖<1.67mmol/L(30mg/dl)" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血糖" }, { "start_idx": 58, "end_idx": 79, "type": "sym", "entity": "3天后<2.2mmol/L(40mg/dl)" }, { "start_idx": 81, "end_idx": 110, "type": "sym", "entity": "低体重儿出生3天内血糖<1.1mmol/L(20mg/dl)" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "血糖" }, { "start_idx": 111, "end_idx": 132, "type": "sym", "entity": "1周后<2.2mmol/L(40mg/dl)" }, { "start_idx": 134, "end_idx": 136, "type": "dis", "entity": "低血糖" } ] }, { "text": "目前有趋势将全血血糖<2.2mmol/L(40mg/dl)新生儿低血糖症。", "entities": [ { "start_idx": 6, "end_idx": 28, "type": "sym", "entity": "全血血糖<2.2mmol/L(40mg/dl)" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血糖" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "新生儿低血糖症" } ] }, { "text": "遗传代谢病导致的新生儿或者婴儿发生低血糖较为少见,主要因碳水化合物代谢缺陷、脂肪酸氧化缺陷及氨基酸代谢缺陷等原因引起。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "低血糖" }, { "start_idx": 28, "end_idx": 36, "type": "dis", "entity": "碳水化合物代谢缺陷" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "脂肪酸氧化缺陷" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "氨基酸代谢缺陷" } ] }, { "text": "最常见的是肝糖原累积病,由于肝脏不能将糖原转变成葡萄糖所造成。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "肝糖原累积病" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "肝脏不能将糖原转变成葡萄糖" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肝脏" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "糖原" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "低血糖常在空腹或者禁食时发作,低血糖症、肝肿大和乳酸血症是这类疾病的突出表现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "低血糖" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "低血糖症" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "肝肿大" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "乳酸血症" } ] }, { "text": "低血糖症也是半乳糖血症和遗传性果糖不耐受症的重要特征,通常在进食含果糖的食物后才出现症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "低血糖症" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "遗传性果糖不耐受症" } ] }, { "text": "除低血糖症进一步加重外,乙酰辅酶A和酮体生成减少。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "低血糖症" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "乙酰辅酶A" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "乙酰辅酶A和酮体生成减少" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "酮体" } ] }, { "text": "因此,虽然有少量酮体产生,这类疾病引起的低血糖,其典型特征仍是非酮性的。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "酮体" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "低血糖" } ] }, { "text": "脂肪酸氧化缺陷最常见的是中链乙酰辅酶A脱氢酶缺乏,除表现为非酮性低血糖症和Reye综合征样表现外,还可表现为猝死。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "脂肪酸氧化缺陷" }, { "start_idx": 12, "end_idx": 23, "type": "dis", "entity": "中链乙酰辅酶A脱氢酶缺乏" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "非酮性低血糖症" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "Reye综合征" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "猝死" } ] }, { "text": "有较多报道诊断为中链乙酰辅酶A脱氢酶缺乏的婴儿,家庭中有同胞死于婴儿猝死综合征。", "entities": [ { "start_idx": 8, "end_idx": 19, "type": "dis", "entity": "中链乙酰辅酶A脱氢酶缺乏" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "婴儿猝死综合征" } ] }, { "text": "任何不明原因死亡的婴儿若在肝脏和肌肉中有脂肪酸沉积,强烈提示有脂肪酸氧化障碍。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肝脏" }, { "start_idx": 13, "end_idx": 24, "type": "sym", "entity": "肝脏和肌肉中有脂肪酸沉积" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肌肉" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "脂肪酸" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "脂肪酸氧化障碍" } ] }, { "text": "极长链脂肪酸乙酰辅酶A脱氢酶缺乏也有低血糖症状,可引起心肌病。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "dis", "entity": "极长链脂肪酸乙酰辅酶A脱氢酶缺乏" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "低血糖" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "心肌病" } ] }, { "text": "患脂肪酸乙酰辅酶A脱氢酶缺乏病的婴儿都可能出现心律失常和心脏骤停。", "entities": [ { "start_idx": 1, "end_idx": 14, "type": "dis", "entity": "脂肪酸乙酰辅酶A脱氢酶缺乏病" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "心律失常" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心脏骤停" } ] }, { "text": "遗传代谢病所致的低血糖病因(表14-14)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "低血糖病" } ] }, { "text": "由于从尿中排出过量的乙酰肉碱,脂肪酸氧化缺陷病导致继发性肉碱缺乏症。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "尿中排出过量的乙酰肉碱" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "乙酰肉碱" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "脂肪酸氧化缺陷病" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "继发性肉碱缺乏症" } ] }, { "text": "对可能发生低血糖者,生后1小时即开始喂(或鼻饲)10%葡萄糖,每次5~10ml/kg,每小时1次,连续3~4次。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "低血糖" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "体重低于2kg或窒息儿复苏困难或延长时,尽快给予5%~10%葡萄糖液2~6ml/kg。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "体重低于2kg" }, { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "体重" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "窒息" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "复苏" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "葡萄糖液" } ] }, { "text": "如用上述方法补充葡萄糖仍不能维持血糖水平可加用氢化可的松5~10mg/(kg•d)。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血糖" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "氢化可的松" } ] }, { "text": "高血糖素(glucagon)0.1~0.3mg/kg肌注,必要时6小时后重复应用,也有一定疗效。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "高血糖素" }, { "start_idx": 5, "end_idx": 12, "type": "dru", "entity": "glucagon" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "肌注" } ] }, { "text": "表14-14遗传代谢病所致的低血糖此外,应积极治疗各种原发病。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "低血糖" } ] }, { "text": "脂肪酸氧化酶缺陷的治疗包括防止禁食,补充足量的葡萄糖,限制脂肪的摄入,提供L-肉碱等。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "脂肪酸氧化酶缺陷" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "防止禁食" }, { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "补充足量的葡萄糖" }, { "start_idx": 27, "end_idx": 33, "type": "pro", "entity": "限制脂肪的摄入" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "提供L-肉碱" } ] }, { "text": "(五)黄疸和肝功能损害遗传代谢病伴有黄疸和肝功能损害的相关疾病和实验室检查(表14-14)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "黄疸" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肝功能损害" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "黄疸" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "肝功能损害" } ] }, { "text": "大部分遗传代谢病引起的胆红素升高为直接胆红素。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "胆红素升高" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "胆红素" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "直接胆红素" } ] }, { "text": "表14-15伴新生儿肝脏损害的遗传代谢病及其实验室检查最明确的伴黄疸的遗传代谢病是半乳糖血症,因半乳糖-1-磷酸转移酶缺乏,导致半乳糖-1-磷酸或其他代谢产物在体内沉积,这些代谢产物对肝脏和其他器官具有直接毒性。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "肝脏损害" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "黄疸" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 48, "end_idx": 60, "type": "dis", "entity": "半乳糖-1-磷酸转移酶缺乏" }, { "start_idx": 64, "end_idx": 83, "type": "sym", "entity": "半乳糖-1-磷酸或其他代谢产物在体内沉积" }, { "start_idx": 64, "end_idx": 71, "type": "bod", "entity": "半乳糖-1-磷酸" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "肝脏" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "器官" } ] }, { "text": "半乳糖对大脑的急性毒性作用不明显。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "半乳糖" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "大脑" } ] }, { "text": "如果半乳糖血症不治疗,将会出现持续性肝脏损害、白内障和严重的智能发育迟缓。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "持续性肝脏损害" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "白内障" }, { "start_idx": 27, "end_idx": 35, "type": "sym", "entity": "严重的智能发育迟缓" } ] }, { "text": "很多患儿在新生儿期死于大肠杆菌性败血症。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "大肠杆菌性败血症" } ] }, { "text": "另一种伴黄疸的代谢性疾病是α1-抗胰蛋白酶缺乏,其临床表现与传统的新生儿肝炎和巨细胞病毒肝炎相似。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "黄疸" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "代谢性疾病" }, { "start_idx": 13, "end_idx": 28, "type": "sym", "entity": "α1-抗胰蛋白酶缺乏" }, { "start_idx": 13, "end_idx": 26, "type": "bod", "entity": "α1-抗胰蛋白酶" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "新生儿肝炎" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "巨细胞病毒肝炎" } ] }, { "text": "因此,所有表现为肝炎综合征的患儿均应测定血清α1-抗胰蛋白酶。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肝炎综合征" }, { "start_idx": 18, "end_idx": 35, "type": "ite", "entity": "测定血清α1-抗胰蛋白酶" } ] }, { "text": "酪氨酸血症的生化特点是血酪氨酸、蛋氨酸升高伴尿酪氨酸排泄增加。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "酪氨酸血症" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "血酪氨酸、蛋氨酸升高" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "血酪氨酸" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "蛋氨酸" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "尿酪氨酸排泄增加" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "尿酪氨酸" } ] }, { "text": "测定尿中的琥珀酰丙酮有助于本病的诊断。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "ite", "entity": "尿中的琥珀酰丙酮" } ] }, { "text": "确诊需要肝脏活检或尸体解剖。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "肝脏活检" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "尸体解剖" } ] }, { "text": "三、支原体目前已知支原体有150多种,其中15种有致病性。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "支原体" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "支原体" } ] }, { "text": "引起人类疾病的常见的有5种:肺炎支原体(M.pneumoniae)、解脲支原体(Ureaplasmaurealyticum)、人型支原体(M.homins)、生殖性支原体(M.genitalium)及发酵支原体(M.ermentans)等。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 20, "end_idx": 31, "type": "mic", "entity": "M.pneumoniae" }, { "start_idx": 34, "end_idx": 38, "type": "mic", "entity": "解脲支原体" }, { "start_idx": 40, "end_idx": 60, "type": "mic", "entity": "Ureaplasmaurealyticum" }, { "start_idx": 63, "end_idx": 67, "type": "mic", "entity": "人型支原体" }, { "start_idx": 69, "end_idx": 76, "type": "mic", "entity": "M.homins" }, { "start_idx": 79, "end_idx": 84, "type": "mic", "entity": "生殖性支原体" }, { "start_idx": 86, "end_idx": 97, "type": "mic", "entity": "M.genitalium" }, { "start_idx": 100, "end_idx": 104, "type": "mic", "entity": "发酵支原体" }, { "start_idx": 106, "end_idx": 116, "type": "mic", "entity": "M.ermentans" } ] }, { "text": "肺炎支原体是学龄期儿童和青壮年非细菌性呼吸道感染的主要病原之一,而人型支原体、生殖性支原体等为机会致病,有时可引起咽喉炎、败血症、腹膜炎、尿道炎和泌尿生殖系统感染。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "非细菌性呼吸道感染" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "人型支原体" }, { "start_idx": 39, "end_idx": 44, "type": "mic", "entity": "生殖性支原体" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "咽喉炎" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "败血症" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "尿道炎" }, { "start_idx": 73, "end_idx": 80, "type": "dis", "entity": "泌尿生殖系统感染" } ] }, { "text": "(一)标本的采集标本包括咽拭子、痰、炎症渗出物、呼吸道和泌尿生殖系统分泌物。", "entities": [ { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "痰" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "炎症渗出物" }, { "start_idx": 24, "end_idx": 36, "type": "bod", "entity": "呼吸道和泌尿生殖系统分泌物" } ] }, { "text": "由于支原体对干燥敏感,标本应立即接种于内含0.5%小牛血清蛋白和200U/ml青霉素大豆胰蛋白酶肉汤转种培养基小瓶中。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "支原体" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "青霉素" } ] }, { "text": "注意体液、痰等标本可经1∶10、1∶100稀释后接种,以减少体液中的抑制物质。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "体液" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "痰" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "体液" } ] }, { "text": "(二)染色及镜检支原体为革兰染色阴性,但不易着色。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "染色" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "镜检" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "支原体" } ] }, { "text": "用吉姆萨染色呈蓝色。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "吉姆萨染色" } ] }, { "text": "此外,还可用暗视野方法、免疫荧光标记方法观察。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "暗视野方法" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "免疫荧光标记方法" } ] }, { "text": "(三)分离培养支原体可人工培养,营养要求高于一般细菌。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "分离培养支原体" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "人工培养" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "细菌" } ] }, { "text": "多数支原体生长缓慢,初次分离培养时,需1周左右或更长时间才能观察出生长现象。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "支原体" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "分离培养" } ] }, { "text": "(四)血清学检测人类经支原体感染后,血清中可出现具有保护性的表面抗原的抗体,同时尚可有部分患儿非特异性冷凝集和GM株链球菌凝集素增高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血清学检测" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "支原体感染" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "血清中" }, { "start_idx": 24, "end_idx": 36, "type": "bod", "entity": "具有保护性的表面抗原的抗体" }, { "start_idx": 47, "end_idx": 65, "type": "sym", "entity": "非特异性冷凝集和GM株链球菌凝集素增高" }, { "start_idx": 47, "end_idx": 53, "type": "ite", "entity": "非特异性冷凝集" }, { "start_idx": 55, "end_idx": 63, "type": "ite", "entity": "GM株链球菌凝集素" } ] }, { "text": "血清学检查有助于支原体感染性疾病的诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "血清学检查" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "支原体感染性疾病" } ] }, { "text": "二、气溶胶吸入疗法的药代动力学吸入疗法的目的是在气道局部作用的同时减少药物的系统性不良反应。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "气溶胶吸入疗法" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "药代动力学吸入疗法" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "气道" } ] }, { "text": "吸入疗法药物周身性吸收存在两种可能的途径:①吸入药物沉积在口腔和咽部的部分,除非经漱口吐掉,不然最终被咽下,经过胃肠道吸收。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "吸入疗法药物周身性吸收" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "口腔" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "咽部" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "胃肠道" } ] }, { "text": "其中有一部分未在肠壁或肝脏首过代谢中灭活,而产生全身不良作用。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肠壁" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肝脏" } ] }, { "text": "②进入气道或肺部部分,目前所用的吸入药物进入气道后基本上以原型通过气道或肺部的庞大表面积吸收。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "气道" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肺部" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "气道" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "气道" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肺部" } ] }, { "text": "局部作用与全身作用的比率取决于以下几个因素:①下呼吸道与口咽部药物沉积之比:药物在支气管内沉积不仅受吸入器内药物载体复合物性质和吸入技巧的影响,而且与使用的吸入装置类型有关(表8-8)。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "下呼吸道" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "口咽部" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "支气管内" }, { "start_idx": 50, "end_idx": 52, "type": "equ", "entity": "吸入器" } ] }, { "text": "在pMDI上连用储雾罐可以解决协同性问题,也可减少了口咽部的药物沉积(减为5%左右)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "equ", "entity": "pMDI" }, { "start_idx": 8, "end_idx": 10, "type": "equ", "entity": "储雾罐" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "口咽部" } ] }, { "text": "②药物经胃肠道吸收和首过代谢后的全身作用:减少口咽部药物沉积和用药后漱口吐掉是减少胃肠道吸收重要手段;药物的首过代谢同样很重要,首过代谢率越高,药物经过胃肠道吸收就越少。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "胃肠道" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "口咽部" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "胃肠道" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "胃肠道" } ] }, { "text": "③药物在气道中的局部活性。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "气道" } ] }, { "text": "④药物在气道和肺中的代谢。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "气道" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肺" } ] }, { "text": "二、触电和雷击【病因】小儿玩弄电器,误触裸露的电线和插头,是导致小儿触电的主要原因。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "触电" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "雷击" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "触电" } ] }, { "text": "近年,随着人民生活水平的提高,家用电器使用增加,儿童触电的意外事故也大大增加。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "触电" } ] }, { "text": "【临床表现】电流对人体的损伤程度主要取决于触电电压的大小和接触时间。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "损伤" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "触电" } ] }, { "text": "其临床主要表现为局部灼伤和全身反应。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "局部灼伤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "全身" } ] }, { "text": "(一)局部灼伤见于电源接触部位,可使局部组织发生严重的灼伤,皮肤可见黄色或褐色干燥灼伤,偶见水泡,与周围皮肤界限清楚,严重灼伤者可深达肌肉和骨骼,甚至于皮肤碳化、骨骼断裂。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "局部灼伤" }, { "start_idx": 18, "end_idx": 28, "type": "sym", "entity": "局部组织发生严重的灼伤" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "局部组织" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "皮肤" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "皮肤可见黄色或褐色干燥灼伤" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "偶见水泡" }, { "start_idx": 49, "end_idx": 57, "type": "sym", "entity": "与周围皮肤界限清楚" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "周围皮肤" }, { "start_idx": 59, "end_idx": 71, "type": "sym", "entity": "严重灼伤者可深达肌肉和骨骼" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "肌肉" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "骨骼" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "皮肤" }, { "start_idx": 76, "end_idx": 79, "type": "sym", "entity": "皮肤碳化" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "骨骼" }, { "start_idx": 81, "end_idx": 84, "type": "sym", "entity": "骨骼断裂" } ] }, { "text": "电击伤造成的组织损伤较一般烧伤重,虽然局部温度不高,但强烈的电流产生的电离作用通常会使局部深部肌肉等组织细胞坏死和血管内血液凝固,累及心脑等脏器者很难存活。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "电击伤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "组织损伤" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "烧伤" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "局部温度不高" }, { "start_idx": 43, "end_idx": 55, "type": "sym", "entity": "局部深部肌肉等组织细胞坏死" }, { "start_idx": 45, "end_idx": 53, "type": "bod", "entity": "深部肌肉等组织细胞" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "坏死" }, { "start_idx": 57, "end_idx": 63, "type": "sym", "entity": "血管内血液凝固" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "血管内血液" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "心脑等脏器" } ] }, { "text": "人是导体,决不能用手直接去推拉或接触触电者,也不能用潮湿的或导电的制品去挑开电线,以免自身触电。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "手" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "触电" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "触电" } ] }, { "text": "对呼吸心跳停止的抢救,详见本篇第二章。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "呼吸心跳停止" } ] }, { "text": "(二)灼伤局部处理局部灼伤处理与一般烫伤处理相同,即清洁创面、消毒包扎,如有其他并发症,如骨折、颅脑损伤、内脏出血,也应立即处理。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "灼伤" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "灼伤" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "烫伤" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "清洁创面" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "消毒包扎" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "骨折" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "颅脑损伤" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "内脏出血" } ] }, { "text": "七、细菌性肺炎(一)支气管肺炎好发于两肺内侧带以及心膈角,病灶表现为多发、散在、大小不等及密度不均匀,常有肺透亮度增强与肺纹理增强。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "支气管肺炎" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "两肺内侧带" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "心膈角" }, { "start_idx": 34, "end_idx": 49, "type": "sym", "entity": "多发、散在、大小不等及密度不均匀" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "肺透亮度增强" }, { "start_idx": 53, "end_idx": 56, "type": "ite", "entity": "肺透亮度" }, { "start_idx": 60, "end_idx": 64, "type": "sym", "entity": "肺纹理增强" } ] }, { "text": "(二)金黄色葡萄球菌肺炎以两下肺野为多,呈小叶分布,涉及一叶和多个肺叶,病灶常表现斑片状密度增高影,边缘模糊。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "金黄色葡萄球菌肺炎" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "以两下肺野为多" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "两下肺野" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "呈小叶分布" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "小叶" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "涉及一叶和多个肺叶" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "一叶和多个肺叶" }, { "start_idx": 41, "end_idx": 53, "type": "sym", "entity": "斑片状密度增高影,边缘模糊" } ] }, { "text": "随着病变的发展,病灶中央可出现低密度,继之出现肺脓疡、肺大疱、胸膜炎、胸腔积液、纵隔积气及液气胸。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "病灶中央可出现低密度" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肺大疱" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "胸腔积液" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "纵隔积气" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "液气胸" } ] }, { "text": "三、鹦鹉热衣原体肺炎鹦鹉热衣原体肺炎(chlamydiapsittacipneumonia)属人畜共患性疾病。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "鹦鹉热衣原体肺炎" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "鹦鹉热衣原体肺炎" }, { "start_idx": 19, "end_idx": 44, "type": "dis", "entity": "chlamydiapsittacipneumonia" } ] }, { "text": "多由吸入含衣原体的鸟类干燥排泄物或污染的尘埃等引起。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "衣原体" } ] }, { "text": "本病临床症状与支气管肺炎相似,但起病较急,全身症状明显如寒战、头痛、肌痛、乏力、发热等,咳嗽剧烈。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "支气管肺炎" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "寒战" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "头痛" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "头" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "肌痛" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肌" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "乏力" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "发热" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "咳嗽剧烈" } ] }, { "text": "如有上述症状及与鸟类、猫等密切接触史,应怀疑本病,并进行相应的病原学检查。", "entities": [ { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "病原学检查" } ] }, { "text": "本病国外首选四环素治疗。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "四环素治疗" } ] }, { "text": "但由于其对小儿骨骼和牙齿发育的不良影响,8岁以内小儿仍首选红霉素治疗,疗程延长至3周左右。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "骨骼" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "牙齿" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "红霉素治疗" } ] }, { "text": "第九章横纹肌肉瘤及其他软组织肉瘤第一节横纹肌肉瘤横纹肌肉瘤(rhadomyosarcoma)是发生自胚胎间叶组织的恶性肿瘤。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "软组织肉瘤" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 31, "end_idx": 45, "type": "dis", "entity": "rhadomyosarcoma" }, { "start_idx": 51, "end_idx": 56, "type": "bod", "entity": "胚胎间叶组织" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "美国每年报道大约250个儿童横纹肌肉瘤的新病例。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "肿瘤发病原因不详,但不排除遗传因素。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" } ] }, { "text": "临床表现的多样性、病理改变的多重性以及发病部位的不同,使横纹肌肉瘤成为小儿肿瘤中最复杂的一种。", "entities": [ { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "小儿肿瘤" } ] }, { "text": "在过去的几十年里,针对肿瘤的不同发生部位及其扩展的范围采用化疗、放疗与手术相结合的措施,患者的生存率有了显著地提高。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肿瘤" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "化疗" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "放疗" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "手术" } ] }, { "text": "最早对横纹肌肉瘤的描述是在1854年,Webner报道了一个21岁舌部横纹肌肉瘤的病人。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "舌部横纹肌肉瘤" } ] }, { "text": "1946年Stout详细报道了一组成人四肢及躯干恶性肿瘤的患者。", "entities": [ { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "四肢及躯干恶性肿瘤" } ] }, { "text": "1952年和1954年Pack分别报道了一些婴儿及儿童横纹肌肉瘤的病例。", "entities": [ { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "1958年Horn和Enterline将横纹肌肉瘤分成以下四种亚型:胚胎型、腺泡型、葡萄簇型和多形型。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "早期的根治性手术方法包括截肢、骨盆及眼眶肿瘤剜除术。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "根治性手术" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "截肢" }, { "start_idx": 15, "end_idx": 24, "type": "pro", "entity": "骨盆及眼眶肿瘤剜除术" } ] }, { "text": "根据肿瘤发生部位不同,生存率不等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" } ] }, { "text": "1961年Pinkel和Pinkren提倡术后预防性化疗及放疗,标志着综合治疗的开端,由此导致1972年横纹肌肉瘤治疗协作组织(intergrouprhabdomyosarcomastudy,IRS)成立。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "化疗" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "放疗" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "综合治疗" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "现在大部分关于横纹肌肉瘤的治疗措施都以此和国际儿科肿瘤协会(SIOP)的研究成果为基础。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 23, "end_idx": 24, "type": "dep", "entity": "儿科" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肿瘤" } ] }, { "text": "【临床表现】横纹肌肉瘤可发生于任何器官或部位,多是由患儿父母最先发现无症状包块。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "无症状包块" } ] }, { "text": "不同部位的肿瘤尚可有部位特征性的症状和体征。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" } ] }, { "text": "不同部位的横纹肌肉瘤各有其临床特点:(一)头颈部头颈部肿瘤根据发生的部位可分为眼眶、脑膜周围和非脑膜周围三种。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "头颈部" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "头颈部肿瘤" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "眼眶" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "脑膜周围" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "非脑膜周围" } ] }, { "text": "眼眶内横纹肌肉瘤可导致眼球凸出、球结膜水肿或眼睑和结膜肿块可能失明、眼肌麻痹,或者两者都有。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "眼眶内横纹肌肉瘤" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "眼球凸出" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "眼球" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "球结膜水肿" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "球结膜" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "眼睑和结膜肿块" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "眼睑" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "结膜" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "失明" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "眼肌麻痹" } ] }, { "text": "脑膜周围肿瘤经常侵蚀颅骨,引起脑神经瘫痪和脑膜刺激症状鼻咽部肿瘤可引起声音改变、气道梗阻、窒息和鼻出血鼻旁窦肿瘤有疼痛、鼻腔分泌物增多、鼻出血症状中耳及乳突部位肿瘤可呈息肉样从耳凸出面神经瘫痪表现颈部淋巴结转移。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "脑膜周围肿瘤" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "经常侵蚀颅骨" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "颅骨" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "脑神经瘫痪" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "脑膜刺激症状" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "脑膜" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "鼻咽部肿瘤" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "声音改变" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "气道梗阻" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "窒息" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "鼻出血" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "鼻旁窦肿瘤" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "疼痛" }, { "start_idx": 60, "end_idx": 66, "type": "sym", "entity": "鼻腔分泌物增多" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "鼻腔分泌物" }, { "start_idx": 68, "end_idx": 72, "type": "sym", "entity": "鼻出血症状" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "鼻出血" }, { "start_idx": 73, "end_idx": 81, "type": "dis", "entity": "中耳及乳突部位肿瘤" }, { "start_idx": 84, "end_idx": 90, "type": "sym", "entity": "息肉样从耳凸出" }, { "start_idx": 88, "end_idx": 88, "type": "bod", "entity": "耳" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "面神经瘫痪" }, { "start_idx": 98, "end_idx": 104, "type": "sym", "entity": "颈部淋巴结转移" }, { "start_idx": 98, "end_idx": 102, "type": "bod", "entity": "颈部淋巴结" } ] }, { "text": "眼眶肿瘤预后最好,而脑膜周围肿瘤预后最差。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "眼眶肿瘤" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "脑膜周围肿瘤" } ] }, { "text": "头颈部横纹肌肉瘤的组织类型大多数为胚胎型。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "头颈部横纹肌肉瘤" } ] }, { "text": "(二)泌尿生殖系泌尿生殖系肿瘤可表现为尿路梗阻、血尿或两者兼有,而盆腔肿物可引起腹痛或肠梗阻,阴道、宫颈、子宫肿瘤可有阴道分泌物睾丸旁肿瘤通常是单侧的、无痛性阴囊内肿块,偶伴腹膜后肿块,多继发于淋巴扩散,占27%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "泌尿生殖系" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "泌尿生殖系肿瘤" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "尿路梗阻" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "尿路" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "血尿" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "盆腔肿物" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "腹痛" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "腹" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 47, "end_idx": 56, "type": "dis", "entity": "阴道、宫颈、子宫肿瘤" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "阴道分泌物" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "睾丸旁肿瘤" }, { "start_idx": 72, "end_idx": 83, "type": "sym", "entity": "单侧的、无痛性阴囊内肿块" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "阴囊内" }, { "start_idx": 85, "end_idx": 91, "type": "sym", "entity": "偶伴腹膜后肿块" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "腹膜后" }, { "start_idx": 93, "end_idx": 100, "type": "sym", "entity": "多继发于淋巴扩散" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "淋巴" } ] }, { "text": "(三)四肢和躯干肢体和躯干部肿瘤表现为肿块,一些患者则先出现淋巴结转移的症状(占12%)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "四肢和躯干" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "肢体和躯干部肿瘤" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "肿块" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "淋巴结" } ] }, { "text": "胸部肿瘤可压迫呼吸。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "胸部肿瘤" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "压迫呼吸" } ] }, { "text": "脊柱旁肿瘤可有脊髓压迫和神经症状腹膜后横纹肌肉瘤可非常大,导致腹痛、消化道或尿路梗阻症状消化道和胆道肿瘤少见,但如发生,则表现为消化道或胆道梗阻心脏肿瘤一般有充血性心力衰竭或心律不齐。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "脊柱旁肿瘤" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "脊髓压迫" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "脊髓" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "神经症状" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "神经" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "腹膜后横纹肌肉瘤" }, { "start_idx": 16, "end_idx": 27, "type": "sym", "entity": "腹膜后横纹肌肉瘤可非常大" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "腹痛" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "腹" }, { "start_idx": 34, "end_idx": 43, "type": "sym", "entity": "消化道或尿路梗阻症状" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "消化道" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "尿路" }, { "start_idx": 44, "end_idx": 51, "type": "dis", "entity": "消化道和胆道肿瘤" }, { "start_idx": 64, "end_idx": 71, "type": "sym", "entity": "消化道或胆道梗阻" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "消化道" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "胆道" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "心脏肿瘤" }, { "start_idx": 79, "end_idx": 85, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "心律不齐" } ] }, { "text": "另一个少见的发病部位是气管和肺。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "气管" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肺" } ] }, { "text": "【诊断】诊断要仔细检查原发病部位和周围淋巴组织。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "周围淋巴组织" } ] }, { "text": "骨扫描可发现骨病变,CT可见肝、脑、心和肺转移与否,头颈部病变要做CT或MRI检查,同时做脑脊液化验。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "骨扫描" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "骨病变" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "CT" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肝" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "脑" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "心" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "肺" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "头颈部病变" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "CT" }, { "start_idx": 36, "end_idx": 40, "type": "pro", "entity": "MRI检查" }, { "start_idx": 45, "end_idx": 49, "type": "pro", "entity": "脑脊液化验" } ] }, { "text": "脊柱旁肿瘤可用MRI观察脊髓浸润情况。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "脊柱旁肿瘤" }, { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "MRI" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "脊髓浸润情况" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脊髓" } ] }, { "text": "【病理】横纹肌肉瘤为向横纹肌分化的肿瘤病变,可发生于任何部位的间叶组织,包括那些没有横纹肌成分的组织。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "横纹肌" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "肿瘤病变" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "间叶组织" }, { "start_idx": 40, "end_idx": 49, "type": "bod", "entity": "没有横纹肌成分的组织" } ] }, { "text": "1958年由Horn和Enterline首先提出了横纹肌肉瘤的诊断标准,随着生物技术的发展,例如免疫分型、细胞和分子遗传学知识的应用,已在某些方面对其进行了修改,内容更为详细,更具有预后判断价值(表11-11)。", "entities": [ { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "免疫分型" }, { "start_idx": 53, "end_idx": 65, "type": "pro", "entity": "细胞和分子遗传学知识的应用" } ] }, { "text": "美国国立癌症研究院(nationalcancerinstitute,NCI)提出采用现有的所有诊断方法,结合临床情况对肿瘤的亚型进行确定。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "癌症" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "肿瘤" } ] }, { "text": "泌尿系横纹肌肉瘤的80%,头颈部的60%,其他部位的50%(不包括四肢和躯干)均属于这种类型。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "泌尿系横纹肌肉瘤" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "头颈部" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "四肢" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "躯干" } ] }, { "text": "梭状细胞胚胎型主要见于睾丸旁横纹肌肉瘤,偶见于头颈部的浅表部位,其预后非常好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "梭状细胞" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "睾丸旁横纹肌肉瘤" }, { "start_idx": 23, "end_idx": 30, "type": "bod", "entity": "头颈部的浅表部位" } ] }, { "text": "表11-23国际横纹肌肉瘤分型葡萄簇样肉瘤是胚胎型横纹肌肉瘤的另一种类型,好原发于内脏空腔器官,如阴道、鼻咽部和胆道。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "葡萄簇样肉瘤" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "胚胎型横纹肌肉瘤" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "内脏空腔器官" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "阴道" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "胆道" } ] }, { "text": "多形型或成人型横纹肌肉瘤在小儿最少见。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "多形型或成人型横纹肌肉瘤" } ] }, { "text": "其特点是可见大的多形性细胞和多核大细胞,以四肢和躯干最多见。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "多形性细胞" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "多核大细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "四肢" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "躯干" } ] }, { "text": "未分化的肉瘤最多见于四肢、头颈部,预后非常差。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "未分化的肉瘤" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "四肢" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "头颈部" } ] }, { "text": "还有一种软组织类型的横纹肌肉瘤也可见于儿童,其不能用传统的方法分类,统称为肉瘤,类型待定。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "肉瘤" } ] }, { "text": "免疫组织化学可利用针对骨骼肌以及生肌蛋白的抗体来显示肿瘤中横纹肌成分。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "免疫组织化学" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "生肌蛋白的抗体" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "肿瘤" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "横纹肌" } ] }, { "text": "Antidesmin、多特异性肌动蛋白(multispecificactin)、肌红蛋白D(myoglobinD,MyoD)都是最敏感的标记物。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "ite", "entity": "Antidesmin" }, { "start_idx": 11, "end_idx": 18, "type": "ite", "entity": "多特异性肌动蛋白" }, { "start_idx": 20, "end_idx": 37, "type": "ite", "entity": "multispecificactin" }, { "start_idx": 40, "end_idx": 44, "type": "ite", "entity": "肌红蛋白D" }, { "start_idx": 46, "end_idx": 55, "type": "ite", "entity": "myoglobinD" }, { "start_idx": 57, "end_idx": 60, "type": "ite", "entity": "MyoD" } ] }, { "text": "vimentin、肌红蛋白、dystrophin、cytokeratin、肌酸激酶(creatinekinase)M和B、S100和神经特异性烯醇化酶用来做进一步的鉴别诊断之用。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "vimentin" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "肌红蛋白" }, { "start_idx": 14, "end_idx": 23, "type": "bod", "entity": "dystrophin" }, { "start_idx": 25, "end_idx": 35, "type": "bod", "entity": "cytokeratin" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "肌酸激酶" }, { "start_idx": 42, "end_idx": 55, "type": "bod", "entity": "creatinekinase" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "M和B" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "S100" }, { "start_idx": 66, "end_idx": 74, "type": "bod", "entity": "神经特异性烯醇化酶" } ] }, { "text": "肌红蛋白D的表达在生肌前体转换为肌肉细胞中有重要意义,而在横纹肌肉瘤中这一过程是受到抑制的。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肌红蛋白D" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "生肌前体" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "肌肉细胞" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "脱氧核糖核酸(DNA)含量(或称倍体)也具有一定的诊断价值。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "脱氧核糖核酸" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "DNA" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "称倍体" } ] }, { "text": "37个无法手术切除的横纹肌肉瘤,8个2倍体的患儿全部死亡,而12个高倍体肿瘤中的10人存活(P<0.0001),但结果缺乏进一步证明。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "高倍体肿瘤" } ] }, { "text": "因为上述生物学、免疫学和细胞学诊断方法在预后判断以及治疗上的重要指导作用,外科医生必须获得足够多的肿瘤送检验,以确保诊断的正确。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "生物学" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "免疫学" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "细胞学诊断方法" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "肿瘤" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "检验" } ] }, { "text": "【分期】IRS的横纹肌肉瘤分级主要是根据临床,尤其是术前和术中的情况,而肿瘤生物学特征并没能予以反映,因此称其为外科病理分期也不为过。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肿瘤" }, { "start_idx": 56, "end_idx": 61, "type": "pro", "entity": "外科病理分期" } ] }, { "text": "比较IRSⅡ,Ⅲ级的病人数量有增加,这是主要是因为手术完全切除率下降所致。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "手术" } ] }, { "text": "有些肿瘤如位于盆腔部位其手术目的就不是完整切除。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "盆腔部位" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "完整切除" } ] }, { "text": "Ⅰ级患儿中42%发生于除膀胱-前列腺外的泌尿生殖系,31%发生于肢体。", "entities": [ { "start_idx": 11, "end_idx": 24, "type": "bod", "entity": "除膀胱-前列腺外的泌尿生殖系" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肢体" } ] }, { "text": "Ⅲ级中46%为头颈部肿瘤。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "头颈部肿瘤" } ] }, { "text": "Ⅳ级中31%为肢体肿瘤,14%为脑膜周围肿瘤,41%为其他类型。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "肢体肿瘤" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "脑膜周围肿瘤" } ] }, { "text": "表11-24IRS横纹肌肉瘤临床分级Lawrence和Gehan等人通过对IRSⅡ的资料进行分析,认定肿瘤部位、大小、侵袭范围及局部淋巴结有无转移对无远处转移的患儿的生存有重要意义。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肿瘤" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "局部淋巴结" } ] }, { "text": "这种分期主要根据肿瘤的生物学特征而非手术切除程度,能够更好地预测预后情况(表11-11)。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "手术切除" } ] }, { "text": "总地来说,肿瘤原发部位和远处转移是决定预后的最有价值的指标。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" } ] }, { "text": "表11-25Lawrence横纹肌肉瘤临床分期表11-26根据Lawrence临床分期的生存率【治疗】横纹肌肉瘤的治疗需要多学科联合进行,包括外科手术、放疗和化疗。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "外科手术" }, { "start_idx": 76, "end_idx": 77, "type": "pro", "entity": "放疗" }, { "start_idx": 79, "end_idx": 80, "type": "pro", "entity": "化疗" } ] }, { "text": "(一)外科手术无论是初次手术还是再次手术均应彻底切除原发肿瘤和周围一定范围的未被侵袭的组织,但是手术必须同时考虑到美容和保护器官功能,大块组织或器官的切除在横纹肌肉瘤的治疗中不再具有价值。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "外科手术" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "手术" }, { "start_idx": 22, "end_idx": 44, "type": "pro", "entity": "彻底切除原发肿瘤和周围一定范围的未被侵袭的组织" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "手术" }, { "start_idx": 57, "end_idx": 58, "type": "pro", "entity": "美容" }, { "start_idx": 60, "end_idx": 65, "type": "pro", "entity": "保护器官功能" }, { "start_idx": 67, "end_idx": 76, "type": "pro", "entity": "大块组织或器官的切除" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "活检以开放手术方法进行比较可靠,针刺或细针穿刺的办法只在比较安全的情况下才考虑。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "活检" }, { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "开放手术方法" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "针刺" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "细针穿刺" } ] }, { "text": "肿瘤组织要足够多,一定要取淋巴结作病理以明确有否转移。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肿瘤组织" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "取淋巴结作病理" } ] }, { "text": "头颈部肿瘤如果无临床症可不做淋巴结活检。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "头颈部肿瘤" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "淋巴结活检" } ] }, { "text": "四肢的横纹肌肉瘤则必须行局部淋巴结评估,因为这将决定放疗的范围。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "四肢的横纹肌肉瘤" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "局部淋巴结评估" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "放疗" } ] }, { "text": "1.再切除术如果首次手术有大体或镜下的肿瘤组织残留,或者术中难以确定周围是否侵袭,那么有必要在辅助治疗实施前再度手术,此为再切除术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "再切除术" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "手术" }, { "start_idx": 16, "end_idx": 16, "type": "equ", "entity": "镜" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "肿瘤组织残留" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "辅助治疗" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "手术" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "再切除术" } ] }, { "text": "Hays的研究表明,四肢和躯干的镜下残余肿瘤(ⅡA级)经肿瘤床再切除手术,可在化疗和放疗前转为临床Ⅰ级,其存活率明显高于没有经再切除术的对照组和首次手术后即为Ⅰ级的病人。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "四肢" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "躯干" }, { "start_idx": 16, "end_idx": 16, "type": "equ", "entity": "镜" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肿瘤" }, { "start_idx": 27, "end_idx": 35, "type": "pro", "entity": "经肿瘤床再切除手术" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "化疗" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "放疗" }, { "start_idx": 63, "end_idx": 66, "type": "pro", "entity": "再切除术" }, { "start_idx": 74, "end_idx": 75, "type": "pro", "entity": "手术" } ] }, { "text": "即使术中没有发现明显的周围组织浸润,只要术前没有估计到是恶性肿瘤而未按其原则手术,均需行再切除术,这尤其适用于四肢和躯干的肿瘤。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "明显的周围组织浸润" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "周围组织" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "手术" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "再切除术" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "四肢和躯干的肿瘤" } ] }, { "text": "2.二次探查手术二次探查手术主要是针对那些对治疗反应欠佳的患儿,其目的是明确肿瘤对治疗的真实反应情况。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "二次探查手术" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "二次探查手术" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "治疗反应欠佳" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肿瘤" } ] }, { "text": "影像学定患者的实际反应时并不完全可靠,ISRⅢ中,75%影像学上对治疗部分或没有反应的Ⅲ级患儿,其病理上可呈现为完全反应或可通过切除残余肿瘤而达到完全反应。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "影像学" }, { "start_idx": 28, "end_idx": 30, "type": "pro", "entity": "影像学" }, { "start_idx": 64, "end_idx": 69, "type": "pro", "entity": "切除残余肿瘤" } ] }, { "text": "当然,影像学为完全反应的大多数(不是所有)病人都被第二次手术证实。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "影像学" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "第二次手术" } ] }, { "text": "二次探查手术对四肢和躯干部肿瘤的治疗有益,对头颈部及Ⅳ型肿瘤患儿效果有限。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "二次探查手术" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "四肢和躯干部肿瘤" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "头颈部及Ⅳ型肿瘤" } ] }, { "text": "(二)化疗化疗或包括化疗的辅助治疗可显著地控制肿瘤的局部发展,改善生存率。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "化疗" }, { "start_idx": 8, "end_idx": 16, "type": "pro", "entity": "包括化疗的辅助治疗" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" } ] }, { "text": "化疗可消灭极微小的肿瘤,使切除困难肿瘤的体积缩小以获得完全切除。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "极微小的肿瘤" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肿瘤" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "完全切除" } ] }, { "text": "增加阿霉素或以阿霉素代替放线菌素D不会进一步提高生存率,而异环磷酰胺和足叶乙甙联合应用可使疗效加强。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "阿霉素" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "阿霉素" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "放线菌素D" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "异环磷酰胺" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "足叶乙甙" } ] }, { "text": "晚期病人可试用:①卡铂、阿霉素、异环磷酰胺和放线菌素D;②异环磷酰胺、放线菌素D和长春新碱联合用药。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "卡铂" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "阿霉素" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "异环磷酰胺" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "放线菌素D" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "异环磷酰胺" }, { "start_idx": 35, "end_idx": 39, "type": "dru", "entity": "放线菌素D" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "长春新碱" } ] }, { "text": "高分级病儿经强化疗后可进行骨髓移植,但这方面的经验还相当有限。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "强化疗" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "骨髓移植" } ] }, { "text": "(三)放疗除Ⅰ级非腺泡型肿瘤或原发于阴道、子宫、外阴部的肿瘤经二次手术已完全切除者无须放疗外,所有其他肿瘤均需要放疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "Ⅰ级非腺泡型肿瘤" }, { "start_idx": 15, "end_idx": 29, "type": "dis", "entity": "原发于阴道、子宫、外阴部的肿瘤" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "二次手术" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "放疗" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肿瘤" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "放疗" } ] }, { "text": "放疗的剂量一般在40~60Gy,主要基于肿瘤的分级和分型。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肿瘤" } ] }, { "text": "镜下残留的病人90%左右经每天150~180cGy,总量41.4Gy的放疗可获得局部控制。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "equ", "entity": "镜" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "放疗" } ] }, { "text": "Ⅳ级患者,不论肿瘤发生在何部位必须放疗。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "放疗" } ] }, { "text": "在总量不变的情况下,将放疗次数增加,可达到高效杀灭肿瘤细胞,减少副作用的效果。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "放疗" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "【特殊部位肿瘤的治疗及结果】(一)头颈部随着综合治疗的应用,原发于头颈部的横纹肌肉瘤手术范围及创伤明显减小。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "特殊部位肿瘤" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "头颈部" }, { "start_idx": 30, "end_idx": 41, "type": "dis", "entity": "原发于头颈部的横纹肌肉瘤" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "手术" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "创伤" } ] }, { "text": "眶骨的局限作用使得眼眶内肿瘤效果最好,而脑膜周围肿瘤因为淋巴循环丰富,预后最差。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "眶骨" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "眼眶内肿瘤" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "脑膜周围肿瘤" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "淋巴" } ] }, { "text": "颈部淋巴结转移仅3%,所以不必常规行淋巴结活检或切除。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "颈部淋巴结" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "淋巴结活检" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "切除" } ] }, { "text": "除了眼睑部位的肿瘤需要切除外,眼眶部位肿瘤的手术仅限于取活检。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "眼睑部位的肿瘤" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "切除" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "眼眶部位肿瘤" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "手术" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "活检" } ] }, { "text": "非眼眶、非脑膜周围的肿瘤有浅表或深部之分,但均不侵犯脑膜。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "非眼眶、非脑膜周围的肿瘤" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "脑膜" } ] }, { "text": "头面部浅表肿瘤很容易早期发现并可以完整切除。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "头面部浅表肿瘤" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "完整切除" } ] }, { "text": "深部肿瘤常发生于腮腺、颊黏膜、喉和口咽部,治疗上类似眼眶部肿瘤,成活率为83%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "深部肿瘤" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腮腺" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "颊黏膜" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "喉" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "口咽部" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "眼眶部肿瘤" } ] }, { "text": "15%的患者有转移,骨质侵蚀是局部复发的标志。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "有转移" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "骨质侵蚀" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "骨质" } ] }, { "text": "接受强化治疗的患者无瘤生存从33%增加到57%,而局部肿瘤失控率从28%降到6%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "强化治疗" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "局部肿瘤" } ] }, { "text": "但最近对中枢神经系统预防应用放疗的方案受到质疑,在IRSⅢ中,放疗和鞘内化疗要根据脑膜侵袭范围、程度而定,而且大多数病人接受放疗的剂量要小于IRSⅡ的病人。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "pro", "entity": "对中枢神经系统预防应用放疗" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "放疗" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "鞘内化疗" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "脑膜" }, { "start_idx": 62, "end_idx": 63, "type": "pro", "entity": "放疗" } ] }, { "text": "但IRSⅢ病人肿瘤无进展存活率与IRSⅡ病人相比并未下降。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肿瘤" } ] }, { "text": "对初期化疗和放疗反应不敏感的头颈部肿瘤患者,要慎重选择颅底手术,以期保持其器官功能和颜面美容。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "放疗" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "头颈部肿瘤" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "颅底手术" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "器官" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "颜面" } ] }, { "text": "在有些病例中,影像学虽然发现有残余肿块,但切除的标本却未证实有肿瘤细胞的存在。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "影像学" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "有残余肿块" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "(二)躯干躯干部的横纹肌肉瘤主要发生于以下3个部位:胸壁、腹壁、脊柱旁。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "躯干" }, { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "躯干部的横纹肌肉瘤" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "胸壁" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "腹壁" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "脊柱旁" } ] }, { "text": "与四肢的肿瘤相比,其相同点是其对其他部位敏感的同一种化疗方案缺乏持久敏感性;不同点是该肿瘤有较高的复发率,而淋巴结转移较少。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "四肢的肿瘤" }, { "start_idx": 14, "end_idx": 36, "type": "sym", "entity": "其对其他部位敏感的同一种化疗方案缺乏持久敏感性" }, { "start_idx": 15, "end_idx": 29, "type": "pro", "entity": "对其他部位敏感的同一种化疗方案" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "肿瘤" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "淋巴结" } ] }, { "text": "目前不再把骨骼外Ewing肉瘤划为横纹肌肉瘤的亚型。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dis", "entity": "骨骼外Ewing肉瘤" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "胸壁横纹肌肉瘤的外科手术要求切除肿瘤组织至周围一定范围的正常组织。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "胸壁横纹肌肉瘤" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "外科手术" }, { "start_idx": 14, "end_idx": 31, "type": "pro", "entity": "切除肿瘤组织至周围一定范围的正常组织" } ] }, { "text": "如果早期切除困难,可以先取病理活检,接受放、化疗,其后再行切除可使局部肿瘤得到控制,提高存活率。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "早期切除" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "病理活检" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "放、化疗" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "切除" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "局部肿瘤" } ] }, { "text": "IRSⅡ和Ⅲ的76名胸壁肿瘤患者,平均诊断年龄为8.5岁,40%为腺泡型,15%为Ⅰ级,20%为Ⅱ级,40%为Ⅲ级,25%为Ⅳ级。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "胸壁肿瘤" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "腺泡" } ] }, { "text": "影响存活率的因素:临床分级,肿瘤大于5cm,局部复发以及远处复发。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "肿瘤大于5cm" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "局部复发" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "远处复发" } ] }, { "text": "腹壁横纹肌肉瘤较少见,治疗方法与胸壁肿瘤相似。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "腹壁横纹肌肉瘤" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "胸壁肿瘤" } ] }, { "text": "存活率与肿瘤的类型、部位及完全切除与否无关。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肿瘤" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "完全切除" } ] }, { "text": "(三)四肢原发于四肢的横纹肌肉瘤占19%,IRSⅢ中Ⅰ级占63%,Ⅱ级占25%,Ⅲ级占20%,Ⅳ型级25%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "四肢" }, { "start_idx": 5, "end_idx": 15, "type": "dis", "entity": "原发于四肢的横纹肌肉瘤" } ] }, { "text": "45%组织分型为腺泡型,12%的病人有区域淋巴结转移。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腺泡" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "区域淋巴结转移" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "淋巴结" } ] }, { "text": "淋巴结转移直接影响预后,阳性者存活率为46%,而阴性者为80%,而且有淋巴结转移者其肿瘤远处转移的可能性较大。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "淋巴结" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "淋巴结" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "肿瘤远处转移" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "肿瘤" } ] }, { "text": "无论肿瘤发生于四肢什么部位,只要能保持肢体的功能,应完整将肿瘤切除。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "四肢" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肢体" }, { "start_idx": 26, "end_idx": 32, "type": "pro", "entity": "完整将肿瘤切除" } ] }, { "text": "完全切除(临床Ⅰ级)比切除不完整者预后要好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "完全切除" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "切除" } ] }, { "text": "Lawrence指出,2/3肿瘤未完整切除的病人应该是能够完整切除的。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "切除" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "完整切除" } ] }, { "text": "肿瘤完全切除者的生存率是70%,而那些应能完整切除而未完整切除病人的仅为48%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "肿瘤完全切除" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "完整切除" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "完整切除" } ] }, { "text": "除非肿瘤位于手、脚末梢,否则不考虑截肢手术。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "手、脚末梢" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "截肢手术" } ] }, { "text": "放疗的范围要根据局部淋巴结情况来定。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "局部淋巴结" } ] }, { "text": "14名发现镜下淋巴结转移的儿童,接受适量的放疗后,只有1人复发。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "equ", "entity": "镜" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "镜下淋巴结转移" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "淋巴结" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "放疗" } ] }, { "text": "病人术后要化疗和放疗。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "化疗" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "放疗" } ] }, { "text": "Ⅰ、Ⅱ级腺泡型肿瘤的化疗强度要大于非腺泡型。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "Ⅰ、Ⅱ级腺泡型肿瘤" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "化疗" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腺泡" } ] }, { "text": "IRSⅡ中,加强化疗者的存活率为77%,而常规方案仅为57%。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "化疗" } ] }, { "text": "未发生转移的四肢横纹肌肉瘤5年存活率(74%)差于眼眶和泌尿生殖系(81%~95%)。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "四肢横纹肌肉瘤" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "眼眶" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "泌尿生殖系" } ] }, { "text": "影响预后的因素有:淋巴结转移、肿瘤超过5cm、远处转移、局部复发、远处复发和年龄超过10岁。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "淋巴结" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "肿瘤超过5cm" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肿瘤" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "远处转移" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "局部复发" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "远处复发" } ] }, { "text": "肿瘤发生在肢体的远、近端及组织类型与预后无明显相关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "肢体的远、近端" } ] }, { "text": "多变量分析结果表明,肿瘤大小、年龄、淋巴结转移及远处转移者预后较差。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "淋巴结" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "远处转移" } ] }, { "text": "(四)泌尿生殖系泌尿生殖系的横纹肌肉瘤占总数的25%,发生部位包括膀胱、前列腺、睾丸旁、阴道、子宫和外阴部,罕见发生于肾脏、输尿管。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "泌尿生殖系" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "泌尿生殖系" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "膀胱" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "前列腺" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "睾丸旁" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "阴道" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "子宫" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "外阴部" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "肾脏" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "输尿管" } ] }, { "text": "其中外阴部、阴道和睾丸旁的预后较好,而膀胱、前列腺的预后较差。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "外阴部" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "阴道" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "睾丸旁" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "膀胱" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "前列腺" } ] }, { "text": "1.膀胱和前列腺部IRSⅢ的结果显示,膀胱和前列腺部横纹肌肉瘤患者在接受加强化疗和早期放疗后,其成活率仅次于头颈部横纹肌肉瘤患者。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "膀胱" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "前列腺部" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "膀胱" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "前列腺部" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "加强化疗" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "早期放疗" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "头颈部横纹肌肉瘤" } ] }, { "text": "其中膀胱横纹肌肉瘤比前列腺横纹肌肉瘤的预后好,但有时原发灶难以确定,临床上经常发现它们的肿块巨大,生长至脐部组织类型多为胚胎型,80%属于IRSⅢ中Ⅲ级。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "膀胱横纹肌肉瘤" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "前列腺横纹肌肉瘤" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "肿块巨大" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "生长至脐部" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "脐部" }, { "start_idx": 54, "end_idx": 62, "type": "sym", "entity": "组织类型多为胚胎型" }, { "start_idx": 64, "end_idx": 75, "type": "sym", "entity": "80%属于IRSⅢ中Ⅲ级" } ] }, { "text": "表11-27膀胱及前列腺横纹肌肉瘤病儿的生存率和膀胱保留率膀胱顶部横纹肌肉瘤通常早期行膀胱部分切除术或接受辅助治疗后再手术。", "entities": [ { "start_idx": 6, "end_idx": 16, "type": "dis", "entity": "膀胱及前列腺横纹肌肉瘤" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "膀胱" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "膀胱顶部横纹肌肉瘤" }, { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "膀胱部分切除术" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "辅助治疗" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "手术" } ] }, { "text": "Hays等人研究了IRS的154个患儿,33例先行膀胱部分切除或化疗后再手术,其3年存活率(79%)与所有膀胱横纹肌肉瘤患者的存活率大致相同。", "entities": [ { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "膀胱部分切除" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "化疗后再手术" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "膀胱横纹肌肉瘤" } ] }, { "text": "行膀胱部分切除存活的26个人中,25人(95%)保留膀胱。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "膀胱部分切除" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "膀胱" } ] }, { "text": "有些膀胱或前列腺横纹肌肉瘤(或两者皆有)的患者,如果对辅助治疗不敏感,在行前列腺切除后,可保留膀胱、尿道重建。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "膀胱或前列腺横纹肌肉瘤" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "辅助治疗" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "前列腺切除" }, { "start_idx": 45, "end_idx": 53, "type": "pro", "entity": "保留膀胱、尿道重建" } ] }, { "text": "如果保留膀胱不可能,则必须用胃或回盲部做可控性尿流改道,以提高生活质量。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "膀胱" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "胃" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "回盲部" }, { "start_idx": 20, "end_idx": 26, "type": "pro", "entity": "可控性尿流改道" } ] }, { "text": "在1年以上的时间里随访活检仍然可以发现肿瘤细胞的存在,只要患儿对化疗敏感或持续部分敏感,那么除了部分切除外,其他任何治疗可推迟进行。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "随访活检" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "化疗" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "部分切除" } ] }, { "text": "2.阴道、会阴和子宫女性生殖器横纹肌肉瘤的传统治疗方法是早期进行组织及器官的根治性切除。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "阴道" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "会阴" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "子宫" }, { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "女性生殖器横纹肌肉瘤" }, { "start_idx": 32, "end_idx": 42, "type": "pro", "entity": "组织及器官的根治性切除" } ] }, { "text": "但随着化疗和放疗的应用,盆腔清除和子宫、阴道切除术又被摒弃。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "放疗" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "盆腔清除" }, { "start_idx": 17, "end_idx": 24, "type": "pro", "entity": "子宫、阴道切除术" } ] }, { "text": "IRSⅡ中,18%的患儿有局部复发,80%接受了完全或部分子宫、阴道切除术。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "局部复发" }, { "start_idx": 24, "end_idx": 36, "type": "pro", "entity": "完全或部分子宫、阴道切除术" } ] }, { "text": "IRSⅢ中,所有原发于阴道、外阴、子宫的病人接受了阿霉素、顺铂和VAC方案,局部肿瘤得到了很好的控制,膀胱、子宫、阴道的保留率高,且局部无复发。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "阴道" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "外阴" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "子宫" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "阿霉素" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "顺铂" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "VAC方案" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "局部肿瘤" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "膀胱" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "子宫" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "阴道" } ] }, { "text": "最近IRSⅢ评估了24名阴道横纹肌肉瘤病人,仅有7人(29%)最终做了器官切除术(5人子宫切除,2人阴道切除);除了1人,其余全部保留了膀胱;局部无复发,存活率达80%。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "阴道横纹肌肉瘤" }, { "start_idx": 35, "end_idx": 39, "type": "pro", "entity": "器官切除术" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "子宫切除" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "阴道切除" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "膀胱" } ] }, { "text": "IRSⅣ中,上述部位肿瘤的化疗和放疗的剂量已经减少。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "化疗" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "放疗" } ] }, { "text": "3.睾丸旁睾丸旁横纹肌肉瘤占7%,占儿童阴囊肿瘤的12%,就诊时多为Ⅰ级,预后较其他部位要好,大多数属于胚胎型,以梭状细胞为主。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "睾丸旁" }, { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "睾丸旁横纹肌肉瘤" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "儿童阴囊肿瘤" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "梭状细胞" } ] }, { "text": "治疗以手术为主,术前要做腹、盆腔CT,观察腹膜后淋巴结情况。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "腹、盆腔CT" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "腹膜后淋巴结" } ] }, { "text": "经腹股沟行睾丸和精索切除,如果术中不能确诊,则先夹住精索血管,保护伤口,切除肿瘤,做冰冻活检。", "entities": [ { "start_idx": 1, "end_idx": 11, "type": "pro", "entity": "腹股沟行睾丸和精索切除" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "先夹住精索血管" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "保护伤口" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "切除肿瘤" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "冰冻活检" } ] }, { "text": "过去常规要行腹膜后淋巴结清扫术。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "腹膜后淋巴结清扫术" } ] }, { "text": "Olive等人研究了19例Ⅰ级且无周围组织浸润或淋巴结转移的睾丸旁横纹肌肉瘤,只有2人局部淋巴结复发,其中1例未化疗。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "周围组织浸润" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "淋巴结" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "睾丸旁横纹肌肉瘤" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "局部淋巴结复发" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "局部淋巴结" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "化疗" } ] }, { "text": "IRSⅢ中,121例睾丸旁横纹肌肉瘤仅2例发生局部淋巴结复发。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "睾丸旁横纹肌肉瘤" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "局部淋巴结复发" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "局部淋巴结" } ] }, { "text": "尽管如此,对腹膜后淋巴结的评估仍有一定意义,据统计每6例临床表现无淋巴结浸润而未做淋巴结活检的病人中有1例将会出现淋巴结复发,有淋巴结转移的病人须接受放疗。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "腹膜后淋巴结" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "淋巴结浸润" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "淋巴结" }, { "start_idx": 41, "end_idx": 45, "type": "pro", "entity": "淋巴结活检" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "淋巴结复发" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "淋巴结" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "淋巴结转移" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "淋巴结" }, { "start_idx": 75, "end_idx": 76, "type": "pro", "entity": "放疗" } ] }, { "text": "9例出现了肠梗阻,8例射精功能丧失,5例下肢淋巴性水肿,4例患慢性腹泻,2例尿道狭窄,4例腹部软组织增生。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "射精功能丧失" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "下肢淋巴性水肿" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "尿道狭窄" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "腹部软组织增生" } ] }, { "text": "接受过环磷酰胺和睾丸放疗患儿的睾丸体积明显减小。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "睾丸放疗" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "睾丸体积明显减小" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "睾丸" } ] }, { "text": "(五)其他部位不包括原发于泌尿系的,腹膜后横纹肌肉瘤占了10%,组织类型为胚胎型或腺泡型,确诊时大多数都有淋巴结累积或远处转移灶,已经不能切除。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "泌尿系" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "腹膜后横纹肌肉瘤" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "腺泡" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "淋巴结累积" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "淋巴结" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "远处转移灶" }, { "start_idx": 69, "end_idx": 70, "type": "pro", "entity": "切除" } ] }, { "text": "胃肠道横纹肌肉瘤罕见,一经发现肿瘤已非常大,切除困难。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "胃肠道横纹肌肉瘤" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "肿瘤已非常大" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "切除" } ] }, { "text": "胆道肿瘤表现为梗阻性黄疸,组织类型为胚胎型和葡萄簇型,要行胰、十二指肠切除术,存活率为40%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "胆道肿瘤" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "梗阻性黄疸" }, { "start_idx": 28, "end_idx": 37, "type": "pro", "entity": "行胰、十二指肠切除术" } ] }, { "text": "已报道少数原发于肺部的横纹肌肉瘤,多数伴有先天性肺囊肿或囊腺样发育异常。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺部" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "先天性肺囊肿" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "囊腺样发育异常" } ] }, { "text": "治疗为肺叶切除术,如果完全切除,效果较好。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "肺叶切除术" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "完全切除" } ] }, { "text": "也有几例原发于皮肤、心脏、乳房的横纹肌肉瘤报道,治疗方法是尽可能切除局部肿瘤。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "皮肤" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "乳房" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "切除局部肿瘤" } ] }, { "text": "(六)转移和复发横纹肌肉瘤最常见的转移部位是肺、骨、骨髓、脑、肝、大网膜、胸膜、腹膜以及远处淋巴结。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "转移和复发" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "转移" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "骨" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "骨髓" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "脑" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肝" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "大网膜" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "胸膜" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "腹膜" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "远处淋巴结" } ] }, { "text": "肿瘤转移后一般预后较差,但有些部位的如膀胱顶和睾丸旁,存活率还好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "肿瘤转移" }, { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "膀胱顶" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "睾丸旁" } ] }, { "text": "化疗和放疗后肺的孤立及局限性的转移灶可予以切除。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "肺的孤立及局限性的转移灶" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "切除" } ] }, { "text": "(七)结果横纹肌肉瘤患者的存活率取决于诊断时肿瘤的临床分级、分期、原发部位。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(八)前景横纹肌肉瘤的治疗发展目标是要在保证疗效的前提下,尽可能减少治疗的副作用和迟发效应;对肿瘤的转移和复发找到更有效的方法。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "肿瘤的转移和复发" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "肿瘤" } ] }, { "text": "新的化疗和其他治疗方法需要不断发展。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "化疗" } ] }, { "text": "外科医生的作用主要在于:①在保留器官功能和外观美容的情况下尽量完整切除肿瘤;②正确的肿瘤分期;③获取足够多的组织进行诊断和预后研究。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dep", "entity": "外科" }, { "start_idx": 31, "end_idx": 36, "type": "pro", "entity": "完整切除肿瘤" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "肿瘤" } ] }, { "text": "五、管瘤性综合征管瘤性综合征是指临床上患有上述某一种血管瘤,有规律地伴有另一些病理情况,而形成几种独立的病理实体或综合征。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "管瘤性综合征" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "管瘤性综合征" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "血管瘤" } ] }, { "text": "(一)遗传性出血性血管扩张症主要表现为皮肤和黏膜的毛细血管扩张,具有显著的出血倾向。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "遗传性出血性血管扩张症" }, { "start_idx": 19, "end_idx": 30, "type": "sym", "entity": "皮肤和黏膜的毛细血管扩张" }, { "start_idx": 19, "end_idx": 28, "type": "bod", "entity": "皮肤和黏膜的毛细血管" }, { "start_idx": 32, "end_idx": 40, "type": "sym", "entity": "具有显著的出血倾向" } ] }, { "text": "病理切片可见扩张的毛细血管壁菲薄,往往仅有一层内皮细胞,直接处于向外菲薄的表皮下面。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "病理切片可见扩张的毛细血管壁菲薄" }, { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "病理切片" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "毛细血管壁" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "往往仅有一层内皮细胞" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "一层内皮细胞" }, { "start_idx": 28, "end_idx": 40, "type": "sym", "entity": "直接处于向外菲薄的表皮下面" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "表皮下面" } ] }, { "text": "该毛细毛细血管瘤常为多发性,平坦或略凸出皮肤,形状不规则,好发于手背和鼻腔黏膜,指压可苍白,易出血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "毛细血管瘤" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "平坦或略凸出皮肤" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "形状不规则" }, { "start_idx": 29, "end_idx": 38, "type": "sym", "entity": "好发于手背和鼻腔黏膜" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "手背" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "鼻腔黏膜" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "指压可苍白" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "指" }, { "start_idx": 46, "end_idx": 48, "type": "sym", "entity": "易出血" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "血" } ] }, { "text": "治疗可采用激素或电凝法。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "激素" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "电凝法" } ] }, { "text": "(二)斯特奇-韦伯综合征系面部葡萄酒色斑合并颅内血管瘤,故对面部葡萄酒色斑要严密注意和检查,如其位于三叉神经第一支眼神经感觉区的皮肤,是并发Sturge-Weber综合征的重要部位,此综合征无根治疗法,常伴有癫痫及智力低下。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "斯特奇-韦伯综合征" }, { "start_idx": 13, "end_idx": 26, "type": "dis", "entity": "面部葡萄酒色斑合并颅内血管瘤" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "面部葡萄酒色斑" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "面部" }, { "start_idx": 50, "end_idx": 65, "type": "bod", "entity": "三叉神经第一支眼神经感觉区的皮肤" }, { "start_idx": 68, "end_idx": 84, "type": "dis", "entity": "并发Sturge-Weber综合征" }, { "start_idx": 96, "end_idx": 99, "type": "pro", "entity": "根治疗法" }, { "start_idx": 104, "end_idx": 105, "type": "dis", "entity": "癫痫" }, { "start_idx": 107, "end_idx": 110, "type": "sym", "entity": "智力低下" } ] }, { "text": "(三)马富西综合征无遗传性,出生时完全正常,尔后逐渐出现单侧软骨发育不良,导致双侧肢体发育不平衡。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "马富西综合征" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "单侧软骨发育不良" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "双侧肢体发育不平衡" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "双侧肢体" } ] }, { "text": "(四)林道-(冯)希佩尔综合征为小脑、脑干、脊髓、视网膜、肝等处生长的单个或多个血管瘤,同时伴有胰腺、肾、肾上腺囊肿或腺瘤。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "林道-(冯)希佩尔综合征" }, { "start_idx": 16, "end_idx": 42, "type": "sym", "entity": "小脑、脑干、脊髓、视网膜、肝等处生长的单个或多个血管瘤" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "小脑" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "脑干" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "脊髓" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "视网膜" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肝" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "血管瘤" }, { "start_idx": 48, "end_idx": 60, "type": "sym", "entity": "胰腺、肾、肾上腺囊肿或腺瘤" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "胰腺" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "肾" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "肾上腺" } ] }, { "text": "(五)Klippel-Trenaunay综合征主要表现为肢体软组织和骨过度增长肥大,皮下静脉曲张,皮肤血管瘤,发病男女差别不大。", "entities": [ { "start_idx": 3, "end_idx": 22, "type": "dis", "entity": "Klippel-Trenaunay综合征" }, { "start_idx": 28, "end_idx": 40, "type": "sym", "entity": "肢体软组织和骨过度增长肥大" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "肢体软组织和骨" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "皮下静脉曲张" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "皮下静脉" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "皮肤血管瘤" } ] }, { "text": "目前尚无规范性的疗法,多数学者主张轻者保守治疗,重者或出现并发症时手术治疗。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "保守治疗" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "手术治疗" } ] }, { "text": "二、机械通气的气体交换机械通气的基本目的是促进有效的通气和气体交换,包括CO2的及时排出和O2的充分摄入,使血气结果在正常范围。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "机械通气" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "机械通气" }, { "start_idx": 36, "end_idx": 49, "type": "ite", "entity": "CO2" }, { "start_idx": 56, "end_idx": 68, "type": "ite", "entity": "O2" }, { "start_idx": 76, "end_idx": 77, "type": "ite", "entity": "血气" } ] }, { "text": "(一)CO2的排出CO2极易从血液弥散到肺泡内,因此血中CO2的排出主要取决于进出肺内的气体总量,即每分肺泡通气量,其计算公式为:每分肺泡通气量=(潮气量-无效腔量)×RR无效腔量是指每次吸入潮气量中分布于气管内,不能进行气体交换的部分气体,因其相对恒定,故增加潮气量或RR,可增加每分肺泡通气量,促进CO2的排出,降低PaCO2,潮气量对CO2的影响大于RR。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "ite", "entity": "CO2" }, { "start_idx": 20, "end_idx": 33, "type": "ite", "entity": "CO2" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血液" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肺泡内" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "血" }, { "start_idx": 50, "end_idx": 63, "type": "ite", "entity": "CO2" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "肺内" }, { "start_idx": 85, "end_idx": 89, "type": "ite", "entity": "肺泡通气量" }, { "start_idx": 98, "end_idx": 104, "type": "ite", "entity": "每分肺泡通气量" }, { "start_idx": 107, "end_idx": 109, "type": "ite", "entity": "潮气量" }, { "start_idx": 111, "end_idx": 114, "type": "ite", "entity": "无效腔量" }, { "start_idx": 117, "end_idx": 118, "type": "ite", "entity": "RR" }, { "start_idx": 119, "end_idx": 122, "type": "ite", "entity": "无效腔量" }, { "start_idx": 127, "end_idx": 131, "type": "ite", "entity": "吸入潮气量" }, { "start_idx": 136, "end_idx": 138, "type": "bod", "entity": "气管内" }, { "start_idx": 164, "end_idx": 166, "type": "ite", "entity": "潮气量" }, { "start_idx": 168, "end_idx": 169, "type": "ite", "entity": "RR" }, { "start_idx": 174, "end_idx": 180, "type": "ite", "entity": "每分肺泡通气量" }, { "start_idx": 184, "end_idx": 197, "type": "ite", "entity": "CO2" }, { "start_idx": 204, "end_idx": 219, "type": "ite", "entity": "PaCO2" }, { "start_idx": 221, "end_idx": 223, "type": "ite", "entity": "潮气量" }, { "start_idx": 225, "end_idx": 238, "type": "ite", "entity": "CO2" }, { "start_idx": 244, "end_idx": 245, "type": "ite", "entity": "RR" } ] }, { "text": "定容型呼吸机的潮气量可通过旋钮直接设置;定压型呼吸机的潮气量主要取决于肺的顺应性和吸、呼气时肺泡内的压力差。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "定容型呼吸机" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "潮气量" }, { "start_idx": 20, "end_idx": 25, "type": "equ", "entity": "定压型呼吸机" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "潮气量" }, { "start_idx": 35, "end_idx": 39, "type": "ite", "entity": "肺的顺应性" }, { "start_idx": 41, "end_idx": 52, "type": "ite", "entity": "吸、呼气时肺泡内的压力差" } ] }, { "text": "一般情况下,肺顺应性在一段时间内相对恒定,故其潮气量主要取决于吸气峰压(peakinspirationpressure,PIP)与PEEP的差值,差值大则潮气量大,反之则小。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "潮气量" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "吸气峰压" }, { "start_idx": 36, "end_idx": 58, "type": "ite", "entity": "peakinspirationpressure" }, { "start_idx": 60, "end_idx": 62, "type": "ite", "entity": "PIP" }, { "start_idx": 65, "end_idx": 68, "type": "ite", "entity": "PEEP" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "潮气量" } ] }, { "text": "通气频率也是影响每分肺泡通气量的重要因素之一,在一定范围内,频率的增加可使每分肺泡通气量增加,可使PaCO2下降。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "通气频率" }, { "start_idx": 8, "end_idx": 14, "type": "ite", "entity": "每分肺泡通气量" }, { "start_idx": 39, "end_idx": 43, "type": "ite", "entity": "肺泡通气量" }, { "start_idx": 49, "end_idx": 64, "type": "ite", "entity": "PaCO2" } ] }, { "text": "此外,患儿在机械通气过程中自主呼吸频率的变化也是影响通气的因素。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "机械通气" }, { "start_idx": 13, "end_idx": 18, "type": "ite", "entity": "自主呼吸频率" } ] }, { "text": "当PaCO2增高时,可通过增大PIP与PEEP的差值(即提高PIP或降低PEEP)或调快呼吸机频率来使PaCO2降低,反之亦然。", "entities": [ { "start_idx": 1, "end_idx": 16, "type": "ite", "entity": "PaCO2" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "PIP" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "PEEP" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "PIP" }, { "start_idx": 47, "end_idx": 50, "type": "ite", "entity": "PEEP" }, { "start_idx": 55, "end_idx": 57, "type": "equ", "entity": "呼吸机" }, { "start_idx": 62, "end_idx": 77, "type": "ite", "entity": "PaCO2" } ] }, { "text": "(二)O2的摄取动脉氧合主要取决于MAP和吸入气氧分数(fractionofinspiredoxygen,FiO2)。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "ite", "entity": "O2" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "MAP" }, { "start_idx": 32, "end_idx": 37, "type": "ite", "entity": "吸入气氧分数" }, { "start_idx": 39, "end_idx": 62, "type": "ite", "entity": "fractionofinspiredoxygen" }, { "start_idx": 64, "end_idx": 78, "type": "ite", "entity": "FiO2" } ] }, { "text": "MAP是一个呼吸周期中施于气道和肺的平均压力,MAP值等于在这个呼吸周期中压力曲线下的面积除以该周期所用的时间,其公式为:MAP=K×(PIP×TI+PEEP×TE)/(TI+TE)式中,K:常数(正弦波为0.5,方形波为1.0);TI:吸气时间;TE:呼气时间。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "MAP" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "气道" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "MAP值" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "MAP" } ] }, { "text": "MAP应用范围一般为5~15cmH2O(0.49~1.47kPa)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "MAP" } ] }, { "text": "从公式可见,提高PIP、PEEP及吸/呼(inspiration/expirationratio,I/E)中任意一项均可使MAP值增大、PaO2提高。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "PIP" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "PEEP" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "吸/呼" }, { "start_idx": 21, "end_idx": 47, "type": "ite", "entity": "inspiration/expirationratio" }, { "start_idx": 49, "end_idx": 51, "type": "ite", "entity": "I/E" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "MAP" }, { "start_idx": 68, "end_idx": 82, "type": "ite", "entity": "PaO2" } ] }, { "text": "在考虑增大MAP时,应注意下列几个问题:①PIP的作用大于PEEP及I/E;②当PEEP达到8cmH2O时,再提高PEEP,PaO2升高则不明显;③过高的MAP可导致肺泡过度膨胀,静脉回流受阻,心排血量减少,氧合降低,并可引起肺气压伤。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "MAP" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "PIP" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "PEEP" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "I/E" }, { "start_idx": 40, "end_idx": 43, "type": "ite", "entity": "PEEP" }, { "start_idx": 68, "end_idx": 71, "type": "ite", "entity": "PEEP" }, { "start_idx": 73, "end_idx": 87, "type": "ite", "entity": "PaO2" }, { "start_idx": 99, "end_idx": 101, "type": "ite", "entity": "MAP" }, { "start_idx": 105, "end_idx": 106, "type": "bod", "entity": "肺泡" }, { "start_idx": 105, "end_idx": 110, "type": "sym", "entity": "肺泡过度膨胀" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "静脉" }, { "start_idx": 112, "end_idx": 117, "type": "sym", "entity": "静脉回流受阻" }, { "start_idx": 119, "end_idx": 124, "type": "sym", "entity": "心排血量减少" }, { "start_idx": 119, "end_idx": 122, "type": "ite", "entity": "心排血量" }, { "start_idx": 126, "end_idx": 129, "type": "sym", "entity": "氧合降低" }, { "start_idx": 126, "end_idx": 127, "type": "ite", "entity": "氧合" }, { "start_idx": 135, "end_idx": 138, "type": "dis", "entity": "肺气压伤" } ] }, { "text": "除增加MAP外,提高FiO2也是直接而有效增加PaO2的方法。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "MAP" }, { "start_idx": 10, "end_idx": 24, "type": "ite", "entity": "FiO2" }, { "start_idx": 34, "end_idx": 48, "type": "ite", "entity": "PaO2" } ] }, { "text": "总之,影响PaCO2的主要参数是RR和PIP与PEEP的差值;影响PaO2的主要参数是MAP(PIP、PEEP和I/E)及FiO2。", "entities": [ { "start_idx": 5, "end_idx": 20, "type": "ite", "entity": "PaCO2" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "RR" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "PIP" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "PEEP" }, { "start_idx": 44, "end_idx": 58, "type": "ite", "entity": "PaO2" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "MAP" }, { "start_idx": 69, "end_idx": 71, "type": "ite", "entity": "PIP" }, { "start_idx": 73, "end_idx": 76, "type": "ite", "entity": "PEEP" }, { "start_idx": 78, "end_idx": 80, "type": "ite", "entity": "I/E" }, { "start_idx": 83, "end_idx": 97, "type": "ite", "entity": "FiO2" } ] }, { "text": "三、基因诊断的策略基因诊断与细胞、病理、生化及免疫等诊断方法比较,其优点是:①在DNA水平了解疾病发生的原因,诊断特异性高;②在个体发育的任何阶段,任何有核细胞都可以进行诊断;③根据突变部位和类型可帮助预测表现型,预后判断;④可诊断致病基因携带者;⑤用于产前诊断;⑥进行准确的遗传咨询;⑦鉴别和分离新基因。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "基因诊断" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "基因诊断" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "细胞" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "病理" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "生化" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "免疫" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "DNA" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "核细胞" }, { "start_idx": 114, "end_idx": 122, "type": "pro", "entity": "诊断致病基因携带者" }, { "start_idx": 127, "end_idx": 130, "type": "pro", "entity": "产前诊断" }, { "start_idx": 138, "end_idx": 141, "type": "pro", "entity": "遗传咨询" }, { "start_idx": 144, "end_idx": 151, "type": "pro", "entity": "鉴别和分离新基因" } ] }, { "text": "遗传病的基因诊断方法分为直接方法与间接方法两种。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "遗传病" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "基因诊断" } ] }, { "text": "1.直接诊断策略指直接检测基因的致病突变的方法。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "直接诊断策略" }, { "start_idx": 9, "end_idx": 22, "type": "pro", "entity": "直接检测基因的致病突变的方法" } ] }, { "text": "当致病基因已分离,DNA序列已知时,应用PCR-RFLP及DNA测序等方法,直接显示基因缺失、插入以及突变,直接作出分子病理诊断。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "致病基因" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "DNA" }, { "start_idx": 20, "end_idx": 27, "type": "pro", "entity": "PCR-RFLP" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "DNA测序" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "基因" }, { "start_idx": 42, "end_idx": 52, "type": "sym", "entity": "基因缺失、插入以及突变" }, { "start_idx": 58, "end_idx": 63, "type": "pro", "entity": "分子病理诊断" } ] }, { "text": "目前包括遗传、血液、消化、内分泌、泌尿及神经等系统数百种遗传病已通过这些方法得到确诊,并应用于产前诊断。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血液" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "消化" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "内分泌" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "泌尿" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "神经" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "遗传病" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "产前诊断" } ] }, { "text": "基因携带致病基因的杂合子是否患病,而且还能揭示个体的基因状态,如是否是携带致病基因的杂合子,是否对某些疾病有易感性或抵抗性等,为遗传咨询和预防疾病提供可靠的依据。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 2, "end_idx": 11, "type": "bod", "entity": "携带致病基因的杂合子" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "基因" }, { "start_idx": 35, "end_idx": 44, "type": "bod", "entity": "携带致病基因的杂合子" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "遗传咨询" } ] }, { "text": "2.基因间接诊断策略指主要用连锁分析方法,即利用与疾病相关基因连锁的遗传多态性标志来检测疾病。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "基因间接诊断策略" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "连锁分析方法" }, { "start_idx": 22, "end_idx": 40, "type": "pro", "entity": "利用与疾病相关基因连锁的遗传多态性标志" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "检测疾病" } ] }, { "text": "间接诊断仅仅提示致病的可能性,而非直接提示致病的原因,主要适用于对于致病基因还不清楚的遗传病诊断,或用直接策略也难以检测时所进行的基因分析。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "间接诊断" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "致病基因" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "遗传病" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "直接策略" }, { "start_idx": 65, "end_idx": 68, "type": "pro", "entity": "基因分析" } ] }, { "text": "所谓连锁是指同一染色体上相互靠近的基因常在一起遗传,它们在遗传中结合在一起的频率大于按照独立分配率期望的频率。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "染色体" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "基因" } ] }, { "text": "在基因诊断中,主要是通过分析与疾病有关的基因连锁的多态性遗传标志来检测疾病。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "基因诊断" }, { "start_idx": 12, "end_idx": 31, "type": "pro", "entity": "分析与疾病有关的基因连锁的多态性遗传标志" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "检测疾病" } ] }, { "text": "一类是RFLP,第二类是串联重复顺序拷贝数不同形式的多态性,或称为微卫星(microsatellite),第三类是单核苷酸多态性(SNP)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "RFLP" }, { "start_idx": 12, "end_idx": 28, "type": "pro", "entity": "串联重复顺序拷贝数不同形式的多态性" }, { "start_idx": 33, "end_idx": 35, "type": "pro", "entity": "微卫星" }, { "start_idx": 57, "end_idx": 63, "type": "pro", "entity": "单核苷酸多态性" }, { "start_idx": 65, "end_idx": 67, "type": "pro", "entity": "SNP" } ] }, { "text": "参考文献1.李仲智,申昆玲,魏新苗,等.18例儿童严重急性呼吸综合征临床分析,中华儿科杂志2003,41(6):574-5742.刘丽,曾华松,卢惠珍,等.儿童传染性非典型肺炎临床诊治体会(附6例临床病例报告).小儿急救医学,2003,10(3):188-1883.曾其毅,刘丽,曾华松,等.广州地区33例儿童严重急性呼吸综合征临床特点和治疗转归.中华儿科杂志,2003,41(6):4084.中华医学会儿科学分会呼吸学组,中华医学会《中华儿科杂志》编辑委员会.儿童SARS专题研讨会纪要.中华儿科杂志,2003,41(6):4175.中华医学会儿科学分会呼吸学组,中华医学会《中华儿科杂志》编辑委员会.儿童严重呼吸综合征临床诊断和诊疗方案(试行).中华儿科杂志,2003.41(6):4086.DrostenC,CuntherS,PreiserW,etal.IdentificationofanovelcoronavirusinpatientswithSevereAcuteRespiratorySyndrome.NEnglJMed,2003,10:120-1207.段红梅申昆玲.儿童SARS特点与免疫关系探讨.中国小儿急救医学,2006,13(3):281-2818.HasogJL,HuangJ.DZH,etal.Th2predontinanmandCD8'memoryTcelldepletioninpatientswithsevereacuterespiratorysyndrome.MicrobesInfect,2005,7(3):427-436", "entities": [ { "start_idx": 23, "end_idx": 33, "type": "dis", "entity": "儿童严重急性呼吸综合征" }, { "start_idx": 41, "end_idx": 42, "type": "dep", "entity": "儿科" }, { "start_idx": 78, "end_idx": 87, "type": "dis", "entity": "儿童传染性非典型肺炎" }, { "start_idx": 153, "end_idx": 163, "type": "dis", "entity": "儿童严重急性呼吸综合征" }, { "start_idx": 176, "end_idx": 177, "type": "dep", "entity": "儿科" }, { "start_idx": 202, "end_idx": 203, "type": "dep", "entity": "儿科" }, { "start_idx": 207, "end_idx": 209, "type": "dep", "entity": "呼吸学" }, { "start_idx": 220, "end_idx": 221, "type": "dep", "entity": "儿科" }, { "start_idx": 231, "end_idx": 236, "type": "dis", "entity": "儿童SARS" }, { "start_idx": 247, "end_idx": 248, "type": "dep", "entity": "儿科" }, { "start_idx": 273, "end_idx": 274, "type": "dep", "entity": "儿科" }, { "start_idx": 278, "end_idx": 280, "type": "dep", "entity": "呼吸学" }, { "start_idx": 302, "end_idx": 310, "type": "dis", "entity": "儿童严重呼吸综合征" }, { "start_idx": 327, "end_idx": 328, "type": "dep", "entity": "儿科" }, { "start_idx": 492, "end_idx": 497, "type": "dis", "entity": "儿童SARS" }, { "start_idx": 510, "end_idx": 513, "type": "dep", "entity": "小儿急救" } ] }, { "text": "颅内高压@第十六章颅内高压【概述】颅内压力(intracranialpressure,ICP)是指颅腔内各种结构,即脑组织、脑血管系统及脑脊液所产生压力的总和,简称颅压或脑压,一般用腰椎穿刺测得的脑脊液压力表示。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "颅内高压" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "颅内压力" }, { "start_idx": 22, "end_idx": 41, "type": "ite", "entity": "intracranialpressure" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "ICP" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "颅腔" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "脑组织" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "脑血管系统" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "脑脊液" }, { "start_idx": 82, "end_idx": 83, "type": "ite", "entity": "颅压" }, { "start_idx": 85, "end_idx": 86, "type": "ite", "entity": "脑压" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 98, "end_idx": 102, "type": "ite", "entity": "脑脊液压力" } ] }, { "text": "颅内高压@通常所说的颅内压,是指在水平侧卧位身体松弛的状态下,经腰椎穿刺接上一定内径的测压管所测得的压力。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "颅内压" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 43, "end_idx": 45, "type": "equ", "entity": "测压管" } ] }, { "text": "颅内高压@测压管(玻璃管或塑料管)以内径2~3mm为宜,太细易有毛细作用使压力偏高,太粗则脑脊液流出过多而使压力偏低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "测压管" }, { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "玻璃管" }, { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "塑料管" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "脑脊液" } ] }, { "text": "颅内高压@颅内压的个体差别较大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@一般正常成人颅内压为70~180mmH2O[(mmH2O)×98.07=Pa]。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@压力在180~200mmH2O,可认为可疑的颅内压增高颅内压增高病理性低颅压,50~70mmH2O之间为可疑的低颅压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "压力" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "病理性低颅压" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "低颅压" } ] }, { "text": "颅内高压@小儿颅内压一般40~100mmH2O,新生儿颅内压为10~14mmH2O,青春期前后颅压达到成人相似水平。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 9, "type": "ite", "entity": "小儿颅内压" }, { "start_idx": 30, "end_idx": 35, "type": "ite", "entity": "新生儿颅内压" }, { "start_idx": 54, "end_idx": 60, "type": "ite", "entity": "青春期前后颅压" } ] }, { "text": "颅内高压@颅腔的内容物主要由三部分组成,即脑、血液和脑脊液。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "颅腔" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "脑" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血液" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑脊液" } ] }, { "text": "颅内高压@在正常状态下,这三部分的总容积是近于恒定的,其中任何一部分的数量改变必将由其余的一部分或两部分的相应改变代偿之,以防止对颅内压的明显影响。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@如果变化过于剧烈,出现失代偿,则出现颅压异常颅内高压@常见影响颅压的因素如下。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "出现失代偿" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "颅压" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "颅压异常" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "颅内高压" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "颅压" } ] }, { "text": "颅内高压@(一)脑脊液的分泌压和流动阻力当脑室系统发生急性闭塞时,在高达700mmH2O的压力下,脑脊液仍然产生,说明存在脑脊液分泌压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑脊液" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "分泌压" }, { "start_idx": 16, "end_idx": 19, "type": "ite", "entity": "流动阻力" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "脑室系统" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "脑室系统发生急性闭塞" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "脑脊液" }, { "start_idx": 67, "end_idx": 72, "type": "ite", "entity": "脑脊液分泌压" } ] }, { "text": "颅内高压@脑脊液在流通过程中也受到一定的阻力,与脑脊液的产生和维持也有一定关系。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脑脊液" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑脊液" } ] }, { "text": "颅内高压@(二)流体静力因素在水平侧卧位时,腰部脑脊液的压力与小脑延髓池和脑室者相等颅内高压@坐位时腰部椎管脑脊液压可高达375小脑延髓池的压力常呈负压顶部的压力也呈明显负压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 22, "end_idx": 29, "type": "ite", "entity": "腰部脑脊液的压力" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "小脑延髓池" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "脑室者" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "颅内高压" }, { "start_idx": 50, "end_idx": 57, "type": "ite", "entity": "腰部椎管脑脊液压" }, { "start_idx": 50, "end_idx": 63, "type": "sym", "entity": "腰部椎管脑脊液压可高达375" }, { "start_idx": 64, "end_idx": 71, "type": "ite", "entity": "小脑延髓池的压力" }, { "start_idx": 64, "end_idx": 75, "type": "sym", "entity": "小脑延髓池的压力常呈负压" }, { "start_idx": 76, "end_idx": 86, "type": "sym", "entity": "顶部的压力也呈明显负压" }, { "start_idx": 76, "end_idx": 80, "type": "ite", "entity": "顶部的压力" } ] }, { "text": "颅内高压@(三)静脉血压静脉内的压力是产生和维持脑脊液压的主要因素之一,全身静脉血压和颅脊腔内静脉血压都影响脑脊液压力。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "静脉血压" }, { "start_idx": 12, "end_idx": 17, "type": "ite", "entity": "静脉内的压力" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "脑脊液压" }, { "start_idx": 36, "end_idx": 41, "type": "ite", "entity": "全身静脉血压" }, { "start_idx": 43, "end_idx": 50, "type": "ite", "entity": "颅脊腔内静脉血压" }, { "start_idx": 54, "end_idx": 58, "type": "ite", "entity": "脑脊液压力" } ] }, { "text": "颅内高压@椎管静脉丛的任何压力改变皆以同等程度反映于脑脊液压胸腔内压力升高咳嗽和用力)时,脑脊液压也以同等程度增高胸内压力经椎静脉扩展到椎静脉丛并阻碍颅内静脉的回流而影响脑脊液吸收。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "椎管静脉丛" }, { "start_idx": 26, "end_idx": 29, "type": "ite", "entity": "脑脊液压" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "胸腔内压力升高" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "胸腔内压力" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "咳嗽" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "脑脊液压" }, { "start_idx": 45, "end_idx": 56, "type": "sym", "entity": "脑脊液压也以同等程度增高" }, { "start_idx": 57, "end_idx": 60, "type": "ite", "entity": "胸内压力" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "椎静脉" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "椎静脉丛" }, { "start_idx": 73, "end_idx": 81, "type": "sym", "entity": "阻碍颅内静脉的回流" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "颅内静脉" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "脑脊液" } ] }, { "text": "颅内高压@腰部椎管静脉丛经穿过椎间孔的腰静脉回流至下腔静脉,当以手压迫腹(脐)部时,因阻碍下腔静脉血液的回流,使椎管静脉丛充盈,从而使脑脊液压增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "腰部椎管静脉丛" }, { "start_idx": 13, "end_idx": 21, "type": "bod", "entity": "穿过椎间孔的腰静脉" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "手" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "腹(脐)部" }, { "start_idx": 43, "end_idx": 53, "type": "sym", "entity": "阻碍下腔静脉血液的回流" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "下腔静脉血液" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "椎管静脉丛" }, { "start_idx": 67, "end_idx": 70, "type": "ite", "entity": "脑脊液压" }, { "start_idx": 67, "end_idx": 72, "type": "sym", "entity": "脑脊液压增高" } ] }, { "text": "颅内高压@脑脊液压较颅内静脉窦的血压高约5~10mmH2O。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "脑脊液压" }, { "start_idx": 10, "end_idx": 17, "type": "ite", "entity": "颅内静脉窦的血压" } ] }, { "text": "颅内高压@当压迫颈静脉时,颅内静脉窦内压力升高,脑脊液压同时升高,临床上腰椎穿刺时常用的压颈试验即是颅内压受静脉压增高直接影响的实际应用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "颈静脉" }, { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "颅内静脉窦内压力升高" }, { "start_idx": 13, "end_idx": 20, "type": "ite", "entity": "颅内静脉窦内压力" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "脑脊液压同时升高" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "脑脊液压" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "压颈试验" }, { "start_idx": 50, "end_idx": 52, "type": "ite", "entity": "颅内压" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "静脉压" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "静脉压增高" } ] }, { "text": "颅内高压@心力衰竭循环淤血胸腔上部或纵隔占位病变压迫上腔静脉者,颅内静脉窦血栓引起闭塞,皆可由于静脉血液回流阻碍而引起颅内压增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "循环淤血" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "胸腔上部" }, { "start_idx": 13, "end_idx": 29, "type": "sym", "entity": "胸腔上部或纵隔占位病变压迫上腔静脉" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "纵隔" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "颅内静脉窦血栓" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "颅内静脉窦" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "闭塞" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "静脉血液" }, { "start_idx": 48, "end_idx": 55, "type": "sym", "entity": "静脉血液回流阻碍" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@(四)动脉血压的影响动脉血压的突然改变,无论升高或降低,皆引起颅内压相应改变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "动脉血压" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "动脉血压" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@变化的程度与血压改变的速度与程度密切相关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "血压" } ] }, { "text": "颅内高压@动脉血压改变较缓或较轻时,由于脑部动脉阻力的相应调整,对颅内压的影响较小。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "动脉血压" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "脑部动脉" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@相反,如血压变化急剧时,则处于失代偿状态,颅内压常明显升高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "血压" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "颅内压" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "颅内压常明显升高" } ] }, { "text": "颅内高压@【病因和发生机制】上述颅腔内三种内容物(脑、血液和脑脊液)体积的增大与颅内占位性病变均可引起颅内压增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "颅腔内" }, { "start_idx": 16, "end_idx": 38, "type": "sym", "entity": "颅腔内三种内容物(脑、血液和脑脊液)体积的增大" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "脑" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血液" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "脑脊液" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "颅内占位性病变" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@因此引起颅压高的主要因素是以下4类:①脑脊液增多,见于交通性与非交通性脑积水血液增多脑血管扩张或出血脑组织体积增大脑水肿;④颅内占位性病变血肿、肿瘤与脑脓肿等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "颅压高" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "颅压" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "脑脊液增多" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑脊液" }, { "start_idx": 32, "end_idx": 42, "type": "dis", "entity": "交通性与非交通性脑积水" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "血液" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "血液增多" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "脑血管" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "脑血管扩张或出血" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "脑组织" }, { "start_idx": 55, "end_idx": 61, "type": "sym", "entity": "脑组织体积增大" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "脑水肿" }, { "start_idx": 67, "end_idx": 73, "type": "dis", "entity": "颅内占位性病变" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "血肿" }, { "start_idx": 77, "end_idx": 78, "type": "dis", "entity": "肿瘤" }, { "start_idx": 80, "end_idx": 82, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "颅内高压@常见原因可归纳为以下6大类:①外伤性;②血管性如出血性或闭塞性脑血管病;③炎症性如脑炎与脑膜炎等;④先天性如婴儿脑积水或狭颅症等;⑤颅内肿瘤;⑥全身性疾病如窒息、肺炎或中毒性痢疾引起的中毒性脑病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "外伤" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血管" }, { "start_idx": 29, "end_idx": 39, "type": "dis", "entity": "出血性或闭塞性脑血管病" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "炎症" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "脑炎" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "婴儿脑积水" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "狭颅症" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "窒息" }, { "start_idx": 86, "end_idx": 87, "type": "dis", "entity": "肺炎" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "中毒性痢疾" }, { "start_idx": 97, "end_idx": 101, "type": "dis", "entity": "中毒性脑病" } ] }, { "text": "颅内高压@这些疾病可由于上述四种因素之一种或多种因素而产生颅内压增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@颅内压增高的发病机制是由于上述各种因素导致颅内f容积代偿失调脑脊液增多脑脊液分泌增多或循环障碍所致,以后者常见,病理生理改变表现为脑积水。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "颅内f容积" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "颅内f容积代偿失调" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "脑脊液" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "脑脊液增多" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "脑脊液" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "脑脊液分泌增多" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "循环障碍" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "脑积水" } ] }, { "text": "颅内高压@脑积水可分为交通性与非交通性两类。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "脑积水" } ] }, { "text": "颅内高压@交通性脑积水者脑脊液能从脑室系统流入蛛网膜下腔,脑脊液的循环障碍发生在蛛网膜下腔;非交通性脑积水也称阻塞性脑积水,阻塞发生在脑室系统,脑脊液不能流入蛛网膜下腔。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "交通性脑积水" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "脑脊液" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "脑室系统" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "脑脊液的循环障碍" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "脑脊液" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "非交通性脑积水" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "阻塞性脑积水" }, { "start_idx": 62, "end_idx": 70, "type": "sym", "entity": "阻塞发生在脑室系统" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "脑室系统" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "脑脊液" }, { "start_idx": 72, "end_idx": 83, "type": "sym", "entity": "脑脊液不能流入蛛网膜下腔" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "蛛网膜下腔" } ] }, { "text": "颅内高压@单纯脑脊液产生过多可见于脉络丛乳头瘤、维生素A缺乏或过量,以及儿童服用四环素等,临床一般不引起严重脑积水。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 13, "type": "sym", "entity": "单纯脑脊液产生过多" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "脑脊液" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "脉络丛乳头瘤" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "四环素" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "严重脑积水" } ] }, { "text": "颅内高压@(二)颅内血容量增加颅内血容量增加颅压高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "颅内血容量" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "颅内血容量增加" }, { "start_idx": 15, "end_idx": 19, "type": "ite", "entity": "颅内血容量" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "颅内血容量增加" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "颅压高" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "颅压" } ] }, { "text": "颅内高压@常见的疾病包括:脑外伤所致的脑血管扩张;蛛网膜下腔出血脑血管畸形或其他原因所致);颅内静脉窦血栓;动脉和静脉血压升高颅内高压@(三)脑组织容积增加脑水肿即过多的水积蓄于脑实质内所致。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "脑外伤" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "脑血管扩张" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "脑血管" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "脑血管畸形" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "颅内静脉窦血栓" }, { "start_idx": 54, "end_idx": 60, "type": "ite", "entity": "动脉和静脉血压" }, { "start_idx": 54, "end_idx": 62, "type": "sym", "entity": "动脉和静脉血压升高" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "颅内高压" }, { "start_idx": 71, "end_idx": 75, "type": "ite", "entity": "脑组织容积" }, { "start_idx": 71, "end_idx": 77, "type": "sym", "entity": "脑组织容积增加" }, { "start_idx": 78, "end_idx": 80, "type": "dis", "entity": "脑水肿" }, { "start_idx": 82, "end_idx": 92, "type": "sym", "entity": "过多的水积蓄于脑实质内" }, { "start_idx": 89, "end_idx": 92, "type": "bod", "entity": "脑实质内" } ] }, { "text": "颅内高压@脑水肿是临床上各种危重症最常见的中枢神经系统并发症,也是颅内压增高的各种因素中最常见者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "脑水肿" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "中枢神经系统并发症" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@其原发病以感染性疾病最多。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "颅内高压@根据病变的范围不同,脑水肿的部位亦有所不同。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "脑水肿" } ] }, { "text": "颅内高压@窒息、心跳呼吸骤停及中毒性脑病等表现为弥漫性的脑水肿;局灶性病变(脑瘤、血肿、脓肿与脑挫裂伤等)则引起病灶周围脑水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "窒息" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "心跳呼吸骤停" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "中毒性脑病" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "弥漫性的脑水肿" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "局灶性病变" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "脑瘤" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "血肿" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "血" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "脓肿" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "脑挫裂伤" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "脑水肿" } ] }, { "text": "颅内高压@根据发生机制脑水肿可分为以下几类。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "脑水肿" } ] }, { "text": "颅内高压@1.血管源性脑水肿见于脑外伤、脑瘤与颅内炎症等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "血管源性脑水肿" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑外伤" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "脑瘤" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "颅内炎症" } ] }, { "text": "颅内高压@由于血脑屏障发生障碍,血浆蛋白、液体与某些离子外溢于细胞外间隙,引起细胞外水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "血脑屏障发生障碍" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "血脑屏障" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "血浆蛋白" }, { "start_idx": 16, "end_idx": 35, "type": "sym", "entity": "血浆蛋白、液体与某些离子外溢于细胞外间隙" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "离子" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "细胞外间隙" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "细胞外水肿" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "细胞外" } ] }, { "text": "颅内高压@病理特点是水肿液中蛋白质增多细胞间隙增宽水肿以白质为主颅内高压@2.细胞毒性脑水肿见于各种原因引起的脑缺血缺氧(呼吸循环暂停及休克等),系细胞代谢障碍所引起。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "水肿液" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "水肿液中蛋白质增多" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "蛋白质" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "细胞间隙" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "细胞间隙增宽" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "水肿" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "水肿以白质为主" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "白质" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "颅内高压" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "细胞毒性脑水肿" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "脑缺血缺氧" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "呼吸循环暂停" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "休克" }, { "start_idx": 74, "end_idx": 79, "type": "dis", "entity": "细胞代谢障碍" } ] }, { "text": "颅内高压@由于脑组织缺血缺氧,引起细胞膜的损害与细胞渗透调节的紊乱细胞内水肿颅内高压@细胞毒性脑水肿的特点为细胞内水肿细胞间隙缩小脑灰白质均受累颅内高压@3.间质性脑水肿常见于非交通性脑积水,脑脊液通过受损的室管膜进入脑室周围的白质,特别是额叶的白质。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "脑组织缺血缺氧" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "脑组织" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "细胞膜的损害" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "细胞" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "细胞渗透调节的紊乱" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "细胞内" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "细胞内水肿" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "颅内高压" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "细胞毒性脑水肿" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "细胞内" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "细胞内水肿" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "细胞" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "细胞间隙缩小" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "脑灰白质" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "脑灰白质均受累" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "颅内高压" }, { "start_idx": 79, "end_idx": 84, "type": "dis", "entity": "间质性脑水肿" }, { "start_idx": 88, "end_idx": 94, "type": "dis", "entity": "非交通性脑积水" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "脑脊液" }, { "start_idx": 101, "end_idx": 106, "type": "bod", "entity": "受损的室管膜" }, { "start_idx": 109, "end_idx": 115, "type": "bod", "entity": "脑室周围的白质" }, { "start_idx": 120, "end_idx": 124, "type": "bod", "entity": "额叶的白质" } ] }, { "text": "颅内高压@间质性脑水肿的特点是脑脊液聚积在室管膜周围白质的细胞外水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "间质性脑水肿" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脑脊液" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "室管膜周围白质" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "细胞外水肿" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "细胞外" } ] }, { "text": "颅内高压@4.渗透压性脑水肿见于急性水中毒、低渗性脱水、急性低钠血症、中枢神经系统感染并发的抗利尿激素异常分泌综合征等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "渗透压性脑水肿" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "急性水中毒" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "低渗性脱水" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "急性低钠血症" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "中枢神经系统感染" }, { "start_idx": 46, "end_idx": 57, "type": "dis", "entity": "抗利尿激素异常分泌综合征" } ] }, { "text": "颅内高压@由于血浆渗透压降低,水分大量内流形成细胞内水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "血浆渗透压降低" }, { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "血浆渗透压" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "水分大量内流" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "细胞内水肿" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "细胞内" } ] }, { "text": "颅内高压@(四)颅内占位性病变主要包括颅内肿瘤、血肿和脓肿,是颅内压增高的常见原因之一。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "颅内占位性病变" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "血肿" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "血" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "脓肿" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@颅内占位性病变引起颅内压增高的机制主要包括:①病变本身引起颅内容积增多继发性脑脊液循环障碍;③病变周围脑水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "颅内占位性病变" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "颅内容积" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "颅内容积增多" }, { "start_idx": 40, "end_idx": 49, "type": "dis", "entity": "继发性脑脊液循环障碍" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "脑水肿" } ] }, { "text": "颅内高压@由于颅压调节的容积代偿功能,小的占位性病变一般不引起颅内压增高的临床症状;但由于颅内容积代偿是有限的,仅为整个颅内腔的5%~10%,因此如占位性病变较大时则出现失代偿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "颅压" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "小的占位性病变" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "占位性病变" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "颅内容积" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "颅内腔" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "占位性病变" }, { "start_idx": 74, "end_idx": 80, "type": "sym", "entity": "占位性病变较大" } ] }, { "text": "颅内高压@占位性病变是否导致颅压高还与其病变生长速度及部位有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "占位性病变" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "颅压高" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "颅压" } ] }, { "text": "颅内高压@增长速度越快则颅压增高越突出;中线附近的占位性病变由于容易引起脑脊液循环障碍,因此更易导致颅压高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "颅压增高" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "中线" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "中线附近的占位性病变" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "占位性病变" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "脑脊液循环障碍" }, { "start_idx": 50, "end_idx": 52, "type": "sym", "entity": "颅压高" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "颅压" } ] }, { "text": "颅内高压@【临床表现】除原发病的相应表现外,颅内压增高的主要表现有头痛、呕吐和视乳头水肿(见于持续时间较长者),称为颅内高压三主征。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "头痛" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "头" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "呕吐" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "颅内高压" } ] }, { "text": "颅内高压@(一)头痛是颅内压增高最常见的症状,可能是由于脑膜、神经或血管受牵拉所致,发生率大约为80%~90%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "头痛" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "头" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "脑膜" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "神经" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血管" } ] }, { "text": "颅内高压@头痛程度差异较大,一般病初时较轻,以后逐渐加重,并呈持续性头痛。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "头痛" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "头" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "持续性头痛" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "头" } ] }, { "text": "颅内高压@头痛的特点是持续性痛、阵发性加重,清晨时加重,用力、咳嗽、弯腰或低头活动时常使头痛加重。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "头痛" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "头" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "持续性痛" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "咳嗽" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "腰" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "头" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "头痛加重" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "头" } ] }, { "text": "颅内高压@头痛部位多在额部、颞部、眼眶或枕部,与病变部位无关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "头痛" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "头" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "额部" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "颞部" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "眼眶" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "枕部" } ] }, { "text": "颅内高压@头痛程度随颅内压的增高而加重。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "头痛" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "头" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "颅内压的增高" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@急性颅内压增高时头痛常很剧烈。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "急性颅内压增高" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "头痛常很剧烈" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "头" } ] }, { "text": "颅内高压@(二)呕吐约2/3病例出现呕吐,常出现于头痛剧烈时,可伴有恶心。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "呕吐" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "呕吐" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "头痛剧烈" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "头" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "恶心" } ] }, { "text": "颅内高压@典型表现为喷射性呕吐,呕吐与进食关系不大而与头痛剧烈程度有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "喷射性呕吐" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "呕吐" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "头痛" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "头" } ] }, { "text": "颅内高压@第Ⅳ脑室和后颅凹的病变较易引起呕吐。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "第Ⅳ脑室" }, { "start_idx": 5, "end_idx": 15, "type": "sym", "entity": "第Ⅳ脑室和后颅凹的病变" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "后颅凹" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "呕吐" } ] }, { "text": "颅内高压@儿童头痛常不显著,呕吐有时是唯一表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "头痛" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "头" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "呕吐" } ] }, { "text": "颅内高压@(三)视乳头水肿是颅内压增高最重要的客观体征,发生率大约为60%~70%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@表现为视神经乳头充血边缘模糊不清,中央凹陷消失,视乳头隆起静脉怒张颅内高压@它出现的时间并不确定,颅内压增高的早期,一般不出现视乳头水肿;婴幼儿由于囟门未闭及颅缝分离,也常无视乳头水肿;急性颅内压增高视乳头水肿较少出现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "视神经" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "视神经乳头充血" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "乳头" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "边缘模糊不清" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "中央凹陷消失" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "视乳头" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "视乳头隆起" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "静脉" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "静脉怒张" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "颅内高压" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "囟门" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "颅缝" }, { "start_idx": 92, "end_idx": 96, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 98, "end_idx": 104, "type": "dis", "entity": "急性颅内压增高" }, { "start_idx": 105, "end_idx": 109, "type": "dis", "entity": "视乳头水肿" } ] }, { "text": "颅内高压@在视乳头水肿的早期,虽有典型的眼底改变,但视力一般没有明显障碍。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "眼底改变" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "眼底" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "视力" } ] }, { "text": "颅内高压@如颅内压增高持续存在或继续发展,视野检查可发现生理盲点扩大,中心视力暗点阵发性黑蒙。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "视野检查" }, { "start_idx": 21, "end_idx": 40, "type": "sym", "entity": "视野检查可发现生理盲点扩大,中心视力暗点" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "视力" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "阵发性黑蒙" } ] }, { "text": "颅内高压@病情再进一步发展,可发生继发性视神经萎缩,视乳头出现苍白视力开始明显减退直至失明。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "继发性视神经萎缩" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "视乳头" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "视乳头出现苍白" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "视力开始明显减退" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "视力" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "失明" } ] }, { "text": "颅内高压@(四)其他表现颅内压增高还可引起头昏耳鸣烦躁不安、嗜睡、复视、意识障碍、头皮静脉怒张血压增高脉搏徐缓一侧或双侧展神经的不全麻痹等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "头" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "头昏" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "耳" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "耳鸣" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "嗜睡" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "复视" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "意识障碍" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "头皮静脉怒张" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "静脉" }, { "start_idx": 47, "end_idx": 48, "type": "ite", "entity": "血压" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "血压增" }, { "start_idx": 51, "end_idx": 52, "type": "ite", "entity": "脉搏" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "脉搏徐缓" }, { "start_idx": 55, "end_idx": 62, "type": "bod", "entity": "一侧或双侧展神经" }, { "start_idx": 55, "end_idx": 67, "type": "sym", "entity": "一侧或双侧展神经的不全麻痹" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "麻痹" } ] }, { "text": "颅内高压@小儿慢性颅压增高常见头颅增大颅缝增宽或分裂囟门饱满隆起头颅叩诊时呈破壶音Macewen征)等表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "小儿慢性颅压增高" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "头颅" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "头颅增大" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "颅缝" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "颅缝增宽或分裂" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "囟门" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "囟门饱满隆起" }, { "start_idx": 32, "end_idx": 48, "type": "sym", "entity": "头颅叩诊时呈破壶音Macewen征" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "头颅叩诊" } ] }, { "text": "颅内高压@颅内压增高严重时,可有生命体征的变化:血压升高脉搏变慢呼吸减慢颅内压增高的危险征兆,提示有发生脑疝之危险。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "血压升高" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "血压" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "脉搏变慢" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "脉搏" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "呼吸减慢" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "脑疝" } ] }, { "text": "颅内高压@(五)脑疝各种原因引起的颅内压增高,都可以导致脑组织由压力高的部位向压力低的部位移位,形成脑疝,颅内局灶占位性病变引起颅内压增高者更容易发生脑疝。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "脑疝" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "脑组织" }, { "start_idx": 28, "end_idx": 46, "type": "sym", "entity": "脑组织由压力高的部位向压力低的部位移位" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "脑疝" }, { "start_idx": 53, "end_idx": 61, "type": "dis", "entity": "颅内局灶占位性病变" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "脑疝" } ] }, { "text": "颅内高压@最常见的脑疝有两类:①小脑幕切迹疝(tentorialnotch),幕上的脑组织(颞叶的海马及钩回)通过小脑幕切迹挤向幕下,又称海马钩回疝;②枕骨大孔疝(tonsillarhernia),幕下的小脑扁桃体及延髓经枕骨大孔疝入椎管内,又称小脑扁桃体疝。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "脑疝" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "小脑幕切迹疝" }, { "start_idx": 23, "end_idx": 36, "type": "dis", "entity": "tentorialnotch" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "幕上的脑组织" }, { "start_idx": 39, "end_idx": 65, "type": "sym", "entity": "幕上的脑组织(颞叶的海马及钩回)通过小脑幕切迹挤向幕下" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "颞叶的海马" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "钩回" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "小脑幕切迹" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "幕下" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "海马钩回疝" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "枕骨大孔疝" }, { "start_idx": 82, "end_idx": 96, "type": "dis", "entity": "tonsillarhernia" }, { "start_idx": 99, "end_idx": 119, "type": "sym", "entity": "幕下的小脑扁桃体及延髓经枕骨大孔疝入椎管内" }, { "start_idx": 99, "end_idx": 109, "type": "bod", "entity": "幕下的小脑扁桃体及延髓" }, { "start_idx": 111, "end_idx": 114, "type": "bod", "entity": "枕骨大孔" }, { "start_idx": 117, "end_idx": 119, "type": "bod", "entity": "椎管内" }, { "start_idx": 123, "end_idx": 128, "type": "dis", "entity": "小脑扁桃体疝" } ] }, { "text": "颅内高压@脑疝不仅使疝入的脑组织受压,而且还会压迫邻近结构,使血液循环和脑脊液循环受阻,进一步加重颅内高压,最终危及生命。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "脑疝" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "使疝入的脑组织受压" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "疝入的脑组织" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "压迫邻近结构" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "血液循环" }, { "start_idx": 31, "end_idx": 42, "type": "sym", "entity": "血液循环和脑脊液循环受阻" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "脑脊液循环" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "颅内高压" } ] }, { "text": "颅内高压@1.小脑幕切迹疝主要表现包括:①颅内压增高剧烈头痛频繁呕吐,程度较脑疝前加重,常伴烦躁不安;②意识改变:可由嗜睡、昏睡到浅昏迷以至深昏迷;③瞳孔改变动眼神经受到压迫牵拉所致,早期病侧瞳孔略缩小光反应稍迟钝,随后逐渐散大,直接及间接光反应消失,晚期因中脑动眼神经核受压麻痹引起双侧瞳孔散大光反应消失;④运动障碍:表现为对侧偏瘫。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "小脑幕切迹疝" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "剧烈头痛" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "头" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "频繁呕吐" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "脑疝" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "意识改变" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "嗜睡" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "昏睡" }, { "start_idx": 65, "end_idx": 67, "type": "sym", "entity": "浅昏迷" }, { "start_idx": 70, "end_idx": 72, "type": "sym", "entity": "深昏迷" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "瞳孔改变" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "瞳孔" }, { "start_idx": 79, "end_idx": 88, "type": "sym", "entity": "动眼神经受到压迫牵拉" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "动眼神经" }, { "start_idx": 94, "end_idx": 100, "type": "sym", "entity": "病侧瞳孔略缩小" }, { "start_idx": 94, "end_idx": 97, "type": "bod", "entity": "病侧瞳孔" }, { "start_idx": 101, "end_idx": 106, "type": "sym", "entity": "光反应稍迟钝" }, { "start_idx": 110, "end_idx": 113, "type": "sym", "entity": "逐渐散大" }, { "start_idx": 115, "end_idx": 124, "type": "sym", "entity": "直接及间接光反应消失" }, { "start_idx": 129, "end_idx": 139, "type": "sym", "entity": "中脑动眼神经核受压麻痹" }, { "start_idx": 129, "end_idx": 135, "type": "bod", "entity": "中脑动眼神经核" }, { "start_idx": 142, "end_idx": 147, "type": "sym", "entity": "双侧瞳孔散大" }, { "start_idx": 142, "end_idx": 145, "type": "bod", "entity": "双侧瞳孔" }, { "start_idx": 148, "end_idx": 152, "type": "sym", "entity": "光反应消失" }, { "start_idx": 155, "end_idx": 158, "type": "sym", "entity": "运动障碍" }, { "start_idx": 163, "end_idx": 166, "type": "sym", "entity": "对侧偏瘫" }, { "start_idx": 165, "end_idx": 166, "type": "dis", "entity": "偏瘫" } ] }, { "text": "颅内高压@脑疝继续发展可引起四肢瘫痪,甚至出现去大脑强直;⑤生命体征改变:表现为血压、脉搏、呼吸及体温的改变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "脑疝" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "四肢瘫痪" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "四肢" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "瘫痪" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "去大脑强直" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "大脑" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "生命体征改变" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "血压" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "脉搏" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "呼吸" }, { "start_idx": 49, "end_idx": 50, "type": "ite", "entity": "体温" } ] }, { "text": "颅内高压@初期血压升高严重时忽高忽低,脉搏减慢呼吸不规则面色潮红大汗淋漓,有时转为苍白、汗闭,体温可高达41℃以上体温不升呼吸、循环衰竭死亡颅内高压@2.枕骨大孔疝颅内高压@【诊断】颅内压增高根据进行性头痛、呕吐和视乳头水肿,一般可做出诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "血压升高" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "血压" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "严重时忽高忽低" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "脉搏减慢" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "脉搏" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "呼吸不规则" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "面色" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "面色潮红" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "大汗淋漓" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "苍白" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "汗闭" }, { "start_idx": 47, "end_idx": 56, "type": "sym", "entity": "体温可高达41℃以上" }, { "start_idx": 47, "end_idx": 48, "type": "ite", "entity": "体温" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "体温不升" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "体温" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "呼吸、循环衰竭" }, { "start_idx": 61, "end_idx": 69, "type": "sym", "entity": "呼吸、循环衰竭死亡" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "颅内高压" }, { "start_idx": 77, "end_idx": 81, "type": "dis", "entity": "枕骨大孔疝" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "颅内高压" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "颅内" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 98, "end_idx": 102, "type": "sym", "entity": "进行性头痛" }, { "start_idx": 101, "end_idx": 101, "type": "bod", "entity": "头" }, { "start_idx": 104, "end_idx": 105, "type": "sym", "entity": "呕吐" }, { "start_idx": 107, "end_idx": 111, "type": "dis", "entity": "视乳头水肿" } ] }, { "text": "颅内高压@应注意早期颅内压增高和婴幼儿视乳头水肿不一定出现,儿童的头痛主诉有时不明显,呕吐可能是唯一的主诉,对小儿的反复头昏、呕吐及头围增大应考虑颅内高压的可能。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "头痛" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "头" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "呕吐" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "反复头昏" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "头" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "呕吐" }, { "start_idx": 66, "end_idx": 69, "type": "sym", "entity": "头围增大" }, { "start_idx": 66, "end_idx": 67, "type": "ite", "entity": "头围" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "颅内高压" } ] }, { "text": "颅内高压@如有视乳头水肿,则颅内压增高的诊断基本可以确定。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@由于病人的自觉症状常比视乳头水肿及头颅X线片中的改变出现要早,故对症状明显的病人不能单凭视乳头正常而排除颅内压增高的可能。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "视乳头水肿" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "头颅X线片" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "视乳头" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@根据病史和体征初步诊断后应选择性进行以下辅助检查,进一步明确颅内压增高的有无及程度,同时寻找引起颅内压增高的病因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "辅助检查" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "颅内高压@(一)神经影像学检查头颅CT检查安全、方便、无创伤,可以了解有无颅内占位性病变或其他病变、中线是否移位以及脑室是否受压变形,有无脑实质饱满脑沟消失外侧裂变窄或消失等脑水肿征象。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "神经影像学检查" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "头颅CT检查" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "颅内占位性病变" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "脑室" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "脑实质" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "脑实质饱满" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "脑沟" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "脑沟消失" }, { "start_idx": 78, "end_idx": 85, "type": "sym", "entity": "外侧裂变窄或消失" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "外侧裂" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "脑水肿" } ] }, { "text": "颅内高压@头颅MRI能够更准确地检查出颅内病变,特别是对脑干和后颅凹的病变优于CT,对脑梗死等急症可在病程早期发现异常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "头颅MRI" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "颅内病变" }, { "start_idx": 28, "end_idx": 36, "type": "dis", "entity": "脑干和后颅凹的病变" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "CT" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "脑梗死" } ] }, { "text": "颅内高压@(二)头颅X线片可提供参考信息。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "头颅X线片" } ] }, { "text": "颅内高压@提示颅内压增高的特征包括颅缝裂开脑回压迹加深蛛网膜颗粒压迹增大加深蝶鞍扩大鞍背及前后床突的吸收或破坏颅内高压@此外,颅骨的局部破坏或增生钙化松果体的移位,病理钙斑等的存在可提示病变的大体性质及方位。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "颅缝裂开" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "颅缝" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "脑回压迹加深" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "脑回" }, { "start_idx": 27, "end_idx": 37, "type": "sym", "entity": "蛛网膜颗粒压迹增大加深" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "蛛网膜颗粒" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "蝶鞍扩大" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "蝶鞍" }, { "start_idx": 42, "end_idx": 54, "type": "sym", "entity": "鞍背及前后床突的吸收或破坏" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "鞍背及前后床突" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "颅内高压" }, { "start_idx": 63, "end_idx": 74, "type": "sym", "entity": "颅骨的局部破坏或增生钙化" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "颅骨" }, { "start_idx": 75, "end_idx": 80, "type": "sym", "entity": "松果体的移位" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "松果体" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "病理钙斑" } ] }, { "text": "颅内高压@(三)脑血管造影检查主要用于明确有无脑血管病变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "脑血管造影检查" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "脑血管病变" } ] }, { "text": "颅内高压@对高度怀疑脑血管畸形的患儿可选择该项检查。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "脑血管畸形" } ] }, { "text": "颅内高压@(四)腰椎穿刺可直接测量颅内压明确诊断,同时可化验脑脊液,对病因诊断提供依据。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "颅内压" }, { "start_idx": 28, "end_idx": 32, "type": "pro", "entity": "化验脑脊液" } ] }, { "text": "颅内高压@脑脊液血性或变黄颅内出血;脑脊液混浊白细胞增多病原学检查阳性颅内炎症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脑脊液" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "脑脊液血性或变黄" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "颅内出血" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑脊液" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "脑脊液混浊" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "白细胞" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "白细胞增多" }, { "start_idx": 28, "end_idx": 32, "type": "pro", "entity": "病原学检查" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "病原学检查阳性" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "颅内炎症" } ] }, { "text": "颅内高压@腰椎穿刺需注意:①有诱发脑疝的危险,因此对颅内压客观体征已很明显的病人禁忌腰椎穿刺检查。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "脑疝" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "颅内压" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "腰椎穿刺检查" } ] }, { "text": "颅内高压@临床怀疑颅压高,为了除外颅内感染等病因而确需腰穿检查时,应先脱水治疗,待颅压稳定后再行检查。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "颅压高" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "外颅内感染" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "腰穿检查" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "脱水治疗" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "颅压" } ] }, { "text": "颅内高压@②有椎管梗阻时经腰穿测压并不能准确代表颅内压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "椎管梗阻" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "经腰穿测压" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@【治疗】(一)一般处理1.密切观察病人的意识、瞳孔、血压、呼吸、脉搏及体温的变化,掌握病情发展的动态,及时采取措施。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "意识" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "瞳孔" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "血压" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "呼吸" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "脉搏" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "体温" } ] }, { "text": "颅内高压@病情稳定者要及时进行原因检查和治疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 11, "end_idx": 21, "type": "pro", "entity": "及时进行原因检查和治疗" } ] }, { "text": "颅内高压@2.吸氧有助于降低颅内压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "吸氧" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@3.保持体液、电解质和酸碱平衡液体入量限于1000ml/(m2•d)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 44, "type": "pro", "entity": "保持体液、电解质和酸碱平衡液体入量限于1000ml/(m2•d)" } ] }, { "text": "颅内高压@含1/3~1/5张含钠液,记录尿量,入量应少于出量。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 5, "end_idx": 16, "type": "pro", "entity": "含1/3~1/5张含钠液" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "记录尿量" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "入量应少于出量" } ] }, { "text": "颅内高压@4.保持呼吸道通畅对意识不清及咳痰困难者要做气管切开术,防止因呼吸不畅而使颅内压进一步增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "意识不清" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "咳痰困难" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "气管切开术" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "呼吸不畅" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "使颅内压进一步增高" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@5.控制惊厥可给予地西泮(安定)每次0.3~0.5mg/kg,静脉注射,半小时后可重复一次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "控制惊厥" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "地西泮" }, { "start_idx": 18, "end_idx": 19, "type": "dru", "entity": "安定" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "静脉注射" } ] }, { "text": "颅内高压@对于反复发作或惊厥持续状态者,可酌情给予苯巴比妥钠或苯妥英钠静脉注射。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "惊厥持续状态" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "苯巴比妥钠" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "静脉注射" } ] }, { "text": "颅内高压@(二)降颅压治疗1.内科治疗常用药物如下。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "降颅压治疗" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "内科治疗" } ] }, { "text": "颅内高压@(1)20%甘露醇:0.25~1.5g/kg,静脉推入,每4~6小时一次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "甘露醇" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "静脉推入" } ] }, { "text": "颅内高压@5~30分钟发挥作用,15~90分钟达最大效力,作用持续3~6小时。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" } ] }, { "text": "颅内高压@(2)甘油:常用10%甘油盐水0.25~1.0g/kg静注,或0.5~2.0g/kg口服,每6~8小时一次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 9, "type": "dru", "entity": "甘油" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "甘油盐水" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "静注" } ] }, { "text": "颅内高压@15~30分钟开始作用,静注后30分钟及口服后60~80分钟达最大作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" } ] }, { "text": "颅内高压@(3)肾上腺皮质激素:常用地塞米松0.2mg/kg静注,每6小时一次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "地塞米松" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "静注" } ] }, { "text": "颅内高压@6~8小时起作用,12~24小时达高峰。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" } ] }, { "text": "颅内高压@(4)戊巴比妥:通过降低脑代谢和脑血流使颅压降低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "戊巴比妥" }, { "start_idx": 15, "end_idx": 23, "type": "pro", "entity": "降低脑代谢和脑血流" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "颅压" } ] }, { "text": "颅内高压@作用迅速,无反跳,不增加颅内出血的危险性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "颅内出血" } ] }, { "text": "颅内高压@该药必须在密切监护下进行,以免因过深的昏迷产生各种并发症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "密切监护" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "过深的昏迷" } ] }, { "text": "颅内高压@起始量3~5mg/kg静注,30分钟后每1~3小时追加静注1~3mg/kg,直至脑电图显示电抑制。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "静注" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "静注" }, { "start_idx": 45, "end_idx": 52, "type": "sym", "entity": "脑电图显示电抑制" }, { "start_idx": 45, "end_idx": 47, "type": "pro", "entity": "脑电图" } ] }, { "text": "颅内高压@(5)冬眠低温疗法:有利于降低脑代谢率减少脑组织的氧耗量,减缓脑水肿的发展,起到脑保护的作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "冬眠低温疗法" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "脑代谢率" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑组织" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "氧耗量" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "脑水肿" }, { "start_idx": 45, "end_idx": 47, "type": "pro", "entity": "脑保护" } ] }, { "text": "颅内高压@(6)过度通气:通过面罩或气管插管给予机械通气,使PaCO2下降至20~25mmHg(2.7~3.3kPa),可使脑血管收缩,脑血流减少而降低颅内压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "过度通气" }, { "start_idx": 15, "end_idx": 16, "type": "equ", "entity": "面罩" }, { "start_idx": 18, "end_idx": 19, "type": "equ", "entity": "气管" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "插管" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "机械通气" }, { "start_idx": 30, "end_idx": 40, "type": "ite", "entity": "PaCO2" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "脑血管" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "脑血流" }, { "start_idx": 82, "end_idx": 84, "type": "ite", "entity": "颅内压" } ] }, { "text": "颅内高压@2.外科治疗严重急性颅内压增高,可进行脑室内、蛛网膜下腔、硬膜下或硬膜外等多种形式穿刺减颅压,必要时置入导管,以便进行随时处理及监测。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "外科治疗" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "严重急性颅内压增高" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑室内" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "硬膜下" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "硬膜外" }, { "start_idx": 46, "end_idx": 50, "type": "pro", "entity": "穿刺减颅压" }, { "start_idx": 57, "end_idx": 58, "type": "equ", "entity": "导管" } ] }, { "text": "颅内高压@必要时进行颅骨开窗减压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "颅骨开窗减压" } ] }, { "text": "颅内高压@对于慢性颅内高压,可根据情况采用脑脊液腹腔分流术治疗,有明显疗效,可降低严重视力损害等并发症发生率。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "慢性颅内高压" }, { "start_idx": 21, "end_idx": 30, "type": "pro", "entity": "脑脊液腹腔分流术治疗" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "严重视力损害" } ] }, { "text": "颅内高压@(三)病因治疗如感染应给予抗生素,硬膜下或硬膜外积液应及时抽出,脑脓肿、肿瘤或其他占位病变的相应手术治疗等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "病因治疗" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "抗生素" }, { "start_idx": 22, "end_idx": 30, "type": "bod", "entity": "硬膜下或硬膜外积液" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "脑脓肿、肿瘤" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "占位病变" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "手术治疗" } ] }, { "text": "颅内高压@(秦炯)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内高压" } ] }, { "text": "第七章性早熟【概述】近100多年来全球儿童青春发育普遍提前,我国儿童的青春发育年龄也在不断提前。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "性早熟" } ] }, { "text": "任何性发育特征初显年龄较正常儿童平均年龄提前2个标准差以上,即儿童性发育启动年龄显著提前者称为性早熟(precociouspuberty)。", "entities": [ { "start_idx": 0, "end_idx": 28, "type": "sym", "entity": "任何性发育特征初显年龄较正常儿童平均年龄提前2个标准差以上" }, { "start_idx": 31, "end_idx": 43, "type": "sym", "entity": "儿童性发育启动年龄显著提前" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "性早熟" }, { "start_idx": 51, "end_idx": 67, "type": "dis", "entity": "precociouspuberty" } ] }, { "text": "性早熟的定义在不同国家或民族有差异,我国将女孩在8岁以前,男性在9岁以前出现第二性征,或者女孩在10岁之前出现月经,定义为性早熟。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "性早熟" }, { "start_idx": 21, "end_idx": 41, "type": "sym", "entity": "女孩在8岁以前,男性在9岁以前出现第二性征" }, { "start_idx": 45, "end_idx": 56, "type": "sym", "entity": "女孩在10岁之前出现月经" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "性早熟" } ] }, { "text": "根据正常青春发育年龄不断提前的趋势,近年有些国家将女性性早熟定在7.5岁以前出现性腺增大和第二性征,或者在9.5岁之前出现月经定义为性早熟。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "性早熟" }, { "start_idx": 32, "end_idx": 48, "type": "sym", "entity": "7.5岁以前出现性腺增大和第二性征" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "性腺" }, { "start_idx": 53, "end_idx": 62, "type": "sym", "entity": "9.5岁之前出现月经" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "性早熟" } ] }, { "text": "性早熟可以分为促性腺激素依赖性,也称真性或中枢性性早熟,以及非促性腺激素依赖性,后者亦称周围性或假性性早熟。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "性早熟" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "促性腺激素" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "真性或中枢性性早熟" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "非促性腺激素" }, { "start_idx": 44, "end_idx": 52, "type": "dis", "entity": "周围性或假性性早熟" } ] }, { "text": "真性性早熟都是同性性早熟,并起源于下丘脑-垂体-性腺轴的活动。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "真性性早熟" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "同性性早熟" }, { "start_idx": 17, "end_idx": 26, "type": "bod", "entity": "下丘脑-垂体-性腺轴" } ] }, { "text": "在假性性早熟中,出现部分第二性征,但未激活正常下丘脑-垂体-性腺轴之间的相互作用中等肥胖者一般发育较早,重度肥胖者则偏迟。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "假性性早熟" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "出现部分第二性征" }, { "start_idx": 23, "end_idx": 32, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "中等肥胖" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "发育较早" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "重度肥胖" } ] }, { "text": "中枢性性早熟国外报道的发病率为0.6%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "中枢性性早熟" } ] }, { "text": "国内未见性早熟的流行病学资料。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "性早熟" } ] }, { "text": "中枢性性早熟的发生率依性别而不同,男孩与女孩之比约23∶1。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "中枢性性早熟" } ] }, { "text": "【病理生理和发病机制】(一)下丘脑-垂体-性腺轴人体从胎儿经青春期到完全的性成熟和生育这一成长过程中,下丘脑(促性腺激素释放激素,GnRH)-垂体(促性腺激素,Gn)-性腺(性激素)轴(HPG轴)的调控和激活也相应发生一系列变化。", "entities": [ { "start_idx": 14, "end_idx": 23, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 51, "end_idx": 97, "type": "bod", "entity": "下丘脑(促性腺激素释放激素,GnRH)-垂体(促性腺激素,Gn)-性腺(性激素)轴(HPG轴)" } ] }, { "text": "下丘脑-垂体-性腺轴主要受两种机制调控:一种为性激素依赖性负反馈调控机制,即一定浓度的性激素能抑制GnRH和Gn的分泌,主要在2~3岁以内发挥作用;一种为中枢神经系统内在的抑制机制,如肾上腺能药物可刺激GnRH释放,内源性脑啡肽可抑制GnRH释放的频率,阿片受体拮抗剂纳曲酮可完全抑制Gn的分泌,主要在3~10岁左右发挥作用。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "性激素" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "性激素" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "GnRH" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "Gn" }, { "start_idx": 77, "end_idx": 82, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 92, "end_idx": 94, "type": "dru", "entity": "肾上腺" }, { "start_idx": 101, "end_idx": 104, "type": "bod", "entity": "GnRH" }, { "start_idx": 108, "end_idx": 113, "type": "bod", "entity": "内源性脑啡肽" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "GnRH" }, { "start_idx": 127, "end_idx": 136, "type": "dru", "entity": "阿片受体拮抗剂纳曲酮" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "Gn" } ] }, { "text": "胎儿12周开始,已有GnRH分泌,促进垂体分泌黄体生成素(LH)和促卵泡刺激素(FSH),胎儿20周时,GnRH分泌达高峰,随即促性腺激素和性激素分泌亦升高。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "GnRH" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "垂体" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "黄体生成素" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "LH" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "促卵泡刺激素" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "FSH" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "GnRH" }, { "start_idx": 64, "end_idx": 77, "type": "sym", "entity": "促性腺激素和性激素分泌亦升高" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "促性腺激素" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "性激素" } ] }, { "text": "此时,因胎盘大量分泌性激素和胎儿中枢神经系统已具备抑制功能,两种调控机制作用使活跃的下丘脑-垂体-性腺轴受到负反馈作用而被抑制。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胎盘" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "性激素" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 42, "end_idx": 51, "type": "bod", "entity": "下丘脑-垂体-性腺轴" } ] }, { "text": "出生后,来自胎盘的性激素中断,负反馈作用减弱,故Gn分泌再度增加中枢神经系统内在的抑制机制和性激素的负反馈作用使下丘脑-垂体-性腺轴保持抑制状态。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "bod", "entity": "来自胎盘的性激素" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "Gn" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "Gn分泌再度增加" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "性激素" }, { "start_idx": 56, "end_idx": 65, "type": "bod", "entity": "下丘脑-垂体-性腺轴" } ] }, { "text": "青春期前,女孩的促卵泡刺激素(FSH)水平高于黄体生成素(LH),女孩的FSH/LH常大于男孩。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "促卵泡刺激素" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "FSH" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "黄体生成素" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "LH" }, { "start_idx": 36, "end_idx": 41, "type": "ite", "entity": "FSH/LH" } ] }, { "text": "无论男女,GnRH注入后LH均呈青春期前反应。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "GnRH" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "LH" } ] }, { "text": "青春发育开始前一年内仅可以见到FSH及LH的24小时分泌量的增加而非分泌频率的增加。", "entities": [ { "start_idx": 15, "end_idx": 31, "type": "sym", "entity": "FSH及LH的24小时分泌量的增加" }, { "start_idx": 15, "end_idx": 28, "type": "ite", "entity": "FSH及LH的24小时分泌量" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "分泌频率的增加" } ] }, { "text": "接近青春期时,中枢神经系统对下丘脑GnRH分泌的抑制作用去除,下丘脑对性激素负反馈的敏感阈逐步上调,即低水平的性激素不足以发挥抑制作用,从而使下丘脑GnRH冲动源激活。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "下丘脑GnRH" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "下丘脑" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "性激素" }, { "start_idx": 51, "end_idx": 57, "type": "sym", "entity": "低水平的性激素" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "性激素" }, { "start_idx": 71, "end_idx": 77, "type": "bod", "entity": "下丘脑GnRH" } ] }, { "text": "GnRH冲动源发生器位于下丘脑中央基底部,下丘脑中央基底部中含有具有转换器作用的GnRH神经元,GnRH神经元可将来自下丘脑的青春发动的神经信号转换为化学信号——GnRH,信号以脉冲式释放,GnRH脉冲式释放的频率和幅度调控垂体Gn的释放。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "GnRH冲动源发生器" }, { "start_idx": 12, "end_idx": 19, "type": "bod", "entity": "下丘脑中央基底部" }, { "start_idx": 21, "end_idx": 28, "type": "bod", "entity": "下丘脑中央基底部" }, { "start_idx": 40, "end_idx": 46, "type": "bod", "entity": "GnRH神经元" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "GnRH神经元" }, { "start_idx": 59, "end_idx": 71, "type": "bod", "entity": "下丘脑的青春发动的神经信号" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "GnRH" }, { "start_idx": 95, "end_idx": 98, "type": "bod", "entity": "GnRH" }, { "start_idx": 112, "end_idx": 115, "type": "bod", "entity": "垂体Gn" } ] }, { "text": "随着GnRH分泌频率和幅度的增多,刺激垂体Gn分泌的频率和幅度也增加性激素的分泌量亦增多GnRH由于分泌量极少,难于检测,但检测Gn的脉冲分泌能间接反映GnRH的脉冲释放情况。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "sym", "entity": "GnRH分泌频率和幅度的增多" }, { "start_idx": 2, "end_idx": 12, "type": "ite", "entity": "GnRH分泌频率和幅度" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "垂体Gn" }, { "start_idx": 19, "end_idx": 33, "type": "sym", "entity": "垂体Gn分泌的频率和幅度也增加" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "性激素" }, { "start_idx": 34, "end_idx": 43, "type": "sym", "entity": "性激素的分泌量亦增多" }, { "start_idx": 44, "end_idx": 54, "type": "sym", "entity": "GnRH由于分泌量极少" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "GnRH" }, { "start_idx": 62, "end_idx": 70, "type": "pro", "entity": "检测Gn的脉冲分泌" }, { "start_idx": 76, "end_idx": 86, "type": "ite", "entity": "GnRH的脉冲释放情况" } ] }, { "text": "青春发育开始时首先可以见到LH夜间脉冲式释放的频度及幅度的增加LH对GnRH注入后的反应增强FSH升高早于LH约一年,且女孩的FSH升高(10~11岁)先于男孩(11~12岁),但GnRH注入后FSH的反应强度与青春期前比较无显著改变。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "LH" }, { "start_idx": 13, "end_idx": 30, "type": "sym", "entity": "LH夜间脉冲式释放的频度及幅度的增加" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "LH" }, { "start_idx": 31, "end_idx": 45, "type": "sym", "entity": "LH对GnRH注入后的反应增强" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "GnRH" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "FSH" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "LH" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "FSH" }, { "start_idx": 63, "end_idx": 67, "type": "sym", "entity": "FSH升高" }, { "start_idx": 90, "end_idx": 93, "type": "bod", "entity": "GnRH" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "FSH" } ] }, { "text": "故青春期GnRH脉冲式释放频率的增加使LH/FSH的比值增加,LH/FSH的比值乳房增大睾丸增大出现阴毛、月经和阴茎的增大乳房增大月经初潮平均历时2~2.5年。", "entities": [ { "start_idx": 4, "end_idx": 17, "type": "sym", "entity": "GnRH脉冲式释放频率的增加" }, { "start_idx": 4, "end_idx": 14, "type": "ite", "entity": "GnRH脉冲式释放频率" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "LH/FSH的比值增加" }, { "start_idx": 19, "end_idx": 27, "type": "ite", "entity": "LH/FSH的比值" }, { "start_idx": 31, "end_idx": 39, "type": "ite", "entity": "LH/FSH的比值" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "乳房" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "乳房增大" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "睾丸" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "睾丸增大" }, { "start_idx": 48, "end_idx": 60, "type": "sym", "entity": "出现阴毛、月经和阴茎的增大" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "阴毛" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "阴茎" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "乳房" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "乳房增大" } ] }, { "text": "男孩从睾丸增大遗精出现平均历时3年。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "睾丸" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "睾丸增大" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "遗精" } ] }, { "text": "性早熟儿童体格发育虽然发生巨大变化,但心理、认知能力和社会心理(社会适应)仍处在儿童期。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "性早熟" }, { "start_idx": 5, "end_idx": 42, "type": "sym", "entity": "体格发育虽然发生巨大变化,但心理、认知能力和社会心理(社会适应)仍处在儿童期" } ] }, { "text": "伴随青春发育女性子宫卵巢的变化(表15-15)。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "子宫卵巢" } ] }, { "text": "不同年龄性别的血清FSH/LH的正常值及LHRH激发后值(表15-15和表15-15)。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "ite", "entity": "血清FSH/LH" }, { "start_idx": 20, "end_idx": 27, "type": "ite", "entity": "LHRH激发后值" } ] }, { "text": "不同年龄性别的血清E2/T的正常值(表15-15)。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "血清E2/T" } ] }, { "text": "中枢性性早熟儿童体内激素的改变类似于正常青春期开始时的改变。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "体内激素" } ] }, { "text": "LH脉冲式释放增加、LH对GnRH注入后的反应增强血中性激素水平上升子宫及卵巢B超大小及形态注:子宫容积=长×宽×高×0.52卵巢容积=长×宽2×0.5表15-10不同年龄男孩LHRH激发实验中FSH、LH的基础值和峰值(U/L)表15-11不同年龄女孩LHRH激发实验中FSH及LH的基础值和峰值(U/L)表15-12不同年龄血清性激素正常值(三)儿童中枢性性早熟的发病机制儿童中枢性性早熟的发病机制较复杂,与神经内分泌功能密切相关。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "LH脉冲式释放增加" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "LH" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "LH" }, { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "LH对GnRH注入后的反应增强" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "GnRH" }, { "start_idx": 25, "end_idx": 29, "type": "ite", "entity": "血中性激素" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "血中性激素水平上升" }, { "start_idx": 34, "end_idx": 40, "type": "pro", "entity": "子宫及卵巢B超" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "子宫" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "卵巢" }, { "start_idx": 94, "end_idx": 101, "type": "pro", "entity": "LHRH激发实验" }, { "start_idx": 103, "end_idx": 105, "type": "ite", "entity": "FSH" }, { "start_idx": 107, "end_idx": 108, "type": "ite", "entity": "LH" }, { "start_idx": 117, "end_idx": 119, "type": "ite", "entity": "U/L" }, { "start_idx": 133, "end_idx": 140, "type": "pro", "entity": "LHRH激发实验" }, { "start_idx": 142, "end_idx": 144, "type": "ite", "entity": "FSH" }, { "start_idx": 146, "end_idx": 147, "type": "ite", "entity": "LH" }, { "start_idx": 156, "end_idx": 158, "type": "ite", "entity": "U/L" }, { "start_idx": 170, "end_idx": 174, "type": "ite", "entity": "血清性激素" }, { "start_idx": 181, "end_idx": 188, "type": "dis", "entity": "儿童中枢性性早熟" }, { "start_idx": 194, "end_idx": 201, "type": "dis", "entity": "儿童中枢性性早熟" } ] }, { "text": "下丘脑GnRH脉冲频率与幅度增加下丘脑-垂体-性腺轴提前兴奋,GnRH脉冲释放明显增强而致中枢性性早熟。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "下丘脑GnRH脉冲频率与幅度增加" }, { "start_idx": 0, "end_idx": 13, "type": "ite", "entity": "下丘脑GnRH脉冲频率与幅度" }, { "start_idx": 16, "end_idx": 25, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 16, "end_idx": 29, "type": "sym", "entity": "下丘脑-垂体-性腺轴提前兴奋" }, { "start_idx": 31, "end_idx": 42, "type": "sym", "entity": "GnRH脉冲释放明显增强" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "GnRH" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "中枢性性早熟" } ] }, { "text": "此外,中枢神经系统的器质性病变也会直接扰乱GnRH脉冲发生器的调控机制而致病。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "中枢神经系统的器质性病变" }, { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 19, "end_idx": 34, "type": "sym", "entity": "扰乱GnRH脉冲发生器的调控机制" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "GnRH脉冲发生器" } ] }, { "text": "除遗传因素以外,性早熟的发生还涉及环境(包括社会、经济及营养)等因素。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "性早熟" } ] }, { "text": "此外,环境雌激素污染问题可能与此也相关,即一些非甾体类激素样物质影响相关激素受体的敏感性,由此干扰性腺功能。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "环境雌激素污染" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "雌激素" }, { "start_idx": 23, "end_idx": 28, "type": "dru", "entity": "非甾体类激素" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "激素受体" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "干扰性腺功能" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "性腺" } ] }, { "text": "根据性早熟的发病机制和病因,可将之分为中枢性性早熟和外周性性早熟:1.中枢性性早熟(centralprecociouspuberty,CPP)亦称完全性或真性性早熟,是指由于下丘脑-垂体-性腺轴功能提前激活,导致性腺发育及功能成熟,与正常青春发育成熟机制完全一致,并可具有一定的生育能力。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "性早熟" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "外周性性早熟" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 42, "end_idx": 65, "type": "dis", "entity": "centralprecociouspuberty" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "CPP" }, { "start_idx": 73, "end_idx": 81, "type": "dis", "entity": "完全性或真性性早熟" }, { "start_idx": 87, "end_idx": 96, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 87, "end_idx": 102, "type": "sym", "entity": "下丘脑-垂体-性腺轴功能提前激活" }, { "start_idx": 106, "end_idx": 107, "type": "bod", "entity": "性腺" } ] }, { "text": "中枢性性早熟主要包括继发于中枢神经系统各种器质性病变和特发性性早熟两大类。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 13, "end_idx": 25, "type": "sym", "entity": "中枢神经系统各种器质性病变" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "特发性性早熟" } ] }, { "text": "特发性性早熟是指经检查未发现患儿提前启动青春发育器质性病因的性早熟。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "特发性性早熟" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "提前启动青春发育" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "性早熟" } ] }, { "text": "此类型以女孩居多(约占女孩CPP的80%~90%),亦是CPP中最常见病因;继发性性早熟以男孩居多,约占男孩性早熟的60%。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "CPP" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "CPP" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "继发性性早熟" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "性早熟" } ] }, { "text": "2.外周性性早熟(peripheralprecociouspuberty)亦称部分性性早熟或假性性早熟,是非受控于下丘脑-垂体-性腺轴功能所致的性发育,有性激素水平的升高性早熟的主要病因(表15-15)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "外周性性早熟" }, { "start_idx": 9, "end_idx": 35, "type": "dis", "entity": "peripheralprecociouspuberty" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "部分性性早熟" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "假性性早熟" }, { "start_idx": 57, "end_idx": 66, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "性激素" }, { "start_idx": 77, "end_idx": 84, "type": "sym", "entity": "性激素水平的升高" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "性早熟" } ] }, { "text": "在中枢性性早熟中,女性患儿约80%~90%属于特发性性早熟,男性患儿则相反,仅占约30%,多数为器质性病变所致的继发性性早熟,故对男孩中枢性性早熟尤应注意探查原发疾病。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "特发性性早熟" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "器质性病变" }, { "start_idx": 56, "end_idx": 61, "type": "dis", "entity": "继发性性早熟" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "中枢性性早熟" } ] }, { "text": "表15-13常见性早熟的病因【临床表现】一般中枢性性早熟的临床特征与正常青春发育程序相似,但临床变异较大,症状发展快慢不一。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "性早熟" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "中枢性性早熟" } ] }, { "text": "女孩首先表现为乳房发育乳头增大乳晕增大大、小阴唇增大色素沉着,阴道出现白色分泌物阴道黏膜细胞出现雌激素依赖性改变子宫及卵巢增大可有成熟性排卵和月经。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "乳房" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "乳房发育" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "乳头" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "乳头增大" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "乳晕" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "乳晕增大" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "大、小阴唇" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "大、小阴唇增大色素沉着" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "阴道" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "阴道出现白色分泌物" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "白色分泌物" }, { "start_idx": 40, "end_idx": 45, "type": "bod", "entity": "阴道黏膜细胞" }, { "start_idx": 40, "end_idx": 55, "type": "sym", "entity": "阴道黏膜细胞出现雌激素依赖性改变" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "雌激素" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "子宫" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "子宫及卵巢增大" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "卵巢" }, { "start_idx": 64, "end_idx": 72, "type": "sym", "entity": "有成熟性排卵和月经" } ] }, { "text": "男孩首先表现为睾丸增大≥4ml容积),阴囊皮肤皱褶增加色素加深,阴茎增长增粗阴毛、腋毛及胡须生长声音变低沉;精子生成肌肉容量增加皮下脂肪减少生长加速,骨成熟加速,骨龄提前,可造成终身高低于靶身高,影响终身高。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "睾丸" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "睾丸增大≥4ml容积" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "阴囊皮肤" }, { "start_idx": 19, "end_idx": 30, "type": "sym", "entity": "阴囊皮肤皱褶增加色素加深" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴茎" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "阴茎增长增粗" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "阴毛" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "阴毛、腋毛及胡须生长" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "腋毛" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "胡须" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "声音变低沉" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "精子" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "精子生成" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "肌肉" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "肌肉容量增加" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "皮下脂肪" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "皮下脂肪减少" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "生长加速" }, { "start_idx": 75, "end_idx": 79, "type": "sym", "entity": "骨成熟加速" }, { "start_idx": 75, "end_idx": 75, "type": "bod", "entity": "骨" }, { "start_idx": 81, "end_idx": 84, "type": "sym", "entity": "骨龄提前" }, { "start_idx": 89, "end_idx": 96, "type": "sym", "entity": "终身高低于靶身高" }, { "start_idx": 98, "end_idx": 102, "type": "sym", "entity": "影响终身高" } ] }, { "text": "松果体部位的肿瘤中约有半数是生殖细胞瘤或星形细胞瘤,其余为由组织学类型不同的肿瘤。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "松果体部位的肿瘤" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "生殖细胞瘤" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "星形细胞瘤" }, { "start_idx": 30, "end_idx": 39, "type": "dis", "entity": "组织学类型不同的肿瘤" } ] }, { "text": "临床表现除性早熟外无其他体征。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "性早熟" } ] }, { "text": "神经内分泌表现常在放射学上发现肿瘤前1~2年即出现。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "放射学" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肿瘤" } ] }, { "text": "下丘脑的症状或体征,如尿崩症、渴感缺乏、高热、不正常的哭笑(痴笑样癫痫)、肥胖以及恶液质,均提示可能有颅内病变。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "下丘脑" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "尿崩症" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "渴感缺乏" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "高热" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "不正常的哭笑" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "痴笑样癫痫" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "肥胖" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "恶液质" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "颅内病变" } ] }, { "text": "视力及视野缺损视神经胶质瘤最早的临床表现,可有颅压增高、头痛及呕吐等神经系统症状和体征。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "视力及视野缺损" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "视神经胶质瘤" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "颅压增高" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "颅压" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "头痛" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "头" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "呕吐" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "神经系统症状和体征" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "神经系统" } ] }, { "text": "外周性性早熟临床表现有第二性征出现,但非青春期发动,一般无性腺增大下丘脑-垂体-性腺轴的活动无关,而与内源性或者外源性性激素水平升高实验室.内分泌激素检查包括测定FSH、LH、雌二醇、睾酮及17-羟孕酮基础值。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "外周性性早熟" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "性腺" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "性腺增大" }, { "start_idx": 33, "end_idx": 42, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 51, "end_idx": 65, "type": "sym", "entity": "内源性或者外源性性激素水平升高" }, { "start_idx": 51, "end_idx": 63, "type": "ite", "entity": "内源性或者外源性性激素水平" }, { "start_idx": 66, "end_idx": 76, "type": "pro", "entity": "实验室.内分泌激素检查" }, { "start_idx": 79, "end_idx": 103, "type": "pro", "entity": "测定FSH、LH、雌二醇、睾酮及17-羟孕酮基础值" } ] }, { "text": "如果第二性征已达青春中期程度时,血清促黄体生成素(LH)基础值可作为初筛,如>5.0IU/L,即可确定其性腺轴已发动,不必再进行促性腺激素释放激素激发试验。", "entities": [ { "start_idx": 16, "end_idx": 30, "type": "ite", "entity": "血清促黄体生成素(LH)基础值" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "性腺轴" }, { "start_idx": 64, "end_idx": 76, "type": "pro", "entity": "促性腺激素释放激素激发试验" } ] }, { "text": "2.促性腺激素释放激素(GnRH)兴奋试验亦称LHRH兴奋试验,其原理是通过GnRH刺激垂体分泌LH和FSH,从而评价垂体促性腺激素细胞储备功能,本试验对性腺轴功能已启动而促性腺激素基础值不升高者是重要的诊断手段,对鉴别中枢性与外周性性早熟具有重要意义。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "pro", "entity": "促性腺激素释放激素(GnRH)兴奋试验" }, { "start_idx": 23, "end_idx": 30, "type": "pro", "entity": "LHRH兴奋试验" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "GnRH" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "垂体" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "LH" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "FSH" }, { "start_idx": 59, "end_idx": 67, "type": "bod", "entity": "垂体促性腺激素细胞" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "性腺轴" }, { "start_idx": 86, "end_idx": 96, "type": "sym", "entity": "促性腺激素基础值不升高" }, { "start_idx": 86, "end_idx": 93, "type": "ite", "entity": "促性腺激素基础值" }, { "start_idx": 110, "end_idx": 119, "type": "dis", "entity": "中枢性与外周性性早熟" } ] }, { "text": "一般采用静脉内注射LHRH(戈那瑞林),按2.5μg/kg(最大剂量≤100μg),于注射0min、30min、60min时采血检测血清LH和FSH。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "静脉内注射" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "LHRH" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "戈那瑞林" }, { "start_idx": 43, "end_idx": 73, "type": "pro", "entity": "注射0min、30min、60min时采血检测血清LH和FSH" } ] }, { "text": "正常青春期或真性性早熟者,LH峰值出现时间在15~30分钟。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "真性性早熟" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "LH" } ] }, { "text": "LH峰值>5.0IU/L及LH峰/FSH峰>0.6可认为其性腺轴功能已经启动。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "LH" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "LH" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "FSH" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "性腺轴" } ] }, { "text": "3.骨龄测定可拍摄左手和腕部X线正位片,骨龄超过实际年龄1岁以上可视为提前,发育越早,则骨龄超前越多。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "骨龄测定" }, { "start_idx": 9, "end_idx": 18, "type": "pro", "entity": "左手和腕部X线正位片" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "骨龄超前" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "骨" } ] }, { "text": "4.B超检查子宫、卵巢及睾丸B超可观察子宫卵巢大小、卵巢内卵泡数目和大小、卵巢有无囊肿和肿瘤以及睾丸有无肿瘤等。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "pro", "entity": "B超检查子宫、卵巢及睾丸" }, { "start_idx": 14, "end_idx": 54, "type": "pro", "entity": "B超可观察子宫卵巢大小、卵巢内卵泡数目和大小、卵巢有无囊肿和肿瘤以及睾丸有无肿瘤等" } ] }, { "text": "5.头颅MRI检查对确诊中枢性性早熟的小年龄女孩和所有男孩应作头颅MRI检查,以排除颅内占位性病变。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "头颅MRI检查" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "头颅MRI检查" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "颅内占位性病变" } ] }, { "text": "【鉴别诊断】(一)单纯性乳房早发育单纯性乳房早发育(prematurethelarche)是女孩不完全性性早熟的特殊表现,起病年龄小,常<2岁,乳腺仅轻度发育,常呈现周期性变化,不伴生长加速和骨龄提前,血清E2和FSH的基础值常有轻度增高,GnRH兴奋试验中FSH峰值增高LH不增高乳房发育过早真性或假性性早熟的第一特征,或可能由外源性雌激素所引起。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "单纯性乳房早发育" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "乳房" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "单纯性乳房早发育" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "乳房" }, { "start_idx": 26, "end_idx": 43, "type": "sym", "entity": "prematurethelarche" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "不完全性性早熟" }, { "start_idx": 72, "end_idx": 87, "type": "sym", "entity": "乳腺仅轻度发育,常呈现周期性变化" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "乳腺" }, { "start_idx": 91, "end_idx": 94, "type": "sym", "entity": "生长加速" }, { "start_idx": 96, "end_idx": 99, "type": "sym", "entity": "骨龄提前" }, { "start_idx": 101, "end_idx": 124, "type": "sym", "entity": "血清E2和FSH的基础值常有轻度增高" }, { "start_idx": 101, "end_idx": 110, "type": "bod", "entity": "血清E2" }, { "start_idx": 112, "end_idx": 114, "type": "bod", "entity": "FSH" }, { "start_idx": 126, "end_idx": 133, "type": "pro", "entity": "GnRH兴奋试验" }, { "start_idx": 135, "end_idx": 141, "type": "sym", "entity": "FSH峰值增高" }, { "start_idx": 135, "end_idx": 137, "type": "bod", "entity": "FSH" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "LH" }, { "start_idx": 147, "end_idx": 152, "type": "sym", "entity": "乳房发育过早" }, { "start_idx": 147, "end_idx": 148, "type": "bod", "entity": "乳房" }, { "start_idx": 153, "end_idx": 160, "type": "dis", "entity": "真性或假性性早熟" } ] }, { "text": "由于本病小部分患者可逐步演变为真性性早熟,故应重视随访,观察女孩乳房早发育的发展过程,争取及时介入治疗。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "真性性早熟" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "乳房早发育" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "乳房" } ] }, { "text": "(二)单纯性阴毛早发育单纯性阴毛早发育(prematurepubarche)属不完全性性早熟的特殊类型,两性均可发病。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "单纯性阴毛早发育" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "阴毛" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "单纯性阴毛早发育" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "阴毛" }, { "start_idx": 20, "end_idx": 36, "type": "sym", "entity": "prematurepubarche" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "不完全性性早熟" } ] }, { "text": "好发于6岁左右,除阴毛外可伴有腋毛发育无其他副性征出现,无性腺发育不发生男性化。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "除阴毛外可伴有腋毛发育" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "阴毛" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "腋毛" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "其他副性征出现" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "性腺发育" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "性腺" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "发生男性化" } ] }, { "text": "部分患儿可有轻度生长加速和骨龄提前,常有家族史。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "轻度生长加速" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "骨龄提前" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "骨" } ] }, { "text": "可能与肾上腺功能早现(adrenarche)以及过早分泌大量雄激素有关。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "肾上腺功能早现" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肾上腺" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "adrenarche" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "过早分泌大量雄激素" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "雄激素" } ] }, { "text": "(三)月经早潮月经早潮(prematuremenarche)为单独发生月经而无其他性早熟表现,大多数女孩仅为1~3次阴道出血促性腺激素正常。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "月经早潮" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "月经早潮" }, { "start_idx": 12, "end_idx": 28, "type": "sym", "entity": "prematuremenarche" }, { "start_idx": 31, "end_idx": 45, "type": "sym", "entity": "单独发生月经而无其他性早熟表现" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "性早熟" }, { "start_idx": 52, "end_idx": 61, "type": "sym", "entity": "仅为1~3次阴道出血" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "阴道" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "促性腺激素" } ] }, { "text": "可能由于卵巢活动引起E2分泌,卵巢B超有时可发现滤泡囊肿。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "卵巢" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "E2" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "卵巢B超" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "滤泡囊肿" } ] }, { "text": "(四)McCune-Albright综合征本症是由于G蛋白α-亚基基因突变,刺激cAMP分泌增加内分泌激素受体,例如FSH及LH受体,有时包括ACTH及TSH受体。", "entities": [ { "start_idx": 3, "end_idx": 20, "type": "dis", "entity": "McCune-Albright综合征" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "G蛋白α-亚基基因突变" }, { "start_idx": 26, "end_idx": 34, "type": "bod", "entity": "G蛋白α-亚基基因" }, { "start_idx": 38, "end_idx": 54, "type": "sym", "entity": "刺激cAMP分泌增加内分泌激素受体" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "cAMP" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "内分泌激素受体" }, { "start_idx": 58, "end_idx": 65, "type": "bod", "entity": "FSH及LH受体" }, { "start_idx": 71, "end_idx": 80, "type": "bod", "entity": "ACTH及TSH受体" } ] }, { "text": "患儿除性早熟征象外,尚伴有皮肤咖啡色素斑骨纤维发育不良卵巢囊肿少数患儿可能同时伴有甲状腺功能亢进或Cushing综合征中枢性性早熟的治疗目的是:①控制或减缓第二性征发育,延迟性成熟过程;②抑制性激素引起的骨成熟,防止骨骺早闭而致成人期矮身材;③同步进行适当的心理和行为指导,从而达到保证儿童理想生长发育的目的。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "性早熟" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "皮肤咖啡色素斑" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "骨纤维发育不良" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "卵巢囊肿" }, { "start_idx": 37, "end_idx": 58, "type": "sym", "entity": "同时伴有甲状腺功能亢进或Cushing综合征" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "甲状腺功能亢进" }, { "start_idx": 49, "end_idx": 58, "type": "dis", "entity": "Cushing综合征" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "性激素" }, { "start_idx": 102, "end_idx": 102, "type": "bod", "entity": "骨" }, { "start_idx": 108, "end_idx": 111, "type": "sym", "entity": "骨骺早闭" }, { "start_idx": 108, "end_idx": 109, "type": "bod", "entity": "骨骺" }, { "start_idx": 117, "end_idx": 119, "type": "sym", "entity": "矮身材" }, { "start_idx": 126, "end_idx": 135, "type": "pro", "entity": "适当的心理和行为指导" } ] }, { "text": "但是并非所有的中枢性性早熟都需要治疗。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "中枢性性早熟" } ] }, { "text": "本病治疗应依据病因而定,下丘脑错构瘤一般不需要神经外科的介入。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "下丘脑错构瘤" }, { "start_idx": 23, "end_idx": 26, "type": "dep", "entity": "神经外科" } ] }, { "text": "对于神经系统的其他病变,治疗应取决于病变的性质和部位进行手术摘除,或化疗及放疗。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "神经系统" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "手术摘除" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "化疗" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "放疗" } ] }, { "text": "无论病因如何,在导致中枢性性早熟的器质性脑部病变患儿中,应用GnRH类似物治疗与特发性性早熟患儿中的治疗同样有效。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "器质性脑部病变" }, { "start_idx": 30, "end_idx": 38, "type": "pro", "entity": "GnRH类似物治疗" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "特发性性早熟" } ] }, { "text": "甲状腺功能减退者给予甲状腺激素补充治疗;先天性肾上腺皮质功能增生者采用皮质激素制剂治疗。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "甲状腺功能减退" }, { "start_idx": 10, "end_idx": 18, "type": "pro", "entity": "甲状腺激素补充治疗" }, { "start_idx": 20, "end_idx": 31, "type": "dis", "entity": "先天性肾上腺皮质功能增生" }, { "start_idx": 35, "end_idx": 42, "type": "pro", "entity": "皮质激素制剂治疗" } ] }, { "text": "1.促性腺激素释放激素类似物(GnRHa)此类药物是将GnRH分子结构中第6位甘氨酸换成D-色氨酸(达菲林和达必佳)及D-亮氨酸(抑那通)等的长效合成激素,其作用原理是利用下丘脑激素类似物竞争性抑制自身分泌的GnRH,减少垂体促性腺激素分泌。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dru", "entity": "促性腺激素释放激素类似物" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "GnRHa" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "GnRH" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "甘氨酸" }, { "start_idx": 44, "end_idx": 48, "type": "dru", "entity": "D-色氨酸" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "达菲林" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "达必佳" }, { "start_idx": 59, "end_idx": 63, "type": "dru", "entity": "D-亮氨酸" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "抑那通" }, { "start_idx": 71, "end_idx": 76, "type": "dru", "entity": "长效合成激素" }, { "start_idx": 86, "end_idx": 93, "type": "dru", "entity": "下丘脑激素类似物" }, { "start_idx": 104, "end_idx": 107, "type": "bod", "entity": "GnRH" }, { "start_idx": 111, "end_idx": 117, "type": "bod", "entity": "垂体促性腺激素" } ] }, { "text": "可按100~120μg/kg用药,每4周肌注一次。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "肌注" } ] }, { "text": "治疗后LH及FSH的分泌下降性征退缩甚至恢复到青春期前水平,骨骼发育减慢GnRHa治疗的适应证女孩要≤11.5岁,男孩≤12.5岁。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "LH及FSH的分泌下降" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "LH" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "FSH" }, { "start_idx": 14, "end_idx": 28, "type": "sym", "entity": "性征退缩甚至恢复到青春期前水平" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "骨骼发育减慢" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "骨骼" }, { "start_idx": 36, "end_idx": 42, "type": "pro", "entity": "GnRHa治疗" } ] }, { "text": "对那些进展缓慢型的特发性性早熟进行密切随访的基础上进一步决定是否需要治疗。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "特发性性早熟" } ] }, { "text": "2.达那唑有抗孕激素和雌激素作用,其作用机制是反馈抑制下丘脑垂体促性腺激素分泌,使体内雌激素水平下降有时见声音粗、毛发增多以及出现粉刺甲羟孕酮(又称安宫黄体酮)由于副作用较大,已不再用于治疗性早熟。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "达那唑" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "孕激素" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "雌激素" }, { "start_idx": 27, "end_idx": 36, "type": "bod", "entity": "下丘脑垂体促性腺激素" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "雌激素水平下降" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "雌激素" }, { "start_idx": 50, "end_idx": 66, "type": "sym", "entity": "有时见声音粗、毛发增多以及出现粉刺" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "毛发" }, { "start_idx": 67, "end_idx": 70, "type": "dru", "entity": "甲羟孕酮" }, { "start_idx": 74, "end_idx": 78, "type": "dru", "entity": "安宫黄体酮" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "性早熟" } ] }, { "text": "五、立克次体国内由立克次体引起的主要疾病是斑疹伤寒、恙虫病和Q热,国外尚有洛矶山斑疹热、地中海斑疹热以及其他一些疾病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "立克次体" }, { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "立克次体" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "斑疹伤寒" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "恙虫病" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "Q热" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "洛矶山斑疹热" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "地中海斑疹热" } ] }, { "text": "立克次体病的病原学诊断主要靠血清抗体的检测,因为立克次体的分离培养比较繁琐,而且有造成实验室传播的危险。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "立克次体病" }, { "start_idx": 14, "end_idx": 20, "type": "ite", "entity": "血清抗体的检测" }, { "start_idx": 24, "end_idx": 27, "type": "mic", "entity": "立克次体" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "分离培养" } ] }, { "text": "血清学试验中,国内外至今最普遍使用的方法仍是变形杆菌凝集试验(即外裴试验)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "血清学试验" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "变形杆菌凝集试验" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "外裴试验" } ] }, { "text": "但变形杆菌凝集试验是一种非特异的试验,在钩端螺旋体病、布鲁菌病、包柔螺旋体感染(莱姆病)、伤寒以及严重肝病和偶于妊娠时也可呈现阳性结果。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "pro", "entity": "变形杆菌凝集试验" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "钩端螺旋体病" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "布鲁菌病" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "包柔螺旋体感染" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "莱姆病" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "伤寒" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "严重肝病" } ] }, { "text": "因为特异性IgG抗体或IgM抗体一般在发病后6天左右出现或明显升高,这时其他试验如变形杆菌凝集试验也可呈现阳性。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "bod", "entity": "特异性IgG抗体或IgM抗体" }, { "start_idx": 41, "end_idx": 54, "type": "sym", "entity": "变形杆菌凝集试验也可呈现阳性" }, { "start_idx": 41, "end_idx": 48, "type": "pro", "entity": "变形杆菌凝集试验" } ] }, { "text": "其他方法,如补体结合试验和立克次体微量凝集试验虽特异性强,但敏感性低;间接血凝和乳胶颗粒凝集试验特异性和敏感性都高,适合于急性感染的诊断,但不适合于血清流行病学的检测。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "补体结合试验" }, { "start_idx": 13, "end_idx": 22, "type": "pro", "entity": "立克次体微量凝集试验" }, { "start_idx": 35, "end_idx": 47, "type": "pro", "entity": "间接血凝和乳胶颗粒凝集试验" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "急性感染" }, { "start_idx": 74, "end_idx": 82, "type": "pro", "entity": "血清流行病学的检测" } ] }, { "text": "最近几年内有人开发出了检测主要几种立克次体(包括洛矶山斑疹热、地中海斑疹热和斑疹伤寒的病原体)共同的特异性DNA序列的PCR方法。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "立克次体" }, { "start_idx": 24, "end_idx": 45, "type": "mic", "entity": "洛矶山斑疹热、地中海斑疹热和斑疹伤寒的病原体" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "DNA" }, { "start_idx": 59, "end_idx": 63, "type": "pro", "entity": "PCR方法" } ] }, { "text": "其敏感度达到了能够检出单个立克次体的程度。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "立克次体" } ] }, { "text": "该方法所用的检测时间不足48小时,因此有利于立克次体病的早期诊断和及时治疗。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "立克次体病" } ] }, { "text": "二、肺动脉吊带肺动脉吊带亦称左肺动脉异常,由Glaevecke和Doehle1897年首次报道近50%患儿伴发动脉导管未闭和左上腔静脉残存。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺动脉吊带" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肺动脉吊带" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "左肺动脉异常" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "左上腔静脉残存" } ] }, { "text": "【病理解剖】在胚胎发育期,左侧第Ⅵ腮弓近端退化或发育不良,导致其与左肺连接中断,致使在右肺动脉橫部与左肺动脉间形成“侧支血管”,该血管跨越右主支气管转向左侧,在气管与食管之间进入左侧肺门,形成包绕右主支气管与气管下段的悬带,产生压迫症状,可伴有气管下段和右主支气管发育不良。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "左侧第Ⅵ腮弓" }, { "start_idx": 13, "end_idx": 38, "type": "sym", "entity": "左侧第Ⅵ腮弓近端退化或发育不良,导致其与左肺连接中断" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "左肺" }, { "start_idx": 43, "end_idx": 48, "type": "bod", "entity": "右肺动脉橫部" }, { "start_idx": 43, "end_idx": 62, "type": "sym", "entity": "右肺动脉橫部与左肺动脉间形成“侧支血管”" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "血管" }, { "start_idx": 69, "end_idx": 73, "type": "bod", "entity": "右主支气管" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "气管" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "食管" }, { "start_idx": 89, "end_idx": 92, "type": "bod", "entity": "左侧肺门" }, { "start_idx": 94, "end_idx": 110, "type": "sym", "entity": "形成包绕右主支气管与气管下段的悬带" }, { "start_idx": 98, "end_idx": 102, "type": "bod", "entity": "右主支气管" }, { "start_idx": 104, "end_idx": 107, "type": "bod", "entity": "气管下段" }, { "start_idx": 114, "end_idx": 115, "type": "sym", "entity": "压迫" }, { "start_idx": 122, "end_idx": 135, "type": "sym", "entity": "气管下段和右主支气管发育不良" }, { "start_idx": 122, "end_idx": 125, "type": "bod", "entity": "气管下段" }, { "start_idx": 127, "end_idx": 131, "type": "bod", "entity": "右主支气管" } ] }, { "text": "【临床表现】主要症状是由于血管压迫后压迫处或压迫处远端气管、支气管发育不良或狭窄引起。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "血管压迫" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血管" }, { "start_idx": 18, "end_idx": 39, "type": "sym", "entity": "压迫处或压迫处远端气管、支气管发育不良或狭窄" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "气管" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "支气管" } ] }, { "text": "【辅助检查】胸部X线检查可发现两侧肺透光度不同,右侧肺过度充气。", "entities": [ { "start_idx": 6, "end_idx": 30, "type": "sym", "entity": "胸部X线检查可发现两侧肺透光度不同,右侧肺过度充气" }, { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "两侧肺" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "右侧肺" } ] }, { "text": "但检查可造成呼吸道梗阻加重。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "呼吸道梗阻" } ] }, { "text": "胸部X线透视检查可发现两侧气管不等粗。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "胸部X线透视检查" }, { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "胸部X线透视检查可发现两侧气管不等粗" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "两侧气管" } ] }, { "text": "如无伴发其他心血管畸形时,不必进行心血管造影检查,正位向头投照肺动脉造影可清晰显示左肺动脉形态及走行。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "心血管畸形" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "心血管造影检查" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "肺动脉造影" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "左肺动脉" } ] }, { "text": "【治疗】异位迷走左肺动脉的治疗方法是经左胸侧后第3或第4肋间切口进胸,游离气管、食管、肺门和左肺动脉,切断结扎动脉韧带后,左支气管后方解剖游离左肺动脉,在靠近右肺动脉起源处切断左肺动脉,缝合右肺动脉切口,将左肺动脉从气管后方拉出,在气管左前方与肺总动脉作对端吻合术。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "异位迷走左肺动脉" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 18, "end_idx": 33, "type": "pro", "entity": "经左胸侧后第3或第4肋间切口进胸" }, { "start_idx": 35, "end_idx": 49, "type": "pro", "entity": "游离气管、食管、肺门和左肺动脉" }, { "start_idx": 51, "end_idx": 59, "type": "pro", "entity": "切断结扎动脉韧带后" }, { "start_idx": 61, "end_idx": 74, "type": "pro", "entity": "左支气管后方解剖游离左肺动脉" }, { "start_idx": 76, "end_idx": 91, "type": "pro", "entity": "在靠近右肺动脉起源处切断左肺动脉" }, { "start_idx": 93, "end_idx": 100, "type": "pro", "entity": "缝合右肺动脉切口" }, { "start_idx": 102, "end_idx": 113, "type": "pro", "entity": "将左肺动脉从气管后方拉出" }, { "start_idx": 115, "end_idx": 131, "type": "pro", "entity": "在气管左前方与肺总动脉作对端吻合术" } ] }, { "text": "术后处理:主动脉弓及其分支畸形病例,由于受压迫的气管软骨环发育不良且较软弱,易于吸气时萎陷,因此术后数日仍需持续气管插管加压,给予高湿度氧吸入;吸除气道分泌物,保证呼吸道通畅。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dis", "entity": "主动脉弓及其分支畸形" }, { "start_idx": 20, "end_idx": 44, "type": "sym", "entity": "受压迫的气管软骨环发育不良且较软弱,易于吸气时萎陷" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "气管软骨环" }, { "start_idx": 54, "end_idx": 61, "type": "pro", "entity": "持续气管插管加压" }, { "start_idx": 63, "end_idx": 70, "type": "pro", "entity": "给予高湿度氧吸入" }, { "start_idx": 72, "end_idx": 78, "type": "pro", "entity": "吸除气道分泌物" }, { "start_idx": 80, "end_idx": 86, "type": "pro", "entity": "保证呼吸道通畅" } ] }, { "text": "有时术后经数周或数月气管和食管压迫症状才完全消失。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "气管和食管压迫症状" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "气管" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "食管" } ] }, { "text": "四、蔓状血管瘤较少见,为包含有小动脉和小静脉吻合的血管瘤,好发于头皮或肢端。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "蔓状血管瘤" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "小动脉" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "小静脉" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "血管瘤" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "头皮" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肢端" } ] }, { "text": "皮下可及血管迂曲,有血管搏动,皮肤温度增高,听诊可闻及血管杂音。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "皮下可及血管迂曲" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮下" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "血管" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "有血管搏动" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血管" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "皮肤温度增高" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "皮肤温度" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "听诊可闻及血管杂音" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "听诊" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血管" } ] }, { "text": "这种血管瘤不会自动消失,故应早期治疗。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "血管瘤" } ] }, { "text": "对局限性蔓状血管瘤(hemangiomaracemosum),唯有手术治疗。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "局限性蔓状血管瘤" }, { "start_idx": 10, "end_idx": 28, "type": "dis", "entity": "hemangiomaracemosum" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "手术治疗" } ] }, { "text": "第三节遗尿症遗尿症(enuresis)又称非器质性遗尿症或功能性遗尿症,通常系指儿童5岁后仍不自主地排尿而尿湿了裤子或床铺,但无明显的器质性病因。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "遗尿症" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "遗尿症" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "非器质性遗尿症" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "功能性遗尿症" }, { "start_idx": 41, "end_idx": 61, "type": "sym", "entity": "儿童5岁后仍不自主地排尿而尿湿了裤子或床铺" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "尿" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "尿" } ] }, { "text": "遗尿症有两种分类的方法。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "遗尿症" } ] }, { "text": "第一种分类是根据遗尿发生的时间而定,当儿童遗尿发生在睡眠中(包括夜间睡眠和午睡),但白天能控制排尿,而且膀胱功能正常,则称为单一症状的夜间遗尿,而当小儿白天清醒时有遗尿,但无神经系统的病变诸如脊柱裂、脊柱损伤等,则称为白日遗尿。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "遗尿" }, { "start_idx": 19, "end_idx": 39, "type": "sym", "entity": "儿童遗尿发生在睡眠中(包括夜间睡眠和午睡)" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "白天能控制排尿" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "膀胱功能正常" }, { "start_idx": 62, "end_idx": 70, "type": "dis", "entity": "单一症状的夜间遗尿" }, { "start_idx": 74, "end_idx": 83, "type": "sym", "entity": "小儿白天清醒时有遗尿" }, { "start_idx": 86, "end_idx": 93, "type": "sym", "entity": "无神经系统的病变" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 100, "end_idx": 103, "type": "dis", "entity": "脊柱损伤" }, { "start_idx": 109, "end_idx": 112, "type": "dis", "entity": "白日遗尿" } ] }, { "text": "第二种分类法将其称为原发性和继发性遗尿,所谓原发性是指小儿从小至就诊时一直有遗尿,而继发性是指小儿曾经停止遗尿至少6个月,以后又发生遗尿。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "原发性和继发性遗尿" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "遗尿" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "尿" }, { "start_idx": 47, "end_idx": 67, "type": "sym", "entity": "小儿曾经停止遗尿至少6个月,以后又发生遗尿" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "尿" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "尿" } ] }, { "text": "【流行病学资料】遗尿病占儿童尿失禁中的95%,其中80%为夜间遗尿,5%为日间遗尿,15%为昼夜均有遗尿。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "遗尿病" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "儿童尿失禁" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "尿" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "日间遗尿" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "尿" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "昼夜均有遗尿" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "尿" } ] }, { "text": "据国外报道,5岁儿童中约10%有遗尿,8岁儿童约7%,以后每岁减少1%。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "遗尿" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "尿" } ] }, { "text": "男孩遗尿多于女孩,但在5~6岁儿童中,女孩多于男孩,随着年龄的增长,男女之比逐渐增加,到11岁男女之比为2∶1。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "遗尿" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" } ] }, { "text": "【病因】遗尿症不是一种疾病,而是有多种原因所致的一个症状,其发生的因素包括遗传、心理和社会因素、睡眠状况、膀胱功能性容量等。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "遗尿症" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "膀胱" } ] }, { "text": "(一)遗传因素遗尿症儿童常有家族史,单卵双胎的孪生儿共同发病的概率高于双卵双胎者,双亲有遗尿症者,在后代中发现有遗尿症者达77%;父母中1人有遗尿症者,子女患遗尿症为44%。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "遗尿症" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "遗尿症" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "遗尿症" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "遗尿症" }, { "start_idx": 79, "end_idx": 81, "type": "dis", "entity": "遗尿症" } ] }, { "text": "然而,仅仅白天有遗尿症的儿童似乎与遗传无关,而那些白天和黑夜均有遗尿的儿童,有明显的男性家族遗传史。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "白天有遗尿症" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "遗尿症" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "白天和黑夜均有遗尿" } ] }, { "text": "Shaffer等(1984)发现阳性家族史在原发性遗尿和继发性遗尿中都很常见,说明遗传因素起一定的作用。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "原发性遗尿" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "继发性遗尿" } ] }, { "text": "最近丹麦的一些研究证实遗尿的显性基因是在第13号染色体上,这一发现为遗尿症的遗传学研究提供了进一步的证据。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "遗尿" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "显性基因" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "第13号染色体" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "遗尿症" } ] }, { "text": "(二)早产遗尿症的流行病学研究证实,早产是儿童日间遗尿最显著的一个高危因素。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "遗尿症" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "儿童日间遗尿" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "尿" } ] }, { "text": "这些早产儿除了有遗尿之外,还往往伴随其他的问题,如注意缺陷多动障碍,有学者提示这可能是轻微神经损伤的缘故。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "遗尿" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "注意缺陷多动障碍" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "轻微神经损伤" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "神经损伤" } ] }, { "text": "实际上夜间遗尿与睡眠深度无关,遗尿可以发生在睡眠任何阶段中,其主要问题是当膀胱充盈时,患儿不能从睡眠中觉醒。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "尿" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "遗尿" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 36, "end_idx": 52, "type": "sym", "entity": "当膀胱充盈时,患儿不能从睡眠中觉醒" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "膀胱" } ] }, { "text": "(四)心理和社会因素强烈的应激因素如幼儿时期的不良遭遇(父母离异、死亡、儿童与父母突然分离、因病住院或意外事故)、初入学不适应新的学习环境等均可导致儿童在控制排尿的关键时期因心理紧张而遗尿。", "entities": [ { "start_idx": 80, "end_idx": 80, "type": "bod", "entity": "尿" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "遗尿" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "尿" } ] }, { "text": "遗尿症儿童也常有较多的行为问题和情绪问题,如多动、抽动、不合群、害羞、脾气古怪等,据报道,遗尿症儿童中约10%有注意缺陷障碍,男孩多于女孩。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "遗尿症" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "有较多的行为问题和情绪问题" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "多动" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "抽动" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "不合群" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "害羞" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "脾气古怪" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "遗尿症" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "注意缺陷障碍" } ] }, { "text": "(五)膀胱容量小遗尿症儿童的膀胱容量较无遗尿的同龄儿童小。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "膀胱容量小" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "膀胱" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "遗尿症" }, { "start_idx": 11, "end_idx": 27, "type": "sym", "entity": "儿童的膀胱容量较无遗尿的同龄儿童小" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "膀胱" } ] }, { "text": "正常儿童的每次尿量约10ml/kg,而遗尿症的小儿其尿量达不到应有的膀胱的容量。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "尿量" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "遗尿症" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "尿量" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "膀胱" } ] }, { "text": "一般来说,这些儿童的平均每次尿量小于10ml/kg,白天排尿频繁(>7次),有尿急现象,晚上遗尿次数可以不止1次,尿量可或多或少。", "entities": [ { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "平均每次尿量小于10ml/kg" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "平均每次尿量" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "白天排尿频繁(>7次)" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "有尿急现象" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "尿" }, { "start_idx": 44, "end_idx": 55, "type": "sym", "entity": "晚上遗尿次数可以不止1次" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "尿" }, { "start_idx": 57, "end_idx": 63, "type": "sym", "entity": "尿量可或多或少" } ] }, { "text": "(六)便秘遗尿症儿童常有便秘的问题,特别多见的是日间遗尿的儿童,这是因为便秘时,直肠壶腹部的粪块强烈地刺激感觉神经,影响大脑对膀胱的充盈的感知而造成遗尿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "便秘" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "便" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "遗尿症" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "便秘" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "便" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "日间遗尿" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "尿" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "便秘" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "便" }, { "start_idx": 40, "end_idx": 56, "type": "sym", "entity": "直肠壶腹部的粪块强烈地刺激感觉神经" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "直肠壶腹部" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "粪块" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "感觉神经" }, { "start_idx": 58, "end_idx": 70, "type": "sym", "entity": "影响大脑对膀胱的充盈的感知" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "大脑" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "膀胱" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "遗尿" }, { "start_idx": 75, "end_idx": 75, "type": "bod", "entity": "尿" } ] }, { "text": "(七)血管加压素的缺乏血管加压素在夜间升高,使儿童在睡眠中尿量减少。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "血管加压素的缺乏" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "血管加压素" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "尿量减少" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" } ] }, { "text": "有一些仅夜间遗尿的儿童因为血管加压素缺乏正常的昼夜分泌节律,致使夜间尿量增多,超过膀胱的容量,造成遗尿。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "尿" }, { "start_idx": 13, "end_idx": 28, "type": "sym", "entity": "血管加压素缺乏正常的昼夜分泌节律" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "血管加压素" }, { "start_idx": 32, "end_idx": 45, "type": "sym", "entity": "夜间尿量增多,超过膀胱的容量" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "尿量" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "膀胱" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "遗尿" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "尿" } ] }, { "text": "患儿常在入眠后不久即遗尿,一般在夜眠最初1/3的时间发生遗尿,且尿渍大,如家长唤醒患儿排尿,则可无遗尿现象。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "入眠后不久即遗尿" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "尿" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "在夜眠最初1/3的时间发生遗尿" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "尿渍大" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "尿渍" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "尿" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "遗尿" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "尿" } ] }, { "text": "【临床表现】据报道,原发性遗尿占大多数,其中尤以夜间遗尿最常见,以男孩多见,夜间遗尿者约有半数每晚尿床,甚至每晚遗尿2~3次,白天过度活动、兴奋、疲劳或躯体疾病后往往遗尿次数增多,日间遗尿较少见。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "原发性遗尿" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "尿" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "遗尿" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "尿" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "每晚尿床" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "尿" }, { "start_idx": 54, "end_idx": 61, "type": "sym", "entity": "每晚遗尿2~3次" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "尿" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "躯体疾病" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "遗尿次数增多" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "尿" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "日间遗尿" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "尿" } ] }, { "text": "遗尿患儿常常伴夜惊、梦游、多动或其他行为障碍。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "遗尿" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "尿" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "夜惊" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "梦游" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "多动" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "其他行为障碍" } ] }, { "text": "【诊断】(一)患儿和家庭的评估在评估的过程中,取得患儿和家庭的信任,这是遗尿症治疗的一个前提。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "患儿和家庭的评估" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "遗尿症" } ] }, { "text": "(二)病史应当详细地采集病史,包括遗尿开始发生的时间,发生的频度,是白天遗尿还是夜间遗尿,是原发性的还是继发性的以及尿量的多少。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "详细地采集病史" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "遗尿" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "白天遗尿" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "尿" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "尿" }, { "start_idx": 58, "end_idx": 59, "type": "ite", "entity": "尿量" } ] }, { "text": "如果是夜间遗尿,每晚遗尿的次数等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "尿" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "遗尿" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "尿" } ] }, { "text": "病史中需了解有关社会心理方面的问题,例如患儿对遗尿的感觉如何;家庭中,父母及患儿,谁对此最为烦恼;父母是否因遗尿而惩罚患儿;患儿是否要求治疗,家庭中最近或经常有无情绪冲突;遗尿对儿童生活有无影响;患儿是否因为遗尿而不能参加集体活动如夏令营、春游等;父母对遗尿的了解程度以及他们对患儿的要求是否合理。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "遗尿" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "尿" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "遗尿" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "尿" }, { "start_idx": 86, "end_idx": 87, "type": "sym", "entity": "遗尿" }, { "start_idx": 87, "end_idx": 87, "type": "bod", "entity": "尿" }, { "start_idx": 104, "end_idx": 105, "type": "sym", "entity": "遗尿" }, { "start_idx": 105, "end_idx": 105, "type": "bod", "entity": "尿" }, { "start_idx": 127, "end_idx": 128, "type": "sym", "entity": "遗尿" }, { "start_idx": 128, "end_idx": 128, "type": "bod", "entity": "尿" } ] }, { "text": "此外,还应询问患儿每天清醒时排尿的次数,有无尿急、尿流细等现象,以排除泌尿系统的器质性病变。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "尿急" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "尿" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "尿流细" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "尿" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "排除泌尿系统" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "器质性病变" } ] }, { "text": "还应了解患儿的大便情况,有无便秘或遗粪情况;在睡眠方面,要了解患儿在睡眠中是否易被唤醒。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "pro", "entity": "应了解患儿的大便情况" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "便秘" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "遗粪" }, { "start_idx": 28, "end_idx": 42, "type": "pro", "entity": "要了解患儿在睡眠中是否易被唤醒" } ] }, { "text": "其他如食物过敏与遗尿的关系也需要考虑。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "食物过敏" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "遗尿" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" } ] }, { "text": "(三)体格检查体格检查的重点是腹部的触诊、生殖器的检查,以及神经系统的检查,另外应观察脊柱下端外观有无小凹及皮肤异常。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "体格检查" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "体格检查" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "腹部的触诊" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "生殖器的检查" }, { "start_idx": 30, "end_idx": 36, "type": "pro", "entity": "神经系统的检查" }, { "start_idx": 41, "end_idx": 57, "type": "pro", "entity": "观察脊柱下端外观有无小凹及皮肤异常" } ] }, { "text": "大多数遗尿症儿童在体格检查中无异常发现。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "遗尿症" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "体格检查" } ] }, { "text": "(四)实验室检查应进行尿常规或尿培养检查以排除尿路感染、慢性肾脏疾病等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "实验室检查" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "尿常规" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "尿培养检查" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "尿路感染" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "慢性肾脏疾病" } ] }, { "text": "尿比重测定排除因血管加压素缺乏所致的遗尿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "尿比重测定" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "血管加压素缺乏" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "血管加压素" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "遗尿" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "尿" } ] }, { "text": "大多数遗尿症儿童的病因并不复杂,但也有少数病例需要作详细的检查。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "遗尿症" } ] }, { "text": "遗尿症的实验室检查流程如图3-3。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "遗尿症" }, { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "实验室检查" } ] }, { "text": "图3-1遗尿症的实验室检查流程(五)诊断标准根据ICD-10精神与行为障碍分类(研究用诊断标准,WHO,1993,中文版)诊断依据为:1.儿童年龄与智龄至少5岁。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "遗尿症" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "实验室检查" } ] }, { "text": "2.不自主地或有意尿床或尿湿裤子,7岁以下每月至少2次,7岁以上每月至少1次。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "sym", "entity": "不自主地或有意尿床或尿湿裤子" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "7岁以下每月至少2次" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "7岁以上每月至少1次" } ] }, { "text": "3.不是癫痫发作或神经系统疾病所致的遗尿,也不是泌尿道结构异常或任何其他非精神科疾病的直接后果。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "癫痫" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "经系统疾病" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "遗尿" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "尿" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "泌尿道结构异常" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "非精神科疾病" } ] }, { "text": "4.不存在符合ICD-10类别标准的任何其他精神障碍的证据如精神发育迟滞、焦虑症、抑郁症等。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "精神障碍" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "精神发育迟滞" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "焦虑症" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "抑郁症" } ] }, { "text": "【鉴别诊断】遗尿症应与下列疾病鉴别:(一)神经系统疾患遗尿也见于骶部发育不良及脊膜膨出患者中。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "遗尿症" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "神经系统疾患遗尿" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "骶部发育不良" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "脊膜膨出" } ] }, { "text": "这类患儿除表现遗尿外,常有下肢无力等表现。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "遗尿" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "尿" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "下肢无力" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "下肢" } ] }, { "text": "骶部X线摄片、神经系统检查常可明确诊断。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "骶部X线摄片" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "神经系统检查" } ] }, { "text": "(二)尿路感染尿路感染可诱发遗尿。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿路感染" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "尿路感染" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "遗尿" } ] }, { "text": "若经过治疗后,尿路症状消失,遗尿现象也随之消失。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "尿路" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "遗尿现象" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" } ] }, { "text": "(三)尿失禁尿失禁常为器质性因素或泌尿系统的结构异常所致,如包茎、尿道口狭窄、糖尿病等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "尿失禁" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "尿失禁" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "泌尿系统的结构异常" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "包茎" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "尿道口狭窄" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "糖尿病" } ] }, { "text": "持续性尿失禁可见于膀胱外翻、尿道下裂以及异位输尿管开口。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "持续性尿失禁" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "膀胱外翻" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "膀胱" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "尿道下裂" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "尿道下" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "异位输尿管开口" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "输尿管开口" } ] }, { "text": "异位输尿管开口多见于女孩,其开口可能在尿道远端及阴道内。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "异位输尿管开口" }, { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "输尿管开口" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "开口可能在尿道远端及阴道内" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "尿道远端" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "阴道内" } ] }, { "text": "(四)尿路梗阻尿路梗阻最常见部位为后尿道瓣膜处,其占男性新生儿尿路梗阻的50%,常伴有膀胱逼尿肌无抑制性收缩,其中25%患儿有尿失禁。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "尿道瓣膜处" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "新生儿尿路梗阻" }, { "start_idx": 43, "end_idx": 53, "type": "sym", "entity": "膀胱逼尿肌无抑制性收缩" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "膀胱逼尿肌" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "尿失禁" } ] }, { "text": "临床上常见的症状是尿流变细,从婴儿时期出现滴尿,后期可出现上尿路功能损害,尿道造影及膀胱检查常可作出诊断。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "尿流变细" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "滴尿" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "尿" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "上尿路功能损害" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "尿道造影" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "膀胱检查" } ] }, { "text": "【治疗】遗尿症应强调综合性的治疗,其内容包括:(一)心理支持和健康教育首先,要对患儿及其家庭提供适当的心理支持和健康教育,寻找家庭环境中的紧张因素,询问患儿对遗尿的想法,向家庭和患儿解释遗尿的原因,进行对症治疗,并掌握患儿遗尿的规律,设法使患儿在觉醒状态下排尿。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "遗尿症" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "心理支持" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "健康教育" }, { "start_idx": 38, "end_idx": 59, "type": "pro", "entity": "要对患儿及其家庭提供适当的心理支持和健康教育" }, { "start_idx": 61, "end_idx": 72, "type": "pro", "entity": "寻找家庭环境中的紧张因素" }, { "start_idx": 74, "end_idx": 83, "type": "pro", "entity": "询问患儿对遗尿的想法" }, { "start_idx": 85, "end_idx": 97, "type": "pro", "entity": "向家庭和患儿解释遗尿的原因" }, { "start_idx": 99, "end_idx": 104, "type": "pro", "entity": "进行对症治疗" }, { "start_idx": 107, "end_idx": 115, "type": "pro", "entity": "掌握患儿遗尿的规律" }, { "start_idx": 117, "end_idx": 129, "type": "pro", "entity": "设法使患儿在觉醒状态下排尿" } ] }, { "text": "对夜间遗尿的患儿,晚餐后应限制液体摄入量,并在睡前将膀胱排空。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 9, "end_idx": 19, "type": "pro", "entity": "晚餐后应限制液体摄入量" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "在睡前将膀胱排空" } ] }, { "text": "遗尿患儿白天应避免过分紧张和疲劳。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "遗尿" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "尿" }, { "start_idx": 4, "end_idx": 15, "type": "pro", "entity": "白天应避免过分紧张和疲劳" } ] }, { "text": "(二)排尿功能训练白天做膀胱扩张训练,具体方法是:让患儿尽量多饮水,白天当患儿欲排尿时,嘱其延缓排尿,直至不能耐受为止。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "排尿功能训练" }, { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "白天做膀胱扩张训练" }, { "start_idx": 28, "end_idx": 32, "type": "pro", "entity": "尽量多饮水" }, { "start_idx": 34, "end_idx": 58, "type": "pro", "entity": "白天当患儿欲排尿时,嘱其延缓排尿,直至不能耐受为止" } ] }, { "text": "这样的训练方法使那些膀胱容量小,二次排尿间隔时间短的遗尿症患儿体会到膀胱胀满的感觉,并延长排尿的间隔时间。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "膀胱" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "膀胱容量小" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "二次排尿间隔时间短" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "遗尿症" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "膀胱胀满的感觉" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "膀胱" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "尿" } ] }, { "text": "对于年长的遗尿儿童,还可作括约肌的训练,以帮助患儿控制排尿。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "遗尿" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "尿" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "作括约肌的训练" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" } ] }, { "text": "对小儿来说,难以作直接的括约肌训练,故训练中采用的是间接的方法。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "直接的括约肌训练" } ] }, { "text": "括约肌训练可分作两个步骤,先是让患儿交替紧闭双眼,然后睁大眼睛,每天做3~5分钟,持续1周。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "括约肌训练" }, { "start_idx": 13, "end_idx": 44, "type": "pro", "entity": "先是让患儿交替紧闭双眼,然后睁大眼睛,每天做3~5分钟,持续1周" } ] }, { "text": "接着教患儿在仰卧位时,双足交替背屈和跖屈。(三)行为疗法该方法安全可靠,有比较确切的疗效,包括下述一系列措施:1.制日程表记录影响遗尿的可能因素,如睡眠时间、傍晚液体摄入量、白天活动情况、情绪等。", "entities": [ { "start_idx": 2, "end_idx": 19, "type": "pro", "entity": "教患儿在仰卧位时,双足交替背屈和跖屈" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "行为疗法" }, { "start_idx": 57, "end_idx": 96, "type": "pro", "entity": "制日程表记录影响遗尿的可能因素,如睡眠时间、傍晚液体摄入量、白天活动情况、情绪等" } ] }, { "text": "2.强化当患儿未出现尿床时,在日程表上贴红星以表示表扬,增强患儿控制遗尿的信心和能力;当患儿出现尿床时,则在次日要求其与家长一起清洁床铺和衣物。", "entities": [ { "start_idx": 2, "end_idx": 41, "type": "pro", "entity": "强化当患儿未出现尿床时,在日程表上贴红星以表示表扬,增强患儿控制遗尿的信心和能力" }, { "start_idx": 43, "end_idx": 70, "type": "pro", "entity": "当患儿出现尿床时,则在次日要求其与家长一起清洁床铺和衣物" } ] }, { "text": "当患儿遗尿时,少量尿液使纱布潮湿而导电,并使电路连通,由此发出警报声而唤醒患儿起床排尿,经反复应用和适当奖赏后,患儿睡眠中尿床的尿渍会逐渐减小,最后当膀胱充盈时会自动起床排尿。", "entities": [ { "start_idx": 0, "end_idx": 86, "type": "pro", "entity": "当患儿遗尿时,少量尿液使纱布潮湿而导电,并使电路连通,由此发出警报声而唤醒患儿起床排尿,经反复应用和适当奖赏后,患儿睡眠中尿床的尿渍会逐渐减小,最后当膀胱充盈时会自动起床排尿" } ] }, { "text": "报警器使用过程中应记录每晚报警叫唤的次数,描述尿渍的大小,从遗尿次数的减少或尿渍变小反映改善的情况,当患儿连续两周无遗尿时,则进入下一阶段的治疗,即在睡前给患儿饮450~900ml的液量,患儿如果膀胱容量较小,液体宜逐渐增多,每次增加60ml左右,夜间仍使用报警器,尽管在下一阶段开始时可使患儿再度出现遗尿,但经过数周后,遗尿现象可消失,当患儿又有连续2周无遗尿,则可停止使用报警器,而且睡前无须再饮水。", "entities": [ { "start_idx": 0, "end_idx": 48, "type": "pro", "entity": "报警器使用过程中应记录每晚报警叫唤的次数,描述尿渍的大小,从遗尿次数的减少或尿渍变小反映改善的情况" }, { "start_idx": 50, "end_idx": 167, "type": "pro", "entity": "当患儿连续两周无遗尿时,则进入下一阶段的治疗,即在睡前给患儿饮450~900ml的液量,患儿如果膀胱容量较小,液体宜逐渐增多,每次增加60ml左右,夜间仍使用报警器,尽管在下一阶段开始时可使患儿再度出现遗尿,但经过数周后,遗尿现象可消失" }, { "start_idx": 169, "end_idx": 200, "type": "pro", "entity": "当患儿又有连续2周无遗尿,则可停止使用报警器,而且睡前无须再饮水" } ] }, { "text": "据报道经过这样的治疗,遗尿复发率可减少到10%~15%。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "遗尿" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" } ] }, { "text": "(四)药物治疗1.丙米嗪此药能减少夜间遗尿,主要作用机制为减轻睡眠深度,使遗尿儿童能觉察到膀胱的涨满。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "药物治疗" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "丙米嗪" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "夜间遗尿" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "遗尿" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "尿" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "膀胱的涨满" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "膀胱" } ] }, { "text": "丙米嗪不良反应为头昏、便秘、心悸、口干和眼花。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "丙米嗪" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "头昏" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "头" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "便秘" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "心悸" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "心" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "口干" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "口" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "眼花" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "眼" } ] }, { "text": "如药物过量,可引起不良反应,症状为抽搐、室性心动过速和意识丧失。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "抽搐" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "室性心动过速" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "意识丧失" } ] }, { "text": "此药不宜在那些家庭环境不稳定的遗尿儿童中应用。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "遗尿" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" } ] }, { "text": "用药过程中,当遗尿纠正后,药物应维持6个月,然后逐渐减量至停药。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "遗尿" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "尿" } ] }, { "text": "2.醋酸去胺加压素这是一种合成的垂体后叶激素,主要用于因血管加压素缺乏的遗尿儿童,这些患儿的膀胱容量正常,常有夜间和(和或)白天遗尿,夜间尿量增多,晨尿比重低,首量为睡前口服0.2mg,如疗效不显著可增至0.4mg,连续使用3个月后停用至少1周,以便评估是否需要继续治疗。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "醋酸去胺加压素" }, { "start_idx": 13, "end_idx": 21, "type": "dru", "entity": "合成的垂体后叶激素" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "血管加压素缺乏" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "血管加压素" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "遗尿" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "尿" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "膀胱容量" }, { "start_idx": 55, "end_idx": 65, "type": "sym", "entity": "夜间和(和或)白天遗尿" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "尿" }, { "start_idx": 67, "end_idx": 72, "type": "sym", "entity": "夜间尿量增多" }, { "start_idx": 69, "end_idx": 70, "type": "ite", "entity": "尿量" }, { "start_idx": 74, "end_idx": 78, "type": "sym", "entity": "晨尿比重低" }, { "start_idx": 74, "end_idx": 77, "type": "ite", "entity": "晨尿比重" } ] }, { "text": "此药对少数儿童可引起头痛、恶心、胃痛或鼻出血。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "头痛" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "头" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "恶心" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "胃痛" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "胃" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "鼻出血" } ] }, { "text": "(五)中医治疗祖国医学认为遗尿系肾气不足,膀胱不能制约小便之故。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "中医治疗" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "遗尿" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "尿" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "肾气不足" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肾气" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "膀胱不能制约小便" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "膀胱" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "小便" } ] }, { "text": "常用各种方剂作补肾治疗如六味地黄丸、桑螵蛸散方加减治之。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "补肾治疗" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "六味地黄丸" }, { "start_idx": 18, "end_idx": 24, "type": "dru", "entity": "桑螵蛸散方加减" } ] }, { "text": "亦可用针刺关元、气海、三阴交、膀胱俞、肾俞等穴,每天针1次,对遗尿有一定的帮助。", "entities": [ { "start_idx": 3, "end_idx": 22, "type": "pro", "entity": "针刺关元、气海、三阴交、膀胱俞、肾俞等穴" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "遗尿" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "尿" } ] }, { "text": "此外,应提供良好的生活环境,避免不良的环境刺激所造成的遗尿。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "提供良好的生活环境" }, { "start_idx": 14, "end_idx": 28, "type": "pro", "entity": "避免不良的环境刺激所造成的遗尿" } ] }, { "text": "当儿童面临挫折和意外时,家长应善于疏导,帮助儿童消除心理紧张,当儿童出现遗尿后,不应责备或体罚,应寻找原因,对症治疗。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "面临挫折和意外" }, { "start_idx": 12, "end_idx": 29, "type": "pro", "entity": "家长应善于疏导,帮助儿童消除心理紧张" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "遗尿" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "尿" }, { "start_idx": 40, "end_idx": 46, "type": "pro", "entity": "不应责备或体罚" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "应寻找原因" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "对症治疗" } ] }, { "text": "遗尿症可能导致儿童各种不良的行为如自尊心低下、情绪问题、学习问题等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "遗尿症" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "自尊心低下" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "情绪问题" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "学习问题" } ] }, { "text": "正是由于这些问题,因此遗尿症既不能忽视,也不能带有责备的处理,应采取积极有效的措施,并仔细随访,避免由此而产生的各种不良后果。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "遗尿症" }, { "start_idx": 14, "end_idx": 29, "type": "pro", "entity": "既不能忽视,也不能带有责备的处理" }, { "start_idx": 31, "end_idx": 61, "type": "pro", "entity": "应采取积极有效的措施,并仔细随访,避免由此而产生的各种不良后果" } ] }, { "text": "三、婴儿肌纤维瘤婴儿肌纤维瘤(myofibroma)首先由Stout在1954年描述,多数为中心型,以后发现有孤立型。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "婴儿肌纤维瘤" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "婴儿肌纤维瘤" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "myofibroma" } ] }, { "text": "病理变化最常见的是皮肤和皮下界限清楚的结节,肌肉、骨骼和内脏的肿瘤边缘模糊肿瘤质硬如瘢痕,表面灰白或淡红色。", "entities": [ { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "皮肤和皮下界限清楚的结节" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "皮肤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "皮下" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肌肉" }, { "start_idx": 22, "end_idx": 36, "type": "sym", "entity": "肌肉、骨骼和内脏的肿瘤边缘模糊" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "骨骼" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "内脏" }, { "start_idx": 37, "end_idx": 52, "type": "sym", "entity": "肿瘤质硬如瘢痕,表面灰白或淡红色" } ] }, { "text": "镜下见丰满的梭形细胞,聚集成束,为高嗜酸性,基质周围有大量的胶原纤维。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "equ", "entity": "镜" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "梭形细胞" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "基质周围" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "胶原纤维" } ] }, { "text": "电镜看到混合的肌母细胞和类平滑肌细胞。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "肌母细胞" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "类平滑肌细胞" } ] }, { "text": "常为单个的皮下或肌肉的肿块神经引起疼痛。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "sym", "entity": "单个的皮下或肌肉的肿块" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "皮下" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肌肉" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "神经" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "疼痛" } ] }, { "text": "第二节大动脉转位大动脉转位(transpositionofthegreatarteries)是指右心房与右心室相连接,后者发出主动脉,而左心室房与左心房相连并发出肺动脉干。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 14, "end_idx": 44, "type": "dis", "entity": "transpositionofthegreatarteries" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "右心房" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "右心室" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "主动脉" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "左心室房" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "左心房" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "肺动脉干" } ] }, { "text": "其发病率占所有先天性心脏病的5%~7%,男性多见,约60%~70%。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "【病理解剖】心房、各静脉窦及房室结的解剖位置均正常。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "病理解剖" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "心房" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "静脉窦" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "房室结" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "解剖" } ] }, { "text": "常合并卵圆孔开放,极少数伴有房间隔缺损。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "卵圆孔开放" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "右心室结构位置正常,右心室壁厚度出生后迅速增加超过左心室。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 10, "end_idx": 27, "type": "sym", "entity": "右心室壁厚度出生后迅速增加超过左心室" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "右心室壁" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "左心室" } ] }, { "text": "膜部室间隔较小甚至缺如。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "膜部室间隔较小甚至缺如" }, { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "膜部室间隔" } ] }, { "text": "当室间隔完整时,室间隔平直,失去正常的曲面,同时左右心室流出道相互平行。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "室间隔" }, { "start_idx": 8, "end_idx": 20, "type": "sym", "entity": "室间隔平直,失去正常的曲面" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "室间隔" }, { "start_idx": 24, "end_idx": 34, "type": "sym", "entity": "左右心室流出道相互平行" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "左右心室流出道" } ] }, { "text": "左室壁的厚度及构型则与患儿年龄、是否伴有其他畸形(动脉导管未闭、肺动脉狭窄、室间隔缺损)有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左室壁" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "畸形" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "这些左室指标在决定外科手术方案时具有重要意义。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "左室指标" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "外科手术" } ] }, { "text": "常见于主动脉在肺动脉的右前方与右心室相连,也可见主动脉在肺动脉正前方、左前方,或极少数位于肺动脉的右后方。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "主动脉" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "肺动脉的右前方" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "右心室" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "主动脉" }, { "start_idx": 28, "end_idx": 37, "type": "bod", "entity": "肺动脉正前方、左前方" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "肺动脉的右后方" } ] }, { "text": "主肺动脉之间相互空间位置的变化与冠状动脉的起源及分布有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "主肺动脉" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "两条冠状动脉通常(>99%)由面对肺动脉的冠状窦发出,故该窦又称为“面对窦”(facingsinuses)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "两条冠状动脉" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "肺动脉的冠状窦" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "面对窦" }, { "start_idx": 39, "end_idx": 51, "type": "bod", "entity": "facingsinuses" } ] }, { "text": "这种结构在外科大动脉转换手术中有很大意义。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "pro", "entity": "外科大动脉转换手术" } ] }, { "text": "冠脉在心脏表面上的分布变异很大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "冠脉" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" } ] }, { "text": "由于主动脉窦位置与主、肺动脉相对位置有关,所以其位置不能仅以左/右或前/后的方法描述,因此,一些分类着重于对冠状动脉起源位置的描述而注重其空间关系。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "主动脉窦" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "主、肺动脉" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "此类方法是假设观察者从非面对窦(non-facingsinuses)部位面向肺动脉干,在其右手侧者称为1号窦或右手窦;左手侧者则称为2号窦或左手窦。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "非面对窦" }, { "start_idx": 16, "end_idx": 32, "type": "bod", "entity": "non-facingsinuses" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "肺动脉干" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "右手侧" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "1号窦" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "右手窦" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "左手侧" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "2号窦" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "左手窦" } ] }, { "text": "但是,该分类方法依据外科医生左右手命名而非解剖位置,可能会出现右冠脉起源于左手窦的情况。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dep", "entity": "外科" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "左右手" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右冠脉" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "左手窦" } ] }, { "text": "为解决这一问题,有作者建议将观察者的视角置于肺动脉的“非面对窦”中,面对主动脉观察。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺动脉" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "非面对窦" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "主动脉" } ] }, { "text": "与正常心脏冠脉命名一样,大动脉转位的三条冠状动脉也是根据其开口部位和在心外膜的走向而定。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "心脏冠脉" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "三条冠状动脉" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "心外膜" } ] }, { "text": "行走于右房室沟的称右冠脉,左前降支在心脏前表面与室间隔平行而下,旋支沿左房室沟下行。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "右房室沟" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "右冠脉" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "左前降支" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "心脏前表面" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "室间隔" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "旋支" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "左房室沟" } ] }, { "text": "此外,三支冠状动脉的近端均可为壁内冠状动脉而在主动脉壁内斜行通过,此时大动脉与冠脉之间没有血管外膜附着。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "bod", "entity": "三支冠状动脉的近端" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "壁内冠状动脉" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "主动脉壁内" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "大动脉" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "冠脉" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "血管外膜" } ] }, { "text": "最常见的冠状类型(约67%)是右冠脉起源于2号窦(即左手窦),左主冠脉起源于1号窦(右手窦)进而分出回旋支和左前降支。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "右冠脉" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "2号窦" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "左手窦" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "左主冠脉" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "1号窦" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "右手窦" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "回旋支" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "左前降支" } ] }, { "text": "其次较为常见的为回旋支由右冠脉分出,约占16%。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "回旋支" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "右冠脉" } ] }, { "text": "其他类型的少见,包括仅单一冠状动脉,左右冠脉反位,左冠脉或右冠脉于主动脉壁间走行等。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "左右冠脉反位" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "左冠脉" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "右冠脉" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "主动脉壁间" } ] }, { "text": "除了壁内冠状动脉畸形外,绝大多数冠状动脉畸形不影响大动脉转换手术(Swith手术)。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "壁内冠状动脉畸形" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "冠状动脉畸形" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "大动脉转换手术" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "Swith手术" } ] }, { "text": "膜部缺损可随年龄增长逐渐减小或自行闭合。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "膜部缺损" } ] }, { "text": "同样,位于室间隔中部的肌部缺损也可变小甚至关闭。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "室间隔中部" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "肌部缺损" } ] }, { "text": "对位不良型室间隔缺损具有较大的临床意义,此时流出道室间隔对位不良,位于右心室内致肺动脉瓣骑跨于右心室之上,严重时可合并肺动脉瓣下室间隔缺损,即Tassig-Bing畸形,出现右室双出口;流出道室间隔明显前移时可致主动脉瓣下狭窄,并伴有主动脉弓发育不全、缩窄或主动脉断离;流出道室间隔向后移位至左心室较少见,但可致左心室流出道梗阻。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "不良型室间隔缺损" }, { "start_idx": 22, "end_idx": 31, "type": "sym", "entity": "流出道室间隔对位不良" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "流出道室间隔" }, { "start_idx": 33, "end_idx": 51, "type": "sym", "entity": "位于右心室内致肺动脉瓣骑跨于右心室之上" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "右心室内" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "右心室之上" }, { "start_idx": 59, "end_idx": 68, "type": "dis", "entity": "肺动脉瓣下室间隔缺损" }, { "start_idx": 71, "end_idx": 83, "type": "dis", "entity": "Tassig-Bing畸形" }, { "start_idx": 87, "end_idx": 91, "type": "sym", "entity": "右室双出口" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "右室" }, { "start_idx": 93, "end_idx": 102, "type": "sym", "entity": "流出道室间隔明显前移" }, { "start_idx": 93, "end_idx": 98, "type": "bod", "entity": "流出道室间隔" }, { "start_idx": 106, "end_idx": 112, "type": "dis", "entity": "主动脉瓣下狭窄" }, { "start_idx": 117, "end_idx": 133, "type": "sym", "entity": "主动脉弓发育不全、缩窄或主动脉断离" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 129, "end_idx": 131, "type": "bod", "entity": "主动脉" }, { "start_idx": 135, "end_idx": 148, "type": "sym", "entity": "流出道室间隔向后移位至左心室" }, { "start_idx": 135, "end_idx": 140, "type": "bod", "entity": "流出道室间隔" }, { "start_idx": 146, "end_idx": 148, "type": "bod", "entity": "左心室" }, { "start_idx": 156, "end_idx": 163, "type": "sym", "entity": "左心室流出道梗阻" }, { "start_idx": 156, "end_idx": 161, "type": "bod", "entity": "左心室流出道" } ] }, { "text": "伴双动脉下型室间隔缺损者罕见,多伴有左位主动脉弓。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "伴双动脉下型室间隔缺损" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "左位主动脉弓" } ] }, { "text": "在大血管转位不伴室间隔缺损患儿中的发生率为4%;在伴室间隔缺损时,约占30%。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "大血管转位" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "室间隔完整时,常见轻度的动力性左室流出道梗阻伴室间隔收缩期向左侧膨出。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "室间隔" }, { "start_idx": 9, "end_idx": 33, "type": "sym", "entity": "轻度的动力性左室流出道梗阻伴室间隔收缩期向左侧膨出" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "左室流出道" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "室间隔" } ] }, { "text": "在新生儿期,因肺血管阻力、左心室压力仍高,这种现象不明显。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "肺血管阻力、左心室压力仍高" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "左心室压力" } ] }, { "text": "膜样纤维环或较少见的纤维肌性隧道可造成固定的流出道梗阻;当伴有室间隔缺损时,左心室流出道梗阻会更严重、更复杂。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "膜样纤维环" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "纤维" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "纤维肌性隧道" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "纤维肌" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "固定的流出道梗阻" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "流出道" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 38, "end_idx": 45, "type": "sym", "entity": "左心室流出道梗阻" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "左心室流出道" } ] }, { "text": "肺动脉瓣下狭窄相对较少见,引起肺动脉瓣下狭窄的因素包括:三尖瓣累赘组织凸入缺损的室间隔,膜部室间隔瘤或二尖瓣前瓣异常腱索组织附着于流出道室间隔等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肺动脉瓣下狭窄" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "肺动脉瓣下狭窄" }, { "start_idx": 28, "end_idx": 42, "type": "sym", "entity": "三尖瓣累赘组织凸入缺损的室间隔" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "三尖瓣累赘组织" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "室间隔" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "膜部室间隔瘤" }, { "start_idx": 51, "end_idx": 70, "type": "sym", "entity": "二尖瓣前瓣异常腱索组织附着于流出道室间隔" }, { "start_idx": 51, "end_idx": 61, "type": "bod", "entity": "二尖瓣前瓣异常腱索组织" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "流出道室间隔" } ] }, { "text": "【病理生理】组织供氧不足是大动脉转位的主要生理异常。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "组织供氧不足" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "大动脉转位" } ] }, { "text": "与正常的血液循环不同,大动脉转位使体循环和肺循环成为相互并行,各自独立的循环系统。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "血液循环" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 17, "end_idx": 39, "type": "sym", "entity": "体循环和肺循环成为相互并行,各自独立的循环系统" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "体循环" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺循环" } ] }, { "text": "在胎儿时期,患儿可以存活且不会影响其正常发育,可能与全部富含氧和葡萄糖的脐静脉血全部进入右心房有关。", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "脐静脉血" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "右心房" } ] }, { "text": "富含氧气的脐静脉血易于通过开放的卵圆孔进入肺循环和降主动脉;含氧量较低的下腔静脉血易通过三尖瓣进入右心室,经升主动脉供给冠脉和大脑,这种异常循环对胎儿生理的影响尚不清楚。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "脐静脉血" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺循环" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "降主动脉" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "含氧量" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "下腔静脉血" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "右心室" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "升主动脉" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "冠脉" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "大脑" }, { "start_idx": 68, "end_idx": 71, "type": "sym", "entity": "异常循环" } ] }, { "text": "出生后,由于体肺循环之间无法混合,氧合后的肺静脉血经右心室仍流入肺循环,而未氧合的体循环血不能进入肺部进行气体交换,因此,只有两系统间存在交通生后才能存活。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "体肺循环" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "肺静脉血" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右心室" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺循环" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "体循环血" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肺部" } ] }, { "text": "常见的心内交通,如卵圆孔未闭、房间隔缺损、室间隔缺损,而在心外可有动脉导管未闭、支气管动静脉侧支。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心内交通" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "卵圆孔未闭" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "心外" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "支气管动静脉侧支" } ] }, { "text": "两个循环间血液混合的程度取决于交换部位的数量、大小及总肺血流量。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "两个循环间血液混合" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "总肺血流量" } ] }, { "text": "当有左心室流出道梗阻或肺血管阻力增高时将导致肺血减少。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "左心室流出道梗阻" }, { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "肺血管阻力增高" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肺血管" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "肺血减少" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肺血" } ] }, { "text": "体肺循环间交通的分流方向取决于两侧心室的顺应性、心室间压力阶差、呼吸相和两大循环的血管阻力。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "体肺循环间交通" }, { "start_idx": 15, "end_idx": 22, "type": "ite", "entity": "两侧心室的顺应性" }, { "start_idx": 24, "end_idx": 30, "type": "ite", "entity": "心室间压力阶差" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "呼吸相" }, { "start_idx": 36, "end_idx": 44, "type": "ite", "entity": "两大循环的血管阻力" } ] }, { "text": "当室间隔完整时,因舒张期左心室顺应性较好,出现心房间右向左分流;而收缩期由于左心房顺应性较差,出现心房水平左向右分流。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "室间隔" }, { "start_idx": 12, "end_idx": 17, "type": "ite", "entity": "左心室顺应性" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "心房间右向左分流" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "心房间" }, { "start_idx": 38, "end_idx": 43, "type": "ite", "entity": "左心房顺应性" }, { "start_idx": 49, "end_idx": 57, "type": "sym", "entity": "心房水平左向右分流" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "心房" } ] }, { "text": "新生儿早期肺血管阻力高时,动脉导管血流呈双向,随着肺血管阻力的下降,分流逐渐从压力高的体循环向肺循环单向进行。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "肺血管阻力高" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肺血管" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "动脉导管血流呈双向" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "动脉导管" }, { "start_idx": 25, "end_idx": 29, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 34, "end_idx": 53, "type": "sym", "entity": "分流逐渐从压力高的体循环向肺循环单向进行" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "体循环" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "肺循环" } ] }, { "text": "而并发室间隔缺损的心室水平分流方式尚不清楚。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "心室水平分流" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心室" } ] }, { "text": "大动脉转位患儿早期并发肺血管疾病已有大量文献报道。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肺血管疾病" } ] }, { "text": "大多数伴有大室间隔缺损的患儿在1岁时可出现严重的肺血管疾病,甚至一些不伴有室间隔缺损的患儿也存在广泛的肺血管组织异常改变。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "大室间隔缺损" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "严重的肺血管疾病" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 48, "end_idx": 59, "type": "sym", "entity": "广泛的肺血管组织异常改变" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "肺血管组织" } ] }, { "text": "此病还可见于合并有大的动脉导管未闭及少数单纯房间隔缺损的患儿。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "单纯房间隔缺损" } ] }, { "text": "肺血管疾病可由多因素引起,如肺动脉高压和高血流量、低氧血症、红细胞增多症、血管收缩因子及血小板聚集异常等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺血管疾病" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "高血流量" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "低氧血症" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "红细胞增多症" }, { "start_idx": 37, "end_idx": 50, "type": "sym", "entity": "血管收缩因子及血小板聚集异常" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "血管收缩因子" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "血小板" } ] }, { "text": "【临床表现】在室间隔完整的大动脉转位新生儿,两大循环间的交换血流量少,存在着明显的青紫(室间隔完整和血液混合不充分的新生儿青紫明显),可在出生后数小时内即被发现,且大多情况下为唯一症状。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "室间隔" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 22, "end_idx": 33, "type": "sym", "entity": "两大循环间的交换血流量少" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "交换血流量" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "明显的青紫" }, { "start_idx": 44, "end_idx": 64, "type": "sym", "entity": "室间隔完整和血液混合不充分的新生儿青紫明显" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "室间隔" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "血液" } ] }, { "text": "体格检查可见明显发绀,外周血管脉搏正常,右心室搏动增强。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "明显发绀" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "外周血管脉搏正常" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "外周血管" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "脉搏" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "右心室搏动增强" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "右心室" } ] }, { "text": "听诊第1、第2心音响亮,第2心音常单一或轻度分裂,心脏杂音柔或缺如。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "听诊第1、第2心音响亮" }, { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "第2心音常单一或轻度分裂" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "心脏杂音柔或缺如" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心脏" } ] }, { "text": "柔和的喷射性杂音可是功能性左心室流出道杂音或是动脉导管未闭引起。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "柔和的喷射性杂音" }, { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "功能性左心室流出道杂音" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "新生儿早期肺血管阻力仍较高,杂音可不明显,但在生后最初的几星期常会于胸骨左下缘出现特征性的全收缩期杂音,可伴或不伴有震颤。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "肺血管阻力仍较高" }, { "start_idx": 5, "end_idx": 9, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "杂音可不明显" }, { "start_idx": 35, "end_idx": 50, "type": "sym", "entity": "骨左下缘出现特征性的全收缩期杂音" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "骨左下缘" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "震颤" } ] }, { "text": "当左室流出道梗阻导致肺动脉狭窄时青紫明显,在胸骨左上缘可闻及响亮的收缩期喷射性杂音。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "左室流出道梗阻" }, { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "左室流出道" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "青紫明显" }, { "start_idx": 21, "end_idx": 40, "type": "sym", "entity": "在胸骨左上缘可闻及响亮的收缩期喷射性杂音" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "胸骨左上缘" } ] }, { "text": "除主动脉瓣靠近前胸壁使第2心音较响外,其他临床表现与法洛四联症相似。", "entities": [ { "start_idx": 1, "end_idx": 16, "type": "sym", "entity": "主动脉瓣靠近前胸壁使第2心音较响" }, { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "前胸壁" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "在伴有前向对位不良的室间隔缺损时,尚需注意是否有左室流出道梗阻的存在。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "前向对位不良的室间隔缺损" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "左室流出道梗阻" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "左室流出道" } ] }, { "text": "若有股动脉搏动减弱而手臂动脉搏动正常或增强提示可能伴有主动脉弓中断或水肿。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "股动脉搏动减弱" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "股动脉" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "搏动" }, { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "手臂动脉搏动正常或增强" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "手臂动脉" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "搏动" }, { "start_idx": 27, "end_idx": 35, "type": "dis", "entity": "主动脉弓中断或水肿" } ] }, { "text": "除此之外,还可能表现有上半身较下半身青紫严重的差异性青紫现象。", "entities": [ { "start_idx": 11, "end_idx": 29, "type": "sym", "entity": "上半身较下半身青紫严重的差异性青紫现象" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "上半身" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "下半身" } ] }, { "text": "【辅助检查】(一)胸部X线出生时心脏大小及肺野均正常,但纵隔阴影狭窄,可能与主、肺动脉呈前后位及因缺氧与应激引起胸腺发育不全有关。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "胸部X线" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心脏" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肺野" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "纵隔阴影狭窄" }, { "start_idx": 38, "end_idx": 46, "type": "sym", "entity": "主、肺动脉呈前后位" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "主、肺动脉" }, { "start_idx": 49, "end_idx": 61, "type": "sym", "entity": "缺氧与应激引起胸腺发育不全" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "胸腺" } ] }, { "text": "随着肺血流的不断增多及充血性心力衰竭的进一步发展,心脏扩大、肺血管影逐渐明显,进而形成“蛋形”心影。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "肺血流的不断增多" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺血流" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心脏" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "肺血管影逐渐明显" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肺血管" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "形成“蛋形”心影" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "心" } ] }, { "text": "当伴有大的室间隔缺损时,心影更大,肺野充血明显。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "大的室间隔缺损" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "室间隔" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "心影更大" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心影" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "肺野充血明显" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肺野" } ] }, { "text": "与之相反,当伴有左心室流出道梗阻时,肺野缺血,心脏大小正常。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "左心室流出道梗阻" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "肺野缺血" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺野" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心脏" } ] }, { "text": "(二)心电图患儿出生后的最初几天心电图常无特异性改变。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "心电图" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "心电图" } ] }, { "text": "额面QRS电轴在+90°~+120°之间,右胸导联主波为R波,右心室占优势。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "额面QRS电轴在+90°~+120°之间" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "右胸导联主波为R波" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "右胸" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "右心室占优势" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心室" } ] }, { "text": "室间隔完整者,几星期内可出现持续电轴右偏和右室肥厚表现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "室间隔" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "持续电轴右偏和右室肥厚" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "右室" } ] }, { "text": "伴大型室间隔缺损时,表现为双室肥大。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "大型室间隔缺损" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "双室肥大" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "双室" } ] }, { "text": "即使有明显左心室流出道梗阻或肺血管阻力增高时单纯左心室肥大也不多见。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "左心室流出道梗阻" }, { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "肺血管阻力增高" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺血管" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "单纯左心室肥大" } ] }, { "text": "(三)超声心动图目前,超声心动图已成为大动脉转位诊断地一种重要的影像学方法,除了作出诊断外,超声心动图检查的目的还包括体肺循环间的分流评估、显示伴发畸形、明确冠状动脉的解剖形态及决定采取何种手术方案。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "超声心动图" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 46, "end_idx": 52, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 59, "end_idx": 68, "type": "pro", "entity": "体肺循环间的分流评估" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "显示伴发畸形" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "畸形" }, { "start_idx": 77, "end_idx": 87, "type": "pro", "entity": "明确冠状动脉的解剖形态" }, { "start_idx": 89, "end_idx": 98, "type": "pro", "entity": "决定采取何种手术方案" } ] }, { "text": "而且,越来越多的球囊房隔造口术也单独在超声心动图导引下得以成功实施。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "球囊房隔造口术" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "超声心动图" } ] }, { "text": "在剑突下通过改变探头方向可同时显示2条大动脉分别自左右心室发出,平行上行(图9-9)。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "在剑突下通过改变探头方向" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "2条大动脉" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "左右心室" } ] }, { "text": "后位的起自左心室的动脉干,发出肺动脉分支,提示为肺动脉干。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "左心室" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "动脉干" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "肺动脉分支" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "肺动脉干" } ] }, { "text": "胸骨旁长短轴切面也同样可显示两根大动脉的平行关系,短轴显示两者相对位置关系更为明确,通常可见主动脉位于肺动脉干的右前方。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "胸骨旁" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "两根大动脉" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "主动脉" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "肺动脉干" } ] }, { "text": "剑突下切面可充分观察心房间的交通道情况,胸骨上窝或高位胸骨旁长轴切面可清楚显示动脉导管未闭动脉导管未闭。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "剑突下" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "心房间的交通道" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "胸骨上窝" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "高位胸骨旁" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "如并发室间隔缺损室间隔缺损、左室流出道梗阻、流出道室间隔对位不良及主动脉弓畸形等二维超声心动图均能显示。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "左室流出道梗阻" }, { "start_idx": 22, "end_idx": 31, "type": "dis", "entity": "流出道室间隔对位不良" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "主动脉弓畸形" }, { "start_idx": 40, "end_idx": 46, "type": "pro", "entity": "二维超声心动图" } ] }, { "text": "多普勒超声可显示流出道梗阻的严重程度。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "多普勒超声" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "流出道梗阻" } ] }, { "text": "此外,超声心动图还能准确评价三条主要冠状动脉的起源及在心外膜表面的走向(图9-9):胸骨旁短轴切面尚可清晰显示冠状动脉在主动脉窦的开口位置,但要全面地评价冠状动脉的走向则需通过多个切面。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "三条主要冠状动脉" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "心外膜表面" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "胸骨旁" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "主动脉窦" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "其次,超声还可探查到一些重要的冠脉异常,如单一冠脉,冠脉在主动脉壁内的行进部分。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "超声" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "冠脉异常" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "冠脉" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "冠脉" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "冠脉" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "主动脉壁" } ] }, { "text": "图9-27超声心动图剑突下切面A.和胸骨上切面;B.显示:大血管呈平行排列Ao:主动脉;LV:左心室;PA:肺动脉;PDA:动脉导管未闭图9-28超声心动图胸骨旁短轴切面可清楚地显示冠状动脉A.左冠状动脉行走于两个大血管前(箭头所示);B.追踪其起源示:单一的主冠状动脉起源于窦2,然后分支成左和右(箭头所示)两根冠状动脉(缩写同图9-9)室间隔完整的大动脉转位婴儿在行大动脉转换术(arterialswitchprocedure)前常需要多次超声检查,以了解室腔大小、室壁厚度、左心室容积和形态。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "超声心动图" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "剑突下" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "胸骨上" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "大血管" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "主动脉" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "左心室" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "肺动脉" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 73, "end_idx": 77, "type": "pro", "entity": "超声心动图" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "胸骨旁" }, { "start_idx": 91, "end_idx": 94, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 97, "end_idx": 101, "type": "bod", "entity": "左冠状动脉" }, { "start_idx": 105, "end_idx": 110, "type": "bod", "entity": "两个大血管前" }, { "start_idx": 130, "end_idx": 134, "type": "bod", "entity": "主冠状动脉" }, { "start_idx": 138, "end_idx": 138, "type": "bod", "entity": "窦" }, { "start_idx": 155, "end_idx": 160, "type": "bod", "entity": "两根冠状动脉" }, { "start_idx": 170, "end_idx": 172, "type": "bod", "entity": "室间隔" }, { "start_idx": 176, "end_idx": 180, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 185, "end_idx": 190, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 192, "end_idx": 214, "type": "pro", "entity": "arterialswitchprocedure" }, { "start_idx": 222, "end_idx": 225, "type": "pro", "entity": "超声检查" }, { "start_idx": 230, "end_idx": 231, "type": "bod", "entity": "室腔" }, { "start_idx": 235, "end_idx": 236, "type": "bod", "entity": "室壁" }, { "start_idx": 240, "end_idx": 242, "type": "bod", "entity": "左心室" } ] }, { "text": "室间隔的弧度可提示左室收缩期压力。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "室间隔" }, { "start_idx": 9, "end_idx": 15, "type": "ite", "entity": "左室收缩期压力" } ] }, { "text": "大动脉转换手术要求左心室流出道大小及肺动脉瓣的功能正常,但动力性左心室流出道梗阻并非大动脉转换术的禁忌证。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "大动脉转换手术" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 29, "end_idx": 39, "type": "dis", "entity": "动力性左心室流出道梗阻" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "大动脉转换术" } ] }, { "text": "(四)心导管检查由于超声心动图检查可以获取详尽的解剖结构信息,心导管检查已很少用于单纯诊断。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心导管检查" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "心导管检查" } ] }, { "text": "在大多数心血管治疗中心,心导管术仅用于球囊房隔造口术。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "心血管" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "心导管术" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "球囊房隔造口术" } ] }, { "text": "尽管如此,球囊房隔造口术仍可在超声心动图导引下成功地进行。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "球囊房隔造口术" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "超声心动图" } ] }, { "text": "而且,在较早开展大动脉转换术的医院,若患儿有足够大的卵圆孔及动脉导管未闭,体肺循环间混合充分时,无须行心导管术。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "体肺循环" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "心导管术" } ] }, { "text": "通过心导管检查详细评价血流动力学情况仅限于复杂型大动脉转位伴严重左室流出道梗阻、多发孔洞型室间隔缺损、主动脉弓畸形及后期可出现肺动脉疾病的患儿。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "心导管检查" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "血流动力学情况" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "复杂型大动脉转位" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "严重左室流出道梗阻" }, { "start_idx": 40, "end_idx": 49, "type": "dis", "entity": "多发孔洞型室间隔缺损" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "主动脉弓畸形" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "肺动脉疾病" } ] }, { "text": "同样,心血管造影术的应用也很少,当疑有水肿时,右心室或升主动脉侧位或长轴位造影可显示水肿的部位。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "心血管造影术" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "水肿" }, { "start_idx": 23, "end_idx": 38, "type": "pro", "entity": "右心室或升主动脉侧位或长轴位造影" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "水肿" } ] }, { "text": "左心室长轴位造影可显示左心室流出道梗阻。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "左心室长轴位造影可显示左心室流出道梗阻" }, { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "左心室长轴位造影" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "左心室流出道" } ] }, { "text": "另外,冠状动脉选择性造影或经静脉主动脉根部球囊堵塞逆向造影可显示冠脉的解剖结构。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "冠状动脉选择性造影" }, { "start_idx": 13, "end_idx": 28, "type": "pro", "entity": "经静脉主动脉根部球囊堵塞逆向造影" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "冠脉" } ] }, { "text": "后前位向头成角投照可清晰见到冠状动脉的开口及其分布和走向。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "后前位向头成角投照" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "但是,超声心动图能可靠地显示几乎所有患儿的冠状动脉解剖结构。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "因此,大部分手术在术前无须行造影检查。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "手术" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "造影检查" } ] }, { "text": "【治疗】(一)术前处理对于有严重缺氧的婴儿,应在转送至三级医院前即行静脉静滴前列腺素E1或前列腺素E2以扩张动脉导管,伴代谢性酸中毒者可静注碳酸氢钠纠正酸中毒。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "术前处理" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "严重缺氧" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "静脉静滴" }, { "start_idx": 38, "end_idx": 43, "type": "dru", "entity": "前列腺素E1" }, { "start_idx": 45, "end_idx": 50, "type": "dru", "entity": "前列腺素E2" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "动脉导管" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 68, "end_idx": 69, "type": "pro", "entity": "静注" }, { "start_idx": 70, "end_idx": 73, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "酸中毒" } ] }, { "text": "在X线或超声心动图的直视监控下,导管自股静脉插入,经下腔静脉至右心房后推开卵圆孔帘膜进入左心房(图9-9),确定导管位于左房后使球囊膨胀,再轻巧快速地回拉导管,使球囊撕裂房间隔。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "X线" }, { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "超声心动图" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "直视监控" }, { "start_idx": 16, "end_idx": 17, "type": "equ", "entity": "导管" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "股静脉" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心房" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "卵圆孔帘膜" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "左心房" }, { "start_idx": 56, "end_idx": 57, "type": "equ", "entity": "导管" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "左房" }, { "start_idx": 63, "end_idx": 67, "type": "pro", "entity": "使球囊膨胀" }, { "start_idx": 70, "end_idx": 78, "type": "pro", "entity": "轻巧快速地回拉导管" }, { "start_idx": 80, "end_idx": 87, "type": "pro", "entity": "使球囊撕裂房间隔" } ] }, { "text": "这种方法可改善体循环的缺氧状态。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "体循环" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "缺氧" } ] }, { "text": "图9-29在超声心动图指导下行球囊心房间隔造口术超声心动图证实膨胀的球囊已经位于左心房RA:右心房RV:右心室LV:左心室(二)手术治疗在大动脉转换术被引入以前,20世纪60年代至80年代初期,大动脉转位患儿的治疗仅局限于心房置换,即在心房水平通过将体循环静脉血导引至左心室,肺静脉血引入右心室以纠正异常的血流动力学。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "超声心动图" }, { "start_idx": 13, "end_idx": 23, "type": "pro", "entity": "下行球囊心房间隔造口术" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "超声心动图" }, { "start_idx": 31, "end_idx": 35, "type": "equ", "entity": "膨胀的球囊" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "左心房" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "RA" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "右心房" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "RV" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "右心室" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "LV" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "左心室" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "手术治疗" }, { "start_idx": 69, "end_idx": 74, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 97, "end_idx": 101, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 111, "end_idx": 114, "type": "pro", "entity": "心房置换" }, { "start_idx": 117, "end_idx": 136, "type": "pro", "entity": "在心房水平通过将体循环静脉血导引至左心室" }, { "start_idx": 138, "end_idx": 146, "type": "pro", "entity": "肺静脉血引入右心室" }, { "start_idx": 150, "end_idx": 157, "type": "sym", "entity": "异常的血流动力学" } ] }, { "text": "Mustard手术时需切除大部分房间隔,在心房内置入板障以建立新的血流通道,补片可为心包组织或化学合成材料。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "Mustard手术" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "切除大部分房间隔" }, { "start_idx": 20, "end_idx": 36, "type": "pro", "entity": "在心房内置入板障以建立新的血流通道" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "心包组织" } ] }, { "text": "与之不同的是,Senning手术补片材料来自患儿自身右心房壁及房间隔。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "pro", "entity": "Senning手术" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "右心房壁" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "房间隔" } ] }, { "text": "由于心房转换术的较高的手术成功率,在先心病术后随访的患儿中此类患儿占有一定的比例。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "心房转换术" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "先心病" } ] }, { "text": "然而,虽然早期效果较好,但由于术后远期的并发症较多,故此类手术目前已很少采用,以下介绍目前采用的手术方法。", "entities": [ { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "手术方法" } ] }, { "text": "1.室间隔完整的大动脉转位对于室间隔完整的大动脉转位患儿,在生后一月内可行大动脉转换术,最佳手术时间是在生后1~2周。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "室间隔完整" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "室间隔完整" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 37, "end_idx": 42, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "手术" } ] }, { "text": "手术是将错位的主肺动脉切断互换,同时将冠状动脉移植到转位后的主动脉上,以达到解剖上的完全纠正。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 3, "end_idx": 14, "type": "pro", "entity": "将错位的主肺动脉切断互换" }, { "start_idx": 18, "end_idx": 33, "type": "pro", "entity": "将冠状动脉移植到转位后的主动脉上" } ] }, { "text": "为了避免因肺动脉较短而使用导管连接新的肺动脉干断端,Lecompte提出新方案,即充分分离肺动脉干及其分支,并将其转移到主动脉前方,使肺动脉干远心端有足够长度可以和主动脉根部吻合,形成新的肺动脉。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肺动脉" }, { "start_idx": 11, "end_idx": 24, "type": "pro", "entity": "使用导管连接新的肺动脉干断端" }, { "start_idx": 41, "end_idx": 52, "type": "pro", "entity": "充分分离肺动脉干及其分支" }, { "start_idx": 55, "end_idx": 64, "type": "pro", "entity": "将其转移到主动脉前方" }, { "start_idx": 66, "end_idx": 88, "type": "pro", "entity": "使肺动脉干远心端有足够长度可以和主动脉根部吻合" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "肺动脉" } ] }, { "text": "冠状动脉自原先的主动脉根部切下,连同其周围的主动脉壁重新植入转位后的主动脉根部,冠脉缺口由补片修复。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "pro", "entity": "冠状动脉自原先的主动脉根部切下" }, { "start_idx": 16, "end_idx": 38, "type": "pro", "entity": "连同其周围的主动脉壁重新植入转位后的主动脉根部" }, { "start_idx": 40, "end_idx": 48, "type": "pro", "entity": "冠脉缺口由补片修复" } ] }, { "text": "尽管有人认为将冠脉转位是引起手术死亡的一个危险因素,但这种方法因能彻底纠正冠状动脉解剖上的异常而在动脉转位术中经常运用。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "将冠脉转位" }, { "start_idx": 37, "end_idx": 46, "type": "sym", "entity": "冠状动脉解剖上的异常" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 49, "end_idx": 53, "type": "pro", "entity": "动脉转位术" } ] }, { "text": "出生后随着肺血管阻力下降,左心室收缩期室内压降低,室间隔向左心室膨出。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "肺血管阻力下降" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肺血管" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "左心室收缩期室内压降低" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "左心室" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "室内压" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "室间隔向左心室膨出" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "室间隔" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "左心室" } ] }, { "text": "一旦时间较长,左心室将得不到正常发育,术后因没有足够左心室容积供给体循环血液,不能适应新的血流动力学要求而失败。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "sym", "entity": "左心室将得不到正常发育" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "左心室" }, { "start_idx": 22, "end_idx": 37, "type": "sym", "entity": "没有足够左心室容积供给体循环血液" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "左心室容积" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "体循环血液" } ] }, { "text": "有时特殊情况下可先行肺动脉环缩术,以达到减轻肺充血,促进左心室发育的目的。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "肺动脉环缩术" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "肺充血" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "左心室" } ] }, { "text": "然而,肺动脉环缩术可使青紫进一步加重和短暂的由急性左心室功能不全造成的低心排血量综合征,一般在术后7~10天就可以恢复。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "肺动脉环缩术" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "青紫进一步加重" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "急性左心室功能不全" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "低心排血量综合征" } ] }, { "text": "对于大于1个月的患儿,可采取在行大动脉转换术前7~10天行肺动脉环缩术和体肺动脉分流术的快速二期手术方法。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "肺动脉环缩术" }, { "start_idx": 36, "end_idx": 42, "type": "pro", "entity": "体肺动脉分流术" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "手术" } ] }, { "text": "2.大动脉转位伴室间隔缺损伴有室间隔缺损但无左心室流出道梗阻的患儿,应在出生后1个月内进行动脉转换加室间隔缺损修补术,其术后生存率与室间隔完整者相同。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "大动脉转位伴室间隔缺损" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "左心室流出道梗阻" }, { "start_idx": 45, "end_idx": 57, "type": "pro", "entity": "动脉转换加室间隔缺损修补术" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "室间隔完整" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "室间隔" } ] }, { "text": "但环缩术后,尤其在流出部室间隔对位不良时,可产生主动脉瓣下狭窄;而且肺动脉环缩术可进一步引起转位后的新主动脉根部扩张和增加新主动脉血液反流的机会。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "环缩术" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "室间隔对位不良" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "室间隔" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "主动脉瓣下狭窄" }, { "start_idx": 34, "end_idx": 39, "type": "pro", "entity": "肺动脉环缩术" }, { "start_idx": 50, "end_idx": 57, "type": "sym", "entity": "新主动脉根部扩张" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "新主动脉根部" }, { "start_idx": 61, "end_idx": 68, "type": "dis", "entity": "新主动脉血液反流" } ] }, { "text": "高肺血管阻力是关闭室间隔缺损的反指征。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "高肺血管阻力" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "高肺血管" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "但尽管存在肺血管疾病,但通常情况下肺动脉氧饱和度仍高于主动脉,单纯行大动脉转换术不关闭室间隔缺损也最终可因提高体动脉氧饱和度而缓解患儿的症状。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肺血管疾病" }, { "start_idx": 17, "end_idx": 29, "type": "sym", "entity": "肺动脉氧饱和度仍高于主动脉" }, { "start_idx": 17, "end_idx": 23, "type": "ite", "entity": "肺动脉氧饱和度" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "主动脉" }, { "start_idx": 34, "end_idx": 39, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 55, "end_idx": 61, "type": "ite", "entity": "体动脉氧饱和度" } ] }, { "text": "3.大动脉转位伴左室流出道梗阻动力性左室流出道梗阻在临床上意义不大,并不是手术的禁忌证。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "sym", "entity": "大动脉转位伴左室流出道梗阻" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "大动脉" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "左室" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "动力性左室流出道梗阻" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "左室" } ] }, { "text": "手术时以补片在室间隔缺损与右心室内的主动脉下圆锥之间建立内隧道,使来自左心室的血液可通过心室内通道流入主动脉,同时,肺动脉总干离断,用人工管道将右心室和离断的远端肺动脉相连。", "entities": [ { "start_idx": 0, "end_idx": 30, "type": "pro", "entity": "手术时以补片在室间隔缺损与右心室内的主动脉下圆锥之间建立内隧道" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "左心室的血液" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "心室内通道" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "主动脉" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "肺动脉总干离断" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "肺动脉总干" }, { "start_idx": 66, "end_idx": 85, "type": "pro", "entity": "用人工管道将右心室和离断的远端肺动脉相连" } ] }, { "text": "病例选择时需要求有足够大的室间隔缺损,以确保术后左心室流出道的通畅,有必要时将前侧的肌部室间隔切去,以扩大室间隔缺损。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 38, "end_idx": 48, "type": "pro", "entity": "将前侧的肌部室间隔切去" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "而当缺损远离累及主动脉瓣下圆锥或异常三尖瓣组织遮挡室间隔缺损时,则需先重建心室间的通道。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "缺损" }, { "start_idx": 8, "end_idx": 29, "type": "sym", "entity": "主动脉瓣下圆锥或异常三尖瓣组织遮挡室间隔缺损" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "主动脉瓣下圆锥" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "三尖瓣组织" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "心室间" } ] }, { "text": "一些学者主张在小儿到4~6岁时手术以便植入成人规格的人工管道。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "手术" }, { "start_idx": 19, "end_idx": 29, "type": "pro", "entity": "植入成人规格的人工管道" } ] }, { "text": "另一种术式由Lecompte提出,手术无须人工管道。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "手术" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "人工管道" } ] }, { "text": "手术方法是,在左心室和主动脉之间建立一个较短的心室内隧道;封闭肺动脉口,将离断的肺动脉直接与右心室流出道连接。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 7, "end_idx": 27, "type": "pro", "entity": "左心室和主动脉之间建立一个较短的心室内隧道" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "封闭肺动脉口" }, { "start_idx": 36, "end_idx": 53, "type": "pro", "entity": "将离断的肺动脉直接与右心室流出道连接" } ] }, { "text": "4.大动脉转位伴主动脉弓异常大动脉转位合并主动脉弓畸形多与室间隔缺损及圆锥隔前向性对位不良伴发。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "sym", "entity": "大动脉转位伴主动脉弓异常" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "大动脉" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 14, "end_idx": 26, "type": "dis", "entity": "大动脉转位合并主动脉弓畸形" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 35, "end_idx": 44, "type": "sym", "entity": "圆锥隔前向性对位不良" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "圆锥隔前" } ] }, { "text": "临床可通过左侧经胸行水肿修补术,是否做肺动脉环缩术视情况而定。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "pro", "entity": "左侧经胸行水肿修补术" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "肺动脉环缩术" } ] }, { "text": "大动脉转换术可紧接在水肿修补术后进行。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "水肿修补术" } ] }, { "text": "圆锥隔前向性对位不良引起明显主动脉瓣下狭窄者需要手术解除,当梗阻严重时则需在右室-肺动脉间放置人工带瓣管道。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "圆锥隔前向性对位不良" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "圆锥隔前" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "明显主动脉瓣下狭窄" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "动脉瓣下狭窄" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "梗阻严重" }, { "start_idx": 38, "end_idx": 52, "type": "pro", "entity": "右室-肺动脉间放置人工带瓣管道" } ] }, { "text": "【预后】未经治疗的大动脉转位患儿临床多表现危重;新生儿早期患儿常见的致死因素包括组织缺氧和酸中毒;在婴儿期为充血性心力衰竭,幼儿期则是以血栓栓塞和渐进性肺血管疾病为主。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "组织缺氧" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "组织" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "酸中毒" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "血栓栓塞" }, { "start_idx": 73, "end_idx": 80, "type": "dis", "entity": "渐进性肺血管疾病" } ] }, { "text": "(一)Mustard和Senning术Sarker等在对226名Mustard手术和132名Senning手术患儿术后长期疗效的比较研究中,发现Senning术术后生存率较Mustard术高。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "pro", "entity": "Mustard和Senning术" }, { "start_idx": 32, "end_idx": 40, "type": "pro", "entity": "Mustard手术" }, { "start_idx": 46, "end_idx": 54, "type": "pro", "entity": "Senning手术" }, { "start_idx": 72, "end_idx": 79, "type": "pro", "entity": "Senning术" }, { "start_idx": 86, "end_idx": 93, "type": "pro", "entity": "Mustard术" } ] }, { "text": "心房转换术术后可出现进行性的窦性心率丧失。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "心房转换术" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "进行性的窦性心率丧失" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "心" } ] }, { "text": "据报道,大约30%~40%行心房转换术(包括Mustard术和Senning术)患儿在术后10年出现节性心律。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "心房转换术" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "Mustard术" }, { "start_idx": 31, "end_idx": 38, "type": "pro", "entity": "Senning术" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "节性心律" } ] }, { "text": "主要的心律失常有交界性心动过缓、窦性心动过缓、室上性心动过速,尤其是房性扑动。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心律失常" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "交界性心动过缓" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "窦性心动过缓" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "房性扑动" } ] }, { "text": "如需监测严重心动过缓的发作和当用药物控制伴严重心动过缓的心动过速患儿时,需做24小时心电监护。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "监测严重心动过缓的发作" }, { "start_idx": 15, "end_idx": 31, "type": "pro", "entity": "用药物控制伴严重心动过缓的心动过速" }, { "start_idx": 38, "end_idx": 45, "type": "pro", "entity": "24小时心电监护" } ] }, { "text": "随访中人工心房内隧道的问题也逐渐出现。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "equ", "entity": "人工心房内隧道" } ] }, { "text": "体循环静脉通道梗阻可无明显的临床症状;而肺静脉通道梗阻时可导致进行性呼吸窘迫、咯血。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "体循环静脉通道梗阻" }, { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "体循环静脉" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "肺静脉通道梗阻" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肺静脉" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "进行性呼吸窘迫" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "咯血" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "血" } ] }, { "text": "球囊膨胀可暂时缓解梗阻状况,在体静脉通路中植入支架可起到较好的效果,在肺静脉通路植入的效果却不理想。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "球囊膨胀" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "球囊" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "梗阻" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "体静脉通路" }, { "start_idx": 23, "end_idx": 24, "type": "equ", "entity": "支架" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "肺静脉通路" } ] }, { "text": "术后随访血管造影有时会见到小的心房内板障瘘,但通常可不予以处理。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "血管造影" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "小的心房内板障瘘" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "心房内板" } ] }, { "text": "对有严重的通道梗阻或补片漏的患儿则需再次手术。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "严重的通道梗阻" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "通道" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "补片漏" } ] }, { "text": "另一需要注意的是术后形态上的右心室长期作为功能左心室的承受能力。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "右心室" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "左心室" } ] }, { "text": "少数约占10%的患儿可出现严重的右心室功能低下,通常还伴发严重的三尖瓣反流。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "严重的右心室功能低下" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "右心室" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "严重的三尖瓣反流" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "三尖瓣" } ] }, { "text": "对于这些患儿,可选择心脏移植或行大动脉转换术,之前如有必要可先行肺动脉环缩术以重新训练左室功能。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "心脏移植" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "肺动脉环缩术" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "左室" } ] }, { "text": "(二)大动脉转换术现在大动脉转换术术后死亡率已不足10%,是目前治疗大动脉转位最常用的一种手术方式。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "大动脉转位" } ] }, { "text": "而伴有室间隔缺损或Taussig-Bing畸形的患儿术后1年、10年、15年生存率分别为81%、80%、80%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 9, "end_idx": 22, "type": "dis", "entity": "Taussig-Bing畸形" } ] }, { "text": "就远期效果而言,行大动脉转换术患儿术后心律失常的发生率少于行心房转位术者,在早期和中期的随访中,超声心动图及心血管造影均提示左心室功能良好。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "心律失常" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "心房转位术" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "超声心动图" }, { "start_idx": 54, "end_idx": 58, "type": "pro", "entity": "心血管造影" }, { "start_idx": 62, "end_idx": 66, "type": "ite", "entity": "左心室功能" } ] }, { "text": "再次手术的原因有肺动脉狭窄、冠状动脉狭窄、转位后的主动脉反流、残留水肿、腔静脉栓塞;在复杂的大血管转位,有时需安装起搏器、关闭心内残余分流以及残余左心室流出道梗阻的疏通等。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "冠状动脉狭窄" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "转位后的主动脉反流" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "主动脉" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "残留水肿" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "腔静脉栓塞" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "大血管转位" }, { "start_idx": 57, "end_idx": 59, "type": "equ", "entity": "起搏器" }, { "start_idx": 61, "end_idx": 68, "type": "pro", "entity": "关闭心内残余分流" }, { "start_idx": 71, "end_idx": 83, "type": "pro", "entity": "残余左心室流出道梗阻的疏通" } ] }, { "text": "肺动脉瓣上狭窄是最常见并发症,随着手术技巧的提高,此发生率已有所下降。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肺动脉瓣上狭窄" } ] }, { "text": "相反,Lecompte术对肺动脉的牵拉引起的肺动脉分支狭窄却少见,球囊扩张术有时效果不明显,支架植入有望可获得长期的效果。", "entities": [ { "start_idx": 3, "end_idx": 28, "type": "sym", "entity": "Lecompte术对肺动脉的牵拉引起的肺动脉分支狭窄" }, { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "Lecompte术" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺动脉" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "肺动脉分支狭窄" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "球囊扩张术" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "支架植入" } ] }, { "text": "新主动脉瓣(原肺动脉瓣)长期功能目前尚不清楚。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "肺动脉瓣" } ] }, { "text": "尽管大多数患儿在术后早期主动脉反流轻微,但一些研究表明新主动脉瓣的反流会随时间而逐渐增多。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "主动脉反流轻微" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "主动脉" }, { "start_idx": 28, "end_idx": 43, "type": "sym", "entity": "主动脉瓣的反流会随时间而逐渐增多" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "主动脉瓣" } ] }, { "text": "大动脉转位后的冠状动脉能否长期生长和保持通畅受到人们的关注。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "尽管大多数患儿的冠脉可保持良好的状态,但有报道大动脉转换术后晚期死亡的主要原因是冠状动脉狭窄所致,冠状动脉梗阻的发生率为7.8%。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "冠脉" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "冠状动脉狭窄" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "冠状动脉梗阻" } ] }, { "text": "尤其重要的是,冠状动脉狭窄或梗阻患儿可无临床症状。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "冠状动脉狭窄或梗阻" } ] }, { "text": "用心肌灌注扫描方法检测心肌灌注不良尚有一定的争议,选择性冠状血管造影术可准确显示冠状动脉梗阻,可作为大动脉转换术后远期了解冠状动脉通畅程度的理想方法。", "entities": [ { "start_idx": 1, "end_idx": 16, "type": "pro", "entity": "心肌灌注扫描方法检测心肌灌注不良" }, { "start_idx": 25, "end_idx": 34, "type": "pro", "entity": "选择性冠状血管造影术" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "冠状动脉梗阻" }, { "start_idx": 50, "end_idx": 55, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "现在,Nikaidoh术和REV术也开始应用于临床,主要针对大动脉转位合并室间隔缺损及严重的肺动脉狭窄而不能行Switch术或Rastelli术的患儿。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "Nikaidoh术" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "REV术" }, { "start_idx": 30, "end_idx": 41, "type": "dis", "entity": "大动脉转位合并室间隔缺损" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 55, "end_idx": 61, "type": "pro", "entity": "Switch术" }, { "start_idx": 63, "end_idx": 71, "type": "pro", "entity": "Rastelli术" } ] }, { "text": "附:重叠综合征重叠综合征(overlappingsyndrome)是指同一个患者同时或先后患有两种或两种以上的风湿性疾病。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "重叠综合征" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "重叠综合征" }, { "start_idx": 13, "end_idx": 31, "type": "dis", "entity": "overlappingsyndrome" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "但它不同于MCTD。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "MCTD" } ] }, { "text": "尽管MCTD临床上有多种病的重叠症状,但它有其自身的诊断标准和特点。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "MCTD" } ] }, { "text": "重叠综合征临床表现为同一时期内并存两种以上风湿性疾病,如系统性红斑狼疮与结节性多动脉炎并存,系统性红斑狼疮与系统性硬化症并存,幼年型特发性关节炎除有严重关节炎和皮下小结外,还有抗核抗体阳性和血管炎,即与系统性红斑狼疮并存。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "重叠综合征" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "结节性多动脉炎" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "系统性硬化症" }, { "start_idx": 63, "end_idx": 71, "type": "dis", "entity": "幼年型特发性关节炎" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "关节炎" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "皮下小结" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "皮下" }, { "start_idx": 88, "end_idx": 93, "type": "sym", "entity": "抗核抗体阳性" }, { "start_idx": 88, "end_idx": 91, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "血管炎" }, { "start_idx": 101, "end_idx": 107, "type": "dis", "entity": "系统性红斑狼疮" } ] }, { "text": "此外,系统性硬化症也可出现抗核抗体阳性、严重关节炎、白细胞减少及溶血性贫血等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "系统性硬化症" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "抗核抗体阳性" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "严重关节炎" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "白细胞减少" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "白细胞" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "重叠综合征也可先后出现两种以上风湿性疾病,如患皮肌炎多年后,又出现双手指关节炎,可视为皮肌炎与特发性关节炎重叠。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "重叠综合征" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "双手指关节炎" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "特发性关节炎" } ] }, { "text": "系统性红斑狼疮的病程进展过程中,又出现了典型的类风湿关节炎的关节畸形,可诊断为系统性红斑狼疮和特发性关节炎的重叠。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "类风湿关节炎" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "关节畸形" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "特发性关节炎" } ] }, { "text": "应用药物应取决于以何种风湿性疾病为主。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "主要药物为肾上腺皮质激素和免疫抑制剂,剂量和用法参照各有关风湿性疾病节。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "本综合征的预后较单一的风湿性疾病要差。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "六、血管内支架在先天性心脏病中的应用血管狭窄性病变可通过球囊血管成形术扩张,但由于血管壁的弹性回缩,球囊导管回撤后血管腔会回到扩张前状态。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "血管内支架" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "血管狭窄性病变" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "球囊血管成形术" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "血管壁" }, { "start_idx": 50, "end_idx": 53, "type": "equ", "entity": "球囊导管" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "血管腔" } ] }, { "text": "虽然20世纪60年代末Dotter首先提出这一设想,并将一螺旋弹簧圈植入实验的周围动脉狭窄,但直至20世纪80年代初才设计出球囊扩张型及自膨胀性支架并应用于临床。", "entities": [ { "start_idx": 27, "end_idx": 35, "type": "pro", "entity": "将一螺旋弹簧圈植入" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "周围动脉狭窄" }, { "start_idx": 62, "end_idx": 73, "type": "equ", "entity": "球囊扩张型及自膨胀性支架" } ] }, { "text": "最初,支架被用于成人周围动脉病变和冠状动脉狭窄性病变。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "equ", "entity": "支架" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "周围动脉病变" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "冠状动脉狭窄性病变" } ] }, { "text": "此后,这一技术被扩展至儿童分支肺动脉狭窄、术后体静脉梗阻及其他先天性与获得性狭窄性病变的治疗。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "儿童分支肺动脉狭窄" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "术后体静脉梗阻" }, { "start_idx": 31, "end_idx": 42, "type": "dis", "entity": "先天性与获得性狭窄性病变" } ] }, { "text": "目前临床上应用的支架有两大类:球囊扩张型支架及自膨胀性支架。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "equ", "entity": "支架" }, { "start_idx": 15, "end_idx": 21, "type": "equ", "entity": "球囊扩张型支架" }, { "start_idx": 23, "end_idx": 28, "type": "equ", "entity": "自膨胀性支架" } ] }, { "text": "前者包括Palmaz-Schatz、Roubin、Strecker、Wiktor、Freedom、Cordis、ACS多链等。", "entities": [ { "start_idx": 4, "end_idx": 16, "type": "equ", "entity": "Palmaz-Schatz" }, { "start_idx": 18, "end_idx": 23, "type": "equ", "entity": "Roubin" }, { "start_idx": 25, "end_idx": 32, "type": "equ", "entity": "Strecker" }, { "start_idx": 34, "end_idx": 39, "type": "equ", "entity": "Wiktor" }, { "start_idx": 41, "end_idx": 47, "type": "equ", "entity": "Freedom" }, { "start_idx": 49, "end_idx": 54, "type": "equ", "entity": "Cordis" }, { "start_idx": 56, "end_idx": 60, "type": "equ", "entity": "ACS多链" } ] }, { "text": "迄今为止,Palmaz-Schatz临床应用最多。", "entities": [ { "start_idx": 5, "end_idx": 17, "type": "equ", "entity": "Palmaz-Schatz" } ] }, { "text": "在自膨胀性支架中,Wallstent和NitinolInStent已接受试验。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "equ", "entity": "自膨胀性支架" }, { "start_idx": 9, "end_idx": 17, "type": "equ", "entity": "Wallstent" }, { "start_idx": 19, "end_idx": 32, "type": "equ", "entity": "NitinolInStent" } ] }, { "text": "Wallstent由于有致血凝性及内膜增殖反应不再应用。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "equ", "entity": "Wallstent" }, { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "致血凝性及内膜增殖反应" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "血" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "内膜" } ] }, { "text": "自膨胀性InStent利用镍钛合金的热形态记忆,其初步的临床结果是令人鼓舞的。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "equ", "entity": "自膨胀性InStent" } ] }, { "text": "在儿科临床实践中大多数应用球囊扩张性Palmaz-Schatz支架。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dep", "entity": "儿科" }, { "start_idx": 13, "end_idx": 32, "type": "equ", "entity": "球囊扩张性Palmaz-Schatz支架" } ] }, { "text": "在儿科患儿中应用最多的是先天性或外科术后肺动脉分支狭窄的支架植入治疗。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dep", "entity": "儿科" }, { "start_idx": 12, "end_idx": 26, "type": "dis", "entity": "先天性或外科术后肺动脉分支狭窄" }, { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "支架植入治疗" } ] }, { "text": "即刻和短期结果肯定:血管直径增加,跨梗阻处峰值收缩压差下降,定量肺灌注扫描示流向支架植入肺的血流增加,右心室压下降。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "血管直径增加" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血管" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "跨梗阻处峰值收缩压差下降" }, { "start_idx": 17, "end_idx": 26, "type": "ite", "entity": "跨梗阻处峰值收缩压差" }, { "start_idx": 30, "end_idx": 49, "type": "sym", "entity": "定量肺灌注扫描示流向支架植入肺的血流增加" }, { "start_idx": 30, "end_idx": 35, "type": "pro", "entity": "定量肺灌注扫" }, { "start_idx": 40, "end_idx": 41, "type": "equ", "entity": "支架" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "肺的血流" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "右心室压下降" }, { "start_idx": 51, "end_idx": 54, "type": "ite", "entity": "右心室压" } ] }, { "text": "在短期随访中,大约30%的病人需要再次支架扩张,但效果良好。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "支架扩张" } ] }, { "text": "支架植入也已用于术后体静脉阻塞、右心室流出道管道阻塞、长段水肿术后和少数未经外科手术的水肿以及肺静脉狭窄性病变的治疗,成功率不一。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "支架植入" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "术后体静脉阻塞" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "右心室流出道管道阻塞" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "长段水肿术后" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "未经外科手术的水肿" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "外科手术" }, { "start_idx": 47, "end_idx": 54, "type": "dis", "entity": "肺静脉狭窄性病变" } ] }, { "text": "在肺动脉闭锁的患儿,支架植入动脉导管可维持其开放以避免或推迟分流手术,而在左心发育不良综合征的患儿支架植入动脉导管可作为心脏移植的过渡治疗方法。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "肺动脉闭锁" }, { "start_idx": 10, "end_idx": 17, "type": "pro", "entity": "支架植入动脉导管" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "分流手术" }, { "start_idx": 37, "end_idx": 45, "type": "dis", "entity": "左心发育不良综合征" }, { "start_idx": 49, "end_idx": 56, "type": "pro", "entity": "支架植入动脉导管" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "心脏移植" } ] }, { "text": "支架植入也用于维持狭窄的主肺动脉侧支血管的开放以增加伴肺动脉闭锁复杂先心病的肺血供。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "支架植入" }, { "start_idx": 9, "end_idx": 19, "type": "bod", "entity": "狭窄的主肺动脉侧支血管" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "肺动脉闭锁" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "复杂先心病" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "肺" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "血" } ] }, { "text": "尽管支架有较常规的球囊血管成形术明确的优点,但仍有一些问题有待将来解决,如释放鞘大、支架纵向僵硬、血栓形成、新内膜增殖、患儿生长问题等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "equ", "entity": "支架" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "球囊血管成形术" }, { "start_idx": 42, "end_idx": 43, "type": "equ", "entity": "支架" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "血栓形成" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "血" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "新内膜增殖" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "新内膜" } ] }, { "text": "附:维生素D过多症长期大量服用或短期超量误服维生素D或对维生素D过于敏感,均可引起维生素D过多症(hypervitaminosisD),临床上出现以高钙血症引起的临床中毒综合征。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "维生素D过多症" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "维生素D" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "维生素D" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "维生素D过多症" }, { "start_idx": 49, "end_idx": 65, "type": "dis", "entity": "hypervitaminosisD" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "高钙血症" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "中毒综合征" } ] }, { "text": "中毒剂量个体差异很大,与维生素D的剂量、应用时间和给药途径有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "中毒" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "维生素D" } ] }, { "text": "通常每天摄入维生素D3000~8000U1~3个月可出现中毒症状。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "维生素D" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "中毒" } ] }, { "text": "【临床表现】主要系因血钙过高和钙盐沉积于身体各组织器官所致。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血钙" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "血钙过高" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "钙盐" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "钙盐沉积于身体各组织器官" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "身体各组织器官" } ] }, { "text": "【实验室检查】血钙增高(>3.0mmol/L),尿钙增加,尿蛋白阳性,血尿素氮增高。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "血钙增高" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "血钙" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "尿钙增加" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "尿钙" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "尿蛋白阳性" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "血尿素氮增高" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "血尿素氮" } ] }, { "text": "X线长骨摄片,临时钙化带过度钙化、密度增高,骨皮质增厚,骨小梁密度增高而模糊,其他组织器官可出现异位钙化灶。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "X线长骨摄片" }, { "start_idx": 7, "end_idx": 20, "type": "sym", "entity": "临时钙化带过度钙化、密度增高" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "临时钙化带" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "骨皮质增厚" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "骨皮质" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "骨小梁密度增高而模糊" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "骨小梁" }, { "start_idx": 39, "end_idx": 52, "type": "sym", "entity": "其他组织器官可出现异位钙化灶" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "其他组织器官" } ] }, { "text": "【处理】立即停用维生素D,处理高钙血症,限制钙盐摄入,给利尿剂加速钙的排泄,同时应用泼尼松或氢氧化铝抑制肠道对钙的吸收。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "维生素D" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "高钙血症" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "限制钙盐摄入" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "利尿剂" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "钙" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "泼尼松" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "氢氧化铝" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "肠道" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "钙" } ] }, { "text": "亦可试用合成降钙素50~100U/d,皮下或肌内注射。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "合成降钙素" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "皮下或肌内注射" } ] }, { "text": "注意水及电解质平衡。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "注意水及电解质平衡" } ] }, { "text": "【预防】加强宣传使家长了解维生素D并非滋补药,应掌握用量及时间。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "维生素D" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "滋补药" } ] }, { "text": "二、胱氨酸尿症胱氨酸尿症(cystinuria)是一种家族性遗传性疾病,为常染色体隐性遗传,是由近端肾小管上皮细胞及空肠黏膜对二碱基氨基酸(包括赖氨酸和精氨酸)及胱氨酸等转运障碍所致。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "胱氨酸尿症" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "胱氨酸尿症" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "cystinuria" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "家族性遗传性疾病" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "常染色体" }, { "start_idx": 48, "end_idx": 56, "type": "bod", "entity": "近端肾小管上皮细胞" }, { "start_idx": 48, "end_idx": 88, "type": "sym", "entity": "近端肾小管上皮细胞及空肠黏膜对二碱基氨基酸(包括赖氨酸和精氨酸)及胱氨酸等转运障碍" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "空肠黏膜" }, { "start_idx": 63, "end_idx": 68, "type": "bod", "entity": "二碱基氨基酸" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "赖氨酸" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "精氨酸" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "胱氨酸" } ] }, { "text": "本病临床罕见,发病率国外统计为1/7000(纯合子为1/40000),男女发病均等,男性症状重,可能与男性泌尿系解剖不同有关。", "entities": [ { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "泌尿系" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "解剖" } ] }, { "text": "【病因与发病机制】本病为常染色体隐性遗传病,由于近端肾小管对胱氨酸回吸收障碍而导致尿中胱氨酸浓度异常增高,在酸性尿中形成大量结石。", "entities": [ { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "常染色体隐性遗传病" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "近端肾小管" }, { "start_idx": 24, "end_idx": 37, "type": "sym", "entity": "近端肾小管对胱氨酸回吸收障碍" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "吸收障碍" }, { "start_idx": 41, "end_idx": 51, "type": "sym", "entity": "尿中胱氨酸浓度异常增高" }, { "start_idx": 41, "end_idx": 47, "type": "ite", "entity": "尿中胱氨酸浓度" }, { "start_idx": 54, "end_idx": 63, "type": "sym", "entity": "酸性尿中形成大量结石" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "尿" } ] }, { "text": "氨基酸跨细胞膜转运由相关转运系统完成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "氨基酸" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "细胞膜" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "转运系统" } ] }, { "text": "该转运单位由氨基酸转运蛋白异聚体组成,包含一重一轻的2个亚单位,自1992年来已发现2种重的亚单位即rBAT及4F2hc,并且很快发现胱氨酸尿症病人存在rBAT基因即SLC3A1突变,后来证实编码rBAT的基因SLC3A1突变是引起Ⅰ型胱氨酸尿症的原因。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "bod", "entity": "氨基酸转运蛋白异聚体" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "胱氨酸尿症" }, { "start_idx": 76, "end_idx": 81, "type": "bod", "entity": "rBAT基因" }, { "start_idx": 83, "end_idx": 88, "type": "bod", "entity": "SLC3A1" }, { "start_idx": 83, "end_idx": 90, "type": "sym", "entity": "SLC3A1突变" }, { "start_idx": 96, "end_idx": 110, "type": "bod", "entity": "编码rBAT的基因SLC3A1" }, { "start_idx": 96, "end_idx": 112, "type": "sym", "entity": "编码rBAT的基因SLC3A1突变" }, { "start_idx": 116, "end_idx": 122, "type": "dis", "entity": "Ⅰ型胱氨酸尿症" } ] }, { "text": "近2年又陆续发现7种轻的亚单位即LAT-1、LAF-2、asc-1、y+LAT-1、Y+LAT-2、xCT和b(0,+)AT,其中b(0,+)AT由SLC7A9基因编码,其突变是引起Ⅱ型、Ⅲ型胱氨酸尿症的原因。", "entities": [ { "start_idx": 74, "end_idx": 81, "type": "bod", "entity": "SLC7A9基因" }, { "start_idx": 86, "end_idx": 87, "type": "sym", "entity": "突变" }, { "start_idx": 91, "end_idx": 100, "type": "dis", "entity": "Ⅱ型、Ⅲ型胱氨酸尿症" } ] }, { "text": "最初认为rBAT与b(0,+)AT一起组成胱氨酸转运单位,但免疫组化分析显示rBAT主要分布在近端小管直部,而b(0,+)AT则在近曲小管中最丰富,因此也有可能rBAT与其他轻的亚单位组成胱氨酸转运单位,而b(0,+)AT也同样与其他重的亚单位组成转运单位。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 30, "end_idx": 35, "type": "pro", "entity": "免疫组化分析" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "近端小管直部" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "近曲小管中" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "胱氨酸" } ] }, { "text": "肾内大量的胱氨酸结石形成后,通过梗阻、压迫或者诱发感染损害肾组织,使病人肾功能减退及尿量减少,尿中胱氨酸更易形成新的结石,如此恶性循环,最终有可能导致肾衰竭。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "肾内大量的胱氨酸结石形成" }, { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "肾内大量的胱氨酸" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "梗阻" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "压迫" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "感染" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "损害肾组织" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "肾功能" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "肾功能减退" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "尿量减少" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "尿量" }, { "start_idx": 47, "end_idx": 59, "type": "sym", "entity": "尿中胱氨酸更易形成新的结石" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "尿中胱氨酸" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "【临床表现】病人出生后即发病,常在双肾出现大量结石时才确诊,根据尿中氨基酸的含量及临床表现可分为Ⅰ、Ⅱ、Ⅲ三型。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "双肾" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "双肾出现大量结石" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "尿中氨基酸" } ] }, { "text": "临床特征如下:(一)尿路胱氨酸结石反复、多发的大量结石是本病的特征。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "尿路胱氨酸结石" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "反复、多发的大量结石" } ] }, { "text": "原因是胱氨酸在酸性尿中溶解度很低,大量胱氨酸,超过尿中的饱和浓度时,形成结石。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 3, "end_idx": 15, "type": "sym", "entity": "胱氨酸在酸性尿中溶解度很低" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "尿" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "结石" } ] }, { "text": "胱氨酸结石呈黄棕色,较硬,大小不等,大者可呈鹿角形,在腹部平片上呈淡薄阴影。", "entities": [ { "start_idx": 0, "end_idx": 36, "type": "sym", "entity": "胱氨酸结石呈黄棕色,较硬,大小不等,大者可呈鹿角形,在腹部平片上呈淡薄阴影" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "腹部平片" } ] }, { "text": "后期可出现高血压,甚至肾衰竭。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "高血压" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "(二)生长发育障碍由于氨基酸丢失引起营养障碍,导致生长迟缓及智能障碍。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "生长发育障碍" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "氨基酸丢失" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "氨基酸" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "营养障碍" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "生长迟缓" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "智能障碍" } ] }, { "text": "(三)少数病人可合并高尿酸血症、遗传性低钙血症、血友病、肌萎缩、遗传性胰腺炎及色素性视网膜炎等。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "高尿酸血症" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "遗传性低钙血症" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "血友病" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肌萎缩" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "遗传性胰腺炎" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "色素性视网膜炎" } ] }, { "text": "【实验室检查】尿中含大量胱氨酸、赖氨酸、精氨酸及鸟氨酸。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "尿" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "赖氨酸" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "精氨酸" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "鸟氨酸" } ] }, { "text": "每日尿胱氨酸增多达730mg/g尿肌酐(正常最高值约18mg/g尿肌酐)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "每日尿胱氨酸增多" }, { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "每日尿胱氨酸" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "尿肌酐" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "尿肌酐" } ] }, { "text": "【诊断】根据临床表现,家族史及尿中排出大量胱氨酸即可确诊。", "entities": [ { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "尿中排出大量胱氨酸" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "胱氨酸" } ] }, { "text": "1.尿胱氨酸结晶检查取晨尿作离心沉淀,光镜下可见六角形扁平状与苯环相似的结晶。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "ite", "entity": "尿胱氨酸结晶检查" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "晨尿" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "离心沉淀" }, { "start_idx": 19, "end_idx": 20, "type": "equ", "entity": "光镜" }, { "start_idx": 19, "end_idx": 37, "type": "sym", "entity": "光镜下可见六角形扁平状与苯环相似的结晶" } ] }, { "text": "结晶出现常提示尿胱氨酸浓度超过200~250mg/L。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "结晶出现" }, { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "尿胱氨酸浓度" }, { "start_idx": 7, "end_idx": 25, "type": "sym", "entity": "尿胱氨酸浓度超过200~250mg/L" } ] }, { "text": "2.氰化硝普钠试验将结石研成粉末,放少许于试管中,加1滴浓氨水,然后再加1滴5%氰化钠,5分钟后再加3滴5%硝普钠,如立即呈现特征性深樱桃红色为阳性,表示存在胱氨酸。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "氰化硝普钠试验" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "结石" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "浓氨水" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "5%氰化钠" }, { "start_idx": 52, "end_idx": 56, "type": "dru", "entity": "5%硝普钠" }, { "start_idx": 59, "end_idx": 70, "type": "sym", "entity": "立即呈现特征性深樱桃红色" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "胱氨酸" } ] }, { "text": "但同型胱氨酸、丙酮酸、全氨基酸尿及某些药物可使该试验呈假阳性,应注意鉴别。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "同型胱氨酸" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "丙酮酸" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "全氨基酸尿" } ] }, { "text": "此外,因尿排胱氨酸可呈波动性,需注意排除假阴性。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胱氨酸" } ] }, { "text": "3.尿胱氨酸高效液相色谱法定量测定对确诊及分型有帮助。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "pro", "entity": "尿胱氨酸高效液相色谱法定量测定" } ] }, { "text": "【治疗】本病为遗传性疾病,无根治办法。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "遗传性疾病" } ] }, { "text": "(一)饮食控制采用低蛋氨酸(胱氨酸最重要的前身)饮食,可在一定程度上减少尿中胱氨酸的含量。", "entities": [ { "start_idx": 3, "end_idx": 25, "type": "pro", "entity": "饮食控制采用低蛋氨酸(胱氨酸最重要的前身)饮食" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "尿中胱氨酸" } ] }, { "text": "(二)增加饮水量多饮水,尤其夜间,以防止尿浓缩时析出胱氨酸结晶。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "pro", "entity": "增加饮水量多饮水,尤其夜间" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "尿浓缩时析出胱氨酸结晶" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "胱氨酸结晶" } ] }, { "text": "每日摄水量至少在4000ml以上,尽量使尿胱氨酸稀释,浓度保持在250mg/L以下,可以防止结石的形成。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "pro", "entity": "每日摄水量至少在4000ml以上" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "尿胱氨酸" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "结石" } ] }, { "text": "(三)碱化尿液服枸橼酸钠或碳酸氢钠,以碱化尿液(使尿pH>7.5),可增加胱氨酸溶解度,防止结石形成。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "碱化尿液" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "枸橼酸钠" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "碱化尿液" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "尿pH" }, { "start_idx": 37, "end_idx": 42, "type": "ite", "entity": "胱氨酸溶解度" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "结石" } ] }, { "text": "一般尿pH7.5时,胱氨酸的溶解度最高,但有促进磷酸钙沉积的危险。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "尿pH" }, { "start_idx": 10, "end_idx": 16, "type": "ite", "entity": "胱氨酸的溶解度" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "促进磷酸钙沉积" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "磷酸钙" } ] }, { "text": "(四)药物治疗D-青霉胺(D-penicillamine)是β-二甲基半胱氨酸,它可使尿中游离胱氨酸减少约50%,同时又可与胱氨酸作用生成可溶性的半胱氨酸-青霉胺二硫化合物从尿中排出,故能防止结石形成。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "药物治疗" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "D-青霉胺" }, { "start_idx": 13, "end_idx": 27, "type": "dru", "entity": "D-penicillamine" }, { "start_idx": 30, "end_idx": 38, "type": "dru", "entity": "β-二甲基半胱氨酸" }, { "start_idx": 43, "end_idx": 51, "type": "sym", "entity": "尿中游离胱氨酸减少" }, { "start_idx": 43, "end_idx": 49, "type": "bod", "entity": "尿中游离胱氨酸" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 69, "end_idx": 85, "type": "bod", "entity": "可溶性的半胱氨酸-青霉胺二硫化合物" }, { "start_idx": 87, "end_idx": 87, "type": "bod", "entity": "尿" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "结石" } ] }, { "text": "巯基丙酚甘氨酸(mercaptopropionglalycine)作用同青霉胺,但毒性较小。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "巯基丙酚甘氨酸" }, { "start_idx": 8, "end_idx": 31, "type": "dru", "entity": "mercaptopropionglalycine" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "青霉胺" } ] }, { "text": "(五)肾结石治疗可考虑用体外震波碎石或手术取石,解除梗阻,保护肾功能。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肾结石" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "体外震波碎石" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "手术取石" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "梗阻" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肾" } ] }, { "text": "(六)并发症治疗包括防治尿路感染及尿路梗阻,尿毒症则予以透析或肾移植等治疗。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "并发症治疗" }, { "start_idx": 10, "end_idx": 20, "type": "pro", "entity": "防治尿路感染及尿路梗阻" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "尿毒症" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "透析" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "肾移植" } ] }, { "text": "第八章遗传性肾脏疾病第一节遗传性进行性肾炎遗传性肾炎,又称Alport综合征(Alport’ssyndrome,AS),属一种家族性慢性进行性肾炎,临床特征以血尿为主,部分病例可表现为蛋白尿或肾病综合征。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "遗传性肾脏疾病" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "遗传性进行性肾炎" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "遗传性肾炎" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "Alport综合征" }, { "start_idx": 39, "end_idx": 54, "type": "dis", "entity": "Alport’ssyndrome" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "AS" }, { "start_idx": 63, "end_idx": 72, "type": "dis", "entity": "家族性慢性进行性肾炎" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "血尿" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "血" }, { "start_idx": 80, "end_idx": 80, "type": "bod", "entity": "尿" }, { "start_idx": 92, "end_idx": 94, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "蛋白" }, { "start_idx": 94, "end_idx": 94, "type": "bod", "entity": "尿" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "常伴有神经性听力障碍及进行性肾功能减退。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "神经性听力障碍" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "进行性肾功能减退" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "肾功能" } ] }, { "text": "且近十年来随着分子生物学的迅猛发展,对于AS的研究已进入分子水平和基因水平。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "AS" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "基因" } ] }, { "text": "【病因及遗传学】AS是一种以血尿及进行性肾脏损害为主要表现的肾小球基底膜(basementmembrane,BM)病,可伴有眼、耳等肾外表现。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "AS" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "血尿" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "进行性肾脏损害" }, { "start_idx": 30, "end_idx": 57, "type": "dis", "entity": "肾小球基底膜(basementmembrane,BM)病" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "眼" }, { "start_idx": 62, "end_idx": 69, "type": "sym", "entity": "眼、耳等肾外表现" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "耳" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "肾外" } ] }, { "text": "电镜下,基底膜表现为弥漫性厚薄不均,可有分层现象,此为特征性的病理改变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "电镜下,基底膜表现为弥漫性厚薄不均,可有分层现象" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "基底膜" } ] }, { "text": "病变的根本原因为基底膜的重要组分Ⅳ型胶原不同α链(α1-α6)的突变,其编码基因称为COL4A1-COL4A6,分别位于不同的染色体上。", "entities": [ { "start_idx": 8, "end_idx": 33, "type": "sym", "entity": "基底膜的重要组分Ⅳ型胶原不同α链(α1-α6)的突变" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "基底膜" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "Ⅳ型胶原不同α链" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "α1-α6" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "编码基因" }, { "start_idx": 42, "end_idx": 54, "type": "bod", "entity": "COL4A1-COL4A6" }, { "start_idx": 60, "end_idx": 66, "type": "bod", "entity": "不同的染色体上" } ] }, { "text": "约85%AS病人为性连锁显性遗传(XLinkedAS,XLAS),致病基因定位于X染色体长臂中段Xq22,为编码Ⅳ型胶原α5链(COL4A5)基因突变所致;其余病人为常染色体隐性遗传(autosomalrecessiveAS,ARAS)和常染色体显性遗传AS(autosomaldominantAS,ADAS),前者致病基因为COL4A3或COL4A4基因,而后者具有遗传多源性。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "AS" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "性连锁显性遗传" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "XLinkedAS" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "XLAS" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "致病基因" }, { "start_idx": 40, "end_idx": 51, "type": "bod", "entity": "X染色体长臂中段Xq22" }, { "start_idx": 54, "end_idx": 74, "type": "sym", "entity": "编码Ⅳ型胶原α5链(COL4A5)基因突变" }, { "start_idx": 54, "end_idx": 72, "type": "bod", "entity": "编码Ⅳ型胶原α5链(COL4A5)基因" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "常染色体" }, { "start_idx": 119, "end_idx": 122, "type": "bod", "entity": "常染色体" }, { "start_idx": 158, "end_idx": 161, "type": "bod", "entity": "致病基因" }, { "start_idx": 163, "end_idx": 177, "type": "bod", "entity": "COL4A3或COL4A4基因" } ] }, { "text": "运用免疫组织化学技术,检测肾组织及皮肤组织中Ⅳ型胶原不同α链的表达,可帮助临床上诊断此病及确定遗传方式。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "免疫组织化学技术" }, { "start_idx": 11, "end_idx": 32, "type": "pro", "entity": "检测肾组织及皮肤组织中Ⅳ型胶原不同α链的表达" } ] }, { "text": "另一方面,人们不仅确定了Alport综合征的致病基因,且已通过各种方法检测出了300余种XLAS和数种ARAS的突变基因,并开始逐步探讨患者基因型与表现型之间的关系。", "entities": [ { "start_idx": 12, "end_idx": 25, "type": "bod", "entity": "Alport综合征的致病基因" }, { "start_idx": 39, "end_idx": 59, "type": "bod", "entity": "300余种XLAS和数种ARAS的突变基因" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "基因" } ] }, { "text": "然而在发病机制方面,从Ⅳ型胶原编码基因上单个或多个碱基突变,直至BM上的相应病变,由于缺乏动态的、系统的研究,人们尚不清楚这其中的许多具体环节。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "bod", "entity": "Ⅳ型胶原编码基因" }, { "start_idx": 11, "end_idx": 28, "type": "sym", "entity": "Ⅳ型胶原编码基因上单个或多个碱基突变" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "BM上的相应病变" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "BM上" } ] }, { "text": "【病理】早期肾脏体积正常或增大,病情进展后肾脏体积逐渐缩小。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肾脏" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "肾脏体积正常或增大" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肾脏" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "肾脏体积逐渐缩小" } ] }, { "text": "在光镜下肾小球正常或轻度上皮细胞增生及系膜基质增加。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "equ", "entity": "光镜" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肾小球" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "轻度上皮细胞增生" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "系膜基质" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "系膜基质增加" } ] }, { "text": "晚期系膜细胞增生,肾小球和肾小管基膜增厚,分层,包氏囊壁增厚并发展到肾小球硬化。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "系膜细胞增生" }, { "start_idx": 9, "end_idx": 22, "type": "sym", "entity": "肾小球和肾小管基膜增厚,分层" }, { "start_idx": 9, "end_idx": 17, "type": "bod", "entity": "肾小球和肾小管基膜" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "包氏囊壁" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "包氏囊壁增厚" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "肾小球硬化" } ] }, { "text": "肾小管细胞萎缩,或伴部分扩张,可有蛋白管型。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肾小管细胞萎缩" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "肾小管细胞萎缩,或伴部分扩张" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "蛋白" } ] }, { "text": "间质可有灶性炎症细胞浸润,也可发展到纤维化。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "间质" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "间质可有灶性炎症细胞浸润" }, { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "灶性炎症细胞" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "纤维化" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "纤维" } ] }, { "text": "40%病例在皮髓质交界处的间质中有泡沫细胞浸润。", "entities": [ { "start_idx": 6, "end_idx": 22, "type": "sym", "entity": "皮髓质交界处的间质中有泡沫细胞浸润" }, { "start_idx": 6, "end_idx": 15, "type": "bod", "entity": "皮髓质交界处的间质中" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "泡沫细胞" } ] }, { "text": "此种泡沫细胞的胞浆含有中性脂肪、黏多糖、胆固醇及磷脂。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "泡沫细胞的胞浆" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "中性脂肪" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "黏多糖" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "胆固醇" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "磷脂" } ] }, { "text": "电子显微镜检查典型者为肾小球及肾小管基膜变薄及不规则增厚。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "电子显微镜" }, { "start_idx": 11, "end_idx": 27, "type": "sym", "entity": "肾小球及肾小管基膜变薄及不规则增厚" }, { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "肾小球及肾小管基膜" } ] }, { "text": "肾小球增厚的基膜伴致密层分裂,或重叠板层样改变,其间含有电子致密颗粒。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "肾小球增厚的基膜" }, { "start_idx": 0, "end_idx": 33, "type": "sym", "entity": "肾小球增厚的基膜伴致密层分裂,或重叠板层样改变,其间含有电子致密颗粒" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "致密层" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "电子致密颗粒" } ] }, { "text": "近年来发现肾小球基底膜(GBM)有变薄、增厚及两者相间病变。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "肾小球基底膜" }, { "start_idx": 5, "end_idx": 28, "type": "sym", "entity": "肾小球基底膜(GBM)有变薄、增厚及两者相间病变" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "GBM" } ] }, { "text": "变薄的GBM可达正常厚度的1/4,而增厚的GBM可达正常厚度的2~5倍,为节段性变薄与增厚的GBM并存。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "变薄的GBM可达正常厚度的1/4" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "GBM" }, { "start_idx": 18, "end_idx": 34, "type": "sym", "entity": "增厚的GBM可达正常厚度的2~5倍" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "GBM" }, { "start_idx": 37, "end_idx": 50, "type": "sym", "entity": "节段性变薄与增厚的GBM并存" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "GBM" } ] }, { "text": "肾小球上皮部分足突可以融合或伴微绒毛形成。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "肾小球上皮部分足突可以融合或伴微绒毛形成" }, { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "肾小球上皮部分足突" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "微绒毛" } ] }, { "text": "免疫荧光检查通常为阴性。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "免疫荧光检查" } ] }, { "text": "偶尔也能见到某些免疫球蛋白如IgM、补体C3等在肾小球内轻度沉积;一般认为系对病变肾小球内的非特异性黏附,无致病意义。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "IgM" }, { "start_idx": 14, "end_idx": 37, "type": "sym", "entity": "IgM、补体C3等在肾小球内轻度沉积" }, { "start_idx": 18, "end_idx": 27, "type": "bod", "entity": "补体C3" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "肾小球内" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "病变肾小球内" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "肾小球内" } ] }, { "text": "【临床表现】(一)肾脏表现多数以持续性或间歇性血尿为主要表现,血尿为肾小球性。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肾脏" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "持续性或间歇性血尿" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "血" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "尿" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "血尿为肾小球性" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "血" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "尿" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "肾小球" } ] }, { "text": "也可表现为程度不等的蛋白尿以及肾病样蛋白尿,常于急性感染时加剧。", "entities": [ { "start_idx": 5, "end_idx": 20, "type": "sym", "entity": "程度不等的蛋白尿以及肾病样蛋白尿" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "蛋白" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肾" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "蛋白" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" } ] }, { "text": "受累男孩的发病可早在出生后第一年,血压升高的发生率和严重性,随年龄而增加,且多发生于男孩。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "血压升高" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "血压" } ] }, { "text": "受累男孩肾脏几乎全部将发展至终末期肾脏病,但进展速度各家系之间有差异,根据终末期肾衰竭的发生年龄可分为青少年型(31岁前发生)和成年型(31岁以后发生)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "终末期肾脏病" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "终末期肾衰竭" } ] }, { "text": "(二)神经性耳聋约30%~40%患者可伴有高频区(4000~8000Hz)神经性耳聋,随着年龄的增长,小儿于学龄期逐渐出现以上症状,男性尤多见。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "神经性耳聋" }, { "start_idx": 21, "end_idx": 41, "type": "dis", "entity": "高频区(4000~8000Hz)神经性耳聋" } ] }, { "text": "两侧耳聋程度可以不完全对称,但为进行性的,耳聋将渐及全音域。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "两侧耳聋程度可以不完全对称,但为进行性的" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "两侧耳" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "耳聋将渐及全音域" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "耳" } ] }, { "text": "脑干电测听显示听力障碍发生于耳蜗部位。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "脑干电测听" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "听力障碍" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "耳蜗" } ] }, { "text": "(三)眼病变约15%患者合并有眼病变,最具特征性的眼部异常为前圆锥形晶状体,即晶状体中央部位突向前房。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "眼病变" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "眼病变" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "眼部异常" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "眼部" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "前圆锥形晶状体" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "晶状体" }, { "start_idx": 39, "end_idx": 45, "type": "bod", "entity": "晶状体中央部位" }, { "start_idx": 39, "end_idx": 49, "type": "sym", "entity": "晶状体中央部位突向前房" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "前房" } ] }, { "text": "确认这一病变常需经眼科裂隙灯检查。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "眼科裂隙灯检查" } ] }, { "text": "其他常见的眼部异常为黄斑周围色素改变,在黄斑区中心凹周围有致密微粒沉着,先天性白内障及眼球震颤等。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "眼部异常" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "眼部" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "黄斑周围色素改变" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "黄斑周围" }, { "start_idx": 20, "end_idx": 34, "type": "sym", "entity": "黄斑区中心凹周围有致密微粒沉着" }, { "start_idx": 20, "end_idx": 27, "type": "bod", "entity": "黄斑区中心凹周围" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "致密微粒" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "先天性白内障" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "眼球震颤" } ] }, { "text": "(四)血液系统异常与肾脏病相关的巨血小板减少症已有报道,此症患者血涂片血小板计数多在(30×109~70×109)/L,血小板呈球形,临床可表现有轻度出血倾向,但极少发生术后严重出血现象。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "血液系统" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "血液系统异常" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾脏病" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "巨血小板减少症" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "血涂片" }, { "start_idx": 35, "end_idx": 39, "type": "ite", "entity": "血小板计数" }, { "start_idx": 82, "end_idx": 84, "type": "ite", "entity": "血小板" }, { "start_idx": 82, "end_idx": 87, "type": "sym", "entity": "血小板呈球形" }, { "start_idx": 95, "end_idx": 100, "type": "sym", "entity": "轻度出血倾向" }, { "start_idx": 98, "end_idx": 98, "type": "bod", "entity": "血" }, { "start_idx": 107, "end_idx": 112, "type": "sym", "entity": "术后严重出血" }, { "start_idx": 112, "end_idx": 112, "type": "bod", "entity": "血" } ] }, { "text": "同时外周血涂片还可见粒细胞甚至巨噬细胞内包涵体。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "外周血涂片" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "粒细胞" }, { "start_idx": 15, "end_idx": 22, "type": "bod", "entity": "巨噬细胞内包涵体" } ] }, { "text": "(五)弥漫性平滑肌瘤某些青少年型Alport综合征家系或患者伴有显著的平滑肌肥大,受累部位常为食管、气管和女性生殖道(如阴蒂、大阴唇及子宫等),出现相应的症状,如吞咽困难及呼吸困难等。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "弥漫性平滑肌瘤" }, { "start_idx": 12, "end_idx": 24, "type": "dis", "entity": "青少年型Alport综合征" }, { "start_idx": 32, "end_idx": 70, "type": "sym", "entity": "显著的平滑肌肥大,受累部位常为食管、气管和女性生殖道(如阴蒂、大阴唇及子宫等)" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "平滑肌" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "食管" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "气管" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "女性生殖道" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "阴蒂" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "大阴唇" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "子宫" }, { "start_idx": 81, "end_idx": 84, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 86, "end_idx": 89, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "【诊断】(一)常用临床症状、体征结合病理学检查来诊断①血尿,多数伴有不等量的蛋白尿。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "血尿" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "蛋白" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "尿" } ] }, { "text": "血尿以镜下为主,或伴以间歇性发作的肉眼血尿。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "血尿" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "尿" }, { "start_idx": 3, "end_idx": 3, "type": "equ", "entity": "镜" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "间歇性发作的肉眼血尿" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" } ] }, { "text": "以肾病综合征形式出现者也需提高警惕。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "③伴同症状,如高频区神经性耳聋,和(或)眼部白内障,圆锥形晶状体等都有诊断价值。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "高频区神经性耳聋" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "眼部白内障" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "圆锥形晶状体" } ] }, { "text": "④肾脏病理显示肾小球基膜广泛不规则增厚、劈裂,并与变薄的基膜并存。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "肾脏病理" }, { "start_idx": 1, "end_idx": 31, "type": "sym", "entity": "肾脏病理显示肾小球基膜广泛不规则增厚、劈裂,并与变薄的基膜并存" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "肾小球基膜" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "基膜" } ] }, { "text": "间质可有较多的泡沫细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "间质" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "间质可有较多的泡沫细胞浸润" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "泡沫细胞" } ] }, { "text": "这种细胞也可见于慢性肾炎及类脂性肾病的膜性肾小球肾炎,但在数量及分布部位上(皮、髓质交界处较多特点也有助于诊断。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "类脂性肾病" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "膜性肾小球肾炎" }, { "start_idx": 38, "end_idx": 44, "type": "bod", "entity": "皮、髓质交界处" } ] }, { "text": "以上几项条件中如有3项即可诊断,但其中病理变化中肾小球基膜的改变不可缺少。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "肾小球基膜" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "肾小球基膜的改变" } ] }, { "text": "(二)基因诊断近年应用分子生物学技术研究Alport基因,尤其是COL4A5基因(长约240碱基,51个外显子),诊断可达基因水平。", "entities": [ { "start_idx": 20, "end_idx": 27, "type": "bod", "entity": "Alport基因" }, { "start_idx": 32, "end_idx": 39, "type": "bod", "entity": "COL4A5基因" } ] }, { "text": "此外,对肾脏组织和皮肤活检组织检测Ⅳ型胶原的α5链的表达,如在上皮的基膜上缺乏也可高度拟为X连锁显性遗传性肾炎,敏感率约75%。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "肾脏组织和皮肤活检" }, { "start_idx": 13, "end_idx": 38, "type": "pro", "entity": "组织检测Ⅳ型胶原的α5链的表达" }, { "start_idx": 42, "end_idx": 49, "type": "sym", "entity": "上皮的基膜上缺乏" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "上皮的基膜上" }, { "start_idx": 56, "end_idx": 65, "type": "dis", "entity": "X连锁显性遗传性肾炎" } ] }, { "text": "【鉴别诊断】(一)良性家族性血尿病人可有阳性家族史。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "良性家族性血尿" } ] }, { "text": "临床主要表现为无症状性单纯性血尿,肾脏病变不呈进行性,故又名良性血尿。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "无症状性单纯性血尿" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "肾脏病变" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "良性血尿" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "血" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "尿" } ] }, { "text": "病理改变光镜下正常。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "equ", "entity": "光镜" } ] }, { "text": "电镜下特征为弥漫性GBM变薄,为正常人的1/3~2/3厚度,肾小球内无电子致密物沉积。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "弥漫性GBM变薄" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "GBM" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "肾小球内" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "电子致密物沉积" } ] }, { "text": "近年来发现家族性薄基底膜病(TBMD)的Ⅳ型胶原α4(COL4A4)有变异,有些性连锁型AS病人可同时合并有薄基底膜病变。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "家族性薄基底膜病" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "TBMD" }, { "start_idx": 20, "end_idx": 54, "type": "sym", "entity": "Ⅳ型胶原α4(COL4A4)有变异" }, { "start_idx": 20, "end_idx": 31, "type": "bod", "entity": "Ⅳ型胶原α4" }, { "start_idx": 33, "end_idx": 50, "type": "bod", "entity": "COL4A4" }, { "start_idx": 58, "end_idx": 63, "type": "dis", "entity": "性连锁型AS" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "薄基底膜病变" } ] }, { "text": "(二)IgA肾病临床表现主要为反复发作的血尿,少数病例仅呈无症状性蛋白尿,或血尿合并有蛋白尿,甚至为肾炎性肾病症状。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "反复发作的血尿" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "尿" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "无症状性蛋白尿" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "蛋白" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "尿" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "血尿" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "血" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "尿" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "蛋白" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "尿" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "肾炎性肾病症状" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "肾" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "肾" } ] }, { "text": "可有高血压、水肿及大量蛋白尿。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "高血压" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "水肿" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "蛋白" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" } ] }, { "text": "病理改变以系膜增生性肾炎为多见,其次为轻微病变及局灶增生性肾炎。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "系膜增生性肾炎" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "轻微病变" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "局灶增生性肾炎" } ] }, { "text": "少数病例呈弥漫增生性肾炎及灶性新月体形成,有时肾小球基膜变薄,该病定义即在免疫病理中显示单纯IgA或以IgA为主的免疫球蛋白沉积于肾小球系膜区。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "弥漫增生性肾炎" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "灶性新月体形成" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "肾小球基膜变薄" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "肾小球基膜" }, { "start_idx": 37, "end_idx": 70, "type": "sym", "entity": "免疫病理中显示单纯IgA或以IgA为主的免疫球蛋白沉积于肾小球系膜区" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "免疫病理" }, { "start_idx": 44, "end_idx": 61, "type": "bod", "entity": "单纯IgA或以IgA为主的免疫球蛋白" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "肾小球系膜区" } ] }, { "text": "一般呈颗粒状或块状分布,部分病例在毛细血管壁上也有IgA沉积,而遗传性肾炎则无免疫沉积。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "颗粒状或块状分布" }, { "start_idx": 17, "end_idx": 29, "type": "sym", "entity": "毛细血管壁上也有IgA沉积" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "毛细血管壁上" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "IgA" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "遗传性肾炎" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "免疫沉积" } ] }, { "text": "肾活体组织检查在电镜下无遗传性肾炎的各种病变。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 8, "end_idx": 9, "type": "equ", "entity": "电镜" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "遗传性肾炎的各种病变" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "遗传性肾炎" } ] }, { "text": "【治疗】遗传性肾炎为一先天性遗传性疾病,至今尚无特效治疗。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "遗传性肾炎" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "先天性遗传性疾病" } ] }, { "text": "以对症及控制并发症为主,预防继发性尿路感染。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "继发性尿路感染" } ] }, { "text": "遇有感染时避免应用肾毒性药物。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "感染" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "肾毒性药物" } ] }, { "text": "终末期肾衰竭阶段则依靠透析疗法维持生命,等待肾移植。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "终末期肾衰竭" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "透析疗法" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "肾移植" } ] }, { "text": "由于Alport综合征病人体内缺乏基膜抗原,在肾移植后可产生抗GBM的抗体,以致移植肾发生抗GBM肾炎(Goodpasture综合征)。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "Alport综合征" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "缺乏基膜抗原" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "基膜抗原" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "肾移植" }, { "start_idx": 23, "end_idx": 36, "type": "sym", "entity": "肾移植后可产生抗GBM的抗体" }, { "start_idx": 30, "end_idx": 36, "type": "bod", "entity": "抗GBM的抗体" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "移植肾" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "抗GBM肾炎" }, { "start_idx": 52, "end_idx": 65, "type": "dis", "entity": "Goodpasture综合征" } ] }, { "text": "故有作者主张对这些病人进行肾移植后应密切追踪尿常规、肾功能及血清抗GBM抗体,至少追踪1年。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "肾移植" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "尿常规" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "肾功能" }, { "start_idx": 30, "end_idx": 37, "type": "ite", "entity": "血清抗GBM抗体" } ] }, { "text": "此期生长发育特点:①男孩骨骼肌肉系统增长大于女孩,女孩的脂肪组织多于男孩;②女性的第二性征出现常早于男孩;③同性别的不同个体的生长发育速率各不相同;④此期的大脑思维能力非常活跃,从家庭走向学校和社会,适应能力和独立意识增强。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "骨骼肌肉系统" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "脂肪组织" } ] }, { "text": "此期身高的增长主要是长骨的生长,骨骼的生长要有充足的钙质,每天需从膳食中摄取钙元素1000mg才能满足机体的需要。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "长骨" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨骼" } ] }, { "text": "另外,由于目前许多处于青春发育期的青少年(尤其是男孩)食欲很好,长期过多食用高热能的食品而发生肥胖,从而增加了成人期患心血管和代谢性疾病的隐患。", "entities": [ { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "肥胖" }, { "start_idx": 59, "end_idx": 67, "type": "dis", "entity": "心血管和代谢性疾病" } ] }, { "text": "七、脊髓内肿瘤脊髓内肿瘤占儿童中枢神经系统肿瘤3%~6%,平均发病年龄10岁,包括星形细胞瘤(70%)、少突胶质细胞瘤和神经节胶质瘤(10%)、室管膜瘤(10%)及恶性胶质瘤(10%),其中多数为高度肿瘤。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "脊髓内肿瘤" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "脊髓内肿瘤" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "儿童中枢神经系统肿瘤" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "星形细胞瘤" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "少突胶质细胞瘤" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "神经节胶质瘤" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "室管膜瘤" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "恶性胶质瘤" }, { "start_idx": 98, "end_idx": 101, "type": "dis", "entity": "高度肿瘤" } ] }, { "text": "肿瘤多涉及数个椎体平面,压迫、侵蚀正常脊髓组织。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "椎体平面" }, { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "压迫、侵蚀正常脊髓组织" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "脊髓组织" } ] }, { "text": "低度肿瘤起病较晚,诊断前常已发病数月,而高度肿瘤起病较快,常为几个星期。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "低度肿瘤起病" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "高度肿瘤" } ] }, { "text": "主要临床症状为疼痛、肢体肌力减退、步态不稳和括约肌功能障碍肿瘤附近背部区域,呈钝痛或刀割样痛,夜间以及用力咳嗽或打喷嚏时加重。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "疼痛" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "肢体肌力减退" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肢体" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "步态不稳" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "括约肌功能障碍" }, { "start_idx": 29, "end_idx": 45, "type": "sym", "entity": "肿瘤附近背部区域,呈钝痛或刀割样痛" }, { "start_idx": 29, "end_idx": 36, "type": "bod", "entity": "肿瘤附近背部区域" }, { "start_idx": 47, "end_idx": 61, "type": "sym", "entity": "夜间以及用力咳嗽或打喷嚏时加重" } ] }, { "text": "手术是治疗的主要手段,应在不加重神经损伤的基础上尽可能切除。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "神经损伤" } ] }, { "text": "低度的星形细胞瘤,经综合治疗,10年生存率达55%~73%,而室管膜瘤达50%~70%,间变性或高度恶性肿瘤生存率极低,常于诊断后数月死亡。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "低度的星形细胞瘤" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "室管膜瘤" }, { "start_idx": 44, "end_idx": 53, "type": "dis", "entity": "间变性或高度恶性肿瘤" } ] }, { "text": "第三节脑面血管瘤病脑面血管瘤病(encephalotrigeminalangiomatosis)又称脑三叉神经血管瘤病或Sturge-Weber综合征,是以面部血管痣、对侧肢体抽搐、偏瘫、同侧颅内钙化、眼球突出或青光眼以及脑部血管畸形、智力低下为特征的一种先天性疾病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "脑面血管瘤病" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "脑面血管瘤病" }, { "start_idx": 16, "end_idx": 46, "type": "dis", "entity": "encephalotrigeminalangiomatosis" }, { "start_idx": 50, "end_idx": 58, "type": "dis", "entity": "脑三叉神经血管瘤病" }, { "start_idx": 60, "end_idx": 74, "type": "dis", "entity": "Sturge-Weber综合征" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "面部血管痣" }, { "start_idx": 84, "end_idx": 89, "type": "sym", "entity": "对侧肢体抽搐" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "对侧肢体" }, { "start_idx": 91, "end_idx": 92, "type": "dis", "entity": "偏瘫" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "同侧颅内钙化" }, { "start_idx": 94, "end_idx": 97, "type": "bod", "entity": "同侧颅内" }, { "start_idx": 101, "end_idx": 104, "type": "sym", "entity": "眼球突出" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "眼球" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "青光眼" }, { "start_idx": 111, "end_idx": 116, "type": "dis", "entity": "脑部血管畸形" }, { "start_idx": 118, "end_idx": 121, "type": "sym", "entity": "智力低下" }, { "start_idx": 128, "end_idx": 132, "type": "dis", "entity": "先天性疾病" } ] }, { "text": "本病较神经纤维瘤病及结节性硬化症少见。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "神经纤维瘤病" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "结节性硬化症" } ] }, { "text": "其发病机制系先天性外、中胚层发育障碍所致,与神经纤维瘤病和结节性硬化症同属斑痣性错构瘤病(phacomatosis)或母斑病。", "entities": [ { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "先天性外、中胚层发育障碍" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "外、中胚层" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "神经纤维瘤病" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "结节性硬化症" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "斑痣性错构瘤病" }, { "start_idx": 45, "end_idx": 56, "type": "dis", "entity": "phacomatosis" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "母斑病" } ] }, { "text": "【临床表现】(一)面部血管痣出生后即有,呈灰红或紫红色,压之不褪色,边缘清楚,扁平或略凹陷。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "面部血管痣" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "面部" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "呈灰红或紫红色" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "压之不褪色" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "边缘清楚" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "扁平或略凹陷" } ] }, { "text": "多位于颜面一侧,偶有两侧。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "颜面" } ] }, { "text": "常沿三叉神经Ⅰ、Ⅱ支范围分布,也可波及第Ⅲ支。", "entities": [ { "start_idx": 1, "end_idx": 21, "type": "sym", "entity": "沿三叉神经Ⅰ、Ⅱ支范围分布,也可波及第Ⅲ支" }, { "start_idx": 2, "end_idx": 11, "type": "bod", "entity": "三叉神经Ⅰ、Ⅱ支范围" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "第Ⅲ支" } ] }, { "text": "有些病例并不按三叉神经范围分布。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "三叉神经范围分布" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "三叉神经范围" } ] }, { "text": "因血管痣的部位与三叉神经的部位相似,以往将本病称为脑三叉神经血管瘤病,实际上与三叉神经无关。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "血管痣" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "三叉神经" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "脑三叉神经血管瘤病" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "三叉神经" } ] }, { "text": "(二)神经系统损害多数患儿在生后数月或数年内神经系统无异常,通常在2~3岁时因发热而诱发出现惊厥或偏瘫。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "神经系统损害" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "神经系统" }, { "start_idx": 38, "end_idx": 50, "type": "sym", "entity": "因发热而诱发出现惊厥或偏瘫" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "惊厥" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "偏瘫" } ] }, { "text": "发作后可有Todd瘫痪,多次发作后可遗有永久性偏瘫。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "Todd瘫痪" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "永久性偏瘫" } ] }, { "text": "约30%~50%的病例其血管痣对侧有中枢性偏瘫,以及偏瘫侧肢体较正常侧发育慢。", "entities": [ { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "血管痣对侧有中枢性偏瘫" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "血管痣对侧" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "中枢性偏瘫" }, { "start_idx": 26, "end_idx": 37, "type": "sym", "entity": "偏瘫侧肢体较正常侧发育慢" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "偏瘫侧肢体" } ] }, { "text": "部分患儿只表现为发作性一过性肢体无力,而无惊厥发作。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "发作性一过性肢体无力" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肢体" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "惊厥" } ] }, { "text": "本病约有一半病人智力受损,双侧脑病变者仅有8%的病人智力正常。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "智力受损" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "双侧脑病变" } ] }, { "text": "智力损害的程度轻重不等,难治性癫痫智力影响较大,偶发惊厥者对智力影响较小。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "智力损害的程度轻重不等" }, { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "难治性癫痫智力影响较大" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "难治性癫痫" }, { "start_idx": 24, "end_idx": 35, "type": "sym", "entity": "偶发惊厥者对智力影响较小" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "惊厥" } ] }, { "text": "部分病例可出现行为障碍。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "行为障碍" } ] }, { "text": "(三)眼部损害40%病人有青光眼,常与面部血管痣同侧。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "眼部损害" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "青光眼" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "面部血管痣" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "面部血管" } ] }, { "text": "双侧面部血管痣的患儿往往出现双侧青光眼。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "双侧面部血管痣" }, { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "双侧面部血管" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "青光眼" } ] }, { "text": "大面积面部血管痣的病人合并青光眼机会较大。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "大面积面部血管痣" }, { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "大面积面部血管" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "青光眼" } ] }, { "text": "青光眼可在出生时出现,也可在出生后数年才被发现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "青光眼" } ] }, { "text": "此外,还可有眼球突出、同侧偏盲(枕叶受累)、角膜血管翳、晶状体混浊、脉络膜血管痣、视网膜血管瘤、视网膜血管怒张、视网膜剥离、视神经萎缩以及视力减退等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "眼球" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "眼球突出" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "同侧偏盲(枕叶受累)" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "枕叶" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "角膜血管" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "角膜血管翳" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "晶状体混浊" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "晶状体" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "脉络膜血管痣" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "视网膜血管瘤" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "视网膜血管" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "视网膜血管怒张" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "视网膜" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "视网膜剥离" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "视神经萎缩" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "视力减退" } ] }, { "text": "(四)其他异常有些病人可伴有内脏血管瘤而引起胃肠道出血或血尿,也有合并其他先天性畸形,如下颌前突、脊柱裂或隐睾等。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "内脏血管瘤" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "胃肠道出血" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "血尿" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "血" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "先天性畸形" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "下颌前突" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "下颌" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "脊柱裂" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "脊柱" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "隐睾" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "睾" } ] }, { "text": "【诊断】有典型面部皮肤改变、癫痫发作及青光眼三主征者,易于作出诊断。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "面部皮肤改变" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "面部皮肤" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "癫痫发作" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "癫痫" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "青光眼" } ] }, { "text": "如仅有皮肤和眼部改变,或仅有癫痫、智力低下等神经系统征象者,需作头颅X线片、颅脑CT或MRI等辅助检查,以协助诊断。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "皮肤和眼部改变" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "眼部" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "癫痫" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "智力低下" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "神经系统征象" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "神经系统" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "头颅X线片" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "颅脑CT" }, { "start_idx": 43, "end_idx": 45, "type": "pro", "entity": "MRI" } ] }, { "text": "头颅X线片可显示颅内钙化影,呈脑回状、线状或双轨状,其中与脑表面外形一致的双轨状钙化影是特征性改变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "头颅X线片" }, { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "头颅X线片可显示颅内钙化影,呈脑回状、线状或双轨状" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "颅内" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脑回" }, { "start_idx": 28, "end_idx": 42, "type": "sym", "entity": "与脑表面外形一致的双轨状钙化影" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "脑表面" } ] }, { "text": "颅内钙化影可见于大脑各叶皮质,多位于一侧,双侧也有报道。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "颅内" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "颅内钙化影" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "大脑各叶皮质" } ] }, { "text": "颅脑CT平扫可见团块状混杂密度病灶,边缘不清,可有钙化影及局部脑萎缩;增强扫描可见异常血管强化影。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "颅脑CT平扫" }, { "start_idx": 0, "end_idx": 33, "type": "sym", "entity": "颅脑CT平扫可见团块状混杂密度病灶,边缘不清,可有钙化影及局部脑萎缩" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "局部脑萎缩" }, { "start_idx": 35, "end_idx": 47, "type": "sym", "entity": "增强扫描可见异常血管强化影" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "增强扫描" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "血管" } ] }, { "text": "颅脑MRI检查在T1和T2加权像均显示低信号影,对钙化的显示不如CT扫描,但可显示软脑膜血管瘤。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "颅脑MRI检查" }, { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "颅脑MRI检查在T1和T2加权像均显示低信号影" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "钙化" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "CT扫描" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "软脑膜血管瘤" } ] }, { "text": "PET和SPECT也可见软脑膜血管瘤。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "PET" }, { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "SPECT" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "软脑膜血管瘤" } ] }, { "text": "MRA和DSA有助于脑部畸形血管的定位及定性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "MRA" }, { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "DSA" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "脑部畸形血管" } ] }, { "text": "青光眼和突眼可手术治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "青光眼" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "突眼" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "手术治疗" } ] }, { "text": "面部血管痣可行整容手术或激光治疗。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "面部血管痣" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "面部血管" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "整容手术" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "激光治疗" } ] }, { "text": "偏瘫患儿可行神经康复治疗。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "偏瘫" }, { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "神经康复治疗" } ] }, { "text": "第十二篇泌尿系统疾病第一章小儿泌尿系统的解剖生理特点第一节解剖特点泌尿系统为一组重要的泌尿排泄器官,也是调节机体水、电解质平衡,维持机体内环境稳定和分泌某些重要激素的一组器官。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "泌尿系统疾病" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "泌尿系统的解剖" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "解剖" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 43, "end_idx": 48, "type": "bod", "entity": "泌尿排泄器官" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "水" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "电解质" }, { "start_idx": 66, "end_idx": 70, "type": "bod", "entity": "机体内环境" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "激素" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "一组器官" } ] }, { "text": "泌尿系统包括肾脏、输尿管、膀胱和尿道。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肾脏" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "输尿管" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "膀胱" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "尿道" } ] }, { "text": "(一)肾脏肾脏呈蚕豆形,左右各一,位于脊柱两侧,居腹膜后方,右肾上方有肝脏,故位置略低于左肾。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肾脏" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "脊柱两侧" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "腹膜后方" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "右肾上方" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肝脏" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "左肾" } ] }, { "text": "左肾上极平第11胸椎,下极平第2腰椎,右肾的上、下极则均低于左肾一个胸椎或一个腰椎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "左肾上极" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "第11胸椎" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "第2腰椎" }, { "start_idx": 19, "end_idx": 25, "type": "bod", "entity": "右肾的上、下极" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "左肾" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "一个胸椎" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "一个腰椎" } ] }, { "text": "新生儿肾脏重约24g,长6cm;至成人,重约150g,长12cm。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" } ] }, { "text": "新生儿肾脏表面凹凸不平,呈分叶状,由于位置较低,因此2岁以下幼儿肾脏容易在腹部扪及;以后随着身长增高,肾脏位置逐渐升高而达腰部。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肾脏" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "腹部" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肾脏" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "腰部" } ] }, { "text": "肾脏的外面有三层被膜,即肾筋膜、肾脂肪囊和肾纤维膜。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肾脏的外面" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "三层被膜" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肾筋膜" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "肾脂肪囊" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "肾纤维膜" } ] }, { "text": "纤维膜紧贴肾实质,易于剥离。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "纤维膜" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾实质" } ] }, { "text": "肾脏内缘中部凹陷,称为肾门,是血管、神经和输尿管出入的门户。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "肾脏内缘中部" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾门" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血管" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "神经" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "输尿管" } ] }, { "text": "肾门以内是肾实质围成的腔隙,称为肾窦,肾窦包括肾盂、肾盏、肾动脉及肾静脉的主要分支及它们周围的疏松结缔组织和脂肪组织。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肾门以内" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾实质" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肾窦" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肾窦" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肾盂" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾盏" }, { "start_idx": 29, "end_idx": 57, "type": "bod", "entity": "肾动脉及肾静脉的主要分支及它们周围的疏松结缔组织和脂肪组织" } ] }, { "text": "肾脏实质由皮质和髓质组成。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肾脏实质" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "皮质" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "髓质" } ] }, { "text": "皮质位于表层,占肾实质的外1/3,内2/3为髓质。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮质" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肾实质" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "髓质" } ] }, { "text": "髓质由8~18个肾锥体组成,呈圆锥状,尖端朝向肾窦,形成肾乳头,底部朝向外侧,与皮质相连,据其结构特点,髓质可分为内带和外带。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "髓质" }, { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "8~18个肾锥体" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肾窦" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肾乳头" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "皮质" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "髓质" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "内带" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "外带" } ] }, { "text": "皮质和髓质并非截然分开,皮质中有许多条髓质放射状插入髓质,称皮质髓放线,髓放线之间的肾皮质称皮质迷路。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮质" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "髓质" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "皮质" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "许多条髓质" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "髓质" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "皮质髓放线" }, { "start_idx": 36, "end_idx": 44, "type": "bod", "entity": "髓放线之间的肾皮质" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "皮质迷路" } ] }, { "text": "部分肾皮质伸入肾锥体之间,称为肾柱。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肾皮质" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾锥体" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肾柱" } ] }, { "text": "肾单位(nephron)是肾脏的基本结构和功能单位,每个肾脏约有100多万个肾单位。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肾单位" }, { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "nephron" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肾脏" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肾脏" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "肾单位" } ] }, { "text": "肾单位由肾小体及其下属的近端肾小管、髓袢的降支和升支以及远端肾小管组成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肾单位" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肾小体" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "近端肾小管" }, { "start_idx": 18, "end_idx": 25, "type": "bod", "entity": "髓袢的降支和升支" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "远端肾小管" } ] }, { "text": "来自腹主动脉的肾动脉在肾门处分为前后两支,再在肾窦内分成3~4条段动脉,继而分成叶间动脉穿行于肾柱之间。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "来自腹主动脉的肾动脉" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肾门处" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肾窦" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "3~4条段动脉" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "叶间动脉" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肾柱" } ] }, { "text": "叶间动脉分支横向走行于皮髓质交界处,称弓状动脉。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "叶间动脉" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "皮髓质交界处" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "弓状动脉" } ] }, { "text": "弓状动脉再发出多个分支放射状伸入皮质迷路,称小叶间动脉,小叶间动脉发出侧支称为入球小动脉,在肾小球内形成毛细血管球,再继而会合成出球小动脉。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "弓状动脉" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "皮质迷路" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "小叶间动脉" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "小叶间动脉" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "入球小动脉" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "肾小球内" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "毛细血管球" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "出球小动脉" } ] }, { "text": "皮质肾单位的出球小动脉再分支形成皮质的肾小管周围毛细血管网,然后会集进入小叶间静脉、弓状静脉以及叶间静脉,最后形成肾静脉。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "bod", "entity": "皮质肾单位的出球小动脉" }, { "start_idx": 16, "end_idx": 28, "type": "bod", "entity": "皮质的肾小管周围毛细血管网" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "小叶间静脉" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "弓状静脉" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "叶间静脉" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "肾静脉" } ] }, { "text": "(二)输尿管婴幼儿输尿管较长而弯曲,管壁肌肉及弹力纤维发育不良,容易受压及扭曲而导致梗阻,造成尿潴留而诱发感染。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "输尿管" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "输尿管" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "管壁肌肉" }, { "start_idx": 18, "end_idx": 30, "type": "sym", "entity": "管壁肌肉及弹力纤维发育不良" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "弹力纤维" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "发育不良" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "扭曲" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "梗阻" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "尿潴留" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "感染" } ] }, { "text": "(三)膀胱婴儿膀胱位置比年长儿及成人高,尿液充盈时可达腹腔内,触诊时容易扪到,随年龄增长逐渐下降至盆腔内。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "膀胱" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "膀胱" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "尿液" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "腹腔内" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "触诊" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "盆腔内" } ] }, { "text": "因此婴儿患尿路感染时可经腹腔穿刺膀胱而获尿液培养。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "尿路感染" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "经腹腔穿刺膀胱" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "尿液培养" } ] }, { "text": "膀胱受脊髓和大脑控制,在正确教养下可于1岁半左右白天养成控制排尿的习惯。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "膀胱" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "脊髓" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "大脑" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "尿" } ] }, { "text": "(四)尿道新生女婴尿道仅长1cm(性成熟期为3~5cm),外口暴露且接近肛门,易受细菌污染。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "尿道" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "尿道" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肛门" }, { "start_idx": 41, "end_idx": 42, "type": "mic", "entity": "细菌" } ] }, { "text": "男婴尿道较长,但常有包茎,积垢时也可引起细菌上行性感染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "尿道" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "包茎" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "积垢" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "细菌上行性感染" } ] }, { "text": "小儿出生后,随着生长发育和代谢的改变,1~2岁时肾脏形态及功能接近成人水平。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肾脏" } ] }, { "text": "第四节先天性肌营养不良先天性肌营养不良(congentialmusculardystrophies,CMDs)为一组不同类型的疾病,其分类(表16-16)。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "先天性肌营养不良" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "先天性肌营养不良" }, { "start_idx": 20, "end_idx": 48, "type": "dis", "entity": "congentialmusculardystrophies" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "CMDs" } ] }, { "text": "表16-15先天性肌营养不良(CMDs)目前有3种类型的基因缺陷已确立,它们是Laminin-α2链缺乏、α7缺乏和Fukuyama肌营养不良。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "先天性肌营养不良" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "CMDs" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "3种类型的基因缺陷" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "3种类型的基因" }, { "start_idx": 39, "end_idx": 51, "type": "dis", "entity": "Laminin-α2链缺乏" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "α7缺乏" }, { "start_idx": 58, "end_idx": 70, "type": "dis", "entity": "Fukuyama肌营养不良" } ] }, { "text": "近年对Lamininα2链缺乏型CMD、FukuyamaCMD、Walker-Warburg综合征以及肌-眼-脑病研究较多。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "dis", "entity": "Lamininα2链缺乏型CMD" }, { "start_idx": 20, "end_idx": 30, "type": "dis", "entity": "FukuyamaCMD" }, { "start_idx": 32, "end_idx": 48, "type": "dis", "entity": "Walker-Warburg综合征" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "肌-眼-脑病" } ] }, { "text": "(一)Laminin-α2链缺乏型CMD此型是常染色体隐性遗传病。", "entities": [ { "start_idx": 3, "end_idx": 19, "type": "dis", "entity": "Laminin-α2链缺乏型CMD" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "常染色体隐性遗传病" } ] }, { "text": "其基因定位于6q22-22,称为LAMA2基因,在某些患者中已证实有该基因的特殊突变(点突变及小缺失)。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "LAMA2基因" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "基因的特殊突变" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "基因" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "点突变" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "小缺失" } ] }, { "text": "本病常于出生后或生后数周出现症状,表现为肌张力低下,运动发育迟缓,关节挛缩,少数患者可出现先天性髋脱位。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "肌张力" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "运动发育迟缓" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "关节挛缩" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "关节" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "先天性髋脱位" } ] }, { "text": "几乎一半患者出现严重的功能障碍,终身不能独立行走。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "严重的功能障碍" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "功能障碍" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "终身不能独立行走" } ] }, { "text": "有些患者除了有脑白质改变外,尚有皮质发育不良和小多脑回等病变。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "脑白质改变" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "脑白质" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "皮质发育不良" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "小多脑回" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "脑回" } ] }, { "text": "大多数患者无智能受损,只有少数患者出现癫痫或智能迟缓。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "智能受损" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "癫痫" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "智能迟缓" } ] }, { "text": "患者血清CK变化很大,病程早期CK水平趋于升高,可达正常值的6~7倍;病程晚期,血清CK逐渐下降,趋于正常。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "血清CK变化很大" }, { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "血清CK" }, { "start_idx": 11, "end_idx": 33, "type": "sym", "entity": "病程早期CK水平趋于升高,可达正常值的6~7倍" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "CK水平" }, { "start_idx": 35, "end_idx": 52, "type": "sym", "entity": "病程晚期,血清CK逐渐下降,趋于正常" }, { "start_idx": 40, "end_idx": 43, "type": "ite", "entity": "血清CK" } ] }, { "text": "肌肉活体组织检查显示非特异性表现,为肌肉纤维数量改变、坏死,肌内膜、结缔组织和脂肪组织增生免疫染色确定可对Laminin-α2链缺乏作特异性诊断。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "肌肉活体组织检查显示非特异性表现" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "肌肉纤维" }, { "start_idx": 18, "end_idx": 28, "type": "sym", "entity": "肌肉纤维数量改变、坏死" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "坏死" }, { "start_idx": 30, "end_idx": 44, "type": "sym", "entity": "肌内膜、结缔组织和脂肪组织增生" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肌内膜" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "结缔组织" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "脂肪组织" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "免疫染色" }, { "start_idx": 53, "end_idx": 65, "type": "dis", "entity": "Laminin-α2链缺乏" } ] }, { "text": "头颅MRI可见髓鞘形成不足。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "头颅MRI" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "头颅MRI可见髓鞘形成不足" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "髓鞘" } ] }, { "text": "(二)Fukuyama先天性肌营养不良(FCMD)该病主要存在于日本,发病率为7/10万~12/10万。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "Fukuyama" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "先天性肌营养不良" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "FCMD" } ] }, { "text": "FCMD为常染色体隐性遗传,研究证实其基因定位于9q31-31。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "FCMD" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "染色体" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "基因" } ] }, { "text": "引起FCMD的特异基因Fukutin为一个插入的逆转换成分。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "FCMD" }, { "start_idx": 7, "end_idx": 17, "type": "bod", "entity": "特异基因Fukutin" } ] }, { "text": "该基因2个独立的点突变已证实与FCMD的发病有关。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "基因2个独立的点突变" }, { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "基因" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "FCMD" } ] }, { "text": "患者常于出生6个月出现症状,一般所能达到的最大运动功能是坐位移行,大多数FCMD患者无法独自行走,20岁左右死亡。", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "FCMD" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "无法独自行走" } ] }, { "text": "所有患者均有严重的智能迟缓,IQ在30~50分,常发生惊厥。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "严重的智能迟缓" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "IQ在30~50分" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "常发生惊厥" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "惊厥" } ] }, { "text": "FCMD的眼部病变相对轻微,包括中到高度近视,视网膜色素上皮斑点化及不同程度视神经萎缩。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "FCMD的眼部病变相对轻微" }, { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "FCMD" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "眼部" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "中到高度近视" }, { "start_idx": 23, "end_idx": 32, "type": "sym", "entity": "视网膜色素上皮斑点化" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "视网膜色素上皮" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "不同程度视神经萎缩" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "视神经萎缩" } ] }, { "text": "(三)Walker-Warburg综合征本征为一组肌肉、眼和脑联合发育缺陷的疾病,至今基因缺陷尚未明确。", "entities": [ { "start_idx": 3, "end_idx": 19, "type": "dis", "entity": "Walker-Warburg综合征" }, { "start_idx": 25, "end_idx": 36, "type": "dis", "entity": "肌肉、眼和脑联合发育缺陷" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "基因缺陷" } ] }, { "text": "患者自出生后不久即表现肌无力及肌张力低下,呼吸困难。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "肌无力" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肌" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "肌张力" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "眼部畸形多样,角膜、虹膜及视神经均可受累严重智力迟缓及癫痫。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "眼部畸形" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "角膜、虹膜及视神经均可受累" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "角膜" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "虹膜" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "视神经" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "严重智力迟缓" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "癫痫" } ] }, { "text": "患者终身不能行走,多于生后数月内死亡。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "终身不能行走" } ] }, { "text": "血清CK增高;肌肉病理学检查非特异性肌纤维变性坏死,肌纤维膜和肌束膜增生头部MRI显示脑回发育不良,脑室扩大,白质密度降低,小脑发育不良。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "血清CK增高" }, { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血清CK" }, { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "肌肉病理学检查" }, { "start_idx": 7, "end_idx": 24, "type": "sym", "entity": "肌肉病理学检查非特异性肌纤维变性坏死" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "非特异性肌纤维变性坏死" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "肌纤维膜和肌束膜增生" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "肌纤维膜" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肌束膜" }, { "start_idx": 36, "end_idx": 40, "type": "pro", "entity": "头部MRI" }, { "start_idx": 36, "end_idx": 48, "type": "sym", "entity": "头部MRI显示脑回发育不良" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "脑回发育不良" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "脑室扩大" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "脑室" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "白质密度降低" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "白质" }, { "start_idx": 62, "end_idx": 67, "type": "sym", "entity": "小脑发育不良" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "小脑" } ] }, { "text": "(四)Santavuori肌营养不良(肌-眼-脑病)本病属常染色体隐性遗传病,多见于芬兰人。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "dis", "entity": "Santavuori肌营养不良" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "肌-眼-脑病" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "常染色体隐性遗传病" } ] }, { "text": "高度近视,视网膜发育不良,视力进行性减退。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "高度近视" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "视网膜发育不良" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "视力进行性减退" } ] }, { "text": "三、气管食管瘘胚胎3周时,原始前肠由其两侧壁向管腔内生嵴,至第5~6周融合成隔,此隔将前肠分为腹侧及背侧两部分,分别形成气管和食管。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "气管食管瘘" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "前肠" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "管腔内" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "隔" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "隔" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "前肠" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "腹" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "背" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "气管" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "食管" } ] }, { "text": "如分隔过程中受血管功能不全、感染、溃疡或某些物质缺乏及遗传因素影响而发生异常,即形成气管食管瘘(tracheoesophagealfistula)。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "血管功能不全" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "感染" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "溃疡" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "物质缺乏" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "气管食管瘘" }, { "start_idx": 48, "end_idx": 71, "type": "dis", "entity": "tracheoesophagealfistula" } ] }, { "text": "85%以上患儿伴有食管闭锁。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "食管闭锁" } ] }, { "text": "将口腔与鼻咽部分泌物清除后,呼吸情况迅速改善。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "bod", "entity": "口腔与鼻咽部分泌物" } ] }, { "text": "气管和食管畸形(trachealandesophagealmalformations)多伴有其他畸形,如脊柱(vertebral)、肛门直肠(anorectal)、心脏(cardial)、肾脏(renal)、桡侧(radial)、肢体畸形(limbabnormalities),称之为VATER或VACTERAL综合征。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "气管和食管畸形" }, { "start_idx": 8, "end_idx": 41, "type": "dis", "entity": "trachealandesophagealmalformations" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "畸形" }, { "start_idx": 52, "end_idx": 137, "type": "dis", "entity": "脊柱(vertebral)、肛门直肠(anorectal)、心脏(cardial)、肾脏(renal)、桡侧(radial)、肢体畸形(limbabnormalities)" }, { "start_idx": 142, "end_idx": 158, "type": "dis", "entity": "VATER或VACTERAL综合征" } ] }, { "text": "导管经鼻或口腔插入,至食管自动返回无法进入胃时,应怀疑此病。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "导管" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "鼻" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "口腔" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "食管" }, { "start_idx": 11, "end_idx": 21, "type": "sym", "entity": "食管自动返回无法进入胃" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "胃" } ] }, { "text": "禁忌用钡剂造影,以防吸入引起化学性肺炎。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "钡剂造影" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "化学性肺炎" } ] }, { "text": "本病是外科急症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "外科" } ] }, { "text": "术前应特别注意避免胃内容物吸入肺部。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "胃内容物" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "胃内容物吸入肺部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺部" } ] }, { "text": "取平卧或侧卧位,定时翻身、拍背和吸痰,亦可留置导管持续吸引食管盲端内的分泌物。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "取平卧或侧卧位" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "定时翻身" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "拍背" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "吸痰" }, { "start_idx": 21, "end_idx": 37, "type": "pro", "entity": "留置导管持续吸引食管盲端内的分泌物" } ] }, { "text": "注意体温、呼吸功能和伴随畸形。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "体温" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "畸形" } ] }, { "text": "二、心室左右心室的解剖形态学特征特点(表9-9)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心室" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "左右心室" } ] }, { "text": "表9-3左右心室解剖形态学特征(一)形态学右心室右心室呈三角形,心室内小梁粗大、稀少且与右心室流入道和流出道平行。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "左右心室" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "解剖" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "形态学右心室" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "右心室" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "心室内小梁" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "心室内小梁粗大" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "右心室流入道" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "流出道" } ] }, { "text": "乳头肌相对较小而多,附着于室间隔表面(多在隔束的后下缘)及游离壁。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "乳头肌" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "室间隔表面" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "隔束的后下缘" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "游离壁" } ] }, { "text": "三尖瓣的数目在婴儿期为两叶(壁瓣及隔瓣),而在老年则为四叶(前、后、隔及圆锥瓣),但整个人生中绝大多数时期为三瓣(前、后及隔瓣)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "两叶" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "壁瓣" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "隔瓣" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "四叶" }, { "start_idx": 30, "end_idx": 38, "type": "bod", "entity": "前、后、隔及圆锥瓣" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "三瓣" }, { "start_idx": 57, "end_idx": 62, "type": "bod", "entity": "前、后及隔瓣" } ] }, { "text": "因此,不能单纯通过瓣膜的数量来判断是否为三尖瓣,而可依据以下两个特征进行判断:①三尖瓣长度相仿;②与二尖瓣不同,三尖瓣为流入道瓣膜。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "瓣膜" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "流入道瓣膜" } ] }, { "text": "正常的右心室有较大的漏斗部形成其流出道。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "右心室" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "漏斗部" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "流出道" } ] }, { "text": "圆锥由三个部分所组成:①远端圆锥隔,延伸至游离壁形成壁束;②近端圆锥隔或称为隔束;③调节束。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "圆锥" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "远端圆锥隔" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "游离壁" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "壁束" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "近端圆锥隔" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "隔束" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "调节束" } ] }, { "text": "解剖学上右心室由四部分组成:①房室通道部;②右心室窦部;③远端圆锥隔;④近端圆锥隔。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "右心室" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "房室通道部" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "右心室窦部" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "远端圆锥隔" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "近端圆锥隔" } ] }, { "text": "右心室流入道流入道由房室通道部及窦部组成,流出道由隔束及壁束所形成。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "右心室流入道" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "房室通道部" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "窦部" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "流出道" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "隔束" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "壁束" } ] }, { "text": "右心室的血供全部由右冠状动脉及其分支供应。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "血" }, { "start_idx": 9, "end_idx": 17, "type": "bod", "entity": "右冠状动脉及其分支" } ] }, { "text": "右心室的传导系统为右束支,右束支在圆锥乳头肌下方穿出并在隔束下缘下行穿过调节束进入前组乳头肌根部后到达右室游离壁。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "右心室的传导系统" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "右束支" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "右束支" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "圆锥乳头肌下方" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "隔束下缘" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "调节束" }, { "start_idx": 41, "end_idx": 48, "type": "bod", "entity": "前组乳头肌根部后" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "右室游离壁" } ] }, { "text": "(二)形态学左心室左心室呈子弹头型,左心室室间隔面上部非常光滑,下部则有较多的细小的斜行肌小梁。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "形态学左心室" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "左心室" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "左心室" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "室间隔面上部" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "斜行肌小梁" } ] }, { "text": "左心室有两组乳头肌,前外侧乳头肌及后内侧乳头肌。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左心室" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "乳头肌" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "前外侧乳头肌" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "后内侧乳头肌" } ] }, { "text": "前外侧乳头肌较高且远离室间隔。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "前外侧乳头肌" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "室间隔" } ] }, { "text": "而后内侧乳头肌靠后且贴近室间隔。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "后内侧乳头肌" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "室间隔" } ] }, { "text": "左心室的乳头肌较大且仅启自左心室游离壁游离壁。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "左心室的乳头肌" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "左心室游离壁" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "游离壁" } ] }, { "text": "与三尖瓣不同二尖瓣前叶明显较后叶长,为左心室流出道及流出道的重要组成部分。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "二尖瓣前叶" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "后叶" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "流出道" } ] }, { "text": "通常情况下,在无冠瓣及左冠瓣下方无肌性圆锥或仅有极小的肌性圆锥。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "无冠瓣" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "左冠瓣下方" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "肌性圆锥" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "极小的肌性圆锥" } ] }, { "text": "在大动脉连接正常时,主动脉无冠瓣与左冠瓣交界直接位于二尖瓣前叶中部之上而无冠瓣与右冠瓣交界则在膜部室间隔之上。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "大动脉" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "主动脉无冠瓣" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "左冠瓣" }, { "start_idx": 26, "end_idx": 34, "type": "bod", "entity": "二尖瓣前叶中部之上" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "无冠瓣" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "右冠瓣" }, { "start_idx": 47, "end_idx": 53, "type": "bod", "entity": "膜部室间隔之上" } ] }, { "text": "左心室可认为是双冠状血管供应的心室(左前降支和回旋支)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左心室" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "双冠状血管" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心室" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "左前降支" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "回旋支" } ] }, { "text": "左室传导系统有两个分布区,上分布区分布至前外侧组乳头肌,下分布区分布至后内侧组乳头肌。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "左室传导系统" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "前外侧组乳头肌" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "后内侧组乳头肌" } ] }, { "text": "左心室同样由与右心室相对应的四部分组成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左心室" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "右心室" } ] }, { "text": "六、病程及预后20世纪60年代小样本提示,儿童CVS发作结束于14岁前,病程中位年龄为6年,发病年越早、病程也越长,小于3岁发病,病程持续3~8年,8岁以后发病,病程分别为5.8、4.9及2.9年。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "儿童CVS" } ] }, { "text": "近年有报道到18岁时75%患儿呕吐停止,27%发展为偏头痛。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "呕吐" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "偏头痛" } ] }, { "text": "对大多数患者来说CVS是偏头痛相关疾病。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "CVS" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "偏头痛" } ] }, { "text": "第三节支气管扩张药物在肺部疾病中的应用支气管扩张剂通过缓解平滑肌痉挛、减轻黏膜充血水肿、减少黏液分泌等机制,缓解支气管痉挛,保持气道通畅,是控制哮喘急性发作的主要药物。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "支气管扩张药物" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "肺部疾病" }, { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "支气管扩张剂" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "平滑肌痉挛" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "黏膜" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "黏膜充血水肿" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "黏液分泌" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "支气管痉挛" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "气道" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "哮喘" } ] }, { "text": "目前常用的支气管扩张剂,根据作用环节不同分为3类:①β2肾上腺素受体激动剂;②茶碱类药物;③抗胆碱能药物。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "支气管扩张剂" }, { "start_idx": 26, "end_idx": 47, "type": "dru", "entity": "β2肾上腺素受体激动剂" }, { "start_idx": 50, "end_idx": 54, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 57, "end_idx": 62, "type": "dru", "entity": "抗胆碱能药物" } ] }, { "text": "一、β2肾上腺素受体激动剂(一)β2肾上腺素受体激动剂分类β肾上腺素受体分为β1和β2两种亚型,药物的效果取决于药物对不同亚型受体的选择性以及不同亚型受体在各器官中的密度和分布。", "entities": [ { "start_idx": 2, "end_idx": 23, "type": "dru", "entity": "β2肾上腺素受体激动剂" }, { "start_idx": 27, "end_idx": 48, "type": "dru", "entity": "β2肾上腺素受体激动剂" }, { "start_idx": 51, "end_idx": 55, "type": "dru", "entity": "β肾上腺素" } ] }, { "text": "β1受体激动剂可增加心肌收缩力、心率和房室节传导速度,而β2受体激动剂则主要分布在支气管上,因而选择性高的β2受体激动剂对心脏的不良反应就会明显减少。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "dru", "entity": "β1受体激动剂" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心肌" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "心率" }, { "start_idx": 30, "end_idx": 36, "type": "ite", "entity": "房室节传导速度" }, { "start_idx": 39, "end_idx": 56, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "支气管上" }, { "start_idx": 75, "end_idx": 92, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "心脏" } ] }, { "text": "β2受体广泛分布在气道的各种效应细胞上,从不同侧面对气道起着调节作用(表8-8)。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "bod", "entity": "β2受体" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "气道的各种效应细胞上" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "气道" } ] }, { "text": "表8-13β2受体激动剂对气道的各种效应细胞的作用常用的β2受体激动剂根据结构不同可以分成5大类,也可根据β2受体选择性、作用的持续时间、有无抗炎作用分为4类(表8-8、表8-8),后者分类对临床更有指导价值。", "entities": [ { "start_idx": 5, "end_idx": 22, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 24, "end_idx": 32, "type": "bod", "entity": "气道的各种效应细胞" }, { "start_idx": 39, "end_idx": 56, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 75, "end_idx": 89, "type": "bod", "entity": "β2受体" } ] }, { "text": "半衰期长的β2受体激动剂,由于作用时间延长、服药次数减少,临床依从性好,对于夜间咳喘的治疗明显优于半衰期短的β2受体激动剂;同时一些半衰期短的β2受体激动剂也通过剂型改良,采用缓释、控释和透皮吸收等方法延长药物释放时间(表8-8)。", "entities": [ { "start_idx": 5, "end_idx": 22, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "夜间咳喘" }, { "start_idx": 65, "end_idx": 82, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 93, "end_idx": 110, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 128, "end_idx": 128, "type": "bod", "entity": "皮" } ] }, { "text": "表8-14常用β2受体激动剂的作用与给药途径的比较注:作用+~+++由弱变强,4级为最强表8-15β2肾上腺素受体激动剂分类注:*这类药物现在已经停止单独使用,多与吸入性糖皮质激素制成混合制剂使用,如信必可、舒利迭表8-16长半衰期或缓释/控释β2受体激动剂注:qN,睡前服对于长效β2受体激动剂福莫特罗和沙美特罗,由于分子结构中加长侧链,使得脂溶性和与受体的亲和力明显增强,作用效价与持续时间延长。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 49, "end_idx": 70, "type": "dru", "entity": "β2肾上腺素受体激动剂" }, { "start_idx": 104, "end_idx": 111, "type": "dru", "entity": "吸入性糖皮质激素" }, { "start_idx": 122, "end_idx": 124, "type": "dru", "entity": "信必可" }, { "start_idx": 126, "end_idx": 128, "type": "dru", "entity": "舒利迭" }, { "start_idx": 134, "end_idx": 161, "type": "dru", "entity": "长半衰期或缓释/控释β2受体激动剂" }, { "start_idx": 172, "end_idx": 191, "type": "dru", "entity": "长效β2受体激动剂" }, { "start_idx": 192, "end_idx": 195, "type": "dru", "entity": "福莫特罗" }, { "start_idx": 197, "end_idx": 200, "type": "dru", "entity": "沙美特罗" } ] }, { "text": "但由于单用长效β2受体激动剂会导致低钾血症等不良反应,目前主要与吸入性糖皮质激素制成合剂使用,不能单独用于临床。", "entities": [ { "start_idx": 5, "end_idx": 24, "type": "dru", "entity": "长效β2受体激动剂" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "低钾血症" }, { "start_idx": 43, "end_idx": 50, "type": "dru", "entity": "吸入性糖皮质激素" } ] }, { "text": "(二)β2受体激动剂的使用方法1.喷射雾化方案喷射雾化通过高压气体冲击液体,产生雾滴;具有雾滴直径均匀,大小适中(1~5μm),对液体中药物成分无影响等优点,适用于各年龄患儿。", "entities": [ { "start_idx": 3, "end_idx": 20, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "喷射雾化" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "喷射雾化" } ] }, { "text": "根据动力源不同,分为以氧气为动力的氧雾化和以压缩泵为动力的泵雾化,一般气体的流量需达到6~8L/min。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "氧雾化" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "压缩泵" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "泵雾化" } ] }, { "text": "β受体激动剂一般与抗胆碱能药物合用喷射雾化吸入,β受体激动剂主要作用于小气道、起效快,但维持时间短;抗胆碱能药物主要作用于大气道、起效相对较慢,但维持时间较长,因而两者合用有互补作用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "β受体激动剂" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "喷射雾化" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "β受体激动剂" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "小气道" }, { "start_idx": 50, "end_idx": 55, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "大气道" } ] }, { "text": "喷射雾化吸入β2受体激动剂,不良反应相对较小,但也要严格掌握用药剂量(表8-8),用药期间注意血钾异常和心血管方面副作用的产生。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "喷射雾化" }, { "start_idx": 6, "end_idx": 23, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "血钾异常" }, { "start_idx": 58, "end_idx": 59, "type": "ite", "entity": "血钾" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "心血管" } ] }, { "text": "表8-17β2受体兴奋剂喷射性雾化2.静脉用药静脉用药多需微量输液泵持续输注,全身不良反应较大,多用于其他治疗无效的重危哮喘患儿;用药期间应注意监测心血管功能和血钾水平。", "entities": [ { "start_idx": 5, "end_idx": 22, "type": "dru", "entity": "β2受体兴奋剂" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "喷射性雾化" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "静脉用药" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "静脉用药" }, { "start_idx": 42, "end_idx": 44, "type": "equ", "entity": "输液泵" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "持续输注" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "重危哮喘" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "心血管" }, { "start_idx": 91, "end_idx": 92, "type": "ite", "entity": "血钾" } ] }, { "text": "(1)沙丁胺醇:负荷量:10μg/kg,静脉滴注(10分钟以上),维持量:0.2~2μg/(kg•min)[最大5μg/(kg•min)]静脉持续滴注。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "沙丁胺醇" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 69, "end_idx": 74, "type": "pro", "entity": "静脉持续滴注" } ] }, { "text": "(2)特布他林:负荷量:10μg/kg,静脉滴注(10分钟以上),维持量:0.1μg/(kg•min),静脉持续滴注;每30分钟增加0.1μg/(kg•min),直到症状缓解或出现不良反应(心动过速、震颤、恶心),最大4μg/(kg•min)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "特布他林" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 52, "end_idx": 57, "type": "pro", "entity": "静脉持续滴注" }, { "start_idx": 95, "end_idx": 98, "type": "sym", "entity": "心动过速" }, { "start_idx": 100, "end_idx": 101, "type": "sym", "entity": "震颤" }, { "start_idx": 103, "end_idx": 104, "type": "sym", "entity": "恶心" } ] }, { "text": "3.吸入用药吸入给药是β2受体激动剂的最佳给药方法,吸入药物直接作用于气道,起效迅速而用药量少,对心血管的不良影响也相对较少。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "吸入用药" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "吸入给药" }, { "start_idx": 11, "end_idx": 28, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "气道" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "心血管" } ] }, { "text": "现在常用的吸入给药方式主要有2种,定量揿压式气雾剂(MDI)和干粉吸入剂。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "吸入给药" }, { "start_idx": 17, "end_idx": 24, "type": "dru", "entity": "定量揿压式气雾剂" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "MDI" }, { "start_idx": 31, "end_idx": 35, "type": "dru", "entity": "干粉吸入剂" } ] }, { "text": "由于干粉吸入剂仅要求有一定的吸气流量,而不需要患儿的配合,因而在儿科领域使用较为广泛。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "干粉吸入剂" }, { "start_idx": 32, "end_idx": 33, "type": "dep", "entity": "儿科" } ] }, { "text": "(1)沙丁胺醇:喘宁蝶(ventodisk)粉剂:0.2mg/囊泡;0.2mg/次,每日3~4次。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "沙丁胺醇" }, { "start_idx": 8, "end_idx": 23, "type": "dru", "entity": "喘宁蝶(ventodisk)粉剂" } ] }, { "text": "速克喘(cyclohalor)粉剂:0.2mg/囊泡;0.2mg/次,每日3~4次。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "dru", "entity": "速克喘(cyclohalor)粉剂" } ] }, { "text": "万托林pMDI:0.1mg/揿;0.1mg/次,每日3~4次。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "万托林pMDI" } ] }, { "text": "(2)特布他林:喘康速pMDI:0.125mg/揿;0.125mg/次,每日3~4次。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "特布他林" }, { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "喘康速pMDI" } ] }, { "text": "博利康尼都保:500μg/吸;500μg/次,3~4次/日。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "博利康尼都保" } ] }, { "text": "4.口服用药口服短效β2受体激动剂,不良反应较多;缓释或控释β2受体激动剂疗效维持时间长,用于防治反复发作性哮喘和夜间哮喘(剂量和用法见表8-8)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "口服" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "口服" }, { "start_idx": 8, "end_idx": 27, "type": "dru", "entity": "短效β2受体激动剂" }, { "start_idx": 36, "end_idx": 58, "type": "dru", "entity": "缓释或控释β2受体激动剂" }, { "start_idx": 71, "end_idx": 77, "type": "dis", "entity": "反复发作性哮喘" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "夜间哮喘" } ] }, { "text": "博利康尼(Brieanyl):2.5mg/片,每次65μg/kg,3次/日。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "博利康尼" }, { "start_idx": 5, "end_idx": 12, "type": "dru", "entity": "Brieanyl" } ] }, { "text": "(三)β受体激动剂常见不良反应各种用法的β2受体激动剂,都存在一定程度的不良反应,需要密切注意:1.心率加快或心律失常虽然现在使用的β2受体激动剂有较强的β2受体选择性,但大剂量使用可以作用于心脏β2受体,引起心率加快或心律失常,尤其在静脉或口服使用时,应密切监护。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "β受体激动剂" }, { "start_idx": 20, "end_idx": 37, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 61, "end_idx": 62, "type": "ite", "entity": "心率" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "心率加快" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "心律失常" }, { "start_idx": 77, "end_idx": 94, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 99, "end_idx": 113, "type": "bod", "entity": "β2受体" }, { "start_idx": 129, "end_idx": 145, "type": "bod", "entity": "心脏β2受体" }, { "start_idx": 149, "end_idx": 150, "type": "ite", "entity": "心率" }, { "start_idx": 149, "end_idx": 152, "type": "sym", "entity": "心率加快" }, { "start_idx": 154, "end_idx": 157, "type": "dis", "entity": "心律失常" }, { "start_idx": 162, "end_idx": 163, "type": "pro", "entity": "静脉" }, { "start_idx": 165, "end_idx": 166, "type": "pro", "entity": "口服" } ] }, { "text": "虽然使用吸入性β2受体激动剂心血管不良反应明显减少,但反复大剂量使用仍可以出现严重的不良反应。", "entities": [ { "start_idx": 4, "end_idx": 24, "type": "dru", "entity": "吸入性β2受体激动剂" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "心血管" } ] }, { "text": "2.肌肉震颤静脉或口服使用β2受体激动剂,可激活横纹肌慢纤维上的β2受体,使之收缩加快,破坏快慢纤维之间的融合现象,引起肌肉震颤;好发于四肢和颈部骨骼肌。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "肌肉震颤" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肌肉" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "静脉" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "口服" }, { "start_idx": 13, "end_idx": 30, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "横纹肌慢纤维" }, { "start_idx": 43, "end_idx": 57, "type": "bod", "entity": "β2受体" }, { "start_idx": 66, "end_idx": 78, "type": "sym", "entity": "破坏快慢纤维之间的融合现象" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "快慢纤维" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "肌肉震颤" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "肌肉" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "四肢" }, { "start_idx": 93, "end_idx": 97, "type": "bod", "entity": "颈部骨骼肌" } ] }, { "text": "吸入给药可以大大减少这一不良反应。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "吸入给药" } ] }, { "text": "3.低钾血症β2受体激动剂可激活横纹肌细胞膜上Na+-K+-ATP酶,促使K+进入细胞内引起血浆钾离子浓度降低,严重者可诱发心律失常。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "低钾血症" }, { "start_idx": 6, "end_idx": 23, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 27, "end_idx": 44, "type": "bod", "entity": "横纹肌细胞膜上Na+-K+-ATP酶" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "细胞内" }, { "start_idx": 57, "end_idx": 63, "type": "ite", "entity": "血浆钾离子浓度" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "血浆钾离子浓度降低" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "心律失常" } ] }, { "text": "长期或静脉使用β2受体激动剂应注意发生低钾血症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "静脉" }, { "start_idx": 7, "end_idx": 24, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "低钾血症" } ] }, { "text": "此外,长期使用β2受体激动剂可以使气道的β2受体数量减少,反应性降低,导致患儿对β2受体激动剂的耐药性。", "entities": [ { "start_idx": 7, "end_idx": 24, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 28, "end_idx": 45, "type": "bod", "entity": "气道的β2受体" }, { "start_idx": 28, "end_idx": 49, "type": "sym", "entity": "气道的β2受体数量减少" }, { "start_idx": 62, "end_idx": 79, "type": "dru", "entity": "β2受体激动剂" } ] }, { "text": "十、脉络丛乳头状瘤占儿童脑肿瘤2%~3%,主要生长在各脑室内。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "脉络丛乳头状瘤" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "儿童脑肿瘤" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "脑室内" } ] }, { "text": "肉眼下肿瘤呈粉红色质软,球样,上有不规则圆形的赘生物组织,似菜花样,质脆、血运丰富。", "entities": [ { "start_idx": 0, "end_idx": 40, "type": "sym", "entity": "肉眼下肿瘤呈粉红色质软,球样,上有不规则圆形的赘生物组织,似菜花样,质脆、血运丰富" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肉眼" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" } ] }, { "text": "显微镜下肿瘤组织由血管和结缔组织形成的乳头样组织被单层柱状上皮覆盖脉络丛乳头状癌(papillomachoroideum)占该肿瘤的10%~20%,呈浸润性生长,组织学上可看到瘤细胞异形性及核分裂象。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "显微镜" }, { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "显微镜下肿瘤组织由血管和结缔组织形成的乳头样组织被单层柱状上皮覆盖" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血管" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "结缔组织" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "乳头样组织" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "单层柱状上皮" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "脉络丛乳头状癌" }, { "start_idx": 41, "end_idx": 59, "type": "dis", "entity": "papillomachoroideum" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "肿瘤" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "瘤细胞" }, { "start_idx": 88, "end_idx": 98, "type": "sym", "entity": "瘤细胞异形性及核分裂象" } ] }, { "text": "无论良恶性肿瘤,皆因乳头容易脱落进入脑脊液,形成脑脊液播散。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "良恶性肿瘤" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "乳头" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑脊液" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑脊液" } ] }, { "text": "另外,为了确定肿瘤的血供应,尚可做脑血管造影或MRI血管造影。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "脑血管造影" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "MRI血管造影" } ] }, { "text": "八、病毒性肺炎(一)腺病毒性肺炎两肺纹理增粗,肺透亮度增高,大片状融合性病变为主,肺脓疡、胸膜炎少见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "病毒性肺炎" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "腺病毒性肺炎" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "两肺" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "两肺纹理增粗" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "肺透亮度增高" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "大片状融合性病变" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "胸膜炎" } ] }, { "text": "(二)呼吸道合胞病毒肺炎病变以两肺内带及下肺野为多,表现为纤细条状,并沿着支气管纹理分布,肺气肿明显。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "呼吸道合胞病毒肺炎" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "两肺内带" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "下肺野" }, { "start_idx": 29, "end_idx": 43, "type": "sym", "entity": "纤细条状,并沿着支气管纹理分布" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "支气管" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "肺气肿" } ] }, { "text": "(三)巨细胞病毒肺炎病变广泛,单侧和双侧均可,肺纹理模糊,细小片状阴影多见,重者出现肺透亮度增强。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "巨细胞病毒肺炎" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "肺纹理模糊" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "细小片状阴影多见" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肺" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "肺透亮度增强" } ] }, { "text": "参考文献1.梁秀龄.神经病学(19卷):神经系统遗传性疾病.北京:人民军医出版社,20012.吴希如,林庆.小儿神经系统疾病基础与临床.北京:人民卫生出版社,20003.梁雅珍,林庆.癫痫与神经皮肤综合征.中国实用儿科杂志,2000;15(9):5524.HerronJ,DarrahR,QuaghebeurG.Intra-cranialmanifestationsoftheneurocutaneoussyndromes.ClinRadiol,2000,55(2):82-825.NorthK.Neurofibromatosistype1.AmJMedGenet,2000,97(2):119-1196.RuggieriM,HusonSM.Theneurofibromatoses.Anoverview.ItalJNeurolSci,1999,20(2):89-897.孙新芬.结节性硬化症.国外医学皮肤性病学分册,1996,22(5):273-2738.HengstschlagerM,RodmanDM,MilolozaA,etal.Tuberoussclerosisgeneproductsinproliferationcontrol.MutatRes,2001,488(3):233-2339.KihiczakNI,SchwartzRA,JozwiakS,etal.Sturge-Webersyndrome.Cutis,2000,65(3):133-13310.祝玉芬,梁志会,杜昱平,等.神经皮肤综合征的临床特征、CT及MRI诊断.中国CT和MRI杂志,2009,7(6):36-3611.FernerRE.Theneurofibromatoses.PractNeurol,2010,10(2):82-82", "entities": [ { "start_idx": 20, "end_idx": 28, "type": "dis", "entity": "神经系统遗传性疾病" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "小儿神经系统疾病" }, { "start_idx": 92, "end_idx": 101, "type": "dis", "entity": "癫痫与神经皮肤综合征" }, { "start_idx": 107, "end_idx": 108, "type": "dep", "entity": "儿科" }, { "start_idx": 390, "end_idx": 395, "type": "dis", "entity": "结节性硬化症" }, { "start_idx": 649, "end_idx": 655, "type": "dis", "entity": "神经皮肤综合征" }, { "start_idx": 662, "end_idx": 663, "type": "pro", "entity": "CT" }, { "start_idx": 665, "end_idx": 669, "type": "pro", "entity": "MRI诊断" }, { "start_idx": 673, "end_idx": 674, "type": "pro", "entity": "CT" }, { "start_idx": 676, "end_idx": 678, "type": "pro", "entity": "MRI" } ] }, { "text": "二、正常影像(一)胸廓形态胸廓形态与肋骨的走向有着密切关系,尤其在新生儿时期,肋骨若倾斜走向呈钟形,肋骨若水平走向则呈圆柱形。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胸廓" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胸廓" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肋骨" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肋骨" }, { "start_idx": 39, "end_idx": 48, "type": "sym", "entity": "肋骨若倾斜走向呈钟形" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "肋骨" }, { "start_idx": 50, "end_idx": 61, "type": "sym", "entity": "肋骨若水平走向则呈圆柱形" } ] }, { "text": "早产儿胸廓中部可出现相对狭小,这与肺充气不足和肋骨软弱有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "早产儿" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "胸廓中部" }, { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "胸廓中部可出现相对狭小" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "肺充气不足" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肋骨" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "肋骨软弱" } ] }, { "text": "一岁以后,胸廓逐渐出现上窄下宽的圆锥形形态。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胸廓" }, { "start_idx": 5, "end_idx": 20, "type": "sym", "entity": "胸廓逐渐出现上窄下宽的圆锥形形态" } ] }, { "text": "(二)胸壁软组织新生儿时期由于皮下脂肪少,使皱褶的皮肤出现纵形或横形的线条状阴影,特别是纵形的线条状阴影易误诊为气胸。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "胸壁软组织" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "皮下脂肪" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "皮下脂肪少" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "皱褶的皮肤" }, { "start_idx": 22, "end_idx": 39, "type": "sym", "entity": "皱褶的皮肤出现纵形或横形的线条状阴影" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "纵形的线条状阴影" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "气胸" } ] }, { "text": "(三)纵隔在新生儿、婴幼儿时期,由于受胸腺、血管及淋巴组织等影响,其纵隔相对较宽,胸腺形态一般呈风帆形,其他有圆锥形、半圆形、三角形,对于较大的胸腺呈右肺上叶大叶性肺炎表现时应引起重视。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胸腺" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血管" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "淋巴组织" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "纵隔" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "胸腺" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "较大的胸腺" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "胸腺" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "右肺上叶" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "大叶性肺炎" } ] }, { "text": "(四)肺门肺门包含了肺动脉、肺静脉、神经、支气管、淋巴等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肺门" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肺门" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺动脉" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺静脉" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "神经" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "支气管" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "淋巴" } ] }, { "text": "左肺门高于右肺门约0.5~1.0cm。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左肺门" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "右肺门" } ] }, { "text": "左肺门上部由肺动脉弓构成,呈圆形和半弧形,下部由左下肺动脉及分支构成;右肺门分为上下两极,相交处称肺门角,上极主要由右上肺动脉及肺静脉构成,下极主要由下叶动脉及叶间动脉构成。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "左肺门上部" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "肺动脉弓" }, { "start_idx": 24, "end_idx": 31, "type": "bod", "entity": "左下肺动脉及分支" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "右肺门" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "肺门角" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "右上肺动脉" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "肺静脉" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "下叶动脉" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "叶间动脉" } ] }, { "text": "(五)肺纹理由肺动脉与肺静脉构成,由肺门向外逐渐伸展,由粗变细,上肺野肺纹理较下肺野稀少,上肺野的肺动脉较水平,而下肺野肺静脉较水平。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肺纹理" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺动脉" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肺静脉" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺门" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "上肺野肺纹理" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "下肺野" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "上肺野的肺动脉" }, { "start_idx": 57, "end_idx": 62, "type": "bod", "entity": "下肺野肺静脉" } ] }, { "text": "(六)气管新生儿、婴儿时期,气管分叉相对较高,接近第三胸椎水平,气管稍有右偏。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "气管" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "气管分叉" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "第三胸椎" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "气管" } ] }, { "text": "(七)横膈正常横膈为边缘光滑、锐利之穹隆状,6个月之内婴儿横膈在第八后肋,左膈高于右膈约1cm之内,正常横膈运动对称,亦有少数呈不对称运动。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "横膈" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "横膈" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "横膈" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "第八后肋" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "左膈" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "右膈" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "横膈" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "不对称运动" } ] }, { "text": "五、HIV感染儿童的结核病据报道,同时感染结核杆菌和HIV的儿童相对少见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "HIV感染" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "结核病" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "感染" }, { "start_idx": 21, "end_idx": 24, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "HIV" } ] }, { "text": "HIV感染儿童的结核病常漏诊,因患者的结核菌素皮试阴性病菌分离困难,且结核病的临床症状与HIV感染的其他许多临床表现相似。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HIV感染" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "结核病" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "结核菌素皮试" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "病菌分离" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "结核病" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "HIV感染" } ] }, { "text": "HIV感染儿童的结核病常更严重、更易播散,肺部症状伴发热体重下降为最常见的表现,HIV感染儿童较其他患儿的肺部病变更易进展为空洞和肺外病变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HIV感染" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "结核病" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肺部" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "肺部症状" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "体重" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "体重下降" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "HIV感染" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "肺部病变" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "空洞" }, { "start_idx": 65, "end_idx": 68, "type": "dis", "entity": "肺外病变" } ] }, { "text": "HIV患儿结核病的最佳治疗方案尚未确定。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "HIV" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "结核病" } ] }, { "text": "大多数专家建议,在HIV抗体阳性对药物敏感的结核病患儿,先给予INH、RFP和PZA,2个月后停PZA,继续INH和RFP治疗,总疗程9~12个月。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "HIV抗体阳性" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "HIV抗体" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "对药物敏感" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "结核病" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "INH" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "RFP" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "PZA" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "PZA" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "INH" }, { "start_idx": 58, "end_idx": 60, "type": "dru", "entity": "RFP" } ] }, { "text": "第三节新生儿缺氧缺血性脑病新生儿缺氧缺血脑病(hypoxic-ischemicencephalopathy,HIE)是围生期缺氧缺血所致的脑损伤,是导致新生儿死亡和发生后遗症的重要原因之一。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "新生儿缺氧缺血性脑病" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "新生儿缺氧缺血脑病" }, { "start_idx": 23, "end_idx": 52, "type": "dis", "entity": "hypoxic-ischemicencephalopathy" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "HIE" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "缺氧" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "缺血" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "血" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "脑损伤" } ] }, { "text": "如积极做好围生期保健,推广正确的复苏方法,降低窒息发生率,HIE的发病率和危害性就可明显降低。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "推广正确的复苏方法" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "窒息" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "HIE" } ] }, { "text": "近年,我国一些大城市,HIE的发病率已开始降低。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "HIE" } ] }, { "text": "【病因】(一)缺氧引起缺氧的原因主要有:①围生期窒息:包括产前、产时和产后窒息;②呼吸暂停:反复呼吸暂停可导致缺氧缺血性脑损伤;③严重呼吸系统疾病。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "缺氧" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "缺氧" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "窒息" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "窒息" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "呼吸暂停" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "呼吸暂停" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "缺氧缺血性脑损伤" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "呼吸系统疾病" } ] }, { "text": "(二)缺血引起缺血的原因主要有:①心搏骤停和心动过缓;②大量失血、休克;③重度心力衰竭。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "缺血" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "血" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "缺血" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "血" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "心搏骤停" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "心动过缓" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "大量失血" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "血" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "休克" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "【发病机制】(一)血流动力学变化缺氧时机体发生潜水反射,为了保证重要生命器官(如脑、心)的血供,脑血管扩张,非重要器官血管收缩,这种自动调节功能使大脑在轻度短期缺氧时不受损伤。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血流" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "缺氧" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "生命器官" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "脑" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "心" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "血" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "脑血管" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "脑血管扩张" }, { "start_idx": 54, "end_idx": 62, "type": "sym", "entity": "非重要器官血管收缩" }, { "start_idx": 54, "end_idx": 60, "type": "bod", "entity": "非重要器官血管" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "大脑" }, { "start_idx": 76, "end_idx": 81, "type": "sym", "entity": "轻度短期缺氧" }, { "start_idx": 85, "end_idx": 86, "type": "dis", "entity": "损伤" } ] }, { "text": "如缺氧继续存在,脑血管自主调节功能失代偿,脑小动脉对灌注压和CO2浓度变化的反应能力减弱,形成压力相关性的被动性脑血流调节过程,当血压降低时脑血流减少,造成动脉边缘带的缺血性损害。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "缺氧" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "脑血管自主调节功能失代偿" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑血管" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "脑小动脉" }, { "start_idx": 21, "end_idx": 49, "type": "sym", "entity": "脑小动脉对灌注压和CO2浓度变化的反应能力减弱" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "灌注压" }, { "start_idx": 30, "end_idx": 40, "type": "ite", "entity": "CO2浓度" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "脑血流" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "血压降低" }, { "start_idx": 71, "end_idx": 72, "type": "ite", "entity": "血压" }, { "start_idx": 76, "end_idx": 80, "type": "sym", "entity": "脑血流减少" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "脑血流" }, { "start_idx": 84, "end_idx": 94, "type": "dis", "entity": "动脉边缘带的缺血性损害" } ] }, { "text": "(二)脑细胞能量代谢衰竭缺氧时,细胞内氧化代谢障碍,只能依靠葡萄糖无氧酵解产生能量,同时产生大量乳酸并堆积在细胞内,导致细胞内酸中毒和脑水肿。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "脑细胞能量代谢衰竭" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "缺氧" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "细胞内氧化代谢障碍" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "细胞内" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "乳酸" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "细胞内" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "细胞内酸中毒" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "脑水肿" } ] }, { "text": "由于无氧酵解产生的能量远远少于有氧代谢,必须通过增加糖原分解和葡萄糖摄取来代偿,从而引起继发性的能量衰竭,致使细胞膜离子泵功能受损,细胞内钠、钙和水增多,造成细胞肿胀和溶解。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "糖原" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "继发性的能量衰竭" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "能量衰竭" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "细胞膜离子泵功能受损" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "细胞膜离子泵" }, { "start_idx": 66, "end_idx": 75, "type": "sym", "entity": "细胞内钠、钙和水增多" }, { "start_idx": 66, "end_idx": 73, "type": "bod", "entity": "细胞内钠、钙和水" }, { "start_idx": 79, "end_idx": 85, "type": "sym", "entity": "细胞肿胀和溶解" } ] }, { "text": "(三)再灌注损伤与氧自由基的作用缺氧缺血时,氧自由基产生增多和清除减少,大量的氧自由基在体内积聚,损伤细胞膜、蛋白质和核酸,致使细胞的结构和功能破坏。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "再灌注损伤" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "氧自由基" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "缺氧" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "缺血" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "氧自由基" }, { "start_idx": 22, "end_idx": 34, "type": "sym", "entity": "氧自由基产生增多和清除减少" }, { "start_idx": 36, "end_idx": 47, "type": "sym", "entity": "大量的氧自由基在体内积聚" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "氧自由基" }, { "start_idx": 49, "end_idx": 60, "type": "sym", "entity": "损伤细胞膜、蛋白质和核酸" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "细胞膜" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "蛋白质" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "核酸" }, { "start_idx": 64, "end_idx": 73, "type": "sym", "entity": "细胞的结构和功能破坏" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "细胞" } ] }, { "text": "黄嘌呤氧化酶和脱氢酶主要集中在微血管的内皮细胞中,致使血管内皮受损,血脑屏障的结构和完整性受到破坏,形成血管源性脑水肿。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "黄嘌呤氧化酶" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "脱氢酶" }, { "start_idx": 15, "end_idx": 23, "type": "bod", "entity": "微血管的内皮细胞中" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "血管内皮" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "血管内皮受损" }, { "start_idx": 34, "end_idx": 48, "type": "sym", "entity": "血脑屏障的结构和完整性受到破坏" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "血脑屏障" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "血管源性脑水肿" } ] }, { "text": "(四)Ca2+内流缺氧时,钙泵活性减弱,导致钙内流。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "bod", "entity": "Ca2+" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "缺氧" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "钙泵活性减弱" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "钙泵" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "钙内流" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "钙" } ] }, { "text": "蛋白酶激活,可催化黄嘌呤脱氢酶变成黄嘌呤氧化酶,后者在恢复氧供和血流时催化次黄嘌呤变成黄嘌呤,同时产生自由基,进一步加重神经细胞的损伤。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "蛋白酶" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "黄嘌呤脱氢酶" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "黄嘌呤氧化酶" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血流" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "次黄嘌呤" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "黄嘌呤" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "自由基" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "神经细胞的损伤" } ] }, { "text": "(五)兴奋性氨基酸的神经毒性作用能量衰竭可致钠泵功能受损,细胞外K+堆积,细胞膜持续去极化,突触前神经元释放大量的兴奋性氨基酸(谷氨酸),同时伴突触后谷氨酸的回摄受损,致使突触间隙内谷氨酸增多,过度激活突触后的谷氨酸受体。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "sym", "entity": "兴奋性氨基酸的神经毒性作用" }, { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "兴奋性氨基酸" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "能量衰竭" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "钠泵功能受损" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "钠泵" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "细胞外K+堆积" }, { "start_idx": 29, "end_idx": 39, "type": "bod", "entity": "细胞外K+" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "细胞膜" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "细胞膜持续去极化" }, { "start_idx": 52, "end_idx": 57, "type": "bod", "entity": "突触前神经元" }, { "start_idx": 52, "end_idx": 73, "type": "sym", "entity": "突触前神经元释放大量的兴奋性氨基酸(谷氨酸)" }, { "start_idx": 63, "end_idx": 68, "type": "bod", "entity": "兴奋性氨基酸" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "突触后" }, { "start_idx": 78, "end_idx": 88, "type": "sym", "entity": "突触后谷氨酸的回摄受损" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 92, "end_idx": 101, "type": "sym", "entity": "突触间隙内谷氨酸增多" }, { "start_idx": 92, "end_idx": 96, "type": "bod", "entity": "突触间隙内" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 103, "end_idx": 115, "type": "sym", "entity": "过度激活突触后的谷氨酸受体" }, { "start_idx": 107, "end_idx": 115, "type": "bod", "entity": "突触后的谷氨酸受体" } ] }, { "text": "非N-甲基-D-门冬氨酸(NMDA)受体激活时,Na+内流,Cl-和H2O也被动进入细胞内,引起神经元的快速死亡;NMDA受体激活时,Ca2+内流,又可导致一系列生化连锁反应,引起迟发性神经元死亡。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "bod", "entity": "非N-甲基-D-门冬氨酸(NMDA)受体" }, { "start_idx": 24, "end_idx": 32, "type": "bod", "entity": "Na+" }, { "start_idx": 36, "end_idx": 44, "type": "bod", "entity": "Cl-" }, { "start_idx": 46, "end_idx": 54, "type": "bod", "entity": "H2O" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "细胞内" }, { "start_idx": 66, "end_idx": 73, "type": "sym", "entity": "神经元的快速死亡" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "神经元" }, { "start_idx": 75, "end_idx": 80, "type": "bod", "entity": "NMDA受体" }, { "start_idx": 85, "end_idx": 94, "type": "bod", "entity": "Ca2+" }, { "start_idx": 114, "end_idx": 121, "type": "sym", "entity": "迟发性神经元死亡" }, { "start_idx": 117, "end_idx": 119, "type": "bod", "entity": "神经元" } ] }, { "text": "当有金属铁存在时,ONOO•能分解产生自由基,OH•和具有很强的细胞毒性作用。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "bod", "entity": "ONOO•" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "自由基" }, { "start_idx": 29, "end_idx": 37, "type": "bod", "entity": "OH•" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "细胞" } ] }, { "text": "此外,NO也可介导谷氨酸的毒性作用,还可通过损害线粒体、蛋白质和DNA而直接引起神经元损伤。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "NO" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 22, "end_idx": 34, "type": "sym", "entity": "损害线粒体、蛋白质和DNA" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "线粒体" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "蛋白质" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "DNA" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "神经元损伤" } ] }, { "text": "缺氧缺血时,Ca2+内流,当细胞内Ca2+积聚到一定水平时,可激活一氧化氮合酶(NOS),合成大量的NO。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "缺氧" }, { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "缺血" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 15, "type": "bod", "entity": "Ca2+" }, { "start_idx": 20, "end_idx": 32, "type": "bod", "entity": "细胞内Ca2+" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "氧化氮合酶" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "NOS" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "NO" } ] }, { "text": "NOS有三种不同的亚型,神经元型和诱导型NOS分别介导早期和晚期神经毒性作用,而内皮细胞型NOS产生的NO能扩张血管而起神经保护作用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "NOS" }, { "start_idx": 12, "end_idx": 22, "type": "bod", "entity": "神经元型和诱导型NOS" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "神经毒性" }, { "start_idx": 40, "end_idx": 52, "type": "bod", "entity": "内皮细胞型NOS产生的NO" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "血管" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "神经" } ] }, { "text": "(七)凋亡与迟发性神经元死亡过去认为缺氧缺血后神经细胞损伤是由于急性能量衰竭造成细胞坏死,但不能解释窒息复苏后患儿可有短暂的相对正常期,而于疾病数小时后出现迟发性脑损伤的表现。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "凋亡" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "迟发性神经元死亡" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "神经元" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "缺氧" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "缺血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "神经细胞损伤" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "急性能量衰竭" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "细胞坏死" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "窒息" }, { "start_idx": 78, "end_idx": 83, "type": "dis", "entity": "迟发性脑损伤" } ] }, { "text": "研究证实缺氧缺血可引起两种不同类型的细胞死亡,即坏死和凋亡。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "缺氧" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "缺血" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "血" }, { "start_idx": 11, "end_idx": 21, "type": "sym", "entity": "两种不同类型的细胞死亡" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "细胞" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "坏死" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "凋亡" } ] }, { "text": "迟发性神经元死亡实质上就是细胞凋亡,在动物模型中检测到一系列凋亡相关基因的表达。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "迟发性神经元死亡" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "神经元" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "细胞凋亡" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "细胞" }, { "start_idx": 27, "end_idx": 35, "type": "bod", "entity": "一系列凋亡相关基因" } ] }, { "text": "总之,HIE的发病机制非常复杂,是由多种机制综合作用所致的一系列生化连锁反应的结果。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "HIE" } ] }, { "text": "大量研究证实多数神经元不是死于缺氧缺血时,而是死于缺氧缺血后数小时至数天,这种迟发性的细胞死亡可通过缺氧缺血后开始的干预来预防或减轻。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "神经元" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "缺氧" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "缺血" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "血" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "缺氧" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "缺血" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "血" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "迟发性的细胞死亡" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "细胞" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "缺氧" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "缺血" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "血" } ] }, { "text": "【病理变化】HIE的病理变化与胎龄、损伤性质和程度密切相关,主要有以下几种病理类型:(一)两侧大脑半球损伤主要见于足月儿,窒息为不完全性,首先发生器官间的血液分流(潜水反射)以保证心、脑血供;随着缺氧持续,血压下降,血流第二次重新分布(脑内分流),即大脑半球的血供由于前脑循环血管收缩而减少,而丘脑、脑干和小脑的血供则由于后脑循环血管扩张而增加。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "HIE" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "损伤" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "两侧大脑半球损伤" }, { "start_idx": 61, "end_idx": 67, "type": "sym", "entity": "窒息为不完全性" }, { "start_idx": 73, "end_idx": 80, "type": "sym", "entity": "器官间的血液分流" }, { "start_idx": 73, "end_idx": 78, "type": "bod", "entity": "器官间的血液" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "潜水反射" }, { "start_idx": 90, "end_idx": 90, "type": "bod", "entity": "心" }, { "start_idx": 92, "end_idx": 92, "type": "bod", "entity": "脑" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "血" }, { "start_idx": 98, "end_idx": 99, "type": "sym", "entity": "缺氧" }, { "start_idx": 103, "end_idx": 104, "type": "ite", "entity": "血压" }, { "start_idx": 103, "end_idx": 106, "type": "sym", "entity": "血压下降" }, { "start_idx": 108, "end_idx": 116, "type": "sym", "entity": "血流第二次重新分布" }, { "start_idx": 108, "end_idx": 109, "type": "bod", "entity": "血流" }, { "start_idx": 118, "end_idx": 121, "type": "sym", "entity": "脑内分流" }, { "start_idx": 118, "end_idx": 119, "type": "bod", "entity": "脑内" }, { "start_idx": 125, "end_idx": 144, "type": "sym", "entity": "大脑半球的血供由于前脑循环血管收缩而减少" }, { "start_idx": 125, "end_idx": 128, "type": "bod", "entity": "大脑半球" }, { "start_idx": 130, "end_idx": 130, "type": "bod", "entity": "血" }, { "start_idx": 134, "end_idx": 139, "type": "bod", "entity": "前脑循环血管" }, { "start_idx": 147, "end_idx": 148, "type": "bod", "entity": "丘脑" }, { "start_idx": 147, "end_idx": 171, "type": "sym", "entity": "丘脑、脑干和小脑的血供则由于后脑循环血管扩张而增加" }, { "start_idx": 150, "end_idx": 151, "type": "bod", "entity": "脑干" }, { "start_idx": 153, "end_idx": 154, "type": "bod", "entity": "小脑" }, { "start_idx": 156, "end_idx": 156, "type": "bod", "entity": "血" }, { "start_idx": 161, "end_idx": 166, "type": "bod", "entity": "后脑循环血管" } ] }, { "text": "因此,大脑半球较易受损,常伴严重脑水肿。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "大脑半球较易受损" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑水肿" } ] }, { "text": "(二)基底节、丘脑和脑干损伤为完全性窒息,两次血流重新分布的代偿机制失效,脑部损害以丘脑和脑干为主,而脑外器官和大脑半球的损害可不严重,脑水肿较轻。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "基底节、丘脑和脑干损伤" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "完全性窒息" }, { "start_idx": 21, "end_idx": 35, "type": "sym", "entity": "两次血流重新分布的代偿机制失效" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血流" }, { "start_idx": 37, "end_idx": 48, "type": "dis", "entity": "脑部损害以丘脑和脑干为主" }, { "start_idx": 51, "end_idx": 62, "type": "dis", "entity": "脑外器官和大脑半球的损害" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "脑水肿" } ] }, { "text": "(三)脑室周围白质软化主要见于早产儿,侧脑室周围缺氧缺血,导致深部白质脑细胞死亡,常呈对称性分布,以后可发生以两下肢受累为主的瘫痪。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "脑室周围白质软化" }, { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "脑室周围白质" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "早产儿" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "侧脑室周围缺氧缺血" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "侧脑室周围" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" }, { "start_idx": 31, "end_idx": 47, "type": "sym", "entity": "深部白质脑细胞死亡,常呈对称性分布" }, { "start_idx": 31, "end_idx": 37, "type": "bod", "entity": "深部白质脑细胞" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "两下肢受累为主的瘫痪" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "两下肢" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "瘫痪" } ] }, { "text": "(四)脑室周围室管膜下/脑室内出血主要见于早产儿,室管膜下生发组织出血,伴脑室内出血。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "dis", "entity": "脑室周围室管膜下/脑室内出血" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "早产儿" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "室管膜下生发组织出血" }, { "start_idx": 25, "end_idx": 32, "type": "bod", "entity": "室管膜下生发组织" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "血" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "脑室内出血" } ] }, { "text": "【临床表现】患儿有严重的宫内窘迫或出生时严重窒息史,出生后12~24小时内出现神经系统症状,根据意识、肌张力改变、原始反射异常、惊厥和脑干受损等表现,可分为轻、中、重三度(表5-5)。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "宫内窘迫" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "严重窒息" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "神经系统" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "肌张力改变" }, { "start_idx": 51, "end_idx": 53, "type": "ite", "entity": "肌张力" }, { "start_idx": 57, "end_idx": 62, "type": "sym", "entity": "原始反射异常" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "惊厥" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "脑干受损" } ] }, { "text": "表5-5新生儿缺氧缺血性脑病临床表现分度(一)轻度主要表现为兴奋,易激惹,肌张力正常,拥抱反射活跃,吸吮反射正常,呼吸平稳,无惊厥。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "新生儿缺氧缺血性脑病" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "兴奋" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "易激惹" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "肌张力" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "拥抱反射活跃" }, { "start_idx": 50, "end_idx": 53, "type": "ite", "entity": "吸吮反射" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "惊厥" } ] }, { "text": "(二)中度表现为嗜睡或抑制,嗜睡或抑制,吸吮反射和拥抱反射减弱,约1/2病例出现惊厥。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "嗜睡" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "嗜睡" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "吸吮反射和拥抱反射减弱" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "吸吮反射" }, { "start_idx": 25, "end_idx": 28, "type": "ite", "entity": "拥抱反射" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "惊厥" } ] }, { "text": "足月儿上肢肌张力降低比下肢严重,提示病变累及矢状窦旁区。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "上肢肌张力降低比下肢严重" }, { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "上肢肌张力" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "下肢" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "病变累及矢状窦旁区" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "矢状窦旁区" } ] }, { "text": "(三)重度患儿处于昏迷状态,肌张力极度低下,松软,拥抱反射、腱反射消失,瞳孔不等大,对光反应差,前囟隆起,惊厥频繁,呼吸不规则或暂停,甚至出现呼吸衰竭。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "昏迷状态" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "肌张力极度低下" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "肌张力" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "松软" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "拥抱反射、腱反射消失" }, { "start_idx": 25, "end_idx": 28, "type": "ite", "entity": "拥抱反射" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "腱反射" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "瞳孔" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "瞳孔不等大" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "对光反应差" }, { "start_idx": 42, "end_idx": 45, "type": "ite", "entity": "对光反应" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "前囟隆起" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "前囟" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "惊厥频繁" }, { "start_idx": 58, "end_idx": 65, "type": "sym", "entity": "呼吸不规则或暂停" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "【诊断】新生儿HIE的诊断主要依据病史和临床表现,但同时要做影像学和其他检查,对病情严重程度及预后进行评价。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "新生儿HIE" }, { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "影像学和其他检查" } ] }, { "text": "(一)影像学检查1.头脑超声检查HIE时,可见普遍回声增强,脑室变窄或消失,提示脑水肿;散在的高回声区,提示散在的脑实质缺血;局限性高回声区,提示该部位有缺血性损害;脑室周围高回声区,多见于侧脑室外角的后方,可能有脑室周围白质软化。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "影像学检查" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "头脑超声检查" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "HIE" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "回声增强" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "脑室" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "脑室变窄或消失" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "脑水肿" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "散在的高回声区" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "脑实质缺血" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "局限性高回声区" }, { "start_idx": 77, "end_idx": 81, "type": "dis", "entity": "缺血性损害" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "脑室周围" }, { "start_idx": 83, "end_idx": 102, "type": "sym", "entity": "脑室周围高回声区,多见于侧脑室外角的后方" }, { "start_idx": 95, "end_idx": 102, "type": "bod", "entity": "侧脑室外角的后方" }, { "start_idx": 107, "end_idx": 114, "type": "sym", "entity": "脑室周围白质软化" }, { "start_idx": 107, "end_idx": 112, "type": "bod", "entity": "脑室周围白质" } ] }, { "text": "2.CT检查轻度表现为散在、局灶性低密度影分布于两个脑叶;中度表现为低密度影超过两个脑叶,白质与灰质的对比模糊;重度表现为大脑半球弥漫性低密度影,白质与灰质界限消失,侧脑室变窄。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "CT检查" }, { "start_idx": 11, "end_idx": 27, "type": "sym", "entity": "散在、局灶性低密度影分布于两个脑叶" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "两个脑叶" }, { "start_idx": 34, "end_idx": 54, "type": "sym", "entity": "低密度影超过两个脑叶,白质与灰质的对比模糊" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "两个脑叶" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "大脑半球" }, { "start_idx": 61, "end_idx": 71, "type": "sym", "entity": "大脑半球弥漫性低密度影" }, { "start_idx": 73, "end_idx": 79, "type": "bod", "entity": "白质与灰质界限" }, { "start_idx": 73, "end_idx": 81, "type": "sym", "entity": "白质与灰质界限消失" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "侧脑室" }, { "start_idx": 83, "end_idx": 87, "type": "sym", "entity": "侧脑室变窄" } ] }, { "text": "正常新生儿(尤其是早产儿)脑水分多,髓鞘发育不成熟,可存在广泛的低密度,因此HIE低密度的诊断CT值应在18以下。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "早产儿" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "脑水分" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "脑水分多" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "髓鞘" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "髓鞘发育不成熟" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "存在广泛的低密度" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "HIE" }, { "start_idx": 41, "end_idx": 49, "type": "ite", "entity": "低密度的诊断CT值" } ] }, { "text": "3.磁共振成像(MRI)MRI不仅能检出急性期HIE的存在、分布和严重性,而且能帮助判断预后,还能发现髓鞘形成是否延迟或异常,以判断神经发育情况。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "磁共振成像" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "MRI" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "MRI" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "急性期HIE" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "髓鞘" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "神经" } ] }, { "text": "(二)脑功能检查1.脑电图(EEG)检查表现为节律紊乱、低波幅背景波上的棘慢波爆发或持续弥漫性慢活动;出现“爆发抑制”、“低电压”甚至“电静息”,则为重度HIE。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "脑功能检查" }, { "start_idx": 10, "end_idx": 19, "type": "pro", "entity": "脑电图(EEG)检查" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "节律紊乱" }, { "start_idx": 28, "end_idx": 49, "type": "sym", "entity": "低波幅背景波上的棘慢波爆发或持续弥漫性慢活动" }, { "start_idx": 51, "end_idx": 71, "type": "sym", "entity": "出现“爆发抑制”、“低电压”甚至“电静息”" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "重度HIE" } ] }, { "text": "2.脑干诱发电位检查表现为出波延迟、潜伏期延长、波幅变平及波脱失。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "脑干诱发电位检查" }, { "start_idx": 2, "end_idx": 31, "type": "sym", "entity": "脑干诱发电位检查表现为出波延迟、潜伏期延长、波幅变平及波脱失" } ] }, { "text": "3.多普勒超声脑血流速度(CBV)测定有助于了解脑灌注情况,高CBV提示存在脑血管麻痹和缺乏自主调节,低CBV提示存在广泛的脑坏死、低灌注甚至无灌流。", "entities": [ { "start_idx": 2, "end_idx": 18, "type": "pro", "entity": "多普勒超声脑血流速度(CBV)测定" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "脑" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "高CBV" }, { "start_idx": 38, "end_idx": 49, "type": "sym", "entity": "脑血管麻痹和缺乏自主调节" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "脑血管" }, { "start_idx": 51, "end_idx": 54, "type": "ite", "entity": "低CBV" }, { "start_idx": 51, "end_idx": 73, "type": "sym", "entity": "低CBV提示存在广泛的脑坏死、低灌注甚至无灌流" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "脑坏死" } ] }, { "text": "(三)脑代谢监测1.磁共振频谱(MRS)MRS是一种无创伤性检测体内化学成分(如脑组织的ATP、磷酸肌酸、乳酸等)的方法,能在活体上测得脑组织的代谢情况,比MRI能更早期敏感地反映缺氧缺血脑损伤。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "脑代谢监测" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "磁共振频谱" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "MRS" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "MRS" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "创伤" }, { "start_idx": 40, "end_idx": 54, "type": "bod", "entity": "脑组织的ATP、磷酸肌酸、乳酸" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "脑组织" }, { "start_idx": 78, "end_idx": 80, "type": "pro", "entity": "MRI" }, { "start_idx": 90, "end_idx": 96, "type": "dis", "entity": "缺氧缺血脑损伤" } ] }, { "text": "2.红外光谱测定技术(NIRS)NIRS是近年来国外新兴的光学诊断技术,可直接测出脑组织中氧合血红蛋白及还原血红蛋白的变化,实际了解脑内氧合情况,间接反映脑血流动力学状况及细胞内生物氧化过程。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "红外光谱测定技术" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "NIRS" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "NIRS" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "光学诊断技术" }, { "start_idx": 41, "end_idx": 57, "type": "bod", "entity": "脑组织中氧合血红蛋白及还原血红蛋白" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "脑内" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "脑血流" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "细胞内" } ] }, { "text": "(四)生化指标测定神经烯醇化酶(NSE)、S-100蛋白(S-100)和脑型肌酸磷酸激酶(CK-BB)存在于神经组织的不同部位,HIE后6~72小时外周血和脑脊液中的水平升高,与脑损害呈正相关,可作为HIE早期诊断的标志物。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "生化指标测定" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "神经烯醇化酶" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "NSE" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "S-100蛋白" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "S-100" }, { "start_idx": 36, "end_idx": 43, "type": "bod", "entity": "脑型肌酸磷酸激酶" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "CK-BB" }, { "start_idx": 54, "end_idx": 62, "type": "bod", "entity": "神经组织的不同部位" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "HIE" }, { "start_idx": 74, "end_idx": 86, "type": "sym", "entity": "外周血和脑脊液中的水平升高" }, { "start_idx": 74, "end_idx": 84, "type": "ite", "entity": "外周血和脑脊液中的水平" }, { "start_idx": 89, "end_idx": 91, "type": "dis", "entity": "脑损害" }, { "start_idx": 100, "end_idx": 102, "type": "dis", "entity": "HIE" } ] }, { "text": "【治疗】HIE是一个多环节、多因素的病理生理过程,患儿对缺氧的耐受性差异很大,因此,HIE的治疗应当根据患者的特点,在缺氧缺血的不同阶段进行针对性的个体化联合治疗,才能提高疗效、减少毒副反应。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "HIE" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "缺氧" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "HIE" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "缺氧" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "缺血" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "血" }, { "start_idx": 70, "end_idx": 80, "type": "sym", "entity": "针对性的个体化联合治疗" } ] }, { "text": "(一)监护对HIE患儿应密切监护,不仅观察神经系统症状还要监护各脏器损害情况。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "监护" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "HIE" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "密切监护" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "观察神经系统症状" }, { "start_idx": 29, "end_idx": 37, "type": "pro", "entity": "监护各脏器损害情况" } ] }, { "text": "(二)维持组织最佳的氧合和灌流重度窒息患儿PaCO2常升高,应改善通气,但要防止PaCO2过低而致脑血流减少,尤其是早产儿可造成脑室周围白质软化,近年发现轻度高碳酸血症有神经保护作用。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "pro", "entity": "维持组织最佳的氧合和灌流" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "重度窒息" }, { "start_idx": 21, "end_idx": 34, "type": "sym", "entity": "PaCO2常升高" }, { "start_idx": 21, "end_idx": 31, "type": "ite", "entity": "PaCO2" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "改善通气" }, { "start_idx": 46, "end_idx": 58, "type": "sym", "entity": "PaCO2过低" }, { "start_idx": 46, "end_idx": 56, "type": "ite", "entity": "PaCO2" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "脑血流" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "脑血流减少" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "早产儿" }, { "start_idx": 76, "end_idx": 81, "type": "bod", "entity": "脑室周围白质" }, { "start_idx": 76, "end_idx": 83, "type": "sym", "entity": "脑室周围白质软化" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "神经" } ] }, { "text": "严重缺氧的新生儿出生时常有低血压,可给予多巴胺和多巴酚丁胺,维持收缩压在50mmHg以上,有利于改善肾脏的灌流和心肌收缩力。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "严重缺氧" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "低血压" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "多巴胺" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "多巴酚丁胺" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "肾脏" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "心肌" } ] }, { "text": "由于缺氧后脑血流自主调节功能障碍,应尽量避免血压的剧烈波动而致颅内出血。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "缺氧" }, { "start_idx": 5, "end_idx": 15, "type": "dis", "entity": "脑血流自主调节功能障碍" }, { "start_idx": 18, "end_idx": 28, "type": "pro", "entity": "尽量避免血压的剧烈波动" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "颅内出血" } ] }, { "text": "(三)适当限制液体入量和控制脑水肿对脑水肿的处理应从控制液体量入手,若有明显颅高压症状和体征,可予甘露醇治疗,每次0.25g/kg,甘露醇虽能减轻脑水肿,但不能改善最终脑损伤的程度,这与成年动物实验结果不同,成年动物脑水肿可加重组织坏死,早期使用甘露醇可减轻HIE的损害程度,而新生儿颅压增高时,HIE可通过颅缝和囟门缓冲减压,对脑灌注的影响不大,因此缺氧缺血后预防性地应用甘露醇无明显神经保护作用。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑水肿" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑水肿" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "颅高压" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "颅" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "甘露醇" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "甘露醇" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "脑水肿" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "脑损伤" }, { "start_idx": 108, "end_idx": 110, "type": "dis", "entity": "脑水肿" }, { "start_idx": 112, "end_idx": 117, "type": "sym", "entity": "加重组织坏死" }, { "start_idx": 114, "end_idx": 117, "type": "dis", "entity": "组织坏死" }, { "start_idx": 123, "end_idx": 125, "type": "dru", "entity": "甘露醇" }, { "start_idx": 129, "end_idx": 131, "type": "dis", "entity": "HIE" }, { "start_idx": 133, "end_idx": 134, "type": "sym", "entity": "损害" }, { "start_idx": 142, "end_idx": 143, "type": "ite", "entity": "颅压" }, { "start_idx": 142, "end_idx": 145, "type": "sym", "entity": "颅压增高" }, { "start_idx": 148, "end_idx": 150, "type": "dis", "entity": "HIE" }, { "start_idx": 154, "end_idx": 155, "type": "bod", "entity": "颅缝" }, { "start_idx": 157, "end_idx": 158, "type": "bod", "entity": "囟门" }, { "start_idx": 165, "end_idx": 165, "type": "bod", "entity": "脑" }, { "start_idx": 176, "end_idx": 177, "type": "sym", "entity": "缺氧" }, { "start_idx": 178, "end_idx": 179, "type": "sym", "entity": "缺血" }, { "start_idx": 179, "end_idx": 179, "type": "bod", "entity": "血" }, { "start_idx": 187, "end_idx": 189, "type": "dru", "entity": "甘露醇" }, { "start_idx": 193, "end_idx": 194, "type": "bod", "entity": "神经" } ] }, { "text": "至于地塞米松对血管源性脑水肿有效,但不能减轻细胞毒性脑水肿,而HIE的脑水肿以细胞毒性为主。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "地塞米松" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "血管源性脑水肿" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "细胞毒性脑水肿" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "HIE" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "脑水肿" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "细胞" } ] }, { "text": "虽有动物实验提示预防性应用地塞米松可减轻HIE,但未能证实缺氧缺血后应用地塞米松有神经保护作用。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "地塞米松" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "HIE" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "缺氧" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "缺血" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "血" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "地塞米松" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "神经" } ] }, { "text": "(四)及时控制惊厥首选苯巴比妥,苯巴比妥不仅可镇静止痉,且可降低脑代谢率,改善脑血流,减轻脑水肿,还有清除自由基的作用。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "惊厥" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "镇静止痉" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脑" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "脑血流" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "脑水肿" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "自由基" } ] }, { "text": "因此,有建议对重度窒息患儿早期(6小时以内)预防性应用苯巴比妥,然而近年的研究未能证实早期应用苯巴比妥的有益效果,所以目前仍推荐在症状出现后才开始抗惊厥治疗。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "重度窒息" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 73, "end_idx": 77, "type": "pro", "entity": "抗惊厥治疗" } ] }, { "text": "可用苯巴比妥,负荷量15~20mg/kg,缓慢静脉注射或肌注,如未能止痉,隔30分钟加用5mg/kg,直至负荷量30mg/kg,给负荷量24小时后,给维持量每天5mg/kg,给1次。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "缓慢静脉注射" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "肌注" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "止痉" } ] }, { "text": "(五)维持适当的血糖水平动物实验证实低血糖会加重HIE,而高血糖能降低脑损伤。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "血糖水平" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "低血糖" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "血糖" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "HIE" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "高血糖" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "血糖" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "脑损伤" } ] }, { "text": "因此,在新生儿缺氧时应维持血糖水平在正常水平(70~120mg/dl)。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "缺氧" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "血糖水平" } ] }, { "text": "(六)其他治疗在HIE的治疗方面有关高压氧、脑代谢激活剂、纳洛酮、维生素C等的应用尚存在许多争议,有待于进一步深入研究,应采用严格的随机对照多中心临床试验。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "HIE" }, { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "脑代谢激活剂" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "纳洛酮" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "维生素C" } ] }, { "text": "(七)早期康复干预0~2岁小儿脑处于快速发育的灵敏期,可塑性强,因此对HIE患儿尽早开始感知刺激和动作训练可促进脑结构和功能代偿,有利于患儿的恢复和减轻后遗症。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "脑" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "HIE" }, { "start_idx": 40, "end_idx": 52, "type": "pro", "entity": "尽早开始感知刺激和动作训练" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "脑" } ] }, { "text": "(八)HIE的治疗展望1.寻找阻断缺氧缺血脑损伤瀑布式发展的神经保护药物氧自由基抑制剂、钙通道阻滞剂、兴奋性氨基酸释放抑制剂及受体阻滞剂。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "HIE" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "缺氧缺血脑损伤" }, { "start_idx": 30, "end_idx": 35, "type": "dru", "entity": "神经保护药物" }, { "start_idx": 36, "end_idx": 42, "type": "dru", "entity": "氧自由基抑制剂" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "钙通道阻滞剂" }, { "start_idx": 51, "end_idx": 61, "type": "dru", "entity": "兴奋性氨基酸释放抑制剂" }, { "start_idx": 63, "end_idx": 67, "type": "dru", "entity": "受体阻滞剂" } ] }, { "text": "2.亚低温疗法近年,亚低温(降低脑温或体温2~4℃)对HIE的神经保护作用已引起了国内外学者的关注。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "亚低温疗法" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "亚低温" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "脑温" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "体温" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "HIE" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "神经" } ] }, { "text": "其作用机制是:降低脑组织的能量需求和耗氧量;改善细胞的能量代谢,减少脑组织的乳酸堆积;保护血脑屏障,减轻脑水肿;抑制有害物质的释放,减少对脑组织的损害;延迟继发性能量衰竭和细胞凋亡,延长治疗时间窗,与其他干预措施起协同的保护作用。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "脑组织" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "细胞" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "脑组织的乳酸" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "血脑屏障" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "脑水肿" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "脑组织的损害" }, { "start_idx": 78, "end_idx": 84, "type": "sym", "entity": "继发性能量衰竭" }, { "start_idx": 86, "end_idx": 89, "type": "sym", "entity": "细胞凋亡" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "细胞" } ] }, { "text": "临床研究显示亚低温有较好的疗效。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "亚低温" } ] }, { "text": "3.神经营养因子实验证实,在HIE的高兴奋阶段后,内源性神经营养因子的表达增加,这可能是一种内源性的神经保护机制。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "神经营养因子实验" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "HIE" }, { "start_idx": 25, "end_idx": 38, "type": "sym", "entity": "内源性神经营养因子的表达增加" }, { "start_idx": 25, "end_idx": 33, "type": "bod", "entity": "内源性神经营养因子" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "神经" } ] }, { "text": "因此,应用外源性神经营养因子改善细胞周围环境,促进受损神经细胞的修复和再生的研究已日益受到重视。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dru", "entity": "外源性神经营养因子" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "细胞周围环境" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "受损神经细胞" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "神经细胞" } ] }, { "text": "参考文献1.沈晓明.临床儿科学.北京:人民卫生出版社,2005年:225-2252.岳少杰.新生儿细菌性脑膜炎诊断中的几个问题.中国实用儿科杂志,2011,26(1):11-113.余佳.化脓性脑膜炎的临床研究进展.儿科药学杂志,2006,12(3):61-634.王艺,王传清,王晓红.细菌性脑膜炎266例病原学与耐药性分析.实用儿科临床杂志,2006(06):355-3555.ChaudhuriA,P.M.Martin,P.G.E.Kennedy,etal.EFNSguidelineonthemanagementofcommunity-acquiredbacterialmeningitis:reportofEFNSTaskForceonacutebacterialmeningitisinolderchildrenandadults.EuropeanJournalofNeurology,2008,15:649-6596.ChaudhuriA.Adjuvantdexamethasoneuseinacutebacterialmeningitis.LancetNeurology,2004,3:54-617.TunkelAIR,HartmanBJ,KaplanSL,etal.PracticeguidelinesfortheManagementofBacterialMeningitis.ClinicalInfectiousDiseases,2004,39:1267-1267", "entities": [ { "start_idx": 46, "end_idx": 54, "type": "dis", "entity": "新生儿细菌性脑膜炎" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 108, "end_idx": 109, "type": "dep", "entity": "儿科" }, { "start_idx": 144, "end_idx": 149, "type": "dis", "entity": "细菌性脑膜炎" }, { "start_idx": 166, "end_idx": 167, "type": "dep", "entity": "儿科" } ] }, { "text": "第七节急性感染性喉炎急性感染性喉炎(acuteinfectiouslaryngitis)系喉黏膜急性弥漫性炎症,婴幼儿多见。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "急性感染性喉炎" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "急性感染性喉炎" }, { "start_idx": 19, "end_idx": 43, "type": "dis", "entity": "acuteinfectiouslaryngitis" }, { "start_idx": 46, "end_idx": 55, "type": "dis", "entity": "喉黏膜急性弥漫性炎症" } ] }, { "text": "由于婴幼儿喉部解剖生理特点,易发生喉痉挛,重者因黏膜高度充血、肿胀,致喉梗阻而引起窒息死亡。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "喉部解剖" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "喉痉挛" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "黏膜高度充血、肿胀" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "黏膜" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "喉梗阻" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "窒息死亡" } ] }, { "text": "常见病原体为流感病毒、副流感病毒、腺病毒等及肺炎链球菌、溶血性链球菌及葡萄球菌等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "病原体" }, { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "流感病毒" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "副流感病毒" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "腺病毒" }, { "start_idx": 22, "end_idx": 26, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 28, "end_idx": 33, "type": "mic", "entity": "溶血性链球菌" }, { "start_idx": 35, "end_idx": 38, "type": "mic", "entity": "葡萄球菌" } ] }, { "text": "本病多为急性上呼吸道感染的一部分,亦可在麻疹、流行性感冒等疾病的过程中发生。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "急性上呼吸道感染" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "麻疹" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "流行性感冒" } ] }, { "text": "如病变累及气管、支气管,则称为急性喉气管支气管炎(croup)。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "病变累及气管、支气管" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "气管" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "支气管" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "急性喉气管支气管炎" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "croup" } ] }, { "text": "初起时多有发热、流涕、咳嗽等症状,继而出现声音嘶哑及典型的犬吠样咳嗽,少数可有呛咳现象。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "流涕" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "咳嗽" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "典型的犬吠样咳嗽" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "呛咳" } ] }, { "text": "病情发展迅速,一般白天症状较轻,夜间可突然憋醒、烦躁不安、出汗,伴吸气性呼吸困难、喉鸣、鼻翼翕动、三凹征、面色苍白、口周发绀。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "突然憋醒" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "出汗" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "吸气性呼吸困难" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "喉" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "喉鸣" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "鼻翼" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "鼻翼翕动" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "三凹征" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "面色苍白" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "面" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "口周发绀" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "口周" } ] }, { "text": "听诊两肺呼吸音降低,吸气时间延长。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" }, { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "听诊两肺呼吸音降低,吸气时间延长" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "两肺" } ] }, { "text": "晚期呼吸渐渐无力而衰竭,发绀加重,意识模糊,并可有抽搐等。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "呼吸渐渐无力而衰竭" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "发绀加重" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "意识模糊" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "抽搐" } ] }, { "text": "根据吸气性呼吸困难的轻重将喉梗阻分为四度:Ⅰ度喉梗阻是指患儿只在活动后才出现吸气性喉喘鸣和呼吸困难,而安静时如常人。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "吸气性呼吸困难" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "喉梗阻" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "Ⅰ度喉梗阻" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "吸气性喉喘鸣" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "Ⅱ度喉梗阻患儿在安静时也出现喉喘鸣和吸气性呼吸困难,但无明显缺氧症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "Ⅱ度喉梗阻" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "喉喘鸣" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "吸气性呼吸困难" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "明显缺氧" } ] }, { "text": "Ⅲ度喉梗阻患儿除Ⅱ度喉梗阻的症状外,可出现缺氧早期的兴奋症状,如阵发性烦躁不安、发绀、出汗、恐惧等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "Ⅲ度喉梗阻" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "Ⅱ度喉梗阻" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "缺氧" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "兴奋症状" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "阵发性烦躁不安" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "发绀" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "出汗" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "恐惧" } ] }, { "text": "胸部听诊呼吸音明显降低,甚至听不到。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸部听诊" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "胸部听诊呼吸音明显降低,甚至听不到" } ] }, { "text": "心音较低钝,心率增快。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "心音较低钝" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "心" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "心率" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "心率增快" } ] }, { "text": "Ⅳ度喉梗阻患儿则出现抑制症状,渐呈衰竭,半昏睡或昏睡状态,由于无力呼吸,表现暂时安静,三凹征不明显,但面色发灰,肺呼吸音几乎完全消失,心音微弱若未得到及时抢救,可发生死亡。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "Ⅳ度喉梗阻" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "衰竭" }, { "start_idx": 20, "end_idx": 27, "type": "sym", "entity": "半昏睡或昏睡状态" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "无力呼吸" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "暂时安静" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "三凹征" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "面" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "面色发灰" }, { "start_idx": 56, "end_idx": 56, "type": "ite", "entity": "肺" }, { "start_idx": 56, "end_idx": 65, "type": "sym", "entity": "肺呼吸音几乎完全消失" }, { "start_idx": 67, "end_idx": 70, "type": "sym", "entity": "心音微弱" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "心" }, { "start_idx": 75, "end_idx": 78, "type": "pro", "entity": "及时抢救" } ] }, { "text": "根据声音嘶哑、犬吠样咳嗽、吸气性呼吸困难、喉鸣等典型症状和体征,诊断不难。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "犬吠样咳嗽" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "吸气性呼吸困难" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "喉鸣" } ] }, { "text": "应注意与急性喉气管支气管炎、急性会厌炎、喉水肿、喉痉挛、喉白喉、咽喉部异物、咽后壁脓肿、先天性喉喘鸣等鉴别。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "急性喉气管支气管炎" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "急性会厌炎" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "喉水肿" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "喉痉挛" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "喉白喉" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "咽喉部异物" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "咽后壁脓肿" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "先天性喉喘鸣" } ] }, { "text": "根据病情口服泼尼松,肌内注射或静脉滴注地塞米松等激素。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "口服" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "泼尼松" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "肌内注射" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "地塞米松" }, { "start_idx": 24, "end_idx": 25, "type": "dru", "entity": "激素" } ] }, { "text": "呼吸困难缓解后即可停药。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "烦躁不安患儿可适当镇静,但应避免使用影响呼吸的药物,更不宜使用冬眠合剂以免影响病情观察和加重呼吸困难。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "镇静" }, { "start_idx": 14, "end_idx": 24, "type": "pro", "entity": "避免使用影响呼吸的药物" }, { "start_idx": 27, "end_idx": 49, "type": "pro", "entity": "不宜使用冬眠合剂以免影响病情观察和加重呼吸困难" } ] }, { "text": "雾化吸入肾上腺素、布地奈德等表面皮质激素可暂时缓解症状,必要时重复使用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "布地奈德" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "表面皮质激素" } ] }, { "text": "十二、病毒性肺炎的药物治疗目前尚缺乏理想的抗病毒药物。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "病毒性肺炎的药物治疗" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "抗病毒药物" } ] }, { "text": "对呼吸道病毒治疗功效较肯定的仅限于流感病毒神经氨酸酶抑制剂和M2蛋白抑制剂(金刚烷胺、金刚乙胺)及雾化吸入利巴韦林。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "呼吸道病毒治疗" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "流感病毒" }, { "start_idx": 21, "end_idx": 28, "type": "dru", "entity": "神经氨酸酶抑制剂" }, { "start_idx": 30, "end_idx": 36, "type": "dru", "entity": "M2蛋白抑制剂" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "金刚烷胺" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "金刚乙胺" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 53, "end_idx": 56, "type": "dru", "entity": "利巴韦林" } ] }, { "text": "1.利巴韦林为广谱抗病毒剂,已广泛用于各类病毒性感染。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "利巴韦林" }, { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "广谱抗病毒剂" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "病毒性感染" } ] }, { "text": "早期应用雾化吸入或静脉给药,有一定疗效,但对重症病毒性肺炎单独使用作用尚不可靠。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "静脉给药" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "重症病毒性肺炎" } ] }, { "text": "10~15mg/(kg•d),必要时30~40mg/(kg•d),分2次静脉滴注,也可肌内注射,或0.1%溶液喷雾吸入。", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "肌内注射" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "喷雾吸入" } ] }, { "text": "国外主要通过雾化吸入治疗严重RSV感染。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "严重RSV感染" } ] }, { "text": "2.金刚烷胺或金刚乙胺可用于流感病毒A感染的防治。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "金刚烷胺" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "金刚乙胺" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "流感病毒A感染" } ] }, { "text": "后者活性比前者强,呼吸道药物浓度亦较高。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "呼吸道药物" } ] }, { "text": "但由于神经系统不良反应、对B型流感病毒无效及耐药株的出现,限制了其在临床的应用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "神经系统不良反应" }, { "start_idx": 13, "end_idx": 18, "type": "mic", "entity": "B型流感病毒" } ] }, { "text": "3.神经氨酸酶抑制剂是一类新型的抗流感病毒药物。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "神经氨酸酶抑制剂" }, { "start_idx": 16, "end_idx": 22, "type": "dru", "entity": "抗流感病毒药物" } ] }, { "text": "目前已用于临床的神经氨酸酶抑制剂包括扎那米韦、奥司他韦(达菲),可选择性抑制A型和B型流感病毒的神经氨酸酶活性,从而改变病毒正常的凝集和释放功能,减轻受感染的程度,缩短病程。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dru", "entity": "神经氨酸酶抑制剂" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "扎那米韦" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "奥司他韦" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "达菲" }, { "start_idx": 38, "end_idx": 52, "type": "mic", "entity": "A型和B型流感病毒的神经氨酸酶" }, { "start_idx": 60, "end_idx": 61, "type": "mic", "entity": "病毒" }, { "start_idx": 76, "end_idx": 77, "type": "dis", "entity": "感染" } ] }, { "text": "前者只能吸入给药,因而婴幼儿患者常无法使用。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "吸入给药" } ] }, { "text": "奥司他韦则口服给药,每次儿童2mg/kg,2次/天。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "奥司他韦" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "口服给药" } ] }, { "text": "4.免疫球蛋白近年来有报道RSV免疫球蛋白静脉使用可显著减轻病情、缩短住院时间,取得较好疗效。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 13, "end_idx": 20, "type": "dru", "entity": "RSV免疫球蛋白" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "静脉使用" } ] }, { "text": "5.干扰素可使受感染细胞转化为抗病毒状态,不断生成具有高度抗病毒活性的蛋白质,从而发挥抗病毒作用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "干扰素" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "受感染细胞" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "抗病毒" }, { "start_idx": 27, "end_idx": 37, "type": "bod", "entity": "高度抗病毒活性的蛋白质" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "抗病毒作用" } ] }, { "text": "可肌内注射、静脉注射或静脉滴注,也可滴鼻或喷雾吸入。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "肌内注射" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "静脉注射" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "滴鼻" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "喷雾吸入" } ] }, { "text": "6.阿昔洛韦(无环鸟苷)主要适用于单纯疱疹病毒、水痘-带状疱疹病毒及CMV感染者。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "阿昔洛韦" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "无环鸟苷" }, { "start_idx": 17, "end_idx": 22, "type": "mic", "entity": "单纯疱疹病毒" }, { "start_idx": 24, "end_idx": 32, "type": "mic", "entity": "水痘-带状疱疹病毒" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "CMV感染" } ] }, { "text": "一般情况下每次5mg/kg,静脉滴注,3次/天,疗程7天。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "7.更昔洛韦(丙氟鸟苷)是抑制CMV作用较强的药物。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "更昔洛韦" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "丙氟鸟苷" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "CMV" } ] }, { "text": "诱导期10mg/(kg•d),2次/天,连用14~21天,静脉滴注;维持量5~7.5mg/(kg•d),1次/天,每周5~7次,静脉滴注,或每次5~10mg/kg,2次/天,口服。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 87, "end_idx": 88, "type": "pro", "entity": "口服" } ] }, { "text": "8.其他白细胞介素-2(IL-2)、胸腺肽、阿糖腺苷、双嘧达莫、聚肌胞、泰瑞宁和丙基乙磺酸及中药制剂。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "白细胞介素-2" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "IL-2" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "胸腺肽" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "阿糖腺苷" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "聚肌胞" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "泰瑞宁" }, { "start_idx": 40, "end_idx": 44, "type": "dru", "entity": "丙基乙磺酸" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "中药制剂" } ] }, { "text": "三、化脓性胸膜炎化脓性胸膜炎(purulentpleurisy)又称脓胸(empyema),是指胸膜腔内有脓液积聚。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "化脓性胸膜炎" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "化脓性胸膜炎" }, { "start_idx": 15, "end_idx": 30, "type": "dis", "entity": "purulentpleurisy" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "脓胸" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "empyema" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "胸膜腔" }, { "start_idx": 48, "end_idx": 56, "type": "sym", "entity": "胸膜腔内有脓液积聚" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "脓液" } ] }, { "text": "多与肺部细菌感染有关,尤其是葡萄球菌感染,其次为肺炎链球菌和流感嗜血杆菌。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肺部细菌感染" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "葡萄球菌感染" }, { "start_idx": 24, "end_idx": 28, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 30, "end_idx": 35, "type": "mic", "entity": "流感嗜血杆菌" } ] }, { "text": "CaksenH等报道32例金黄色葡萄球菌肺炎中37.5%发生脓胸。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "金黄色葡萄球菌肺炎" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "脓胸" } ] }, { "text": "少数可由于肺脓疡破裂、胸膜穿刺或外科创伤、纵隔感染、膈下感染等引起。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肺脓疡破裂" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "胸膜穿刺" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "外科创伤" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "纵隔感染" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "膈下感染" } ] }, { "text": "早期表现与细菌性肺炎相似。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "细菌性肺炎" } ] }, { "text": "经抗生素治疗后可有数天的间隔期,随后出现急性中毒症状中毒症状,如面色灰白、食欲缺乏、精神委靡、高热、频咳、胸痛、呼吸困难,有时发绀。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "急性中毒" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "中毒" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "面色灰白" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "面" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "精神委靡" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "高热" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "频咳" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "胸痛" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "胸" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "有时发绀" } ] }, { "text": "积脓多时,患侧肋间隙饱满、呼吸运动减弱、听诊肺呼吸音消失,心脏及支气管受压而移向对侧。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "积脓多" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "脓" }, { "start_idx": 5, "end_idx": 27, "type": "sym", "entity": "患侧肋间隙饱满、呼吸运动减弱、听诊肺呼吸音消失" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "患侧肋间隙" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "听诊" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "心脏及支气管受压而移向对侧" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "心脏" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "支气管" } ] }, { "text": "少量积脓时可无明显体征,仅叩诊浊音、听诊呼吸音减低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "少量积脓" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "脓" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "叩诊浊音" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "叩诊" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "听诊呼吸音减低" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "听诊" } ] }, { "text": "葡萄球菌所致脓胸常并发支气管胸膜瘘和脓气胸,亦可发生化脓性心包炎、肺脓疡、肋骨骨髓炎或脑膜炎、败血症等,后者在肺炎链球菌和流感嗜血杆菌感染者更为多见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "脓胸" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "支气管胸膜瘘" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脓气胸" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "化脓性心包炎" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "肋骨骨髓炎" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "败血症" }, { "start_idx": 55, "end_idx": 68, "type": "dis", "entity": "肺炎链球菌和流感嗜血杆菌感染" } ] }, { "text": "体位改变后胸片无变化包裹性脓胸。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "胸片" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "包裹性脓胸" } ] }, { "text": "金黄色葡萄球菌引起者,脓液极为黏稠,呈黄色或黄绿色。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 11, "end_idx": 24, "type": "sym", "entity": "脓液极为黏稠,呈黄色或黄绿色" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脓液" } ] }, { "text": "肺炎链球菌引起者亦较稠厚,呈黄色。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "较稠厚" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "呈黄色" } ] }, { "text": "链球菌引起者脓液稀薄,呈米汤样。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "链球菌" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "脓液稀薄,呈米汤样" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脓液" } ] }, { "text": "在胸水常规基础上均应进行胸水培养和涂片革兰染色找细菌,同时送血培养。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "胸水" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "胸水培养" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "涂片革兰染色" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "细菌" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "送血培养" } ] }, { "text": "乳胶凝集试验可能有助于病原诊断。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "乳胶凝集试验" } ] }, { "text": "外周血白细胞计数和中性粒细胞比例常增高,血沉加快。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "外周血白细胞计数和中性粒细胞比例常增高" }, { "start_idx": 0, "end_idx": 15, "type": "ite", "entity": "外周血白细胞计数和中性粒细胞比例" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "血沉" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "血沉加快" } ] }, { "text": "(一)一般疗法卧床休息,给予高热量、富含蛋白质、维生素的饮食,补充损失的蛋白质,纠正水、电解质紊乱,必要时少量多次输血。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "卧床休息" }, { "start_idx": 12, "end_idx": 29, "type": "pro", "entity": "给予高热量、富含蛋白质、维生素的饮食" }, { "start_idx": 31, "end_idx": 38, "type": "pro", "entity": "补充损失的蛋白质" }, { "start_idx": 40, "end_idx": 48, "type": "pro", "entity": "纠正水、电解质紊乱" }, { "start_idx": 50, "end_idx": 58, "type": "pro", "entity": "必要时少量多次输血" } ] }, { "text": "(二)抗生素治疗最好根据体外药物敏感试验结果选用对致病菌敏感的抗生素。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "体外药物敏感试验" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "致病菌" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "抗生素" } ] }, { "text": "葡萄球菌感染首选耐酶青霉素,如苯甲异唑青霉素、乙氧萘青霉素或万古霉素;肺炎链球菌感染可选用青霉素,但近年来青霉素耐药率明显增高,对重症或青霉素治疗无效者应使用头孢噻肟、头孢曲松或万古霉素;流感嗜血杆菌可选用头孢呋辛、头孢噻肟、头孢曲松或阿奇霉素。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌感染" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "耐酶青霉素" }, { "start_idx": 15, "end_idx": 21, "type": "dru", "entity": "苯甲异唑青霉素" }, { "start_idx": 23, "end_idx": 28, "type": "dru", "entity": "乙氧萘青霉素" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "万古霉素" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "肺炎链球菌感染" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "青霉素" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "青霉素" }, { "start_idx": 68, "end_idx": 72, "type": "pro", "entity": "青霉素治疗" }, { "start_idx": 79, "end_idx": 82, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 84, "end_idx": 87, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 89, "end_idx": 92, "type": "dru", "entity": "万古霉素" }, { "start_idx": 94, "end_idx": 99, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 103, "end_idx": 106, "type": "dru", "entity": "头孢呋辛" }, { "start_idx": 108, "end_idx": 111, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 113, "end_idx": 116, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 118, "end_idx": 121, "type": "dru", "entity": "阿奇霉素" } ] }, { "text": "抗生素疗程至少3~4周。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "抗生素" } ] }, { "text": "(三)胸腔闭锁引流脓液稀薄者,可每日或隔日用粗针穿刺抽脓。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "胸腔闭锁引流" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "脓液稀薄" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "脓液" }, { "start_idx": 16, "end_idx": 27, "type": "pro", "entity": "每日或隔日用粗针穿刺抽脓" } ] }, { "text": "若效果不明显,可安置肋间硅胶管或导尿管,行水封式引流。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "pro", "entity": "安置肋间硅胶管或导尿管" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "行水封式引流" } ] }, { "text": "但目前多数学者认为,如胸穿抽出脓液,应立即放管通过水封式或负压引流,而不应该通过反复穿刺抽吸排脓。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "胸穿" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脓液" }, { "start_idx": 25, "end_idx": 32, "type": "pro", "entity": "水封式或负压引流" }, { "start_idx": 40, "end_idx": 47, "type": "pro", "entity": "反复穿刺抽吸排脓" } ] }, { "text": "引流管内径应尽可能大。", "entities": [] }, { "text": "多房性包裹性脓胸可能需数根引流管。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "多房性包裹性脓胸" } ] }, { "text": "引流时间一般为1周左右。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "引流" } ] }, { "text": "局部注入抗生素并不能提高疗效,并有局部不良反应。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "局部注入" }, { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "抗生素" } ] }, { "text": "有文献报道局部注入溶纤维性药物如尿激酶等可促进胸水引流排出。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "局部注入" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "溶纤维性药物" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "尿激酶" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "胸水引流" } ] }, { "text": "参考文献1.杨锡强等.儿童免疫学.北京:人民卫生出版社,20012.蒋明等.风湿病学.北京:科学出版社,19983.BehrmanRE,KliegmanRM,JensonHB.NelsonTextbookofPediatrics,16thed,W.B.SaunersCo,Philadelphia,20004.GenslerL,DavisJCJr.Recognitionandtreatmentofjuvenileonsetspondyloarthritis.CurrOpinRheumatol,2006,18(5):507-5075.AmbrogioOrlando,SaraRenna,GiovanniPerricone,etal.Gastrointestinallesionsassociatedwithspondyloarthropathies.WorldJGastroenterol,2009,May28,15(20):2443-24436.邝伟英,韩形欣,吴凤歧,等.女童强直性关节炎10例分析.中华儿科杂志,2001,39(11):7007.魏华,李小峰.儿童强直性脊柱炎误诊风湿性关节炎30例临床分析.山西医药杂志,2003,32(3):289", "entities": [ { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "风湿病" }, { "start_idx": 447, "end_idx": 452, "type": "dis", "entity": "强直性关节炎" }, { "start_idx": 461, "end_idx": 462, "type": "dep", "entity": "儿科" }, { "start_idx": 492, "end_idx": 497, "type": "dis", "entity": "强直性脊柱炎" }, { "start_idx": 500, "end_idx": 505, "type": "dis", "entity": "风湿性关节炎" } ] }, { "text": "四、治疗原则(一)儿童肿瘤的主要治疗手段1.手术手术是非血液淋巴系统恶性肿瘤的主要治疗手段之一。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "手术" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "手术" }, { "start_idx": 27, "end_idx": 37, "type": "dis", "entity": "非血液淋巴系统恶性肿瘤" } ] }, { "text": "术前应有充分准备,明确手术目的,在预知不能完全或大部分切除时主张先作病理活检以明确诊断,然后先行化疗,使肿瘤缩小、分期前移后再手术。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "手术" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "病理活检" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "化疗" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "肿瘤" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "手术" } ] }, { "text": "2.放疗多种儿童肿瘤对放疗敏感,主要有淋巴系统恶性肿瘤、神经母细胞瘤、肾母细胞瘤、部分脑瘤、尤因肉瘤、横纹肌肉瘤。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "放疗" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "放疗" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "淋巴系统恶性肿瘤" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "脑瘤" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "放疗有明确的近远期不良反应,并可因此而影响长期生存者的远期生活质量,因此并非每个对放疗敏感的肿瘤均接受放疗,只有在明确放疗能改善其预后或利大于弊的条件下才采用放疗。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "放疗" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "肿瘤" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "放疗" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "放疗" }, { "start_idx": 79, "end_idx": 80, "type": "pro", "entity": "放疗" } ] }, { "text": "3.化疗对未完整切除或有任何残留可能的儿童肿瘤有指征给予适当的化疗,以减少复发率,增加治愈率。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "化疗" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "化疗" } ] }, { "text": "(二)分型分组分治原则1.同一种肿瘤有不同的病理形态或免疫、细胞遗传学亚型,它们对同样的治疗手段可有不同的敏感性,因此需了解这些差异分别给予不同的治疗。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" } ] }, { "text": "2.同一肿瘤在诊断时处于不同的疾病阶段,需接受不同强度的治疗,避免早期或低危险组病人接受不必要的过强的治疗,影响远期的生存质量。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(三)对治疗反应儿童肿瘤对治疗有与成人不同的反应,主要表现为以下三点:1.总体上对化疗、放疗的敏感性高于成人,因此儿童肿瘤治愈率高于成人,在合理治疗下儿童肿瘤总体治愈率可达50%。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "化疗" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "放疗" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 75, "end_idx": 78, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "由于对治疗较敏感,肿瘤高负荷病人在初始化疗时易发生肿瘤细胞溶解综合征,出现水电解质紊乱、肾功能不全、DIC等情况,应特别予以重视。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "化疗" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "肿瘤细胞溶解综合征" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "水电解质紊乱" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "肾功能不全" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "DIC" } ] }, { "text": "2.对化疗的近期耐受优于成人,儿童所采用的剂量有时不能被成人所接受。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" } ] }, { "text": "第十五篇内分泌疾病第一章生长激素缺乏症【概述】身材矮小是指在相似生活环境下,儿童身高低于同种族、同年龄、同性别个体正常身高2个标准差(s)以上,或者低于正常儿童生长曲线第3百分位数。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "内分泌疾病" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "生长激素缺乏症" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "身材矮小" }, { "start_idx": 29, "end_idx": 89, "type": "sym", "entity": "在相似生活环境下,儿童身高低于同种族、同年龄、同性别个体正常身高2个标准差(s)以上,或者低于正常儿童生长曲线第3百分位数" } ] }, { "text": "在众多因素中,内分泌的生长激素(GH)对身高的影响起着十分重要的作用。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "生长激素" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "GH" } ] }, { "text": "患儿因GH缺乏所导致的矮小,称为生长激素缺乏症(growthhormonedeficiency),以前又称为垂体性侏儒症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "GH缺乏" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "GH" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "矮小" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "生长激素缺乏症" }, { "start_idx": 24, "end_idx": 46, "type": "dis", "entity": "growthhormonedeficiency" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "垂体性侏儒症" } ] }, { "text": "GH缺乏症是儿科临床常见的内分泌疾病之一,大多为散发性,少部分为家族性遗传。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "GH缺乏症" }, { "start_idx": 6, "end_idx": 7, "type": "dep", "entity": "儿科" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "内分泌疾病" } ] }, { "text": "【流行病学】特发性GH缺乏症在英国、德国和法国人群中的发病率约为18/100万~24/100万人,瑞典的发病率约62/100万人,美国报道的发病率最高,约287/100万人。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "特发性GH缺乏症" } ] }, { "text": "在20世纪80年代末,北京协和医院调查了103753名年龄在6~15岁的中小学生身高,发现202人低于第3百分位数,其中12例诊断生长激素缺乏症,发病率为115/100万人。", "entities": [ { "start_idx": 65, "end_idx": 71, "type": "dis", "entity": "生长激素缺乏症" } ] }, { "text": "【病理生理和病因分类】(一)病理生理1.生长激素基因生长激素由腺垂体嗜酸性粒细胞分泌,其基因GH1的表达产物含191个氨基酸,分子量22kD,属非糖基化蛋白质激素,GH的半衰期为15~30分钟。", "entities": [ { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "生长激素基因" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "生长激素" }, { "start_idx": 31, "end_idx": 39, "type": "bod", "entity": "腺垂体嗜酸性粒细胞" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "基因GH1" }, { "start_idx": 55, "end_idx": 61, "type": "bod", "entity": "191个氨基酸" }, { "start_idx": 72, "end_idx": 80, "type": "bod", "entity": "非糖基化蛋白质激素" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "GH" } ] }, { "text": "人类GH基因定位于第17号染色体长臂q22~24区带,由5个外显子和4个内含子组成。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "GH基因" }, { "start_idx": 9, "end_idx": 25, "type": "bod", "entity": "第17号染色体长臂q22~24区带" } ] }, { "text": "GH基因突变包括错义突变、无义突变及移码突变等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "GH基因突变" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "GH基因" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "错义突变" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "无义突变" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "移码突变" } ] }, { "text": "2.GH的分泌在胎龄3个月内,垂体尚无GH分泌,其后血中GH水平逐步增高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "GH" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "垂体" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "GH" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "血中GH水平逐步增高" }, { "start_idx": 26, "end_idx": 31, "type": "ite", "entity": "血中GH水平" } ] }, { "text": "至12周时,GH血浓度可达到60μg/L,30周时达130μg/L,以后GH浓度逐渐下降,出生时为30μg/L,以后进一步下降。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "GH血浓度" }, { "start_idx": 36, "end_idx": 39, "type": "ite", "entity": "GH浓度" } ] }, { "text": "GH分泌一般呈脉冲式释放,昼夜波动大,在分泌低峰时,常难以测到,一般在夜间深睡眠后的早期分泌最高。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "GH" } ] }, { "text": "在血循环中,大约50%的GH与生长激素结合蛋白(GHBP)结合,以GH-GHBP复合物的形式存在。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "血循环" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "GH" }, { "start_idx": 15, "end_idx": 22, "type": "bod", "entity": "生长激素结合蛋白" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "GHBP" }, { "start_idx": 33, "end_idx": 42, "type": "bod", "entity": "GH-GHBP复合物" } ] }, { "text": "3.GH的分泌调节在垂体生长激素细胞中,GH基因的表达受三种下丘脑激素的控制:生长激素释放激素(GHRH)刺激GH释放,生长抑素则抑制GH释放,以及Ghrelin的调节。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "GH" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "垂体生长激素细胞" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "GH基因" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "下丘脑激素" }, { "start_idx": 39, "end_idx": 46, "type": "bod", "entity": "生长激素释放激素" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "GHRH" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "GH" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "生长抑素" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "GH" }, { "start_idx": 74, "end_idx": 80, "type": "bod", "entity": "Ghrelin" } ] }, { "text": "GHRH和生长抑素的交替性分泌可以解释GH的节律性分泌。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "GHRH" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "生长抑素" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "GH" } ] }, { "text": "GH的分泌高峰发生在GHRH的分泌高峰,同时又是生长抑素分泌的低谷。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "GH" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "GHRH" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "生长抑素" } ] }, { "text": "GH分泌呈脉冲式,其高峰在睡眠期间。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "GH" } ] }, { "text": "Ghrelin由下丘脑的弓形核产生,胃部也产生较大量的Ghrelin。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "Ghrelin" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "下丘脑的弓形核" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胃部" }, { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "Ghrelin" } ] }, { "text": "GH的释放受下丘脑-垂体-门脉循环和体循环的Ghrelin水平的影响,饥饿能刺激Ghrelin释放入体循环,而进食能抑制Ghrelin释放入体循环。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "GH" }, { "start_idx": 6, "end_idx": 16, "type": "bod", "entity": "下丘脑-垂体-门脉循环" }, { "start_idx": 18, "end_idx": 28, "type": "bod", "entity": "体循环的Ghrelin" }, { "start_idx": 40, "end_idx": 46, "type": "bod", "entity": "Ghrelin" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "体循环" }, { "start_idx": 60, "end_idx": 66, "type": "bod", "entity": "Ghrelin" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "体循环" } ] }, { "text": "4.GH与受体的结合GH通过与靶细胞表面的受体分子相结合而发挥作用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "GH" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "受体" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "GH" }, { "start_idx": 15, "end_idx": 24, "type": "bod", "entity": "靶细胞表面的受体分子" } ] }, { "text": "GH受体是一个具有620个氨基酸的单链分子;GH受体有细胞外区,单体的跨膜区以及胞浆区。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "GH受体" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "620个氨基酸" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "GH受体" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "细胞外区" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "单体的跨膜区" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "胞浆区" } ] }, { "text": "细胞外区的蛋白水解片段,循环于血浆中,充当为一种GH结合蛋白。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "bod", "entity": "细胞外区的蛋白水解片段" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血浆" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "GH结合蛋白" } ] }, { "text": "与细胞因子受体族的其他成分一样,GH受体的胞浆区缺乏内在的激酶活性,而GH的结合,可以诱导受体的二聚作用和一种与受体相连的Jak2的活性。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "bod", "entity": "细胞因子受体族" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "GH受体的胞浆区" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "酶" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "GH" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "受体" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "受体" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "Jak2" } ] }, { "text": "该激酶和其他蛋白质底物的磷酸化作用可引起一系列的反应。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "激酶" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "蛋白质底物" } ] }, { "text": "5.GH的生理作用GH的生理作用非常广泛,既促进生长,也调节代谢。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "GH" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "GH" } ] }, { "text": "其主要作用是:①促进骨生长;②促进蛋白质合成;③促进脂肪降解;④对糖代谢作用复杂,能减少外周组织对葡萄糖的利用,亦降低细胞对胰岛素的敏感性;⑤促进水、矿物质代谢;⑥促进脑功能效应,增强心肌功能,提高免疫功能等作用。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "骨" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "蛋白质" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "脂肪" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "糖" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "外周组织" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "细胞" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "胰岛素" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "水" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "矿物质" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "脑" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "心肌" } ] }, { "text": "6.类胰岛素生长因子-1(IGF-1)IGF-1为肝脏对GH反应时产生的一种多肽,这是一种单链多肽,由70个氨基酸组成,基因定位于第12号染色体长臂,含有6个外显子,IGF-1与胰岛素具有相当的同源性。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "bod", "entity": "类胰岛素生长因子-1" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "IGF-1" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "IGF-1" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肝脏" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "GH" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "多肽" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "单链多肽" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "氨基酸" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "基因" }, { "start_idx": 65, "end_idx": 73, "type": "bod", "entity": "第12号染色体长臂" }, { "start_idx": 83, "end_idx": 87, "type": "bod", "entity": "IGF-1" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "胰岛素" } ] }, { "text": "血中90%的IGF-1由肝脏合成,其余由成纤维细胞及胶原等细胞在局部合成。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "IGF-1" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肝脏" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "胶原" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "细胞" } ] }, { "text": "GH通过增加IGF-1的合成,介导其促进有丝分裂的作用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "GH" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "IGF-1" } ] }, { "text": "循环中的IGF-1与数种不同的结合蛋白相结合,其中主要的一种是分子量为150kD的复合物IGFBP3,IGFBP3在GH缺乏症的儿童中是降低的,但在因其他原因引起矮小的儿童中则仍在正常范围。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "循环中的IGF-1" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "结合蛋白" }, { "start_idx": 44, "end_idx": 55, "type": "bod", "entity": "IGFBP3" }, { "start_idx": 57, "end_idx": 68, "type": "bod", "entity": "IGFBP3" }, { "start_idx": 70, "end_idx": 74, "type": "dis", "entity": "GH缺乏症" }, { "start_idx": 93, "end_idx": 94, "type": "sym", "entity": "矮小" } ] }, { "text": "(二)病因分类根据下丘脑-GH-IGF生长轴功能缺陷,病因可分为原发性、继发性GH缺乏症,单纯性GH缺乏症或多种垂体激素缺乏。", "entities": [ { "start_idx": 9, "end_idx": 21, "type": "bod", "entity": "下丘脑-GH-IGF生长轴" }, { "start_idx": 9, "end_idx": 25, "type": "sym", "entity": "下丘脑-GH-IGF生长轴功能缺陷" }, { "start_idx": 32, "end_idx": 43, "type": "dis", "entity": "原发性、继发性GH缺乏症" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "单纯性GH缺乏症" }, { "start_idx": 54, "end_idx": 61, "type": "sym", "entity": "多种垂体激素缺乏" }, { "start_idx": 54, "end_idx": 59, "type": "bod", "entity": "多种垂体激素" } ] }, { "text": "1.原发性(1)遗传:正常生长激素功能的维持,需要下丘脑GHRH的分泌到GH、IGF-1的分泌,受体效应都要完整,目前下丘脑-垂体-IGF-1轴的多种基因都已发现突变,导致功能障碍,包括与垂体发育有关的基因缺陷、GH、IGF-1的编码基因和受体基因,例如PROP-1、POU1F1、GHRH、GHRH受体、GH、GH受体、IGF-1以及IGF-1受体等。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "生长激素" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "下丘脑GHRH" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "GH" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "IGF-1" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "受体" }, { "start_idx": 59, "end_idx": 82, "type": "sym", "entity": "下丘脑-垂体-IGF-1轴的多种基因都已发现突变" }, { "start_idx": 59, "end_idx": 76, "type": "bod", "entity": "下丘脑-垂体-IGF-1轴的多种基因" }, { "start_idx": 86, "end_idx": 89, "type": "dis", "entity": "功能障碍" }, { "start_idx": 93, "end_idx": 104, "type": "sym", "entity": "与垂体发育有关的基因缺陷" }, { "start_idx": 93, "end_idx": 102, "type": "bod", "entity": "与垂体发育有关的基因" }, { "start_idx": 106, "end_idx": 123, "type": "bod", "entity": "GH、IGF-1的编码基因和受体基因" }, { "start_idx": 127, "end_idx": 132, "type": "bod", "entity": "PROP-1" }, { "start_idx": 134, "end_idx": 139, "type": "bod", "entity": "POU1F1" }, { "start_idx": 141, "end_idx": 144, "type": "bod", "entity": "GHRH" }, { "start_idx": 146, "end_idx": 151, "type": "bod", "entity": "GHRH受体" }, { "start_idx": 153, "end_idx": 154, "type": "bod", "entity": "GH" }, { "start_idx": 156, "end_idx": 159, "type": "bod", "entity": "GH受体" }, { "start_idx": 161, "end_idx": 165, "type": "bod", "entity": "IGF-1" }, { "start_idx": 168, "end_idx": 174, "type": "bod", "entity": "IGF-1受体" } ] }, { "text": "(2)特发性:下丘脑功能异常,神经递质-神经激素信号传导途径的缺陷。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "下丘脑" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "下丘脑功能异常" }, { "start_idx": 15, "end_idx": 32, "type": "sym", "entity": "神经递质-神经激素信号传导途径的缺陷" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "神经递质" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "神经激素信号" } ] }, { "text": "各种先天原因引起的垂体不发育、发育不良,空蝶鞍及视中隔发育异常等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "垂体" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "垂体不发育" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "发育不良" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "空蝶鞍及视中隔发育异常" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "空蝶鞍" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "视中隔" } ] }, { "text": "2.继发性(1)肿瘤:下丘脑、垂体或颅内其他肿瘤,例如颅咽管瘤、神经纤维瘤以及错构瘤等可影响GH的分泌,造成GH缺乏。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肿瘤" }, { "start_idx": 11, "end_idx": 23, "type": "dis", "entity": "下丘脑、垂体或颅内其他肿瘤" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "神经纤维瘤" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "错构瘤" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "GH" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "GH缺乏" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "GH" } ] }, { "text": "(2)放射性损伤:下丘脑、垂体肿瘤放疗后,有一大部分存在生长激素缺乏,患急性淋巴细胞白血病的儿童,接受预防性头颅照光者也属于这一类。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "放射性损伤" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "下丘脑、垂体肿瘤" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "放疗" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "生长激素缺乏" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "生长激素" }, { "start_idx": 36, "end_idx": 44, "type": "dis", "entity": "急性淋巴细胞白血病" }, { "start_idx": 51, "end_idx": 57, "type": "pro", "entity": "预防性头颅照光" } ] }, { "text": "放疗和化疗引起典型的生长缓慢见于治疗1~2年后,由于GH缺乏,患者身高逐渐偏离正常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "生长缓慢" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "GH缺乏" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "GH" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "身高逐渐偏离正常" } ] }, { "text": "除GH缺乏外,亦可有TSH和ACTH缺乏发生。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "GH缺乏" }, { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "GH" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "TSH和ACTH缺乏" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "TSH" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "ACTH" } ] }, { "text": "(3)头部创伤:任何疾病损伤下丘脑、垂体柄及腺垂体均可导致垂体激素缺乏。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "头部创伤" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "损伤" }, { "start_idx": 12, "end_idx": 24, "type": "sym", "entity": "损伤下丘脑、垂体柄及腺垂体" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "下丘脑" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "垂体柄" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "腺垂体" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "垂体激素缺乏" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "垂体激素" } ] }, { "text": "由于这种病变是非选择性的,常存在多种垂体激素缺乏,例如在产伤、手术损伤以及颅底骨折等情况发生时。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "垂体激素缺乏" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "垂体激素" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "产伤" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "手术损伤" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "颅底骨折" } ] }, { "text": "创伤还包括儿童受虐待、牵引产、缺氧及出血性梗死等损伤垂体、垂体柄及下丘脑。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "受虐待" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "牵引产" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "缺氧" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "出血性梗死" }, { "start_idx": 24, "end_idx": 35, "type": "sym", "entity": "损伤垂体、垂体柄及下丘脑" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "垂体" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "垂体柄" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "下丘脑" } ] }, { "text": "【临床表现】GH缺乏症的部分患儿出生时有难产史、窒息史或者胎位不正,以臀位和足位产多见。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "GH缺乏症" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "难产" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "窒息" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "胎位不正" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "臀位和足位产" } ] }, { "text": "随年龄的增长,生长缓慢程度也增加,体型较实际年龄幼稚。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "生长缓慢程度也增加" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "体型较实际年龄幼稚" } ] }, { "text": "自幼食欲低下。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "食欲低下" } ] }, { "text": "患儿智力正常。", "entities": [] }, { "text": "出牙、换牙及骨龄落后。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "出牙、换牙及骨龄落后" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "牙" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "牙" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "骨" } ] }, { "text": "青春发育大多延缓(与骨龄成熟程度有关)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "青春发育大多延缓" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "骨" } ] }, { "text": "多种垂体激素缺乏患者根据病因有不同的激素缺乏和相应的临床表现。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "多种垂体激素缺乏" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "垂体激素" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "激素缺乏" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "激素" } ] }, { "text": "垂体MRI表现多数为腺垂体发育不良,蝶鞍常增大或正常,但患者中也有少数表现出增大的垂体(腺垂体增生)、垂体囊性肿物(似颅咽管瘤,或Rathke囊肿)或插入垂体前后叶之间的信号不增强的垂体肿物。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "垂体MRI" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "腺垂体发育不良" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "蝶鞍常增大" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "蝶鞍" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "增大的垂体" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "垂体" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "腺垂体增生" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "垂体囊性肿物" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "垂体" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 65, "end_idx": 72, "type": "dis", "entity": "Rathke囊肿" }, { "start_idx": 77, "end_idx": 81, "type": "bod", "entity": "垂体前后叶" }, { "start_idx": 91, "end_idx": 94, "type": "sym", "entity": "垂体肿物" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "垂体" } ] }, { "text": "但以垂体激素缺乏症状为主诉就诊者仅约10%。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "垂体激素缺乏" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "垂体激素" } ] }, { "text": "外科手术后可首先出现垂体功能减退。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "外科手术" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "垂体功能减退" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "垂体" } ] }, { "text": "【实验室检查】1.血GH测定血清GH呈脉冲式分泌,半衰期较短,随机取血检测GH无诊断价值,不能区别正常人与GH缺乏症。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 9, "end_idx": 29, "type": "sym", "entity": "血GH测定血清GH呈脉冲式分泌,半衰期较短" }, { "start_idx": 9, "end_idx": 17, "type": "ite", "entity": "血GH测定血清GH" }, { "start_idx": 33, "end_idx": 38, "type": "ite", "entity": "取血检测GH" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "GH缺乏症" } ] }, { "text": "通过GH刺激试验,GH缺乏或低水平可明确诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "GH刺激试验" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "GH缺乏或低水平" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "GH" } ] }, { "text": "临床多采用药物激发试验来判断垂体分泌GH状况(表15-15),常用药物激发剂有胰岛素、精氨酸、L-多巴及可乐定。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "药物激发试验" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "垂体" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "GH" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "药物激发剂" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "胰岛素" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "精氨酸" }, { "start_idx": 47, "end_idx": 54, "type": "dru", "entity": "L-多巴及可乐定" } ] }, { "text": "由于各种药物激发GH反应途径不同,各种试验的敏感性及特异性亦有差异,故通常采用至少2种作用途径不同的药物进行激发试验才能作为判断的结果。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "GH" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "激发试验" } ] }, { "text": "当两个不同激发试验的GH峰值均低于10μg/L时可确诊为GHD。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "pro", "entity": "激发试验的GH峰值" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "GHD" } ] }, { "text": "一般认为两种试验若GH峰值均<5μg/L,为完全性GH缺乏症;GH峰值在5.1~9.9μg/L为部分性GH缺乏;GH峰值≥10μg/L为正常反应。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "GH峰值" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "完全性GH缺乏症" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "GH峰值" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "部分性GH缺乏" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "GH" }, { "start_idx": 56, "end_idx": 59, "type": "ite", "entity": "GH峰值" } ] }, { "text": "单次试验约有20%的正常儿童呈阴性反应。", "entities": [] }, { "text": "GH激发试验前需禁食8小时以上。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "GH激发试验" } ] }, { "text": "表15-1GH缺乏症诊断常用药物激发试验2.血清IGF-1及IGFBP3测定血循环中IGF-1大多与IGFBP3结合(95%以上),IGFBP3有运送和调节IGF-1的功能,两者分泌模式与GH不同,IGF-1呈非脉冲性分泌和较少日夜波动,故血中浓度稳定,并与GH水平呈一致关系,是检测下丘脑-GH-IGF生长轴功能的指标。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "GH缺乏症" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "药物激发试验" }, { "start_idx": 22, "end_idx": 37, "type": "ite", "entity": "血清IGF-1及IGFBP3测定" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "血循环" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "IGF-1" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "IGFBP3" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "IGFBP3" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "IGF-1" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "GH" }, { "start_idx": 99, "end_idx": 103, "type": "bod", "entity": "IGF-1" }, { "start_idx": 106, "end_idx": 117, "type": "sym", "entity": "脉冲性分泌和较少日夜波动" }, { "start_idx": 120, "end_idx": 120, "type": "bod", "entity": "血" }, { "start_idx": 129, "end_idx": 130, "type": "bod", "entity": "GH" }, { "start_idx": 140, "end_idx": 159, "type": "pro", "entity": "检测下丘脑-GH-IGF生长轴功能的指标" } ] }, { "text": "IGF-1浓度与年龄有关,亦受其他内分泌激素和营养状态影响。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "ite", "entity": "IGF-1浓度" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "其他内分泌激素" } ] }, { "text": "3.影像学检查颅脑磁共振显像(MRI)可显示蝶鞍容积大小,垂体前、后叶大小,可诊断垂体不发育、发育不良,空蝶鞍及视中隔发育不良等,在区分蝶鞍饱满还是空蝶鞍上MRI优于CT。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "影像学检查" }, { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "颅脑磁共振显像" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "MRI" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "蝶鞍" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "垂体前、后叶" }, { "start_idx": 41, "end_idx": 50, "type": "sym", "entity": "垂体不发育、发育不良" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "垂体" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "发育不良" }, { "start_idx": 52, "end_idx": 62, "type": "sym", "entity": "空蝶鞍及视中隔发育不良" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "空蝶鞍" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "视中隔" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "发育不良" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "蝶鞍" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "空蝶鞍" }, { "start_idx": 78, "end_idx": 80, "type": "pro", "entity": "MRI" }, { "start_idx": 83, "end_idx": 84, "type": "pro", "entity": "CT" } ] }, { "text": "并且可发现颅咽管瘤、神经纤维瘤及错构瘤等肿瘤。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "神经纤维瘤" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "错构瘤" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肿瘤" } ] }, { "text": "生长激素缺乏者,骨成熟常明显延迟。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "生长激素缺乏" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "骨成熟常明显延迟" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "骨" } ] }, { "text": "TSH和GH同时缺乏者骨龄延迟更加明显。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "TSH和GH同时缺乏者骨龄延迟更加明显" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "TSH" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "GH" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "骨" } ] }, { "text": "4.染色体检查对女性矮小伴青春期发育延迟者应常规作染色体检查,以排除染色体病,如Turner综合征等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "染色体检查" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "矮小伴青春期发育延迟" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "染色体检查" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "染色体病" }, { "start_idx": 40, "end_idx": 48, "type": "dis", "entity": "Turner综合征" } ] }, { "text": "5.其他垂体功能检查除了确定GHD诊断外,根据临床表现可选择性地检测血TSH、T3、T4、PRL、ACTH、皮质醇及LHRH激发试验等,以判断有无甲状腺和性腺激素等缺乏。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "其他垂体功能检查" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "GHD" }, { "start_idx": 32, "end_idx": 77, "type": "pro", "entity": "检测血TSH、T3、T4、PRL、ACTH、皮质醇及LHRH激发试验" }, { "start_idx": 85, "end_idx": 95, "type": "sym", "entity": "甲状腺和性腺激素等缺乏" }, { "start_idx": 85, "end_idx": 92, "type": "bod", "entity": "甲状腺和性腺激素" } ] }, { "text": "垂体功能减退时血浆PRL水平升高,强烈提示病变在下丘脑而不是垂体。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "垂体功能减退时血浆PRL水平升高" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "垂体" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "血浆PRL" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "下丘脑" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "垂体" } ] }, { "text": "【诊断与鉴别诊断】1.对身高低于同种族、同年龄、同性别正常儿童平均身高2个标准差或第3百分位数以下者都应分析原因,仔细了解母亲孕期、围生期、喂养和疾病等情况,结合体格检查和实验室资料,进行综合分析诊断和鉴别诊断。", "entities": [ { "start_idx": 81, "end_idx": 84, "type": "pro", "entity": "体格检查" } ] }, { "text": "GHD患儿的年增长速率往往<5cm,骨龄延迟一般可大于2年以上,GH激发峰值<10μg/L。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "GHD" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "年增长速率往往<5cm" }, { "start_idx": 18, "end_idx": 30, "type": "sym", "entity": "骨龄延迟一般可大于2年以上" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "骨" }, { "start_idx": 32, "end_idx": 37, "type": "ite", "entity": "GH激发峰值" } ] }, { "text": "2.家族性矮小症父母身高都矮,身高常在第3百分位数左右,但其年增长速率>5cm,骨龄与年龄相称,智能与性发育均正常,GH激发峰值>10μg/L。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "家族性矮小症" }, { "start_idx": 58, "end_idx": 63, "type": "ite", "entity": "GH激发峰值" } ] }, { "text": "3.体质性青春期延迟属正常发育中的一种变异,较为常见。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "体质性青春期延迟" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "正常发育中的一种变异" } ] }, { "text": "出生时及生后数年生长无异常,以后则逐年的身高增长及成熟缓慢,尤于青春发育前或即将进入青春发育期时,性发育出现可延迟数年。", "entities": [ { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "逐年的身高增长及成熟缓慢" }, { "start_idx": 49, "end_idx": 58, "type": "sym", "entity": "性发育出现可延迟数年" } ] }, { "text": "4.宫内发育迟缓本症可由母孕期营养或供氧不足、胎盘存在病理性因素、宫内感染以及胎儿基因组遗传印迹等因素导致胎儿宫内发育障碍。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "宫内发育迟缓本症" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "营养或供氧不足" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "胎盘存在病理性因素" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胎盘" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "宫内感染" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "胎儿基因组遗传印迹" }, { "start_idx": 53, "end_idx": 60, "type": "sym", "entity": "胎儿宫内发育障碍" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "宫内" } ] }, { "text": "初生时多为足月小样儿,散发起病,无家族史,亦无内分泌异常。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "内分泌异常" } ] }, { "text": "出生后极易发生低血糖,生长缓慢。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "低血糖" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "生长缓慢" } ] }, { "text": "5.染色体异常典型Turner综合征不难鉴别,但部分患儿系因X染色体结构异常(如等臂畸形及部分缺失等)或各种嵌合体所致病。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "dis", "entity": "染色体异常典型Turner综合征" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "X染色体结构异常" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "臂畸形及部分缺失" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "嵌合体" } ] }, { "text": "其临床表现不甚典型,常仅以生长迟缓为主,应进行染色体核型分析鉴别。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "生长迟缓" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "染色体核型分析" } ] }, { "text": "21-三体综合征除身材矮小外,同时伴有智能落后及特殊面容等特征,故临床诊断一般不易混淆。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "身材矮小" }, { "start_idx": 19, "end_idx": 30, "type": "sym", "entity": "智能落后及特殊面容等特征" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "面容" } ] }, { "text": "6.骨骼发育异常如各种骨、软骨发育不良等,都有特殊的体态和外貌,可选择进行骨骼X线片及相关溶酶体酶学测定、基因分析等,以明确诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "骨骼发育异常" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨骼" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "各种骨、软骨发育不良" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "各种骨、软骨" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "发育不良" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "特殊的体态和外貌" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "骨骼X线片" }, { "start_idx": 45, "end_idx": 51, "type": "pro", "entity": "溶酶体酶学测定" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "基因分析" } ] }, { "text": "7.其他包括心、肝、肾等慢性疾病,长期营养不良,遗传代谢病(如黏多糖病及糖原累积症等),以及精神心理压抑等因素导致者,都应通过对病史、体检资料分析和必要的特殊检查予以鉴别。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "心、肝、肾等慢性疾病" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "长期营养不良" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "营养不良" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "糖原累积症" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "精神心理压抑" } ] }, { "text": "【治疗】对生长激素缺乏症的治疗主要采用基因重组人生长激素替代治疗。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "生长激素缺乏症" }, { "start_idx": 19, "end_idx": 31, "type": "pro", "entity": "基因重组人生长激素替代治疗" } ] }, { "text": "无论特发性或继发性GH缺乏性矮小均可用GH治疗。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "特发性或继发性GH缺乏性矮小" }, { "start_idx": 19, "end_idx": 20, "type": "dru", "entity": "GH" } ] }, { "text": "但是对颅内肿瘤术后导致的继发性生长激素缺乏症患者需做好解释,对恶性肿瘤或有潜在肿瘤恶变者及严重糖尿病患者禁用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "颅内肿瘤术" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "继发性生长激素缺乏症" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "潜在肿瘤恶变" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "肿瘤" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "糖尿病" } ] }, { "text": "生长激素替代治疗剂量采用0.1U/(kg•d),于每晚睡前半小时皮下注射,可选择在上臂、大腿前侧和腹壁、脐周等部位注射。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "生长激素替代治疗" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "皮下注射" }, { "start_idx": 41, "end_idx": 58, "type": "pro", "entity": "上臂、大腿前侧和腹壁、脐周等部位注射" } ] }, { "text": "GH治疗第1年的效果最好,以后随治疗时间延长GH效果减低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "GH治疗" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "GH效果减低" }, { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "GH" } ] }, { "text": "少数患者在用GH治疗过程中可出现甲状腺激素水平下降,故须监测甲状腺功能,必要时予甲状腺激素补充治疗。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "GH治疗" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "甲状腺激素水平下降" }, { "start_idx": 16, "end_idx": 22, "type": "ite", "entity": "甲状腺激素水平" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "监测甲状腺功能" }, { "start_idx": 40, "end_idx": 48, "type": "pro", "entity": "甲状腺激素补充治疗" } ] }, { "text": "应用GH治疗后的副反应包括假性脑瘤,股骨头脱位,并加重脊柱侧弯及血糖暂时性升高等,但糖尿病的发生率极少。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "GH治疗" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "假性脑瘤" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "股骨头" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "股骨头脱位" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "加重脊柱侧弯" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "脊柱" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "血糖暂时性升高" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "血糖" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "糖尿病" } ] }, { "text": "对于伴有其他垂体激素缺乏者需进行相应的替代治疗。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "其他垂体激素缺乏" }, { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "其他垂体激素" } ] }, { "text": "TSH缺乏者可完全用甲状腺素替代。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "TSH缺乏" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "TSH" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "甲状腺素" } ] }, { "text": "对于ACTH缺乏的患者,适当的补充氢化可的松,剂量不超过10mg/(m2•24h),在患病或手术前需增加剂量。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "ACTH缺乏" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "ACTH" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "氢化可的松" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "手术" } ] }, { "text": "对于促性腺激素缺乏者,当骨龄接近青春期时需用性激素治疗。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "促性腺激素缺乏" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "骨龄" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "性激素" } ] }, { "text": "蛋白同化类固醇药物可促进生长,但是该类药物可加速骨龄发育,加快骨骺融合,对最终身高无明显改善。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dru", "entity": "蛋白同化类固醇药物" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "骨龄" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "骨骺" } ] }, { "text": "第十篇血液系统疾病第一章血液系统的发育及儿童期的生理特点第一节血液系统发育(一)胎儿期造血1.中胚叶造血期血细胞的发育生成始于胚胎的中胚叶,在胚胎发育第3~4周,卵黄囊中形成许多血岛并开始造血,血岛细胞分为内外两个部分,外围细胞发育为血管的原始内皮细胞,中间的细胞形成原始的血细胞。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "血液系统" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "血液系统" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "血液系统" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "血" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "中胚叶" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "血" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "血细胞" }, { "start_idx": 63, "end_idx": 68, "type": "bod", "entity": "胚胎的中胚叶" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "胚胎" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "卵黄囊" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "血岛" }, { "start_idx": 95, "end_idx": 95, "type": "bod", "entity": "血" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "血岛细胞" }, { "start_idx": 110, "end_idx": 113, "type": "bod", "entity": "外围细胞" }, { "start_idx": 117, "end_idx": 125, "type": "bod", "entity": "血管的原始内皮细胞" }, { "start_idx": 127, "end_idx": 131, "type": "bod", "entity": "中间的细胞" }, { "start_idx": 134, "end_idx": 139, "type": "bod", "entity": "原始的血细胞" } ] }, { "text": "至胚胎6周,中胚叶造血开始减退,到10周时接近停止,由肝脏造血取而代之。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "胚胎" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "中胚叶造血开始减退" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "中胚叶" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "血" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肝脏" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "血" } ] }, { "text": "2.肝脾造血期自胚胎第8周开始,至5个月时达高峰,以后逐渐减退,至出生时肝内仅有少量造血灶,至生后4~5天完全停止造血。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肝" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "脾" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "血" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胚胎" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肝内" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "造血灶" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "血" } ] }, { "text": "在肝脏开始造血2个月后,脾脏也参与造血,但造血功能不强,持续时间短,至胚胎5个月时基本停止。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肝脏" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "血" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脾脏" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "血" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "血" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "胚胎" } ] }, { "text": "3.骨髓造血期胚胎第6周出现骨髓,至胚胎4~5个月时开始造血,不久就成为主要的造血器官。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨髓" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "血" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "胚胎" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "骨髓" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胚胎" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "血" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "造血器官" } ] }, { "text": "4.胸腺胚胎期即是T淋巴细胞发育成熟和增殖的主要部位。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胸腺" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胚胎" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "(二)出生后造血为胚胎期造血的继续,主要造血部位在骨髓,特殊情况下可出现骨髓外造血。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "血" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胚胎" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "骨髓" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "骨髓" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "血" } ] }, { "text": "1.骨髓造血骨髓为出生后的主要造血器官,能生成各系血细胞。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨髓" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "骨髓" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "造血器官" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "各系血细胞" } ] }, { "text": "婴幼儿时期全身的骨髓几乎均为红髓,约自5~7岁起长骨骨干中红髓开始转化为黄髓(脂肪组织),随年龄增长更多的红髓由黄髓替代,至18岁左右红髓只限于长骨的两端和不规则骨,但黄髓仍有潜在的造血功能,当造血需要增加时,黄髓重新转化为红髓进行代偿性造血。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "全身的骨髓" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "红髓" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "长骨骨干中红髓" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "黄髓" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "脂肪组织" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "红髓" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "黄髓" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "红髓" }, { "start_idx": 72, "end_idx": 76, "type": "bod", "entity": "长骨的两端" }, { "start_idx": 78, "end_idx": 81, "type": "bod", "entity": "不规则骨" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "黄髓" }, { "start_idx": 92, "end_idx": 92, "type": "bod", "entity": "血" }, { "start_idx": 98, "end_idx": 98, "type": "bod", "entity": "血" }, { "start_idx": 105, "end_idx": 106, "type": "bod", "entity": "黄髓" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "红髓" }, { "start_idx": 120, "end_idx": 120, "type": "bod", "entity": "血" } ] }, { "text": "2.骨髓外造血正常情况下,出生后骨髓外造血极少,但在某些疾病情况下,如严重感染、溶血性贫血时造血增加,并在造血增加的需求超过骨髓的代偿能力时,骨髓外造血组织恢复造血,称为“髓外造血”,临床特点为肝、脾、淋巴结因代偿性造血而增大外周血出现有核红细胞和粒细胞核左移现象黄髓很少,也即骨髓的代偿性造血储备力很少,一旦造血需要增加时,容易出现髓外代偿性造血。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "骨髓外" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "血" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨髓" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "血" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "感染" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "造血增加" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "血" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "造血增加" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "血" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "骨髓" }, { "start_idx": 71, "end_idx": 77, "type": "bod", "entity": "骨髓外造血组织" }, { "start_idx": 71, "end_idx": 81, "type": "sym", "entity": "骨髓外造血组织恢复造血" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "血" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "髓外" }, { "start_idx": 86, "end_idx": 89, "type": "sym", "entity": "髓外造血" }, { "start_idx": 89, "end_idx": 89, "type": "bod", "entity": "血" }, { "start_idx": 97, "end_idx": 97, "type": "bod", "entity": "肝" }, { "start_idx": 97, "end_idx": 112, "type": "sym", "entity": "肝、脾、淋巴结因代偿性造血而增大" }, { "start_idx": 99, "end_idx": 99, "type": "bod", "entity": "脾" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "淋巴结" }, { "start_idx": 109, "end_idx": 109, "type": "bod", "entity": "血" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "外周血" }, { "start_idx": 113, "end_idx": 131, "type": "sym", "entity": "外周血出现有核红细胞和粒细胞核左移现象" }, { "start_idx": 119, "end_idx": 127, "type": "bod", "entity": "核红细胞和粒细胞核" }, { "start_idx": 132, "end_idx": 133, "type": "bod", "entity": "黄髓" }, { "start_idx": 132, "end_idx": 135, "type": "sym", "entity": "黄髓很少" }, { "start_idx": 139, "end_idx": 140, "type": "bod", "entity": "骨髓" }, { "start_idx": 146, "end_idx": 146, "type": "bod", "entity": "血" }, { "start_idx": 156, "end_idx": 156, "type": "bod", "entity": "血" }, { "start_idx": 167, "end_idx": 168, "type": "bod", "entity": "髓外" }, { "start_idx": 167, "end_idx": 173, "type": "sym", "entity": "髓外代偿性造血" }, { "start_idx": 173, "end_idx": 173, "type": "bod", "entity": "血" } ] }, { "text": "二、支气管舒张试验痉挛收缩的气道自然舒张或经支气管舒张剂治疗后舒张,此现象称为气道可逆性。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "支气管舒张试验" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "痉挛" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "气道" }, { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "支气管舒张剂" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "气道" } ] }, { "text": "可以通过吸入支气管舒张剂前后肺功能指标的变化来了解气道舒缓反应的方法,称为支气管舒张试验。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "吸入" }, { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "支气管舒张剂" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "肺功能" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "气道" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "支气管舒张试验" } ] }, { "text": "最常用的支气管舒张药物为β2受体兴奋剂。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "支气管舒张药物" }, { "start_idx": 12, "end_idx": 29, "type": "dru", "entity": "β2受体兴奋剂" } ] }, { "text": "评价指标有多种,依据检测方式不同进行选择,但目前最常用并公认的是FEV1。", "entities": [ { "start_idx": 32, "end_idx": 46, "type": "ite", "entity": "FEV1" } ] }, { "text": "若FEV1的改善率>基础值的12%以上(成人同时要求用药后FEV1绝对值增加200ml),则舒张试验阳性。", "entities": [ { "start_idx": 1, "end_idx": 15, "type": "ite", "entity": "FEV1" }, { "start_idx": 40, "end_idx": 54, "type": "ite", "entity": "FEV1" }, { "start_idx": 68, "end_idx": 71, "type": "pro", "entity": "舒张试验" } ] }, { "text": "注意事项:1.检测前FEV1<预计值70%2.支气管舒张试验前应停用β2受体兴奋剂、胆碱能受体阻滞剂、茶碱等。", "entities": [ { "start_idx": 10, "end_idx": 24, "type": "ite", "entity": "FEV1" }, { "start_idx": 34, "end_idx": 40, "type": "pro", "entity": "支气管舒张试验" }, { "start_idx": 45, "end_idx": 62, "type": "dru", "entity": "β2受体兴奋剂" }, { "start_idx": 64, "end_idx": 71, "type": "dru", "entity": "胆碱能受体阻滞剂" }, { "start_idx": 73, "end_idx": 74, "type": "dru", "entity": "茶碱" } ] }, { "text": "短效β2受体兴奋剂停用4~6小时,口服β2受体兴奋剂、茶碱等停用12小时,长效或缓释β2受体兴奋剂、茶碱等停用24小时以上。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "dru", "entity": "短效β2受体兴奋剂" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "口服" }, { "start_idx": 30, "end_idx": 47, "type": "dru", "entity": "β2受体兴奋剂" }, { "start_idx": 49, "end_idx": 50, "type": "dru", "entity": "茶碱" }, { "start_idx": 59, "end_idx": 81, "type": "dru", "entity": "长效或缓释β2受体兴奋剂" }, { "start_idx": 83, "end_idx": 84, "type": "dru", "entity": "茶碱" } ] }, { "text": "婴幼儿目前尚无统一的支气管激发试验或舒张试验的方法和标准,仅有少数研究者的各自研究报道,如用干燥冷空气刺激作为激发的方法等。", "entities": [ { "start_idx": 10, "end_idx": 21, "type": "pro", "entity": "支气管激发试验或舒张试验" } ] }, { "text": "在婴幼儿舒张试验中,绝大部分人用沙丁胺醇,测试方法可以用脉冲振荡法、体描法和潮气法等,但是报道的结果不一。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "婴幼儿舒张试验" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "沙丁胺醇" }, { "start_idx": 28, "end_idx": 32, "type": "pro", "entity": "脉冲振荡法" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "体描法" }, { "start_idx": 38, "end_idx": 40, "type": "pro", "entity": "潮气法" } ] }, { "text": "六、沙门菌肺炎由伤寒、副伤寒、鼠伤寒或其他非伤寒沙门菌引起,发生于沙门菌感染的病程中,较为少见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "沙门菌肺炎" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "伤寒" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "副伤寒" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "鼠伤寒" }, { "start_idx": 21, "end_idx": 26, "type": "mic", "entity": "非伤寒沙门菌" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "沙门菌感染" } ] }, { "text": "可表现为大叶性肺炎或支气管肺炎症状。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "大叶性肺炎" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "支气管肺炎" } ] }, { "text": "较为特殊的表现为痰常呈血性或带血丝。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "痰常呈血性或带血丝" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "痰" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "血" } ] }, { "text": "在沙门菌感染的病程中,如发生呼吸道症状如咳嗽、气急,即使无肺部体征,也应进行摄片。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "沙门菌感染" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "呼吸道症状" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "呼吸道" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "咳嗽" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "气急" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺部" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "摄片" } ] }, { "text": "如有肺炎改变应考虑为沙门菌肺炎(salmonellapneumonia)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肺炎" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "沙门菌肺炎" }, { "start_idx": 16, "end_idx": 34, "type": "dis", "entity": "salmonellapneumonia" } ] }, { "text": "在美国,约20%沙门菌株对氨苄西林耐药。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "沙门菌株" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "如描述性研究提示单侧与双侧视网膜母细胞瘤发病年龄不同,由此提出了两次基因突变是形成肿瘤的发病基础的假设,据此可设计另一个研究来证明这一假设。", "entities": [ { "start_idx": 8, "end_idx": 19, "type": "dis", "entity": "单侧与双侧视网膜母细胞瘤" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "基因突变" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(二)生态研究研究肿瘤发病与不同区域人群生活习性间的关系,如吸烟、喝咖啡及特殊饮食习惯与肿瘤发病间的关系,在发现有肯定的关系时可提出进一步的致病机制假设,由另一个研究来检测假设是否成立。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "肿瘤" } ] }, { "text": "儿童肿瘤常不能完全符合这些要求。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "第三节马蹄肾马蹄肾是肾脏融合畸形中最常见的一种。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "肾脏融合畸形" } ] }, { "text": "与其他融合肾相区别的主要特征是肾脏纵立于脊柱两侧,结构完整,只是其相对应的两肾下极被横跨脊柱的实质性组织或纤维组织相连接。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "融合肾" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肾脏" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "脊柱两侧" }, { "start_idx": 32, "end_idx": 59, "type": "sym", "entity": "其相对应的两肾下极被横跨脊柱的实质性组织或纤维组织相连接" }, { "start_idx": 32, "end_idx": 40, "type": "bod", "entity": "其相对应的两肾下极" }, { "start_idx": 42, "end_idx": 56, "type": "bod", "entity": "横跨脊柱的实质性组织或纤维组织" } ] }, { "text": "1521年DeCarpi医师在尸体解剖中首先发现马蹄肾,1564年Botallo对其作了更为详细的描述,1820年Morgagni首次报道了发生于马蹄肾的肾脏病变。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "尸体解剖" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "肾脏病变" } ] }, { "text": "至今为止,基本上每一种肾脏病变都已在马蹄肾上发现。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "肾脏病变" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "马蹄肾" } ] }, { "text": "马蹄肾的发生率约为0.25%,男女之比约为2∶1。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "马蹄肾" } ] }, { "text": "马蹄肾有家族中发病的报告,也见于同卵孪生儿中,但遗传倾向并不明确。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "马蹄肾" } ] }, { "text": "马蹄肾发生于胚胎第5和第6周间,此时输尿管芽已进入肾基质。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胚胎" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "输尿管芽" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肾基质" } ] }, { "text": "一般认为输尿管芽进入肾实质是在肾脏旋转及其上升发生以前,故马蹄肾的肾盂多位于肾实质腹侧,整个肾脏位置比较低,一般其峡部因肠系膜下动脉的阻碍而位于其下方。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "输尿管芽" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肾实质" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肾脏" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肾盂" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "肾实质腹侧" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "整个肾脏位置比较低" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "整个肾脏" }, { "start_idx": 57, "end_idx": 74, "type": "sym", "entity": "峡部因肠系膜下动脉的阻碍而位于其下方" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "峡部" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "肠系膜下动脉" } ] }, { "text": "马蹄肾发生的具体过程至今尚未完全明了,1931年Boyden报告一例迄今为止最小的6周马蹄胚胎肾,他认为,当胚胎4.5周时,两肾的位置靠得非常近,因某种因素作用造成了其下极融合。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "马蹄胚胎肾" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "胚胎" }, { "start_idx": 62, "end_idx": 71, "type": "sym", "entity": "两肾的位置靠得非常近" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "两肾" }, { "start_idx": 84, "end_idx": 87, "type": "sym", "entity": "下极融合" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "下极" } ] }, { "text": "也有认为脐动脉或髂总动脉解剖位置异常改变了肾脏原来的旋转和上升路径,导致其下极融合,而峡部的形成则有认为是后肾生肾细胞异常迁移所致。", "entities": [ { "start_idx": 4, "end_idx": 19, "type": "sym", "entity": "脐动脉或髂总动脉解剖位置异常改变" }, { "start_idx": 4, "end_idx": 13, "type": "pro", "entity": "脐动脉或髂总动脉解剖" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肾脏" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "下极融合" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "下极" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "峡部的形成" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "峡部" }, { "start_idx": 53, "end_idx": 62, "type": "sym", "entity": "后肾生肾细胞异常迁移" }, { "start_idx": 53, "end_idx": 58, "type": "bod", "entity": "后肾生肾细胞" } ] }, { "text": "95%左右的马蹄肾在其下极处相互连接,在一小部分病人,两肾在上极之间由峡部相连。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "下极处相互连接" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "下极处" }, { "start_idx": 27, "end_idx": 38, "type": "sym", "entity": "两肾在上极之间由峡部相连" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "两肾" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "上极之间" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "峡部" } ] }, { "text": "峡部通常位于腰3、腰4椎体及腹主动脉和下腔静脉的前方、肠系膜下动脉起点的下方,也有报道峡部在腹主动脉和下腔静脉之间穿行或完全位于其两者背侧。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "峡部" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "腰3、腰4椎体" }, { "start_idx": 14, "end_idx": 25, "type": "bod", "entity": "腹主动脉和下腔静脉的前方" }, { "start_idx": 27, "end_idx": 37, "type": "bod", "entity": "肠系膜下动脉起点的下方" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "峡部" }, { "start_idx": 46, "end_idx": 56, "type": "bod", "entity": "腹主动脉和下腔静脉之间" } ] }, { "text": "马蹄肾的肾盏数目正常,但多朝向背侧。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肾盏" } ] }, { "text": "输尿管往往起始于肾盂上端,但其在膀胱开口多是正常的,很少发生异位。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "输尿管" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "肾盂上端" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "膀胱开口" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "异位" } ] }, { "text": "马蹄肾病人的1/3至少伴发一种畸形,而患多种先天性畸形的新生儿或婴儿多有马蹄肾。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "畸形" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "先天性畸形" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "马蹄肾" } ] }, { "text": "伴发畸形多发生于骨骼系统、心血管系统和中枢神经系统,生殖系统畸形发生率也比较高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "畸形" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "骨骼系统" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "心血管系统" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "生殖系统畸形" } ] }, { "text": "1/3左右的马蹄肾病人没有症状,很多情况下马蹄肾是在超声检查或尸检时被偶然发现。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "超声检查" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "尸检" } ] }, { "text": "其他检查可有CT及逆行输尿管插管造影等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "CT" }, { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "逆行输尿管插管造影" } ] }, { "text": "Glenn对马蹄肾患儿随访10年,其中60%始终没有症状,13%有尿路感染或腹痛,17%有结石,仅25%的患儿需手术来解除梗阻或去除结石。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "尿路感染" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "腹痛" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "腹" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "结石" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "手术" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "梗阻" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "结石" } ] }, { "text": "第三节衣原体感染衣原体为专性细胞内寄生,含有DNA、RNA、核糖体和细胞壁。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "衣原体感染" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "衣原体" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "细胞内" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "DNA" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "RNA" }, { "start_idx": 30, "end_idx": 32, "type": "mic", "entity": "核糖体" }, { "start_idx": 34, "end_idx": 36, "type": "mic", "entity": "细胞壁" } ] }, { "text": "衣原体不能产生ATP,可被认为是能量寄生菌。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "衣原体" }, { "start_idx": 16, "end_idx": 20, "type": "mic", "entity": "能量寄生菌" } ] }, { "text": "已知的衣原体属有4种:沙眼衣原体(C.trachomatis,CT)、肺炎衣原体(C.pneumoniae,CP)、鹦鹉热衣原体(C.psittacosis)、家畜衣原体(C.pecorum)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "mic", "entity": "衣原体" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 17, "end_idx": 29, "type": "mic", "entity": "C.trachomatis" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "CT" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 41, "end_idx": 52, "type": "mic", "entity": "C.pneumoniae" }, { "start_idx": 54, "end_idx": 55, "type": "mic", "entity": "CP" }, { "start_idx": 58, "end_idx": 63, "type": "mic", "entity": "鹦鹉热衣原体" }, { "start_idx": 65, "end_idx": 77, "type": "mic", "entity": "C.psittacosis" }, { "start_idx": 80, "end_idx": 84, "type": "mic", "entity": "家畜衣原体" }, { "start_idx": 86, "end_idx": 94, "type": "mic", "entity": "C.pecorum" } ] }, { "text": "沙眼衣原体可引起男性尿道炎、女性宫颈炎及输卵管炎和婴儿的结膜炎和肺炎;肺炎衣原体正日益被认为是呼吸道疾病,包括儿童和成人的支气管炎和肺炎的常见病原。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "男性尿道炎" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "女性宫颈炎" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "输卵管炎" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "结膜炎" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "肺炎" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "呼吸道疾病" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "支气管炎" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "肺炎" } ] }, { "text": "一、沙眼衣原体感染【病因】沙眼(trachomatis)是世界范围内造成失明最重要的可预防疾病。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "沙眼衣原体感染" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "沙眼" }, { "start_idx": 16, "end_idx": 26, "type": "dis", "entity": "trachomatis" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "失明" } ] }, { "text": "该病为眼与眼之间传播,苍蝇常为储存宿主。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "眼" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "眼" } ] }, { "text": "沙眼初为滤泡性结膜炎,多发生于童年,滤泡愈合形成结膜瘢痕,引起睑内翻,使睫毛擦伤角膜,经常性的角膜外伤性溃疡引起瘢痕和失明,细菌性双重感染也参与了瘢痕的形成。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "沙眼" }, { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "滤泡性结膜炎" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "结膜" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "结膜瘢痕" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "睑" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "睑内翻" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "睫毛" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "睫毛擦伤角膜" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "角膜" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "角膜外伤性溃疡" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "瘢痕" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "失明" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "细菌性双重感染" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "瘢痕" } ] }, { "text": "【临床表现】(一)包涵体结膜炎沙眼衣原体是新生儿结膜炎的最常见已知病原。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "包涵体结膜炎" }, { "start_idx": 15, "end_idx": 19, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "新生儿结膜炎" } ] }, { "text": "结膜炎是新生儿衣原体感染的主要的临床表现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "结膜炎" }, { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "新生儿衣原体感染" } ] }, { "text": "临床表现不一,从轻症的结膜充血伴少量黏液样分泌物到严重的结膜炎伴大量脓性分泌物、球结膜充血和伪膜形成。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "结膜" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "结膜充血" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "少量黏液样分泌物" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "分泌物" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "结膜炎" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "大量脓性分泌物" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "分泌物" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "球结膜" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "球结膜充血" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "血" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "伪膜形成" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "伪膜" } ] }, { "text": "结膜脆性大,用拭子轻擦可出血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "结膜" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "出血" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "血" } ] }, { "text": "(二)肺炎沙眼衣原体感染的母亲所生的新生儿中10%~20%发生沙眼衣原体肺炎。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肺炎" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "沙眼衣原体感染" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "沙眼衣原体肺炎" } ] }, { "text": "常在生后1~3个月时隐匿起病,表现为持续性咳嗽、呼吸急促、无发热和喘息。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "持续性咳嗽" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "发热" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "喘息" } ] }, { "text": "听诊可闻湿啰音。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "听诊可闻湿啰音" }, { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" } ] }, { "text": "特征性的实验室检查为末梢血嗜酸性粒细胞增多,胸片常表现为肺气肿伴间质和肺泡浸润影。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 18, "type": "ite", "entity": "末梢血嗜酸性粒细胞" }, { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "末梢血嗜酸性粒细胞增多" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "胸片" }, { "start_idx": 22, "end_idx": 39, "type": "sym", "entity": "胸片常表现为肺气肿伴间质和肺泡浸润影" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "间质" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺泡" } ] }, { "text": "(三)成人和青少年的眼、生殖器感染沙眼衣原体为30%~50%男性非淋球菌性尿道炎的病因,是35岁以下男性附睾炎的主要原因,从生殖器到眼的自行接种可引起包涵体结膜炎。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "眼、生殖器感染" }, { "start_idx": 17, "end_idx": 21, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "非淋球菌性尿道炎" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "附睾炎" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "生殖器" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "眼" }, { "start_idx": 70, "end_idx": 71, "type": "pro", "entity": "接种" }, { "start_idx": 75, "end_idx": 80, "type": "dis", "entity": "包涵体结膜炎" } ] }, { "text": "沙眼衣原体感染女性子宫颈内膜,可引起黏液性子宫颈炎,但常无症状。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "沙眼衣原体感染" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "子宫颈内膜" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "黏液性子宫颈炎" } ] }, { "text": "沙眼衣原体可感染尿道,引起尿痛伴无菌性脓尿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "感染尿道" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "尿痛" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "菌性脓尿" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" } ] }, { "text": "【诊断】1.沙眼可临床诊断世界卫生组织建议以下4条符合两条以上可诊断:上睑结膜的淋巴样滤泡、典型的结膜瘢痕、血管翳和角膜上缘滤泡。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "沙眼" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "上睑结膜" }, { "start_idx": 35, "end_idx": 44, "type": "sym", "entity": "上睑结膜的淋巴样滤泡" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "滤泡" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "典型的结膜瘢痕" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "结膜" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "血管" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "血管翳" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "角膜上缘" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "角膜上缘滤泡" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "滤泡" } ] }, { "text": "2.围产期感染最佳标准仍为从结膜或鼻咽部分离到沙眼衣原体。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "围产期感染" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "结膜" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "鼻咽" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "分离" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "沙眼衣原体" } ] }, { "text": "此外,还可用酶联免疫法检测沙眼衣原体的抗原或抗体。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "酶联免疫法" }, { "start_idx": 11, "end_idx": 23, "type": "pro", "entity": "检测沙眼衣原体的抗原或抗体" } ] }, { "text": "3.通过从男性尿道和女性子宫内膜分离到病原可明确诊断。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "尿道" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "子宫内膜" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "分离" } ] }, { "text": "【治疗】采用红霉素治疗婴儿结膜炎和肺炎。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "红霉素" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "结膜炎" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肺炎" } ] }, { "text": "对男性和非妊娠妇女,美国疾病控制中心推荐采用多西环素或阿奇霉素治疗,对妊娠妇女采用红霉素治疗。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "多西环素" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "红霉素" } ] }, { "text": "二、磁共振成像学磁共振成像学(magneticresonanceimaging,MRI)是20世纪80年代继CT之后又一项新的影像诊断技术。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "磁共振成像学" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "磁共振成像学" }, { "start_idx": 15, "end_idx": 38, "type": "pro", "entity": "magneticresonanceimaging" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "MRI" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "CT" }, { "start_idx": 63, "end_idx": 68, "type": "pro", "entity": "影像诊断技术" } ] }, { "text": "MRI在显示小儿畸形以及脱髓鞘疾病方面有独特价值,而对显示肿瘤、炎症以及血管性疾患方面也不比CT和DSA逊色,因此已成为小儿疾患可选择,有时甚至是首选检查方法。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "MRI" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "小儿畸形" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "脱髓鞘" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肿瘤" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "炎症" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "血管性疾患" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "CT" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "DSA" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "小儿疾患" } ] }, { "text": "(一)基本原理MRI是借助接收器探测人体组织内蕴藏量最丰富的氢原子,在磁共振过程中所发出的电磁波信号,从而测出其氢原子的浓度及其弛豫时间(T1和T2),作为成像的参数,通过电子计算机运算和处理,如同CT一样进行图像重建;通过波谱分析,还可了解组织器官及病变组织的代谢功能、生理和生化信息。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "MRI" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "人体组织内" }, { "start_idx": 35, "end_idx": 37, "type": "pro", "entity": "磁共振" }, { "start_idx": 99, "end_idx": 100, "type": "pro", "entity": "CT" }, { "start_idx": 112, "end_idx": 115, "type": "pro", "entity": "波谱分析" }, { "start_idx": 121, "end_idx": 124, "type": "bod", "entity": "组织器官" }, { "start_idx": 126, "end_idx": 129, "type": "sym", "entity": "病变组织" }, { "start_idx": 128, "end_idx": 129, "type": "bod", "entity": "组织" } ] }, { "text": "(二)检查方法一次完整的MRI检查须同时采用T1加权、T2加权和质子密度加权成像,根据横断面、矢状面或冠状面不同平面的图像以作出正确的诊断。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "MRI检查" } ] }, { "text": "MRI与CT比较,其优点为:①任意方位断层,如可取横轴位、矢状位以及冠状位;②组织对比度高(对白质疾病、水肿以及肿瘤显示较清晰);③无骨伪影;④脊髓可显示;⑤能显示神经核团。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "MRI" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "CT" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "白质疾病" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "水肿" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "肿瘤" }, { "start_idx": 67, "end_idx": 69, "type": "sym", "entity": "骨伪影" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "骨" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "脊髓" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "神经核团" } ] }, { "text": "缺点在于:对钙化及骨病变的诊断较差。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "钙化" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "骨病变" } ] }, { "text": "(三)临床应用1.颅脑肿瘤MRI能较满意地显示肿瘤的内部结构,尤其是它所具有的三维成像特点,为手术方案的拟订、放疗计划的确定以及立体针吸活组织的入路选择提供了更多的信息,尤其适用于颅后窝和颅底附近疾患的检查。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "颅脑肿瘤" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "MRI" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "手术" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "放疗" }, { "start_idx": 64, "end_idx": 70, "type": "pro", "entity": "立体针吸活组织" }, { "start_idx": 90, "end_idx": 99, "type": "dis", "entity": "颅后窝和颅底附近疾患" } ] }, { "text": "MRI可定出肿瘤的部位;根据肿瘤信号强度初步作出组织来源的定性诊断;根据肿瘤边缘是否清楚、规则作出良恶性初步诊断;能清晰地显示肿瘤血供及其与颅内大血管的关系。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "MRI" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肿瘤" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肿瘤" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "组织" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肿瘤" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "肿瘤" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "血" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "颅内大血管" } ] }, { "text": "在常规MRI检查之后行GdDTPA增强MRI扫描,可提高平扫阴性颅内肿瘤的显示率,对于平扫已显示的脑肿瘤,增强扫描也有助于明确肿瘤的边缘,并帮助作定性诊断。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "常规MRI检查" }, { "start_idx": 11, "end_idx": 23, "type": "pro", "entity": "GdDTPA增强MRI扫描" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "平扫" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "平扫" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "脑肿瘤" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "增强扫描" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "肿瘤" } ] }, { "text": "2.脑血管疾病脑血管疾病包括脑梗死、脑出血、静脉窦血栓形成、颅内动脉瘤、脑血管畸形以及基底异常血管网症。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "脑血管疾病" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "脑血管疾病" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑梗死" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑出血" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "静脉窦血栓" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "颅内动脉瘤" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "脑血管畸形" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "基底异常血管网症" } ] }, { "text": "对于前3种疾病,MRI的长处主要是基于其多参数成像,即同一被检层面可得到3幅MRI图像,分别揭示了血肿内部T1、质子密度以及T2的变化。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "MRI" }, { "start_idx": 38, "end_idx": 40, "type": "pro", "entity": "MRI" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "血肿" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "血" } ] }, { "text": "对于后3种疾病,MRI的优点主要取决于它在不使用造影剂情况下可满意地显示血管及其内部结构,可作多方向切层以及去骨作用。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "MRI" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "血管及其内部" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "骨" } ] }, { "text": "而MRI对于烟雾病的显示是CT所不能比拟的。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "MRI" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "烟雾病" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "CT" } ] }, { "text": "3.脑变性病和脑白质病一些脑变性病和脑白质病MRI具有特征性的表现如肾上腺白质脑病、多发性硬化以及中心性脑桥髓鞘溶解等,MRI不仅能诊断这些疾患,而且对疾病的程度也作出判断,并可随访观察疗效。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "脑变性病" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "脑白质病" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "脑变性病" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "脑白质病" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "MRI" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "肾上腺白质脑病" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "多发性硬化" }, { "start_idx": 49, "end_idx": 57, "type": "dis", "entity": "中心性脑桥髓鞘溶解" }, { "start_idx": 60, "end_idx": 62, "type": "pro", "entity": "MRI" } ] }, { "text": "但不少脑变性病和脑白质病尚缺乏较特异性MRI表现颅内感染性疾病有些脑部炎症如硬脑膜下积脓、脑囊虫、单纯疱疹病毒性脑炎以及少数真菌感染,诊断主要依靠MRI所见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "脑变性病" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "脑白质病" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "MRI" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "颅内感染性疾病" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "脑部炎症" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "硬脑膜下积脓" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "脑囊虫" }, { "start_idx": 49, "end_idx": 57, "type": "dis", "entity": "单纯疱疹病毒性脑炎" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "真菌感染" }, { "start_idx": 73, "end_idx": 75, "type": "pro", "entity": "MRI" } ] }, { "text": "但是,许多脑部炎症其MRI所见与CT所见一样,缺乏诊断特异性,MRI只是在显示病变大小、范围、数目、病变内部结构以及病变与邻近结构关系方面比CT向前迈进了一大步。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "脑部炎症" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "MRI" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "CT" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "MRI" }, { "start_idx": 70, "end_idx": 71, "type": "pro", "entity": "CT" } ] }, { "text": "5.先天性颅脑发育不全与其他检查方法比较,MRI以安全、无损伤以及行之有效而著称。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "先天性颅脑发育不全" }, { "start_idx": 21, "end_idx": 23, "type": "pro", "entity": "MRI" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "损伤" } ] }, { "text": "其中DandyWalker因MRI可作任意方向的切层,有利于显示小脑的异常改变并明确其与第4脑室的关系。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "MRI" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "小脑的异常改变" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "小脑" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "第4脑室" } ] }, { "text": "胼胝体发育不全在矢状切面检查,显示最为真切。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "胼胝体发育不全" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "矢状切面检查" } ] }, { "text": "Huntington舞蹈病显示尾状核萎缩。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "Huntington舞蹈病" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "尾状核萎缩" } ] }, { "text": "Wilson病显示对称性豆状核和丘脑异常。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "Wilson病" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "对称性豆状核" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "豆状核" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "丘脑异常" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "丘脑" } ] }, { "text": "6.颅脑损伤MRI检查时间较长,病情重笃者不宜接受此项检查,一些躁动的外伤病人,在检查前务必使用药物以使病人安静,以完成检查。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颅脑损伤" }, { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "MRI" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "躁动" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "外伤" } ] }, { "text": "另外,头部有金属异物者不能作MRI扫描。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "头部" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "MRI扫描" } ] }, { "text": "7.脊柱和脊髓疾病MRI以高软组织分辨能力和矢状面的使用更为重要。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "脊柱和脊髓疾病" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "MRI" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "软组织" } ] }, { "text": "MRI能清晰地观察各处椎体、椎间盘的髓核和纤维软骨环,以及其他附件、前后纵韧带等影像,可直接显示脊柱先天性畸形、椎间盘病变、椎管狭窄、脊柱炎性疾病(包括脊髓炎、硬膜外脓肿、蛛网膜炎以及类风湿性关节炎)、椎管肿瘤以及椎管内血管畸形,此外,MRI还应用于脊柱外伤的诊断。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "MRI" }, { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "椎体、椎间盘的髓核" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "纤维软骨环" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "前后纵韧带" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "脊柱先天性畸形" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "椎间盘病变" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "椎管狭窄" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "脊柱炎性疾病" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "脊髓炎" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "硬膜外脓肿" }, { "start_idx": 86, "end_idx": 89, "type": "dis", "entity": "蛛网膜炎" }, { "start_idx": 92, "end_idx": 98, "type": "dis", "entity": "类风湿性关节炎" }, { "start_idx": 101, "end_idx": 104, "type": "dis", "entity": "椎管肿瘤" }, { "start_idx": 107, "end_idx": 113, "type": "dis", "entity": "椎管内血管畸形" }, { "start_idx": 118, "end_idx": 120, "type": "pro", "entity": "MRI" }, { "start_idx": 125, "end_idx": 128, "type": "dis", "entity": "脊柱外伤" } ] }, { "text": "8.骨骼肌肉系统疾病MRI能明确地显示骨骼、肌肉病灶的部位和范围,对进行性肌萎缩、肌炎、萎缩性肌强直,以及其他包括代谢性在内的肌病均可显示受累肌群的某些特征性信号,从而进行诊断和鉴别诊断。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "骨骼肌肉系统疾病" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "MRI" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "骨骼、肌肉病灶" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "骨骼" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肌肉" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "进行性肌萎缩" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "肌炎" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "萎缩性肌强直" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "肌病" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "肌群" } ] }, { "text": "(四)磁共振血管造影(MRA)MRA的应用可大大地提高脑血管病变的诊断能力。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "磁共振血管造影" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "MRA" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "MRA" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "脑血管病变" } ] }, { "text": "MRA技术可分为二维和三维两种方式,二维MRA对慢血流(如静脉)的病变检查最佳,三维能够提供较薄的层厚和较高的空间分辨率,且由于回波时间短,可减少造成对狭窄过度估计的伪影。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "MRA技术" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "二维MRA" }, { "start_idx": 24, "end_idx": 36, "type": "pro", "entity": "慢血流(如静脉)的病变检查" }, { "start_idx": 76, "end_idx": 77, "type": "sym", "entity": "狭窄" }, { "start_idx": 83, "end_idx": 84, "type": "sym", "entity": "伪影" } ] }, { "text": "二、心脏外形的演变在原始心管形成期,其外层的心外肌膜始基在腹侧融合形成心包腔。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心脏" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "原始心管" }, { "start_idx": 19, "end_idx": 27, "type": "bod", "entity": "外层的心外肌膜始基" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "腹侧" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "心包腔" } ] }, { "text": "背侧不融合,形成心系膜(meso-cardium)。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "心系膜" }, { "start_idx": 12, "end_idx": 23, "type": "bod", "entity": "meso-cardium" } ] }, { "text": "心系膜在心管的背侧,首尾梁端相接,中段消失,因此心管在心包腔中完全游离。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "心系膜" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "心管的背侧" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "首尾梁端" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心管" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "心包腔" } ] }, { "text": "心管的生长速度远较心包腔的生长速度快,而且心管各部生长不平衡,在胚胎24天左右心管先后发生四个收缩环和相应的四个膨大部分,由头至尾,依此为动脉干、心球、心室、心房与静脉窦,与此同时心管发生扭转,心球转至右尾侧位。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心管" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "心包腔" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "心管各部" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "心管各部生长不平衡" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胚胎" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "左右心管" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "动脉干" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "心球" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "心室" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "心房" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "静脉窦" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "心管" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "心球" } ] }, { "text": "其中心球与心室最为膨大且向尾端移动。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心球" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心室" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "尾端" } ] }, { "text": "至胚胎2~9天左右,心室已降至心房水平之下,心房显露于心室的后上方(头端)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "胚胎" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心室" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心房" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心房" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "心室的后上方" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "头端" } ] }, { "text": "此时,心脏外形已基本形成。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" } ] }, { "text": "心房及心室表面产生纵沟,分心脏为左右房室,心房腹侧纵沟纵沟中有动脉干经过。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "心房及心室表面" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "纵沟" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "左右房室" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "心房腹侧纵沟纵沟中" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "动脉干" } ] }, { "text": "但此时心脏仍为单一的管道,血流尚未分开。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "管道" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血流" } ] }, { "text": "由静脉窦流入的血液由动脉干流出。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "bod", "entity": "静脉窦流入的血液" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "动脉干" } ] }, { "text": "参考文献1.急性呼吸道抗生素合理使用指南(试用)(上部分).中华儿科杂志,1999,37(12):748-7482.急性呼吸道抗生素合理使用指南(试用)(下部分).中华儿科杂志,2001,39(6):379-3793.CrawfordSE,DaumR.BacterialPneumonia,LungAbscess,andEmpyema.In:TaussigLMandLandauLIeds.PediatricRespiratorymedicine.2nded.Philadelphia:Mosby,2008,501-5014.GlobalInitiativeforAsthma(GINA).Globalstrategyforasthmamanagementandprevention;2008.Availableat:http://www.ginasthma.org.AccessedDecember20085.TheSanfordguidetoantimicrobialtherapy2006.36thed.AntimicrobialTherapyInc.,Sperryville,VT,20066.ZachMS,OberwaldnerB.ChestPhysiotherapy.In:TaussigLMandLandauLIeds.PediatricRespiratoryMedicine.2nded.Philadelphia:Mosby,2008:241-241", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dru", "entity": "急性呼吸道抗生素" }, { "start_idx": 33, "end_idx": 34, "type": "dep", "entity": "儿科" }, { "start_idx": 59, "end_idx": 66, "type": "dru", "entity": "急性呼吸道抗生素" }, { "start_idx": 85, "end_idx": 86, "type": "dep", "entity": "儿科" } ] }, { "text": "第十一章支气管哮喘支气管哮喘(asthma)是儿科常见的呼吸道疾病之一,我国儿童哮喘患病率约为0.5%~2%,个别地区高达5%,哮喘的患病率仍呈上升趋势。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "asthma" }, { "start_idx": 23, "end_idx": 24, "type": "dep", "entity": "儿科" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "呼吸道疾病" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "哮喘" } ] }, { "text": "但儿童哮喘在不同年龄具有不同的病因、发病机制,甚至有不同的病理特征,在疾病治疗和预后方面也存在很大的不同。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "儿童哮喘" } ] }, { "text": "第一节支气管哮喘的病因与发病机制一、病因及发病机制(一)5岁以下儿童喘息5岁以下儿童易患喘息性疾病,但其喘息发作的病因、发病机制与自然病程具有很大的不同。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "喘息" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "喘息性疾病" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "喘息" } ] }, { "text": "根据起病年龄及预后可以将5岁以下儿童喘息分成3种临床表型,其病因也有明显的不同:1.早期一过性喘息多见于早产和父母吸烟者,喘息主要是由于环境因素、宫内发育异常或感染导致肺发育延迟所致,年龄的增长使肺的发育逐渐成熟,大多数患儿在生后3岁之内喘息逐渐消失。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "喘息" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "早期一过性喘息" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "喘息" }, { "start_idx": 73, "end_idx": 78, "type": "sym", "entity": "宫内发育异常" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "宫内" }, { "start_idx": 80, "end_idx": 81, "type": "dis", "entity": "感染" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "肺" }, { "start_idx": 84, "end_idx": 88, "type": "sym", "entity": "肺发育延迟" }, { "start_idx": 98, "end_idx": 98, "type": "bod", "entity": "肺" }, { "start_idx": 119, "end_idx": 120, "type": "sym", "entity": "喘息" } ] }, { "text": "2.早期起病的持续性喘息(指3岁前起病)主要表现为与急性呼吸道病毒感染(小于2岁的儿童通常为呼吸道合胞病毒感染,2岁以上的儿童与鼻病毒等其他病毒感染有关)相关的反复喘息,本人无特应症表现,也无家族过敏性疾病史。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "持续性喘息" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "急性呼吸道病毒感染" }, { "start_idx": 46, "end_idx": 54, "type": "dis", "entity": "呼吸道合胞病毒感染" }, { "start_idx": 64, "end_idx": 66, "type": "mic", "entity": "鼻病毒" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "病毒感染" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "反复喘息" }, { "start_idx": 96, "end_idx": 102, "type": "dis", "entity": "家族过敏性疾病" } ] }, { "text": "其原因可能是病毒感染导致的一过性气道反应性增高,随着年龄增大,呼吸道病毒感染减少,症状逐渐减轻,喘息症状一般持续至学龄期,部分患儿在12岁时仍然有症状。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "病毒感染" }, { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "一过性气道反应性增高" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "呼吸道病毒感染" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "喘息" } ] }, { "text": "3.迟发性喘息/哮喘这些儿童有典型的特应症背景,往往伴有湿疹,哮喘症状常迁延持续至成人期,气道有典型的哮喘病理特征。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "迟发性喘息/哮喘" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "湿疹" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "哮喘" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "气道" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "哮喘病理特征" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "哮喘病" } ] }, { "text": "(二)儿童哮喘60%~80%的5岁以上儿童哮喘与呼吸道过敏有关,气道有大量嗜酸性粒细胞、肥大细胞、淋巴细胞等炎性细胞浸润及广泛的黏膜上皮细胞脱落;主要由持续反复吸入低剂量变应原引起,可以使气道反应性明显持续的增加。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "呼吸道过敏" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "呼吸道" }, { "start_idx": 32, "end_idx": 71, "type": "sym", "entity": "气道有大量嗜酸性粒细胞、肥大细胞、淋巴细胞等炎性细胞浸润及广泛的黏膜上皮细胞脱落" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "气道" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "嗜酸性粒细胞" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 61, "end_idx": 69, "type": "bod", "entity": "广泛的黏膜上皮细胞" }, { "start_idx": 94, "end_idx": 105, "type": "sym", "entity": "气道反应性明显持续的增加" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "气道" } ] }, { "text": "由于呼吸道尘螨过敏的表达需要2年左右的时间,因而儿童过敏性哮喘多在2岁左右开始起病。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "呼吸道尘螨过敏" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "呼吸道" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "尘螨" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "儿童过敏性哮喘" } ] }, { "text": "(三)咳嗽变异性哮喘发病机制与支气管哮喘相似,其只咳不喘的原因或机制还不是非常清楚,部分学者认为可能为气道炎症和气道高反应没有达到哮喘发作的程度;另一些学者认为慢性气道炎症主要集中在中央气道,大气道平滑肌收缩刺激肌梭内咳嗽感受器引起剧烈咳嗽,而没有小气道阻塞表现。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "咳嗽变异性哮喘" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 25, "end_idx": 25, "type": "sym", "entity": "咳" }, { "start_idx": 27, "end_idx": 27, "type": "sym", "entity": "喘" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "气道炎症" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "气道高反应" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "哮喘" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "慢性气道炎症" }, { "start_idx": 91, "end_idx": 94, "type": "bod", "entity": "中央气道" }, { "start_idx": 96, "end_idx": 101, "type": "bod", "entity": "大气道平滑肌" }, { "start_idx": 106, "end_idx": 113, "type": "bod", "entity": "肌梭内咳嗽感受器" }, { "start_idx": 116, "end_idx": 119, "type": "sym", "entity": "剧烈咳嗽" }, { "start_idx": 124, "end_idx": 128, "type": "sym", "entity": "小气道阻塞" }, { "start_idx": 124, "end_idx": 126, "type": "bod", "entity": "小气道" } ] }, { "text": "第六节巨输尿管一般认为,小儿输尿管的直径大于0.7cm是巨输尿管。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "巨输尿管" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "输尿管" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "但巨输尿管更多是因为肾积水而被发现,随之的影像学检查再显示出输尿管全程的扩张及迂曲,从而做出巨输尿管的诊断。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "巨输尿管" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾积水" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "影像学检查" }, { "start_idx": 21, "end_idx": 40, "type": "sym", "entity": "影像学检查再显示出输尿管全程的扩张及迂曲" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "输尿管" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "患儿常合并肾盂、肾盏的扩张和积水。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "sym", "entity": "肾盂、肾盏的扩张和积水" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肾盂" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肾盏" } ] }, { "text": "巨输尿管诊治的关键是依其原因确定合理的治疗方案。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "【分类】巨输尿管按原因可分为反流性、梗阻性、既反流又梗阻性和非反流非梗阻性四类。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "(一)反流性巨输尿管主要由膀胱输尿管反流而致。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "反流性巨输尿管" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "又可进一步分为原发性和继发性的,这一部分我们在膀胱输尿管反流章节予以详述。", "entities": [ { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "(二)既反流又梗阻性巨输尿管所占病人不多,据报道膀胱输尿管反流的病人中有梗阻的约占2%。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "既反流又梗阻性巨输尿管" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "梗阻" } ] }, { "text": "(三)梗阻性巨输尿管由输尿管远端的功能性或机械性梗阻导致。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "梗阻性巨输尿管" }, { "start_idx": 11, "end_idx": 25, "type": "sym", "entity": "输尿管远端的功能性或机械性梗阻" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "输尿管远端" } ] }, { "text": "1.原发性梗阻性巨输尿管由于近膀胱3~4cm的远端输尿管的一段失去蠕动能力,导致尿液无法以正常速率排入膀胱所致。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "原发性梗阻性巨输尿管" }, { "start_idx": 14, "end_idx": 36, "type": "sym", "entity": "近膀胱3~4cm的远端输尿管的一段失去蠕动能力" }, { "start_idx": 14, "end_idx": 30, "type": "bod", "entity": "近膀胱3~4cm的远端输尿管的一段" }, { "start_idx": 40, "end_idx": 52, "type": "sym", "entity": "尿液无法以正常速率排入膀胱" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "尿液" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "膀胱" } ] }, { "text": "电镜观察肌肉细胞之间的胶原纤维增生,干扰了细胞之间的紧密连接,阻止了正常电传导及蠕动阻止了正常电传导及蠕动。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "电镜" }, { "start_idx": 4, "end_idx": 16, "type": "sym", "entity": "肌肉细胞之间的胶原纤维增生" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "肌肉细胞" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "胶原纤维增生" }, { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "干扰了细胞之间的紧密连接" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "细胞之间" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "阻止了正常电传导及蠕动" }, { "start_idx": 42, "end_idx": 52, "type": "sym", "entity": "阻止了正常电传导及蠕动" } ] }, { "text": "2.继发性梗阻性巨输尿管常见于神经源性膀胱或非神经源性排尿功能障碍及下尿路梗阻(如后尿道瓣膜)。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "继发性梗阻性巨输尿管" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 22, "end_idx": 32, "type": "dis", "entity": "非神经源性排尿功能障碍" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "下尿路梗阻" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "后尿道瓣膜" } ] }, { "text": "其他尚包括输尿管腔内或周围的肿瘤梗阻或压迫、输尿管周围慢性炎症导致的纤维化等。", "entities": [ { "start_idx": 5, "end_idx": 20, "type": "sym", "entity": "输尿管腔内或周围的肿瘤梗阻或压迫" }, { "start_idx": 5, "end_idx": 12, "type": "bod", "entity": "输尿管腔内或周围" }, { "start_idx": 22, "end_idx": 30, "type": "dis", "entity": "输尿管周围慢性炎症" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "纤维化" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "纤维" } ] }, { "text": "(四)非反流非梗阻性巨输尿管1.原发性非反流非梗阻性巨输尿管在反流、梗阻以及所有继发的因素都被排除后,原发性非反流非梗阻性巨输尿管的诊断才能考虑。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "非反流非梗阻性巨输尿管" }, { "start_idx": 16, "end_idx": 29, "type": "dis", "entity": "原发性非反流非梗阻性巨输尿管" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "反流" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "梗阻" }, { "start_idx": 51, "end_idx": 64, "type": "dis", "entity": "原发性非反流非梗阻性巨输尿管" } ] }, { "text": "从文献报道来看,这种类型的巨输尿管主要还是见于新生儿中。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "形成这种变异的原因并不清楚,可能与肾脏发育过程中的某个环节有关,如由于肾小球滤过率、肾血管阻力以及肾小球浓缩能力缘由,胎儿时的尿量可4~6倍于其出生后的尿量,这时如有输尿管远端梗阻,即使是一过性的梗阻,也可形成输尿管扩张。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肾脏" }, { "start_idx": 35, "end_idx": 40, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 42, "end_idx": 46, "type": "ite", "entity": "肾血管阻力" }, { "start_idx": 49, "end_idx": 55, "type": "ite", "entity": "肾小球浓缩能力" }, { "start_idx": 63, "end_idx": 64, "type": "ite", "entity": "尿量" }, { "start_idx": 76, "end_idx": 77, "type": "ite", "entity": "尿量" }, { "start_idx": 83, "end_idx": 89, "type": "dis", "entity": "输尿管远端梗阻" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "一过性的梗阻" }, { "start_idx": 105, "end_idx": 107, "type": "bod", "entity": "输尿管" }, { "start_idx": 105, "end_idx": 109, "type": "sym", "entity": "输尿管扩张" } ] }, { "text": "持续存在的输尿管皱折、尚未成熟的输尿管蠕动功能、婴幼儿膀胱的顺应性差以及输尿管本身的结构异常也都可能引起原发性非反流非梗阻性巨输尿管的发生。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "持续存在的输尿管皱折" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "输尿管" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "输尿管" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "膀胱的顺应性差" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "膀胱" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "输尿管本身的结构异常" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "输尿管" }, { "start_idx": 52, "end_idx": 65, "type": "dis", "entity": "原发性非反流非梗阻性巨输尿管" } ] }, { "text": "2.继发性非反流非梗阻性巨输尿管这一类巨输尿管比过去认识的要多。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "继发性非反流非梗阻性巨输尿管" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "如急性尿路感染时,细菌的内毒素可抑制输尿管的蠕动而发生巨输尿管。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "急性尿路感染" }, { "start_idx": 9, "end_idx": 14, "type": "mic", "entity": "细菌的内毒素" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "输尿管" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "糖尿病及尿崩症患儿尿量的增加也可导致。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "糖尿病" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "尿崩症" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "尿量" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "尿量的增加" } ] }, { "text": "另一种就是李状腹综合征的情况,输尿管有非梗阻性的扩张。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "李状腹综合征" }, { "start_idx": 15, "end_idx": 25, "type": "sym", "entity": "输尿管有非梗阻性的扩张" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "输尿管" } ] }, { "text": "【临床表现】巨输尿管依其发生原因不同而各有特征性的表现。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "如反流性的以尿路感染为主要征象;梗阻性的可有腹痛及呕吐等情况;输尿管扩张巨大时腹部可触及肿块。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "尿路感染" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "梗阻" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "腹痛" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "腹" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "输尿管扩张巨大" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "输尿管" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "腹部可触及肿块" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "腹部" } ] }, { "text": "继发性巨输尿管症往往是在原发病检查时被发现。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "继发性巨输尿管症" } ] }, { "text": "出生后即成为新生儿巨输尿管。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "新生儿巨输尿管" } ] }, { "text": "【诊断】对有泌尿系统症状或有生长发育迟缓的小儿都应进行泌尿系统的影像学检查。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "泌尿系统症状" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "生长发育迟缓" }, { "start_idx": 27, "end_idx": 36, "type": "pro", "entity": "泌尿系统的影像学检查" } ] }, { "text": "1.超声简便易行,除诊断外还是主要的随访手段。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "超声" } ] }, { "text": "从波士顿儿童医院的经验来看,超声观察肾积水的发展情况是决定巨输尿管治疗的主要手段。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "超声" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肾积水" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "2.静脉尿路造影(IVU)可粗略了解肾功能情况及明确上尿路形态。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "静脉尿路造影" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "IVU" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肾" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "上尿路" } ] }, { "text": "大部分巨输尿管在IVU上都可得到显示。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "巨输尿管" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "IVU" } ] }, { "text": "3.排尿性膀胱尿道造影(VCUG)可发现反流性巨输尿管及继发性巨输尿管的原发病,如后尿道瓣膜、神经性膀胱、膀胱憩室和输尿管囊肿等。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "VCUG" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "反流性巨输尿管" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "继发性巨输尿管" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "神经性膀胱" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "膀胱憩室" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "输尿管囊肿" } ] }, { "text": "可以评价膀胱和尿道的情况。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "膀胱" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "尿道" } ] }, { "text": "4.磁共振尿路显像(MRU)巨输尿管和肾盂的积水、扩张,在磁共振的T2相上可以显示。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "磁共振尿路显像" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "MRU" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "巨输尿管" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "肾盂的积水、扩张" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肾盂" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "磁共振" } ] }, { "text": "5.利尿性肾图可以注射呋塞米得有关肾功能的准确信息,通过注射呋塞米,则利于区分功能性或机械性的梗阻。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "利尿性肾图" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "注射" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "呋塞米" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肾" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "注射" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "呋塞米" }, { "start_idx": 39, "end_idx": 48, "type": "dis", "entity": "功能性或机械性的梗阻" } ] }, { "text": "【治疗】巨输尿管的治疗依其种类不同而各有特点,目标是保护肾功能。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "巨输尿管" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "保护肾功能" } ] }, { "text": "(一)继发性巨输尿管继发性巨输尿管的治疗关键是原发病的治疗,如后尿道瓣膜病人有40%~60%有输尿管反流;电灼瓣膜后反流有1/3缓解,1/3可被药物控制,只1/3需手术。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "继发性巨输尿管" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "继发性巨输尿管" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "原发病的治疗" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "输尿管反流" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "电灼瓣膜" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "反流" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "药物控制" }, { "start_idx": 82, "end_idx": 83, "type": "pro", "entity": "手术" } ] }, { "text": "而糖尿病和尿路感染引起的根本不需治疗。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "糖尿病" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "尿路感染" } ] }, { "text": "(二)原发性梗阻性或非梗阻性巨输尿管包括输尿管膀胱连接部及以上部位的梗阻,如输尿管狭窄、瓣膜、闭锁、异位输尿管开口及远端无蠕动功能输尿管等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "原发性梗阻性" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "非梗阻性巨输尿管" }, { "start_idx": 20, "end_idx": 35, "type": "dis", "entity": "输尿管膀胱连接部及以上部位的梗阻" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "输尿管狭窄" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "瓣膜" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "闭锁" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "异位输尿管" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "开口及远端无蠕动功能" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "开口" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "远端" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "输尿管" } ] }, { "text": "胎儿和新生儿的巨输尿管主要是这一类。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "巨输尿管" } ] }, { "text": "对新生儿或婴幼儿进行手术的效果从随访来看并不令人满意,8个月前手术的其再手术率可达12%,因此如肾功能能维持稳定,尿路感染能够控制,应首选随访观察。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "进行手术" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "手术" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "手术" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "肾" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "尿路感染" } ] }, { "text": "此期间,可每3~6个月复查超声和尿常规,如输尿管扩张有改善则可继续随访观察,但复查的间隔可以延长。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "超声" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "尿常规" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "输尿管扩张" } ] }, { "text": "如肾和输尿管的积水及扩张仍然严重,没有改善,可在1岁后选择手术治疗。", "entities": [ { "start_idx": 1, "end_idx": 15, "type": "sym", "entity": "肾和输尿管的积水及扩张仍然严重" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肾" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "输尿管" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "手术治疗" } ] }, { "text": "巨输尿管再植入膀胱前需进行裁剪。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "巨输尿管" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "植入膀胱" } ] }, { "text": "三、食物和呕吐物吸入除食物本身的刺激外,反流的胃酸亦是肺损伤的重要决定因素。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "食物和呕吐物吸入" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "反流的胃酸" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肺损伤" } ] }, { "text": "肺部可闻广泛湿啰音和哮鸣音呼吸道黏膜易继发细菌感染。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "肺部可闻广泛湿啰音和哮鸣音" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "细菌感染" } ] }, { "text": "参考文献1.苏林,许春娣,陈舜年,等.儿童功能性消化不良的胃动力学改变与普瑞博思的疗效.中国实用儿科杂志,1999,14(10):612-6142.田虹,周汉建,陈小良,等.功能性消化不良及十二指肠溃疡的若干因素分析.胃肠病学和肝病杂志,1999,8(4):275-2753.王元骏,彭玄杰.小剂量多虑平治疗功能性消化不良对照研究.健康心理学杂志,1999,7(4)∶468-4694.张艳玲,张玉琳.功能性消化不良儿童胆囊运动障碍24例临床研究.中国实用儿科杂志,1999,14(7):427-4285.张万明,王岚,郑敏,等.儿童功能性消化不良与幽门螺杆菌的关系.实用儿科临床杂志,1998,13(3):131-1316.BjornssonES,AbrahamssonH.Contractilepatternsinpatientswithseverechronicdyspepsia.AmJGastroenterol,1999,94(1):54-647.HoltmannG,TalleyNJ,MithchellH,etal.AntibodyreponsetospecificH.piloriantigenesinfunctionaldyspepsia,duodenalulcerdisease,andhealth.AmJGastroenterol,1998,93(8):1222-12228.HeikkinenM,MayK,MegraudF,etal.AssociationofCagApositiveandCagAnegativeH.pyloristrainswithpatientssymptomsandgastritisinprimarycarepatientswithfunctionalupperabdominalcomplaints.ScandJGastroenterol,1998,33(1):31-319.KaulA,RudolphCd.Gastrointestinalmanometrystudiesinchildren.JClinGastroenterol,1998,27(3):187-18710.KlinkenbergknolEc,NelvsF,DentJ,etal.Longtermomeprazoletreatmentinresistancegastroesophagealrefluxdisease.Gastroenterology.2000,118(4):661-66911.McCarthyC,kPatchettS,CoclinsRM,etal.LongtermprospectivestudyoftheHelicobauterpyloriinnonulcerdyspepsia.DigDisSci,1995,40:114-11412.SandhuBK,MoultrieJ,DunhamM,etal.SilentGastrooesophagealrefluxmaybeacommoncauseoffoodrefusalandfailuretothrive.6thcongressoftheAsian,Pan,PacIFicsocieryofPaediatricGastrorntrologyandnutrition,1999,6∶79-8013.中华医学会消化病学分会.中国慢性胃炎共识意见.胃肠病学,2006,11:674-68414.HeikkinenM,MayK,MegraudF,etal.AssociationofCagApositiveandCagAnegativeH.pyloristrainswithpatients’symptomsandgastritisinprimarycarepatientswithfunctionalupperabdominalcomplaints.ScandJGastroenterol,1998,33(1):31-3115.罗金燕.功能性消化不良的再认识—罗马Ⅲ标准解读.中华内科杂志,2009,48:104-10416.TackJ,TalleyNY,CamilleriM,etal.Functionalgastroduodenaldisorders.Gastroenterology,2006,130:1466-1466", "entities": [ { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "儿童功能性消化不良" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "胃动力学改变" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "胃" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "普瑞博思" }, { "start_idx": 48, "end_idx": 49, "type": "dep", "entity": "儿科" }, { "start_idx": 87, "end_idx": 93, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 95, "end_idx": 100, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 109, "end_idx": 111, "type": "dis", "entity": "胃肠病" }, { "start_idx": 114, "end_idx": 115, "type": "dis", "entity": "肝病" }, { "start_idx": 149, "end_idx": 151, "type": "dru", "entity": "多虑平" }, { "start_idx": 154, "end_idx": 160, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 201, "end_idx": 207, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 208, "end_idx": 215, "type": "dis", "entity": "儿童胆囊运动障碍" }, { "start_idx": 228, "end_idx": 229, "type": "dep", "entity": "儿科" }, { "start_idx": 265, "end_idx": 273, "type": "dis", "entity": "儿童功能性消化不良" }, { "start_idx": 275, "end_idx": 279, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 286, "end_idx": 287, "type": "dep", "entity": "儿科" }, { "start_idx": 1406, "end_idx": 1409, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 1415, "end_idx": 1417, "type": "dis", "entity": "胃肠病" }, { "start_idx": 1659, "end_idx": 1665, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 1681, "end_idx": 1682, "type": "dep", "entity": "内科" } ] }, { "text": "四、儿童肿瘤流行病学研究意义1.儿童肿瘤比成人提供更多的致癌基因信息,同时也可提供致肿瘤的环境因素信息。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 29, "end_idx": 29, "type": "dis", "entity": "癌" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "肿瘤" } ] }, { "text": "2.儿童肿瘤发病率低,促使儿童肿瘤专家相互协作,共同验证临床及各种理论性的假设,加速了肿瘤研究的进展,向成人肿瘤专家提供了很好的协作研究榜样。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "肿瘤" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "成人肿瘤" } ] }, { "text": "3.根据目前儿童肿瘤的治愈率,预计将来16~44岁成年人群中儿童肿瘤生存者为1/900人。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "由于这些人的治疗史和可能存在的基因因素,在儿童期肿瘤后的20年内第二肿瘤的发病率估计接近8%~9%。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "基因" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "儿童期肿瘤" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "第二肿瘤" } ] }, { "text": "从流行病角度,这些人代表了一个特殊的群体,对这一群体的研究可能会发现致癌的基因和环境因素,有利于肿瘤预防和干预措施的研究。", "entities": [ { "start_idx": 35, "end_idx": 35, "type": "dis", "entity": "癌" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "基因" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "肿瘤" } ] }, { "text": "第四章糖尿病【概述】糖尿病(diabetesmellitus,DM)是一种常见的,慢性的代谢综合征,其基本的生化特点是高血糖,并由于胰岛素绝对或者相对缺乏而造成糖、脂肪及蛋白质代谢紊乱。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "糖尿病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "糖尿病" }, { "start_idx": 14, "end_idx": 29, "type": "dis", "entity": "diabetesmellitus" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "DM" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "慢性的代谢综合征" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "高血糖" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "胰岛素" }, { "start_idx": 66, "end_idx": 76, "type": "sym", "entity": "胰岛素绝对或者相对缺乏" }, { "start_idx": 80, "end_idx": 91, "type": "dis", "entity": "糖、脂肪及蛋白质代谢紊乱" } ] }, { "text": "儿童原发性糖尿病主要分为三大类:①1型糖尿病,因胰岛β细胞破坏、胰岛素分泌绝对缺乏所造成,必须使用胰岛素治疗,故又称胰岛素依赖型糖尿病(IDDM),95%儿童期糖尿病属此类型;②2型糖尿病,肌肉、肝脏和脂肪组织的胰岛素抵抗为主,伴胰岛β细胞分泌胰岛素不足或相对缺乏,亦称非胰岛素依赖型糖尿病(NIDDM),在儿童期发病者较少,但由于我国近年来发生的儿童肥胖症明显增多,发病率有增加趋势;③其他特殊类型糖尿病:如青少年早发的2型糖尿病(maturity-onsettypediabetesoftheyoung,MODY),包括HNF-1α、葡萄糖激酶及HNF-4α等基因缺陷,这是一类常染色体显性的单基因遗传病,属非胰岛素依赖型糖尿病,儿童极为罕见。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "儿童原发性糖尿病" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "1型糖尿病" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "胰岛β细胞" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "胰岛β细胞破坏" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "胰岛素" }, { "start_idx": 32, "end_idx": 40, "type": "sym", "entity": "胰岛素分泌绝对缺乏" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "胰岛素" }, { "start_idx": 58, "end_idx": 66, "type": "dis", "entity": "胰岛素依赖型糖尿病" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "IDDM" }, { "start_idx": 77, "end_idx": 82, "type": "dis", "entity": "儿童期糖尿病" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "2型糖尿病" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "肌肉" }, { "start_idx": 95, "end_idx": 110, "type": "sym", "entity": "肌肉、肝脏和脂肪组织的胰岛素抵抗" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "肝脏" }, { "start_idx": 101, "end_idx": 104, "type": "bod", "entity": "脂肪组织" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "胰岛素" }, { "start_idx": 115, "end_idx": 119, "type": "bod", "entity": "胰岛β细胞" }, { "start_idx": 115, "end_idx": 131, "type": "sym", "entity": "胰岛β细胞分泌胰岛素不足或相对缺乏" }, { "start_idx": 122, "end_idx": 124, "type": "bod", "entity": "胰岛素" }, { "start_idx": 135, "end_idx": 144, "type": "dis", "entity": "非胰岛素依赖型糖尿病" }, { "start_idx": 146, "end_idx": 150, "type": "dis", "entity": "NIDDM" }, { "start_idx": 174, "end_idx": 178, "type": "dis", "entity": "儿童肥胖症" }, { "start_idx": 200, "end_idx": 202, "type": "dis", "entity": "糖尿病" }, { "start_idx": 211, "end_idx": 215, "type": "dis", "entity": "2型糖尿病" }, { "start_idx": 217, "end_idx": 252, "type": "dis", "entity": "maturity-onsettypediabetesoftheyoung" }, { "start_idx": 254, "end_idx": 257, "type": "dis", "entity": "MODY" }, { "start_idx": 262, "end_idx": 285, "type": "sym", "entity": "HNF-1α、葡萄糖激酶及HNF-4α等基因缺陷" }, { "start_idx": 262, "end_idx": 283, "type": "bod", "entity": "HNF-1α、葡萄糖激酶及HNF-4α等基因" }, { "start_idx": 291, "end_idx": 303, "type": "dis", "entity": "常染色体显性的单基因遗传病" }, { "start_idx": 306, "end_idx": 315, "type": "dis", "entity": "非胰岛素依赖型糖尿病" } ] }, { "text": "还有线粒体糖尿病等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "线粒体糖尿病" } ] }, { "text": "本章主要叙述儿童期1型糖尿病。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "儿童期1型糖尿病" } ] }, { "text": "【流行病学】世界各国、各地区儿童糖尿病发病率不同。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "儿童糖尿病" } ] }, { "text": "我国22个地区15岁以下儿童糖尿病平均发病率为0.56/10万,其中北京0.90/10万,上海0.83/10万(1989—1993)。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "儿童糖尿病" } ] }, { "text": "随着社会经济的发展,儿童时期的糖尿病与成年人一样,有逐年升高趋势。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "糖尿病" } ] }, { "text": "【病因机制和病理生理】1.病因机制(1)流行病学调查提示,糖尿病的发生与种族、地理环境、生活方式、饮食及感染等有关。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "糖尿病" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "感染" } ] }, { "text": "儿童糖尿病各年龄均可发病,但以5~7岁和10~13岁两组年龄多见,婴幼儿糖尿病较少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童糖尿病" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "婴幼儿糖尿病" } ] }, { "text": "随着经济发展和生活方式的改变,儿童糖尿病亦有逐年增高趋势。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "儿童糖尿病" } ] }, { "text": "(2)自身免疫:环境因素有病毒感染:CoxsackieB组病毒、EB病毒及腮腺炎病毒等;牛乳蛋白:过早、过多地摄入牛乳制品,其中酪蛋白作为抗原,触发糖尿病发生。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "病毒感染" }, { "start_idx": 18, "end_idx": 30, "type": "mic", "entity": "CoxsackieB组病毒" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "EB病毒" }, { "start_idx": 37, "end_idx": 41, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "糖尿病" } ] }, { "text": "牛乳中牛胰岛素可能引起破坏人β细胞功能的免疫反应。", "entities": [ { "start_idx": 11, "end_idx": 23, "type": "sym", "entity": "破坏人β细胞功能的免疫反应" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "人β细胞" } ] }, { "text": "自身抗原有谷氨酸脱羧酶(GAD)、胰岛素、胰岛抗原及胰岛细胞抗原,产生相应的自身抗体如GAD抗体、胰岛细胞抗体(ICA)和胰岛素自身抗体(IAA)等。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "谷氨酸脱羧酶" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "GAD" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "胰岛素" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "胰岛抗原" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "胰岛细胞抗原" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "GAD抗体" }, { "start_idx": 49, "end_idx": 54, "type": "bod", "entity": "胰岛细胞抗体" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "ICA" }, { "start_idx": 61, "end_idx": 67, "type": "bod", "entity": "胰岛素自身抗体" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "IAA" } ] }, { "text": "(3)遗传易感:遗传因素在1型糖尿病的发病过程中起着重要的作用。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "1型糖尿病" } ] }, { "text": "目前已知该病为多基因遗传病,有多个基因与糖尿病的遗传易感性有关。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "多基因遗传病" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "多个基因" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "糖尿病" } ] }, { "text": "目前研究最多的是1型糖尿病与人类白细胞抗原(HLA)D区的Ⅱ类抗原基因,后者位于第6号染色体短臂(6p21.3)。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "1型糖尿病" }, { "start_idx": 14, "end_idx": 34, "type": "bod", "entity": "人类白细胞抗原(HLA)D区的Ⅱ类抗原基因" }, { "start_idx": 40, "end_idx": 47, "type": "bod", "entity": "第6号染色体短臂" }, { "start_idx": 49, "end_idx": 54, "type": "bod", "entity": "6p21.3" } ] }, { "text": "人群调查发现1型糖尿病的发病与HLAⅡ类抗原DR3、DR4有关,单卵双胎先后发生糖尿病的一致性为35%~50%,如同时有HLA-DR3/DR4者发生糖尿病一致性为70%。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "1型糖尿病" }, { "start_idx": 15, "end_idx": 30, "type": "bod", "entity": "HLAⅡ类抗原DR3" }, { "start_idx": 32, "end_idx": 40, "type": "bod", "entity": "DR4" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "单卵双胎" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "糖尿病" }, { "start_idx": 72, "end_idx": 78, "type": "bod", "entity": "HLA-DR3" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "DR4" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "糖尿病" } ] }, { "text": "近年研究发现,HLA-DQα链第52位精氨酸及DQβ链第57位非门冬氨酸等位基因为1型糖尿病易感性基因;HLA-DQα链第52位非精氨酸及DQβ链第57位门冬氨酸等为糖尿病保护基因。", "entities": [ { "start_idx": 7, "end_idx": 39, "type": "bod", "entity": "HLA-DQα链第52位精氨酸及DQβ链第57位非门冬氨酸等位基因" }, { "start_idx": 41, "end_idx": 50, "type": "bod", "entity": "1型糖尿病易感性基因" }, { "start_idx": 52, "end_idx": 67, "type": "bod", "entity": "HLA-DQα链第52位非精氨酸" }, { "start_idx": 69, "end_idx": 80, "type": "bod", "entity": "DQβ链第57位门冬氨酸" }, { "start_idx": 83, "end_idx": 89, "type": "bod", "entity": "糖尿病保护基因" } ] }, { "text": "因此HLA-Ⅱ类分子DRDQα1-DQβ1的结构是影响1型糖尿病的易感性和保护性的主要因素。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "bod", "entity": "HLA-Ⅱ类分子DRDQα1-DQβ1" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "1型糖尿病" } ] }, { "text": "2.病理生理糖尿病患儿由于胰岛素分泌不足或缺如,使葡萄糖的利用(进入细胞)量减少,而增高的胰高血糖素、生长激素和皮质醇等却又促进肝糖原分解和葡萄糖异生,脂肪和蛋白质分解加速,造成血糖增高和细胞外液渗透压增高、细胞内液向细胞外转移。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "糖尿病" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "胰岛素" }, { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "胰岛素分泌不足或缺如" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 25, "end_idx": 39, "type": "sym", "entity": "葡萄糖的利用(进入细胞)量减少" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "细胞" }, { "start_idx": 42, "end_idx": 58, "type": "sym", "entity": "增高的胰高血糖素、生长激素和皮质醇" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "胰高血糖素" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "生长激素" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "皮质醇" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "肝糖原" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "脂肪" }, { "start_idx": 76, "end_idx": 85, "type": "sym", "entity": "脂肪和蛋白质分解加速" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "蛋白质" }, { "start_idx": 89, "end_idx": 92, "type": "sym", "entity": "血糖增高" }, { "start_idx": 89, "end_idx": 90, "type": "ite", "entity": "血糖" }, { "start_idx": 94, "end_idx": 102, "type": "sym", "entity": "细胞外液渗透压增高" }, { "start_idx": 94, "end_idx": 100, "type": "ite", "entity": "细胞外液渗透压" }, { "start_idx": 104, "end_idx": 113, "type": "sym", "entity": "细胞内液向细胞外转移" }, { "start_idx": 104, "end_idx": 107, "type": "bod", "entity": "细胞内液" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "细胞外" } ] }, { "text": "当血糖浓度超过肾阈值时,即产生糖尿。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "血糖浓度" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "肾阈值" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "糖尿" } ] }, { "text": "自尿液排出的葡萄糖量可达200~300g/d,导致渗透性利尿,临床出现多尿症状,每日丢失大量的水分和电解质,因而造成严重的电解质失衡和慢性脱水。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "ite", "entity": "尿液排出的葡萄糖量" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "渗透性利尿" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "多尿" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "尿" }, { "start_idx": 42, "end_idx": 52, "type": "sym", "entity": "丢失大量的水分和电解质" }, { "start_idx": 44, "end_idx": 52, "type": "bod", "entity": "大量的水分和电解质" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "电解质失衡" }, { "start_idx": 67, "end_idx": 70, "type": "sym", "entity": "慢性脱水" } ] }, { "text": "由于机体的代偿作用,患儿渴感增加,饮水增多;又因为组织不能利用葡萄糖,能量不足而产生饥饿感,引起多食。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "渴感增加" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "饮水增多" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "能量不足" }, { "start_idx": 42, "end_idx": 44, "type": "sym", "entity": "饥饿感" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "多食" } ] }, { "text": "胰岛素不足和胰岛素拮抗激素,如胰高糖素、肾上腺素、皮质醇及生长激素的增高,促进了脂肪分解,血中脂肪酸增高,肌肉和胰岛素依赖性组织即利用这类游离脂肪酸供能以弥补细胞内葡萄糖不足,而过多的游离脂肪酸在进入肝脏后则在胰高糖素等生酮激素作用下加速氧化,导致乙酰乙酸、β-羟丁酸等酮体累积在各种体液中,形成酮症酸中毒。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "胰岛素" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "胰岛素不足" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "胰岛素拮抗激素" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "胰高糖素" }, { "start_idx": 15, "end_idx": 35, "type": "sym", "entity": "胰高糖素、肾上腺素、皮质醇及生长激素的增高" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "皮质醇" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "生长激素" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "脂肪" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "血中脂肪酸" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "血中脂肪酸增高" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肌肉" }, { "start_idx": 56, "end_idx": 63, "type": "bod", "entity": "胰岛素依赖性组织" }, { "start_idx": 69, "end_idx": 73, "type": "bod", "entity": "游离脂肪酸" }, { "start_idx": 79, "end_idx": 86, "type": "sym", "entity": "细胞内葡萄糖不足" }, { "start_idx": 79, "end_idx": 84, "type": "bod", "entity": "细胞内葡萄糖" }, { "start_idx": 89, "end_idx": 96, "type": "sym", "entity": "过多的游离脂肪酸" }, { "start_idx": 92, "end_idx": 96, "type": "bod", "entity": "游离脂肪酸" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "肝脏" }, { "start_idx": 105, "end_idx": 108, "type": "bod", "entity": "胰高糖素" }, { "start_idx": 105, "end_idx": 120, "type": "sym", "entity": "胰高糖素等生酮激素作用下加速氧化" }, { "start_idx": 110, "end_idx": 113, "type": "bod", "entity": "生酮激素" }, { "start_idx": 124, "end_idx": 127, "type": "bod", "entity": "乙酰乙酸" }, { "start_idx": 129, "end_idx": 133, "type": "bod", "entity": "β-羟丁酸" }, { "start_idx": 135, "end_idx": 136, "type": "bod", "entity": "酮体" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "体液" }, { "start_idx": 148, "end_idx": 152, "type": "dis", "entity": "酮症酸中毒" } ] }, { "text": "血渗透压升高、水和电解质紊乱以及酮症酸中毒等代谢失常的发生,最终都造成中枢神经系统的损伤,甚至导致意识障碍或昏迷。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "血渗透压升高" }, { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血渗透压" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "水和电解质紊乱" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "酮症酸中毒" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "代谢失常" }, { "start_idx": 35, "end_idx": 43, "type": "dis", "entity": "中枢神经系统的损伤" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "意识障碍" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "昏迷" } ] }, { "text": "【临床表现】胰岛细胞破坏90%左右可出现糖尿病临床症状。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "胰岛细胞" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "胰岛细胞破坏" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "糖尿病" } ] }, { "text": "各年龄均可发病,小至新生儿糖尿病,但以5~7岁和10~13岁两组年龄多见,患病率男女无性别差异。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "新生儿糖尿病" } ] }, { "text": "除消瘦外,一般无阳性体征发现。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "消瘦" } ] }, { "text": "【实验室.血糖增高,空腹血糖>7.0mmol/L,随机血糖≥11.1mmol/L。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "血糖增高" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "血糖" }, { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "空腹血糖>7.0mmol/L" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "空腹血糖" }, { "start_idx": 25, "end_idx": 39, "type": "sym", "entity": "随机血糖≥11.1mmol/L" }, { "start_idx": 25, "end_idx": 28, "type": "ite", "entity": "随机血糖" } ] }, { "text": "测定治疗前的糖化血红蛋白(HbA1c)以估计高血糖的持续时间,这有利于进行治疗前后的对照以判断疗效,正常人<6%,未治疗患者常大于正常的2倍以上。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "ite", "entity": "测定治疗前的糖化血红蛋白(HbA1c)" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "高血糖" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "血糖" } ] }, { "text": "若糖尿病患者血糖控制水平<8.3mmol/L时,HbA1c常<7%,为最理想的控制水平。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "糖尿病" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "血糖" }, { "start_idx": 24, "end_idx": 28, "type": "ite", "entity": "HbA1c" } ] }, { "text": "若HbA1c>9%,发生糖尿病微血管并发症的危险性明显增加。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "ite", "entity": "HbA1c" }, { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "HbA1c>9%" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "糖尿病微血管并发症" } ] }, { "text": "3.血电解质酮症酸中毒时血电解质紊乱,应测血Na、K、Cl、CO2CP、血pH及血浆渗透压。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "血电解质酮症酸中毒" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "血电解质紊乱" }, { "start_idx": 20, "end_idx": 50, "type": "ite", "entity": "测血Na、K、Cl、CO2CP、血pH及血浆渗透压" } ] }, { "text": "4.血脂代谢紊乱期血清胆固醇及甘油三酯均明显增高。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "血脂代谢紊乱" }, { "start_idx": 9, "end_idx": 23, "type": "sym", "entity": "血清胆固醇及甘油三酯均明显增高" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "血清胆固醇" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "甘油三酯" } ] }, { "text": "5.尿液检测尿糖增高及尿酮体阳性。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "尿液检测" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "尿糖增高" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "尿糖" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "尿酮体阳性" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "尿酮体" } ] }, { "text": "6.葡萄糖耐量试验(OGTT)1型糖尿病一般不需做OGTT,仅用于无明显症状、尿糖偶尔阳性而血糖正常或稍增高的患儿。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "葡萄糖耐量试验" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "OGTT" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "1型糖尿病" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "OGTT" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "尿糖偶尔阳性" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "尿糖" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "血糖" } ] }, { "text": "通常采用口服葡萄糖法。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "口服" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "试验当日禁食,于清晨按1.75g/kg口服葡萄糖(最大量不超过75g),3~5分钟内服完;在口服0、120分钟分别采血测血糖浓度。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "口服" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "口服" }, { "start_idx": 57, "end_idx": 58, "type": "pro", "entity": "采血" }, { "start_idx": 59, "end_idx": 63, "type": "ite", "entity": "测血糖浓度" } ] }, { "text": "7.抗体测定检测抗体GAD、IAA、IA2和ICA,主要用于1型糖尿病诊断和鉴别诊断。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "抗体测定" }, { "start_idx": 6, "end_idx": 24, "type": "pro", "entity": "检测抗体GAD、IAA、IA2和ICA" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "1型糖尿病" } ] }, { "text": "【诊断和鉴别诊断】(一)诊断1型糖尿病的诊断根据脱水、体重不增、多饮多尿、高血糖、糖尿和酮尿便能迅速判定。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "1型糖尿病" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "脱水" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "体重" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "多饮" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "多尿" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "尿" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "高血糖" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "血糖" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "糖尿" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "糖" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "尿" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "酮尿" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "酮" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "尿" } ] }, { "text": "糖尿病诊断标准如下:(1)空腹血糖≥7.0mmol/L(≥126mg/dl);(2)随机血糖≥11.1mmol/L(≥200mg/dl);(3)OGTT2h血糖≥11.1mmol/L(≥200mg/dl)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "糖尿病" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "空腹血糖" }, { "start_idx": 13, "end_idx": 37, "type": "sym", "entity": "空腹血糖≥7.0mmol/L(≥126mg/dl)" }, { "start_idx": 42, "end_idx": 67, "type": "sym", "entity": "随机血糖≥11.1mmol/L(≥200mg/dl)" }, { "start_idx": 42, "end_idx": 45, "type": "ite", "entity": "随机血糖" }, { "start_idx": 72, "end_idx": 101, "type": "sym", "entity": "OGTT2h血糖≥11.1mmol/L(≥200mg/dl)" }, { "start_idx": 72, "end_idx": 79, "type": "ite", "entity": "OGTT2h血糖" } ] }, { "text": "凡符合上述任何一条即可诊断为糖尿病。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "糖尿病" } ] }, { "text": "儿童1型糖尿病一旦出现临床症状、尿糖阳性、空腹血糖达7.0mmol/L以上和随机血糖在11.1mmol/L以上,不需做糖耐量试验就能确诊。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "儿童1型糖尿病" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "尿糖阳性" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "尿糖" }, { "start_idx": 21, "end_idx": 34, "type": "sym", "entity": "空腹血糖达7.0mmol/L" }, { "start_idx": 21, "end_idx": 24, "type": "ite", "entity": "空腹血糖" }, { "start_idx": 38, "end_idx": 54, "type": "sym", "entity": "随机血糖在11.1mmol/L以上" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "随机血糖" }, { "start_idx": 59, "end_idx": 63, "type": "pro", "entity": "糖耐量试验" } ] }, { "text": "若OGTT后2h血糖7.8~11.1mmol/L,为糖耐量减低。", "entities": [ { "start_idx": 1, "end_idx": 23, "type": "sym", "entity": "OGTT后2h血糖7.8~11.1mmol/L" }, { "start_idx": 1, "end_idx": 9, "type": "ite", "entity": "OGTT后2h血糖" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "糖耐量减低" }, { "start_idx": 26, "end_idx": 26, "type": "ite", "entity": "糖" } ] }, { "text": "空腹血糖6.1~7.0mmol/L为空腹血糖损害(IFG)。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "空腹血糖6.1~7.0mmol/L" }, { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "空腹血糖" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "空腹血糖损害" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "空腹血糖" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "IFG" } ] }, { "text": "糖耐量损害是指处于正常体内稳态葡萄糖与糖尿病之间的代谢阶段。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "糖耐量损害" }, { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "糖耐量" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "糖尿病" } ] }, { "text": "空腹葡萄糖浓度超过正常值的上限,则当静脉给予葡萄糖时发生急性胰岛素分泌反应丧失以及发生微血管和大血管并发症的危险性进行性增大。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "空腹葡萄糖浓度超过正常值的上限" }, { "start_idx": 0, "end_idx": 6, "type": "ite", "entity": "空腹葡萄糖浓度" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "静脉给予" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "急性胰岛素分泌反应丧失" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "胰岛素" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "微血管" }, { "start_idx": 43, "end_idx": 52, "type": "sym", "entity": "微血管和大血管并发症" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "大血管" } ] }, { "text": "许多存在糖耐量损害的个体,其日常生活中的血糖是正常的,而且糖化血红蛋白水平也可能正常或接近正常,仅当进行标准的口服葡萄糖耐量试验时才表现出高血糖。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "糖耐量损害" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "糖耐量" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "血糖" }, { "start_idx": 29, "end_idx": 34, "type": "ite", "entity": "糖化血红蛋白" }, { "start_idx": 55, "end_idx": 63, "type": "pro", "entity": "口服葡萄糖耐量试验" }, { "start_idx": 69, "end_idx": 71, "type": "sym", "entity": "高血糖" }, { "start_idx": 70, "end_idx": 71, "type": "ite", "entity": "血糖" } ] }, { "text": "(二)鉴别诊断1.儿童2型糖尿病胰岛素抵抗为主伴胰岛素相对分泌不足,或胰岛素分泌不足伴或不伴胰岛素抵抗,属多基因遗传,近年来发病率有增高趋势。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "儿童2型糖尿病" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "胰岛素抵抗" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "胰岛素" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "胰岛素相对分泌不足" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "胰岛素" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "胰岛素分泌不足" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "胰岛素" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "胰岛素抵抗" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "胰岛素" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "基因" } ] }, { "text": "约1/3患儿无临床症状,有时因肥胖就诊,给予糖耐量试验后才发现。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肥胖" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "糖耐量试验" } ] }, { "text": "一般无酮症酸中毒,但在应激情况下也会发生。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "酮症酸中毒" } ] }, { "text": "血C肽水平正常或增高,各种自身抗体ICA、IAA及GAD均阴性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "血C肽水平" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "自身抗体" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "ICA" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "IAA" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "GAD" } ] }, { "text": "饮食控制、锻炼或口服降糖药治疗有效。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "饮食控制" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "锻炼" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "口服降糖药治疗" } ] }, { "text": "2.青少年型糖尿病(MODY)为单基因遗传的常染色体显性遗传病,是一种特殊类型的非胰岛素依赖性糖尿病。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "青少年型糖尿病" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "MODY" }, { "start_idx": 16, "end_idx": 30, "type": "dis", "entity": "单基因遗传的常染色体显性遗传病" }, { "start_idx": 40, "end_idx": 49, "type": "dis", "entity": "非胰岛素依赖性糖尿病" } ] }, { "text": "至今发现MODY有5种类型及其相关基因。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "MODY" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "基因" } ] }, { "text": "治疗同2型糖尿病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "2型糖尿病" } ] }, { "text": "3.肾性糖尿病无糖尿病症状,多在体检或者做尿常规检查时发现,血糖正常,胰岛素分泌正常。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肾性糖尿病" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "糖尿病" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "体检" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "尿常规检查" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "血糖" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "胰岛素" } ] }, { "text": "也可见于范可尼综合征及近端肾小管功能障碍时。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "范可尼综合征" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "近端肾小管功能障碍" } ] }, { "text": "4.假性高血糖短期大量食入或者输入葡萄糖液,可使尿糖暂时阳性,血糖升高。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "假性高血糖" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "输入" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "葡萄糖液" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "尿糖暂时阳性" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "尿糖" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "血糖升高" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "血糖" } ] }, { "text": "另外,在应急状态时血糖也可一过性升高,需注意鉴别。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "血糖" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "血糖也可一过性升高" } ] }, { "text": "【治疗】儿童糖尿病强调综合治疗,应加强对患者或者家庭的健康教育,使患儿能长期维持血糖接近正常水平,保证儿童获得正常的生活和活动。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "儿童糖尿病" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "综合治疗" }, { "start_idx": 17, "end_idx": 30, "type": "pro", "entity": "加强对患者或者家庭的健康教育" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "血糖" } ] }, { "text": "治疗目的是:①消除糖尿病症状;②避免或减少酮症酸中毒及低血糖产生;③维持儿童正常生长和性发育;④解除患儿心理障碍;⑤防止中晚期并发症出现。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "糖尿病" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "酮症酸中毒" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "低血糖" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "心理障碍" } ] }, { "text": "1.胰岛素替代治疗(1)胰岛素制剂和作用:目前所用的胰岛素主要为基因重组技术合成人胰岛素。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "胰岛素替代治疗" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "胰岛素制剂" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "胰岛素" }, { "start_idx": 32, "end_idx": 43, "type": "dru", "entity": "基因重组技术合成人胰岛素" } ] }, { "text": "短、中效配合使用,每日2次注射方案在国内外均较普遍。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "注射" } ] }, { "text": "表15-5胰岛素的种类和作用时间(2)新诊患儿的初始治疗:开始胰岛素治疗应选用短效胰岛素(RI),初始剂量应根据患儿体重计算,每天0.5~1.0U/kg,分4次于早、中、晚餐前30分钟皮下注射,临睡前再注射一次。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "胰岛素" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "胰岛素治疗" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "短效胰岛素" }, { "start_idx": 45, "end_idx": 46, "type": "dru", "entity": "RI" }, { "start_idx": 92, "end_idx": 95, "type": "pro", "entity": "皮下注射" }, { "start_idx": 101, "end_idx": 102, "type": "pro", "entity": "注射" } ] }, { "text": "每日胰岛素总量的分配:早餐前30%~40%、中餐前20%~30%、晚餐前30%以及临睡前10%。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "胰岛素" } ] }, { "text": "以后可过渡到短、中效胰岛素配合使用。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dru", "entity": "短、中效胰岛素" } ] }, { "text": "(3)胰岛素的调节:一般当饮食和运动量固定时血糖是调节胰岛素的根据。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "胰岛素" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "血糖" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "胰岛素" } ] }, { "text": "用RI时应根据每餐后及下一餐前的血糖调节次日该餐前的胰岛素剂量。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "RI" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "血糖" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "胰岛素" } ] }, { "text": "每次增加或减少胰岛素的剂量不宜过大,以1~2U为宜。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "胰岛素" } ] }, { "text": "7~10岁糖尿病儿使用CSII能够改善代谢,CSII在低龄患儿也取得了好的疗效。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "糖尿病" }, { "start_idx": 11, "end_idx": 14, "type": "equ", "entity": "CSII" }, { "start_idx": 22, "end_idx": 25, "type": "equ", "entity": "CSII" } ] }, { "text": "血糖控制的程度主要取决于患者遵循糖尿病自我监测的严格性,而与使用的胰岛素种类无关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血糖" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "糖尿病" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "胰岛素" } ] }, { "text": "大多数运用胰岛素泵治疗的患者都能减少低血糖频度和严重低血糖发作的疗效。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "胰岛素泵治疗" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "低血糖" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血糖" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "严重低血糖" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血糖" } ] }, { "text": "CSII不会发生体重异常增加。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "CSII" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "体重异常增加" }, { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "体重" } ] }, { "text": "慢性胰岛素过量(Somogyi反应)是指胰岛素(尤其是晚餐前中效胰岛素)慢性过量,凌晨2~3时易发生低血糖,低血糖又引发反调节激素分泌增高,清晨出现高血糖,即低-高血糖反应。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "慢性胰岛素过量" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "Somogyi反应" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "胰岛素" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "中效胰岛素" }, { "start_idx": 50, "end_idx": 52, "type": "sym", "entity": "低血糖" }, { "start_idx": 51, "end_idx": 52, "type": "ite", "entity": "血糖" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "低血糖" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "血糖" }, { "start_idx": 60, "end_idx": 68, "type": "sym", "entity": "反调节激素分泌增高" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "反调节激素" }, { "start_idx": 74, "end_idx": 76, "type": "sym", "entity": "高血糖" }, { "start_idx": 75, "end_idx": 76, "type": "ite", "entity": "血糖" }, { "start_idx": 79, "end_idx": 85, "type": "sym", "entity": "低-高血糖反应" } ] }, { "text": "如清晨尿糖阴性或弱阳性,而尿酮体阳性,则提示夜间低血糖,应检测早晨2~3时血糖,并减少晚餐前或睡前胰岛素用量。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "尿糖" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "尿酮体" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "尿酮体阳性" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "夜间低血糖" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "血糖" }, { "start_idx": 29, "end_idx": 38, "type": "ite", "entity": "检测早晨2~3时血糖" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "胰岛素" } ] }, { "text": "2.营养管理营养管理的目的是使血糖能控制在要求达到的范围内,既要保证儿童正常生长,又避免肥胖,营养师应定期进行营养评估和指导。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "营养管理" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "营养管理" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "血糖" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "肥胖" }, { "start_idx": 55, "end_idx": 61, "type": "pro", "entity": "营养评估和指导" } ] }, { "text": "患者的饮食应基于个人口味和嗜好,且必须与胰岛素治疗同步进行。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "胰岛素治疗" } ] }, { "text": "碳水化合物成分应主要来自淀粉类,高纤维成分的食品有利于促进血糖控制,使食物的消化和吸收时间延长,血糖水平上升较慢。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "血糖" }, { "start_idx": 48, "end_idx": 51, "type": "ite", "entity": "血糖水平" } ] }, { "text": "要限制食用蔗糖及精制糖,包括碳酸饮料,防止糖类吸收过快引起血糖的大幅波动。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "血糖" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "血糖的大幅波动" } ] }, { "text": "如果运动不引起低血糖,则不必调节饮食和胰岛素,运动可使肌肉对葡萄糖利用增加,血糖的调节得以改善。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "低血糖" }, { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "血糖" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "胰岛素" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肌肉" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "血糖" } ] }, { "text": "4.糖尿病酮症酸中毒(DKA)是由于胰岛素缺乏或胰岛素效能不足引起的代谢异常的最终后果,胰岛素效能不足是指应激时拮抗激素阻断胰岛素的作用。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "糖尿病酮症酸中毒" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "DKA" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "胰岛素缺乏" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "胰岛素" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "胰岛素效能不足" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "胰岛素" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "代谢异常" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "胰岛素效能不足" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "胰岛素" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "拮抗激素" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "胰岛素" } ] }, { "text": "临床症状取决于酮症酸中毒的程度,有大量酮尿、血离子间隙增加、和pH下降,血清渗透压增高提示高渗性脱水。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "酮症酸中毒" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "大量酮尿" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "酮" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "血离子间隙增加" }, { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "血离子间隙" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "pH下降" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "pH" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "血清渗透压增高" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "血清渗透压" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "高渗性脱水" } ] }, { "text": "DKA是糖尿病最常见的死亡原因,大多是由于脑水肿的原因。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "DKA" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "糖尿病" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑水肿" } ] }, { "text": "见排尿后即加入氯化钾3~6mmol/kg。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "尿" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "氯化钾" } ] }, { "text": "(2)胰岛素应用:采用小剂量胰岛素持续静脉输入,儿童胰岛素用量为0.1U/(kg•h),加入生理盐水中输入,要检测血糖,防止血糖下降过快。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "胰岛素" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "胰岛素" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "静脉输入" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "胰岛素" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "生理盐水" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "输入" }, { "start_idx": 55, "end_idx": 58, "type": "ite", "entity": "检测血糖" }, { "start_idx": 62, "end_idx": 67, "type": "sym", "entity": "血糖下降过快" }, { "start_idx": 62, "end_idx": 63, "type": "ite", "entity": "血糖" } ] }, { "text": "(3)监测:每小时监测血糖一次,每2~4小时重复一次电解质、血糖、尿糖及血气分析,直至酸中毒纠正。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "监测血糖" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "电解质" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "血糖" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "尿糖" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "血气分析" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "酸中毒" } ] }, { "text": "由于糖尿病是慢性终生性疾病,因此对本病的管理和监控非常重要。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "糖尿病" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "慢性终生性疾病" } ] }, { "text": "血糖应控制在餐前4.4~6.7mmol/L(80~120mg/L)、餐后血糖<8.3~10mmol/L(150~180mg/L),每日平均血糖应<8.3mmol/L(150mg/L)为理想,微血管并发症的发生可以明显减少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血糖" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "餐后血糖" }, { "start_idx": 65, "end_idx": 70, "type": "ite", "entity": "每日平均血糖" }, { "start_idx": 95, "end_idx": 100, "type": "sym", "entity": "微血管并发症" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "微血管" } ] }, { "text": "(3)尿微量白蛋白排泄率测定:一般每年检测1~2次,以监测早期糖尿病肾病的发生。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "ite", "entity": "尿微量白蛋白排泄率测定" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "糖尿病肾病" } ] }, { "text": "第五节插管患者的肺功能检查及影响因素插管患者的肺功能检查除使用特定检测方法外,还须注意影响肺功能检测的因素。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "插管" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "肺功能检查" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "插管" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "肺功能检查" }, { "start_idx": 45, "end_idx": 49, "type": "pro", "entity": "肺功能检测" } ] }, { "text": "(一)频率、流速及容积的因素许多研究证实,呼吸系统存在频率依赖性。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "呼吸系统" } ] }, { "text": "即当通气频率改变时,呼吸机制也将发生改变,通常频率增快,气道阻力增高,弹性上升。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "频率增快" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "气道" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "气道阻力增高" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "弹性上升" } ] }, { "text": "因此肺功能检测必须考虑到频率依赖性。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "肺功能检测" } ] }, { "text": "气道阻力和气管插管阻力同时还存在流速依赖性,即流速上升,阻力增大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "气道" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "气管插管" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "流速上升" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "阻力增大" } ] }, { "text": "呼吸肌完全放松时的压力-容量曲线(图8-8)(即顺应性)呈S形,这意味着呼吸系统在高肺容积和低肺容积时顺应性小。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "ite", "entity": "呼吸肌完全放松时的压力-容量曲线" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "顺应性" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 36, "end_idx": 54, "type": "sym", "entity": "呼吸系统在高肺容积和低肺容积时顺应性小" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肺" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肺" }, { "start_idx": 51, "end_idx": 53, "type": "ite", "entity": "顺应性" } ] }, { "text": "正常时肺的顺应性居于中间的陡坡段,随吸气压力的变化而相应增减;在等肺容积时(中度通气)顺应性最大。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "顺应性" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "肺容积" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "顺应性" } ] }, { "text": "当肺膨胀时,由于肺底胶原纤维的牵拉而使气道开放,气道与组织的关系被称为机械互依,这意味着当肺容积增加时,阻力下降。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 13, "type": "ite", "entity": "肺底胶原纤维" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "气道" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "气道" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "组织" }, { "start_idx": 45, "end_idx": 47, "type": "ite", "entity": "肺容积" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "肺容积增加" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "阻力下降" } ] }, { "text": "相反,机械通气中,吸气时阻力受肺组织和胸壁的影响而逐渐增加,肺容积对总阻力的影响依肺容积对气道及组织的阻力影响而定。", "entities": [ { "start_idx": 12, "end_idx": 28, "type": "sym", "entity": "阻力受肺组织和胸壁的影响而逐渐增加" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺组织" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胸壁" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "肺容积" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "肺容积" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "气道" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "组织" } ] }, { "text": "Crs在机械呼吸(不包括插管的CPAP患者)的孩子明显低于自主呼吸的孩子,Rrs在插管的孩子明显高于非插管者。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "Crs" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "插管" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "CPAP" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "Rrs" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "插管" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "插管" } ] }, { "text": "在ICU中监测肺功能时,必须考虑频率、流速及容积的影响。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "ICU" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "监测肺功能" } ] }, { "text": "(二)气管插管(TT)的影响TT存在时对呼吸力学有一系列影响。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "气管" } ] }, { "text": "首先TT增加了呼吸系统的阻力,其阻力值取决于插管的内径、长度及内表面的情况。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "呼吸系统" } ] }, { "text": "其次TT也可以绕过上呼吸道,这样就把维持婴儿肺容量的主要机制给破坏了。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "肺容量" } ] }, { "text": "另外因为带套囊的TT很少应用于儿科及NICU,故TT周围漏气使呼吸力学变得更加复杂。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dep", "entity": "儿科" }, { "start_idx": 18, "end_idx": 21, "type": "dep", "entity": "NICU" } ] }, { "text": "应该认真考虑TT对被动流速-容量曲线呼气支的影响。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "ite", "entity": "被动流速-容量曲线" } ] }, { "text": "由于阻力是流速依赖性的,TT使呼气支向容量轴方向凹陷。", "entities": [] }, { "text": "在一个计算机模型中,3.5mmTT的流速依赖性阻力可抵消4倍时间常数不稳定造成的呼气支曲线的改变。", "entities": [ { "start_idx": 40, "end_idx": 44, "type": "ite", "entity": "呼气支曲线" } ] }, { "text": "因此,TT存在的情况下,被动呼吸流速-容量曲线呼气支的形态可提示时间常数不稳定的一定程度。", "entities": [ { "start_idx": 12, "end_idx": 22, "type": "ite", "entity": "被动呼吸流速-容量曲线" } ] }, { "text": "(三)机械呼吸和自主呼吸婴幼儿及儿童的机械通气中,通气的压力可在气道开口处测量,这意味着呼吸力学可通过气道开口处的压力和流速来测定。", "entities": [ { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "气道开口处" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "气道开口处" } ] }, { "text": "而自主呼吸时,通气所需的压力是由呼吸肌来完成的,不能在气道开口处测量,在这种情况下,要测量经肺压。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "气道开口处" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "测量经肺压" } ] }, { "text": "(四)漏气的影响TT周围的漏气是机械通气时肺功能测定的主要问题。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "肺功能测定" } ] }, { "text": "不同的呼吸机在通气时,漏气情况也不一样,定容型呼吸机通常产生一个固定的吸气流速,因此在吸气时间恒定时,容积也是恒定的。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "呼吸机" }, { "start_idx": 20, "end_idx": 25, "type": "equ", "entity": "定容型呼吸机" } ] }, { "text": "TT周围的漏气可使VT及峰压下降,但不会改变流速曲线外观(即F-V环形态)。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "VT" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "VT及峰压下降" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "峰压" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "流速曲线" } ] }, { "text": "压力限制性呼吸机可产生一个最初的高吸气流速,此流速在达到峰压时几乎降到零。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "equ", "entity": "压力限制性呼吸机" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "峰压" } ] }, { "text": "TT周围漏气可降低VT,但不会降低压力。", "entities": [ { "start_idx": 9, "end_idx": 21, "type": "ite", "entity": "VT" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "压力" } ] }, { "text": "在以上两种情况下,通过计算吸气时流经TT的气体(用呼吸流速描记仪)会高估给患者的气量。", "entities": [ { "start_idx": 25, "end_idx": 31, "type": "equ", "entity": "呼吸流速描记仪" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "气量" } ] }, { "text": "在机械通气时,大多数测量肺功能的计算机都会对流速信号进行自动校正。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "equ", "entity": "测量肺功能的计算机" } ] }, { "text": "然而患者在检测肺功能时,若TT周围有明显漏气,其数据是不可靠的。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "检测肺功能" } ] }, { "text": "尽管还没有关于漏气限度的系统研究报道,但有些作者认为:临床上漏气量达10%,仍是可以接受的,但是实际上5%左右的漏气即可对阻力及顺应性产生明显的影响。", "entities": [ { "start_idx": 64, "end_idx": 66, "type": "ite", "entity": "顺应性" } ] }, { "text": "在机械通气时,有学者发现RDS早产儿应用肺表面活性物质后肺功能(跨肺压、肺泡膜两边的氧浓度差、VT、MV、吸气流速、顺应性)有很大改善,主要在自主呼吸(CPAP)患者。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "RDS" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "早产儿" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "肺功能" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "跨肺压" }, { "start_idx": 36, "end_idx": 45, "type": "ite", "entity": "肺泡膜两边的氧浓度差" }, { "start_idx": 47, "end_idx": 48, "type": "ite", "entity": "VT" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "MV" }, { "start_idx": 53, "end_idx": 56, "type": "ite", "entity": "吸气流速" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "顺应性" } ] }, { "text": "第六章立克次体感染立克次体病(Rickettsia)是一类威胁人类健康的人兽共患病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "立克次体感染" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "立克次体病" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "Rickettsia" } ] }, { "text": "立克次体病是由各种立克次体感染导致的人兽共患自然疫源性疾病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "立克次体病" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "立克次体感染" }, { "start_idx": 18, "end_idx": 28, "type": "dis", "entity": "人兽共患自然疫源性疾病" } ] }, { "text": "近年来世界范围内新发立克次体病不断出现,如人粒细胞无形体病及人单核细胞埃立克体病等。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "立克次体病" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "人粒细胞无形体病" }, { "start_idx": 30, "end_idx": 39, "type": "dis", "entity": "人单核细胞埃立克体病" } ] }, { "text": "无论传统立克次体病还是新发立克次体病,其临床共同特点主要是不明原因高热(≥38℃)、头痛、肌肉酸痛、皮疹、粒细胞小板减少,一般抗病毒药及抗生素治疗无效,而对多西环素、四环素、氯霉素等敏感。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "传统立克次体病" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "新发立克次体病" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "高热(≥38℃)" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "头痛" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肌肉" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "肌肉酸痛" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "皮疹" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "皮" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "粒细胞小板" }, { "start_idx": 53, "end_idx": 59, "type": "sym", "entity": "粒细胞小板减少" }, { "start_idx": 63, "end_idx": 72, "type": "pro", "entity": "抗病毒药及抗生素治疗" }, { "start_idx": 78, "end_idx": 81, "type": "dru", "entity": "多西环素" }, { "start_idx": 83, "end_idx": 85, "type": "dru", "entity": "四环素" }, { "start_idx": 87, "end_idx": 89, "type": "dru", "entity": "氯霉素" } ] }, { "text": "下面主要介绍传统的立克次体,如斑疹伤寒、恙虫病、Q热;新发立克次体病,如人粒细胞无形体病。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "立克次体" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "斑疹伤寒" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "恙虫病" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "Q热" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "新发立克次体病" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "人粒细胞无形体病" } ] }, { "text": "第一节斑疹伤寒斑疹伤寒(rockymountainspottedfever)是由斑疹伤寒立克次体引起的急性传染病,分为典型的流行性斑疹伤寒及地方性鼠型斑疹伤寒。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "斑疹伤寒" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "斑疹伤寒" }, { "start_idx": 12, "end_idx": 36, "type": "dis", "entity": "rockymountainspottedfever" }, { "start_idx": 40, "end_idx": 47, "type": "mic", "entity": "斑疹伤寒立克次体" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "流行性斑疹伤寒" }, { "start_idx": 70, "end_idx": 78, "type": "dis", "entity": "地方性鼠型斑疹伤寒" } ] }, { "text": "【病原及流行病学】病原体为立克次体。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "立克次体" } ] }, { "text": "流行性斑疹伤寒由普氏立克次体引起,人是基本宿主,传染媒介为人身上的体虱,见于我国北方,冬春季流行。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "流行性斑疹伤寒" }, { "start_idx": 8, "end_idx": 13, "type": "mic", "entity": "普氏立克次体" } ] }, { "text": "地方性斑疹伤寒由莫氏立克次体引起,鼠为基本宿主,传染媒介为人蚤和鼠蚤,见于我国各地,四季散发,夏秋季较多。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "地方性斑疹伤寒" }, { "start_idx": 8, "end_idx": 13, "type": "mic", "entity": "莫氏立克次体" } ] }, { "text": "人对斑疹伤寒普遍易感。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "斑疹伤寒" } ] }, { "text": "体虱和鼠蚤叮咬患者,吸血时将附着于红细胞及血小板上的立克次体吸入胃内,立克次体在胃肠壁上皮细胞中不断繁殖增生。", "entities": [ { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 17, "end_idx": 29, "type": "mic", "entity": "红细胞及血小板上的立克次体" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胃内" }, { "start_idx": 35, "end_idx": 38, "type": "mic", "entity": "立克次体" }, { "start_idx": 40, "end_idx": 47, "type": "bod", "entity": "胃肠壁上皮细胞中" } ] }, { "text": "再叮咬健康人时将大量立克次体随粪便排泄于皮肤上,立克次体经伤口而进入人体。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "mic", "entity": "立克次体" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "粪便" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "皮肤上" }, { "start_idx": 24, "end_idx": 27, "type": "mic", "entity": "立克次体" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "伤口" } ] }, { "text": "也可经由虱、蚤干燥粪便或碾碎感染虱蚤时通过皮肤黏膜进入人体而感染。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "感染" } ] }, { "text": "【发病机制及病理改变】立克次体进入人体后,经血行至全身各器官,主要病变在微血管,在内皮细胞中增殖并有毒素作用,使血管壁发生炎症反应,细胞浸润,形成斑疹伤寒结节,内皮细胞坏死堵塞血管。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "立克次体" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "血" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "全身各器官" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "病变在微血管" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "微血管" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "内皮细胞中" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "血管壁" }, { "start_idx": 56, "end_idx": 64, "type": "sym", "entity": "血管壁发生炎症反应" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "细胞" }, { "start_idx": 66, "end_idx": 69, "type": "sym", "entity": "细胞浸润" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "斑疹伤寒" }, { "start_idx": 77, "end_idx": 78, "type": "dis", "entity": "结节" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "内皮细胞坏死" }, { "start_idx": 80, "end_idx": 89, "type": "sym", "entity": "内皮细胞坏死堵塞血管" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "血管" } ] }, { "text": "神经系统的变化典型,最多见于大脑的底部和皮层以及延髓等处。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "神经系统" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "大脑的底部" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮层" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "延髓" } ] }, { "text": "血管病变涉及心肌、肾脏、睾丸、肾上腺等处。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "血管病变涉及心肌、肾脏、睾丸、肾上腺" }, { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "血管病变" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "心肌" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肾脏" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "睾丸" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾上腺" } ] }, { "text": "可有轻的前驱症,发热畏寒,并多以寒战开始。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "轻的前驱症" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "畏寒" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "寒战" } ] }, { "text": "皮疹是本病的特征,最早在发病后3~5天出现,第5~8天出疹较多。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "皮疹" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "皮" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "出疹较多" } ] }, { "text": "皮疹形态各有不同,包括斑丘疹、丘疹、出血疹、混合疹等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "皮疹" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "皮" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "斑丘疹" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "丘疹" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "出血疹" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "混合疹" } ] }, { "text": "皮疹主要分布于躯干及四肢,包括手掌和脚底,颜面部较少见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "皮疹" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "皮" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "躯干" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "四肢" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "手掌" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脚底" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "颜面部" } ] }, { "text": "多先出现于胸腹部,由斑丘疹逐渐融合成红斑或中央有出血点的紫斑。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "胸腹部" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "斑丘疹" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "红斑" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "中央有出血点的紫斑" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" } ] }, { "text": "退疹后皮肤留有褐色色素沉着,出现皮屑脱落。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "sym", "entity": "疹" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 3, "end_idx": 19, "type": "sym", "entity": "皮肤留有褐色色素沉着,出现皮屑脱落" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "皮屑" } ] }, { "text": "在未出疹前,做束臂试验,可显示出皮疹,对早期诊断有帮助。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "出疹" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "束臂试验" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "皮疹" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "皮" } ] }, { "text": "但轻症病例可无或极少皮疹。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "皮疹" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "皮" } ] }, { "text": "此外可见眼结膜及咽部充血,咽黏膜可见出血点。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "眼结膜" }, { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "眼结膜及咽部充血" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "咽部" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "咽黏膜" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "咽黏膜可见出血点" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" } ] }, { "text": "肝脾多肿大,尤以脾大较为显著。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肝脾多肿大" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肝" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "脾" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "脾" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "脾大较为显著" } ] }, { "text": "重症病例可出现神经系统症状,如头痛、神情呆滞、烦躁不安、谵妄、昏迷、脑膜刺激征、病理反射等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "神经系统" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "头痛" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "头" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "神情呆滞" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "谵妄" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "昏迷" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "脑膜刺激征" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "脑膜" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "病理反射" } ] }, { "text": "脉细速,血压下降,奔马律,心力衰竭。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "脉细速" }, { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "血压" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "血压下降" }, { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "奔马律" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "地方性斑疹伤寒病情一般较轻,较少出现神经系统症状和并发肺炎。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "地方性斑疹伤寒" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "神经系统" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "肺炎" } ] }, { "text": "临床症状以发热、头痛、皮疹为主要表现。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "头痛" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "头" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "皮疹" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "皮" } ] }, { "text": "部分病例合并多脏器损害。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "多脏器损害" } ] }, { "text": "基层诊断主要依靠临床表现结合外斐试验OX19阳性。", "entities": [ { "start_idx": 14, "end_idx": 23, "type": "sym", "entity": "外斐试验OX19阳性" }, { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "外斐试验OX19" } ] }, { "text": "近年因抗菌药物的应用,临床症状趋于不典型,而外斐试验OX19阳性多出现于发病后第5~17天,平均为11~15天。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "抗菌药物" }, { "start_idx": 22, "end_idx": 31, "type": "sym", "entity": "外斐试验OX19阳性" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "外斐试验OX19" } ] }, { "text": "(二)实验室检查血清外斐反应缺乏敏感性和特异性,最好用标准立克次体抗原做补体结合试验等特异性血清试验,双份血清抗体效价4倍升高可确诊。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "实验室检查" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "血清外斐反应" }, { "start_idx": 26, "end_idx": 41, "type": "pro", "entity": "用标准立克次体抗原做补体结合试验" }, { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "特异性血清试验" }, { "start_idx": 51, "end_idx": 62, "type": "sym", "entity": "双份血清抗体效价4倍升高" }, { "start_idx": 51, "end_idx": 58, "type": "ite", "entity": "双份血清抗体效价" } ] }, { "text": "或者从组织中找到立克次体,或应用聚合酶链反应检测立克次体核酸。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "组织中" }, { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "立克次体" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "聚合酶链反应检测" }, { "start_idx": 24, "end_idx": 29, "type": "mic", "entity": "立克次体核酸" } ] }, { "text": "(三)除外其他疾病需与Q热、恙虫病、流行性出血热、洛杉矶斑疹伤寒等鉴别。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "Q热" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "恙虫病" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "流行性出血热" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "洛杉矶斑疹伤寒" } ] }, { "text": "【治疗和预防】(一)治疗氯霉素为治疗斑疹伤寒的特效药,四环素也有效。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "氯霉素" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "斑疹伤寒" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "四环素" } ] }, { "text": "剂量为50mg/(kg•d),分3次口服。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "口服" } ] }, { "text": "同时服用甲氧苄啶(TMP)可提高疗效,剂量为10mg/(kg•d),分2次口服。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "甲氧苄啶" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "TMP" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "口服" } ] }, { "text": "重症可用静脉滴注,热退后减量口服5~7天。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 9, "end_idx": 9, "type": "sym", "entity": "热" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "口服" } ] }, { "text": "此外还应加强对症支持治疗。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "对症支持治疗" } ] }, { "text": "(二)预防注意个人清洁卫生,消灭虱蚤及流行区鼠类。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "注意个人清洁卫生" }, { "start_idx": 14, "end_idx": 23, "type": "pro", "entity": "消灭虱蚤及流行区鼠类" } ] }, { "text": "及早诊断和隔离患者,对可能与病原体短期接触的人可采用药物预防。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "及早诊断和隔离患者" }, { "start_idx": 10, "end_idx": 29, "type": "pro", "entity": "对可能与病原体短期接触的人可采用药物预防" } ] }, { "text": "接种斑疹伤寒疫苗可增强抵抗力,减轻病情,降低病死率,但不能预防感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "接种" }, { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "斑疹伤寒疫苗" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "感染" } ] }, { "text": "第三节常用治疗方案一、喷射雾化方案(一)应用原理通过高压气体冲击液体,产生雾滴,它具有雾滴直径均匀,大小适中(1~5μm),对液体中药物成分无影响等优点。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "喷射雾化" } ] }, { "text": "(二)应用原则原则为:①平喘药物可用拟肾上腺素和抗胆碱能药物合用,拟肾上腺素药物起效快,但维持时间短;抗胆碱能药物起效相对较慢,但维持时间较长,因而两者合用有互补作用。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "平喘药物" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "拟肾上腺素" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 33, "end_idx": 39, "type": "dru", "entity": "拟肾上腺素药物" }, { "start_idx": 51, "end_idx": 56, "type": "dru", "entity": "抗胆碱能药物" } ] }, { "text": "②如要用雾化吸入糖皮质激素,最好先吸入平喘药物,再吸糖皮质激素,以增加糖皮质激素的吸入量。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "平喘药物" }, { "start_idx": 25, "end_idx": 25, "type": "pro", "entity": "吸" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 35, "end_idx": 39, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "③要严格掌握用药剂量(表8-8),用药期间注意心血管方面副作用的产生。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "心血管" } ] }, { "text": "四、高血钾型肾小管酸中毒(Ⅳ型)高血钾型肾小管酸中毒(hyperkalemicrenaltubularacidosis)又称全远端型肾小管酸中毒(generalizeddistalrenaltubularacidosis),是由于醛固酮分泌不足或肾小管对其反应低下所致高血氯性代谢性酸中毒及持续高钾血症。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "高血钾型肾小管酸中毒(Ⅳ型)" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "高血钾型肾小管酸中毒" }, { "start_idx": 27, "end_idx": 58, "type": "dis", "entity": "hyperkalemicrenaltubularacidosis" }, { "start_idx": 62, "end_idx": 71, "type": "dis", "entity": "全远端型肾小管酸中毒" }, { "start_idx": 73, "end_idx": 109, "type": "dis", "entity": "generalizeddistalrenaltubularacidosis" }, { "start_idx": 115, "end_idx": 117, "type": "bod", "entity": "醛固酮" }, { "start_idx": 115, "end_idx": 121, "type": "sym", "entity": "醛固酮分泌不足" }, { "start_idx": 123, "end_idx": 125, "type": "bod", "entity": "肾小管" }, { "start_idx": 123, "end_idx": 131, "type": "sym", "entity": "肾小管对其反应低下" }, { "start_idx": 134, "end_idx": 143, "type": "dis", "entity": "高血氯性代谢性酸中毒" }, { "start_idx": 145, "end_idx": 150, "type": "dis", "entity": "持续高钾血症" } ] }, { "text": "【病因】几乎所有Ⅳ型RTA均继发于其他疾病,罕见有原发性者。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "Ⅳ型RTA" } ] }, { "text": "常见继发性病因有:(一)单纯醛固酮缺乏如失盐性先天性肾上腺增生、醛固酮缺乏症以及艾迪生病。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "单纯醛固酮缺乏" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "醛固酮" }, { "start_idx": 20, "end_idx": 30, "type": "dis", "entity": "失盐性先天性肾上腺增生" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "醛固酮缺乏症" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "艾迪生病" } ] }, { "text": "(二)慢性肾脏疾病伴肾素和醛固酮分泌不足如糖尿病肾病、紫癜性肾炎、镰状细胞肾病、肾硬化及间质性肾炎等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "慢性肾脏疾病" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肾素" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "肾素和醛固酮分泌不足" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "醛固酮" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "糖尿病肾病" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "紫癜性肾炎" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "镰状细胞肾病" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "肾硬化" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "间质性肾炎" } ] }, { "text": "(三)急性肾小球肾炎伴肾素和醛固酮分泌不足。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "急性肾小球肾炎" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾素" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "肾素和醛固酮分泌不足" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "醛固酮" } ] }, { "text": "(四)肾小管对醛固酮反应性降低如婴儿原发性假性醛固酮缺乏及继发性假性醛固酮缺乏(包括婴儿尿路梗阻、婴儿肾静脉血栓形成和氯分流综合征即Gordon综合征)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肾小管" }, { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "肾小管对醛固酮反应性降低" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "醛固酮" }, { "start_idx": 16, "end_idx": 27, "type": "dis", "entity": "婴儿原发性假性醛固酮缺乏" }, { "start_idx": 29, "end_idx": 38, "type": "dis", "entity": "继发性假性醛固酮缺乏" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "婴儿尿路梗阻" }, { "start_idx": 49, "end_idx": 55, "type": "dis", "entity": "婴儿肾静脉血栓" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "氯分流综合征" }, { "start_idx": 66, "end_idx": 74, "type": "dis", "entity": "Gordon综合征" } ] }, { "text": "(五)药物和毒素补充氯化钾过多,使用过量保钾利尿剂、肝素以及前列腺素抑制剂等。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "氯化钾" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "保钾利尿剂" }, { "start_idx": 26, "end_idx": 36, "type": "dru", "entity": "肝素以及前列腺素抑制剂" } ] }, { "text": "【发病机制】醛固酮是调节Na+-K+和Na+-H+交换的主要内分泌激素。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "醛固酮" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "内分泌激素" } ] }, { "text": "当醛固酮不足或肾小管对醛固酮反应性降低时,Na+-K+和Na+-H+交换减少,肾小管对Na+重吸收减少丢失增多,泌H+及排K+障碍,因而出现高血钾型酸中毒。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "醛固酮" }, { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "醛固酮不足" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾小管" }, { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "肾小管对醛固酮反应性降低" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "醛固酮" }, { "start_idx": 21, "end_idx": 37, "type": "sym", "entity": "Na+-K+和Na+-H+交换减少" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肾小管" }, { "start_idx": 39, "end_idx": 50, "type": "sym", "entity": "肾小管对Na+重吸收减少" }, { "start_idx": 56, "end_idx": 86, "type": "sym", "entity": "泌H+及排K+障碍" }, { "start_idx": 92, "end_idx": 98, "type": "dis", "entity": "高血钾型酸中毒" } ] }, { "text": "(二)常有一定程度的肾小球功能受损但本病往往在慢性肾功能不全出现前即有高血氯性代谢性酸中毒及高钾血症。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肾小球功能受损" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "慢性肾功能不全" }, { "start_idx": 35, "end_idx": 44, "type": "dis", "entity": "高血氯性代谢性酸中毒" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "高钾血症" } ] }, { "text": "肾小球滤过率降低但通常GFR>20min/(min•1.73m2),而且GFR下降难以解释酸中毒。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肾小球" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "肾小球滤过率降低" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "GFR" }, { "start_idx": 11, "end_idx": 38, "type": "sym", "entity": "GFR>20min/(min•1.73m2)" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "GFR" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "GFR下降" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "酸中毒" } ] }, { "text": "(三)无糖尿、氨基酸尿及高磷尿症等近端肾小管的功能障碍。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "糖尿" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "糖" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "尿" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "氨基酸尿" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "氨基酸" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "尿" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "高磷尿症" }, { "start_idx": 17, "end_idx": 26, "type": "dis", "entity": "近端肾小管的功能障碍" } ] }, { "text": "(四)血尿生化改变与pRTA类似尿排出量增加,尿氨生成减少。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "血尿生化改变" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "pRTA" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "尿排出量增加" }, { "start_idx": 16, "end_idx": 19, "type": "ite", "entity": "尿排出量" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "尿氨生成减少" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "尿氨" } ] }, { "text": "酸中毒时,尿可呈酸性,但尿氨仍然减少。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "酸中毒" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "尿可呈酸性" }, { "start_idx": 5, "end_idx": 5, "type": "ite", "entity": "尿" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "尿氨仍然减少" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "尿氨" } ] }, { "text": "(五)本病通常不出现肾钙化与肾结石,骨损害仅见于尿毒症患者。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "肾钙化" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肾" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肾结石" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "骨损害" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "尿毒症" } ] }, { "text": "【诊断和鉴别诊断】诊断本病时应注意与高血钾远端肾小管性酸中毒鉴别。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "高血钾" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "远端肾小管性酸中毒" } ] }, { "text": "两者均表现为高血钾和酸中毒,但本病在酸血症时,尿pH<5.5,而且如果肾小球滤过率无明显下降(>40ml/min),在碱化尿液后,尿与血CO2分压之差>2.66kPa(20mmHg)当尿丢失增多而血浓度正常时,易与pRTA混淆,鉴别要点在于本病在酸血症时,排出减少,可用硫酸钠滴注试验鉴别。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "高血钾" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "酸中毒" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "酸血症" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "尿pH" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "尿pH<5.5" }, { "start_idx": 35, "end_idx": 40, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "碱化尿液" }, { "start_idx": 65, "end_idx": 83, "type": "ite", "entity": "尿与血CO2分压" }, { "start_idx": 65, "end_idx": 101, "type": "sym", "entity": "尿与血CO2分压之差>2.66kPa(20mmHg)" }, { "start_idx": 103, "end_idx": 107, "type": "sym", "entity": "尿丢失增多" }, { "start_idx": 103, "end_idx": 103, "type": "bod", "entity": "尿" }, { "start_idx": 109, "end_idx": 111, "type": "ite", "entity": "血浓度" }, { "start_idx": 118, "end_idx": 121, "type": "dis", "entity": "pRTA" }, { "start_idx": 134, "end_idx": 136, "type": "dis", "entity": "酸血症" }, { "start_idx": 146, "end_idx": 152, "type": "pro", "entity": "硫酸钠滴注试验" } ] }, { "text": "【治疗】(一)降低血钾1.限制钾摄入<30mmol/d,避免用含钾药物。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "降低血钾" }, { "start_idx": 13, "end_idx": 26, "type": "pro", "entity": "限制钾摄入<30mmol/d" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "避免用含钾药物" } ] }, { "text": "2.排钾利尿剂DHCT2mg/(kg•d)或呋塞米每次2mg/kg,每日1~2次。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "排钾利尿剂" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "DHCT" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "呋塞米" } ] }, { "text": "(二)补碱碳酸氢钠1.5~2mmol/(kg•d),既可纠酸中毒又能降低血钾浓度。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "补碱" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "酸中毒" }, { "start_idx": 36, "end_idx": 39, "type": "ite", "entity": "血钾浓度" } ] }, { "text": "(三)盐皮质激素治疗氟氢可的松(fludrocortisone)0.01mg/(kg•d),可纠正酸中毒并降低血钾。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "盐皮质激素治疗" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "氟氢可的松" }, { "start_idx": 16, "end_idx": 30, "type": "dru", "entity": "fludrocortisone" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "酸中毒" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "血钾" } ] }, { "text": "第三节钩虫病钩虫病(hookwormdiseases)的病原体是钩虫,钩虫共有3种,即十二指肠钩虫、美洲钩虫和锡兰钩虫,其中以前两者引起人类感染最为重要。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "钩虫病" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "钩虫病" }, { "start_idx": 10, "end_idx": 25, "type": "dis", "entity": "hookwormdiseases" }, { "start_idx": 32, "end_idx": 33, "type": "mic", "entity": "钩虫" }, { "start_idx": 35, "end_idx": 36, "type": "mic", "entity": "钩虫" }, { "start_idx": 43, "end_idx": 48, "type": "mic", "entity": "十二指肠钩虫" }, { "start_idx": 50, "end_idx": 53, "type": "mic", "entity": "美洲钩虫" }, { "start_idx": 55, "end_idx": 58, "type": "mic", "entity": "锡兰钩虫" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "感染" } ] }, { "text": "我国钩虫平均感染率为6.12%,多数为美洲钩虫和十二指肠钩虫混合感染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "钩虫" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "感染" }, { "start_idx": 19, "end_idx": 33, "type": "dis", "entity": "美洲钩虫和十二指肠钩虫混合感染" } ] }, { "text": "钩虫幼虫生活在温暖、潮湿的泥土中,穿透皮肤使人感染。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "钩虫幼虫" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "皮肤" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "感染" } ] }, { "text": "喝饮含幼虫的生水或有食土癖者可通过消化道感染。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "幼虫" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "消化道" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "感染" } ] }, { "text": "幼虫从皮下组织或肠壁移行进入血液循环随血流到达肺部穿过血管壁进入肺泡腔,沿小支气管壁向上游走,在咽部被吞咽而进入消化道,最后定位于小肠上段,2~4周即发育为成虫。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "幼虫" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "皮下组织" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肠壁" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "血液循环" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血流" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "血管壁" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺泡腔" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "小支气管壁" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "咽部" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "消化道" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "小肠上段" }, { "start_idx": 78, "end_idx": 79, "type": "mic", "entity": "成虫" } ] }, { "text": "钩虫成虫是半透明状,呈米黄色或淡红色(吸血后)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "钩虫成虫" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "血" } ] }, { "text": "幼虫可通过胎盘进入胎儿体内发育为成虫,称为先天感染,但比较少见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "幼虫" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胎盘" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "胎儿体内" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "成虫" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "先天感染" } ] }, { "text": "【病理改变】主要为十二指肠及空肠黏膜广泛出血及溃疡。", "entities": [ { "start_idx": 9, "end_idx": 24, "type": "sym", "entity": "十二指肠及空肠黏膜广泛出血及溃疡" }, { "start_idx": 9, "end_idx": 17, "type": "bod", "entity": "十二指肠及空肠黏膜" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" } ] }, { "text": "体内每条十二指肠钩虫每天导致的失血量约为0.14~0.4ml,十二指肠钩虫引起的失血量大于美洲钩虫。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "mic", "entity": "十二指肠钩虫" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "失血" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "血" }, { "start_idx": 31, "end_idx": 36, "type": "mic", "entity": "十二指肠钩虫" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "失血" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "血" }, { "start_idx": 45, "end_idx": 48, "type": "mic", "entity": "美洲钩虫" } ] }, { "text": "严重感染时可引起大量失血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "严重感染" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "大量失血" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" } ] }, { "text": "长期失血后体内的铁储备量可逐渐耗尽而形成严重的小细胞低色素性贫血及营养不良。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "失血" }, { "start_idx": 5, "end_idx": 16, "type": "sym", "entity": "体内的铁储备量可逐渐耗尽" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "体内的铁" }, { "start_idx": 20, "end_idx": 31, "type": "dis", "entity": "严重的小细胞低色素性贫血" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "营养不良" } ] }, { "text": "严重贫血可使心、肝、肾细胞的脂肪变性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "严重贫血" }, { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "心、肝、肾细胞的脂肪变性" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "心、肝、肾细胞" } ] }, { "text": "【临床表现】轻重不一,一般无明显全身症状,以贫血为主。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "贫血" } ] }, { "text": "(一)钩蚴所致的症状当钩蚴侵入皮肤时,局部有匍行丘疹或小疱疹,有痒感。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "钩蚴" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "钩蚴" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "局部有匍行丘疹或小疱疹" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "痒感" } ] }, { "text": "由于抓痒可引起继发感染,局部淋巴结亦可肿大。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "继发感染" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "局部淋巴结亦可肿大" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "局部淋巴结" } ] }, { "text": "当钩蚴侵入血液循环在体内移行时,可引起蠕动移行病及嗜酸性粒细胞增多症。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "钩蚴" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "血液循环" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "蠕动移行病" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "嗜酸性粒细胞增多症" } ] }, { "text": "(二)成虫引起的症状主要为失血性贫血,食欲缺乏及消化不良、腹泻而致营养缺乏。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "成虫" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "失血性贫血" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "消化不良" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "腹泻" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "腹" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "营养缺乏" } ] }, { "text": "营养及发育差,精神委靡,表情淡漠,不愿活动。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "营养及发育差" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "精神委靡" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "表情淡漠" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "不愿活动" } ] }, { "text": "有时烦躁不安、眩晕、心悸、气短等贫血的症状。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "眩晕" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "心悸" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "心" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "气短" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "贫血" } ] }, { "text": "【诊断】主要依靠从粪便中检出虫卵以确定诊断。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "粪便" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "虫卵" } ] }, { "text": "【治疗】应及早采取综合治疗措施,以免发生严重贫血及营养不良。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "严重贫血" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "营养不良" } ] }, { "text": "(一)一般疗法必须注意改善患儿的营养状况,给高蛋白质、多维生素饮食。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "pro", "entity": "注意改善患儿的营养状况" }, { "start_idx": 21, "end_idx": 32, "type": "pro", "entity": "给高蛋白质、多维生素饮食" } ] }, { "text": "(二)驱虫疗法驱钩药物的选择,应根据钩虫虫种及其他肠道寄生虫的合并感染情况选择高效、低毒的药物。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "驱虫疗法" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "驱钩药物" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "钩虫" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "肠道寄生虫" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "感染" } ] }, { "text": "为了提高疗效,尤其是驱美洲钩虫的疗效,可采用交替用药和联合用药。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "驱美洲钩虫" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "交替用药" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "联合用药" } ] }, { "text": "1.甲苯咪唑每次100mg,每天2次,连服3天,或单服1剂300mg。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "甲苯咪唑" } ] }, { "text": "2.丙硫咪唑成人400mg顿服,10天后再重复给药1次即可。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "丙硫咪唑" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "顿服" } ] }, { "text": "对十二指肠钩虫的疗效优于美洲钩虫。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "mic", "entity": "十二指肠钩虫" }, { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "美洲钩虫" } ] }, { "text": "一般虫卵阴转率为94%~98%。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "虫卵" } ] }, { "text": "3.左旋咪唑对十二指肠钩虫疗效较好。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "左旋咪唑" }, { "start_idx": 7, "end_idx": 12, "type": "mic", "entity": "十二指肠钩虫" } ] }, { "text": "虫卵阴转率为80%~96%,美洲钩虫为5%~25%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "虫卵" }, { "start_idx": 14, "end_idx": 17, "type": "mic", "entity": "美洲钩虫" } ] }, { "text": "也可用于皮肤涂搽,治疗经皮肤感染的美洲钩虫。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "皮肤涂搽" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "皮肤" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "感染" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "美洲钩虫" } ] }, { "text": "4.噻嘧啶基质10mg/kg顿服,可连服3天,虫卵阴转率达95%以上;美洲钩虫为62.5%~80.0%。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "噻嘧啶基质" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "顿服" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "虫卵" }, { "start_idx": 35, "end_idx": 38, "type": "mic", "entity": "美洲钩虫" } ] }, { "text": "5.伊维菌素0.2mg/kg顿服,可使虫卵减少率为70.6%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "伊维菌素" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "顿服" }, { "start_idx": 19, "end_idx": 20, "type": "mic", "entity": "虫卵" } ] }, { "text": "6.联合用药甲苯咪唑加噻嘧啶,或噻嘧啶加左旋咪唑联合治疗,可提高疗效。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "联合用药" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "甲苯咪唑" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "噻嘧啶" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "噻嘧啶" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "左旋咪唑" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "联合治疗" } ] }, { "text": "(三)对局部钩蚴性皮炎的治疗1.皮肤透热法把手足发痒的部位浸泡在50℃以上热水中,约30分钟左右,或用毛巾放在50~60℃热水中,取出后呈半干状态,使之紧贴在皮炎部位,每半分钟换一次,连续10分钟。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "局部钩蚴性皮炎" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "皮肤透热法" }, { "start_idx": 21, "end_idx": 47, "type": "pro", "entity": "把手足发痒的部位浸泡在50℃以上热水中,约30分钟左右" }, { "start_idx": 50, "end_idx": 97, "type": "pro", "entity": "用毛巾放在50~60℃热水中,取出后呈半干状态,使之紧贴在皮炎部位,每半分钟换一次,连续10分钟" } ] }, { "text": "2.局部涂2%~4%碘液、15%噻苯唑油膏、或5%硫磺炉甘石洗剂。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "局部涂" }, { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "碘液" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "噻苯唑油膏" }, { "start_idx": 25, "end_idx": 31, "type": "dru", "entity": "硫磺炉甘石洗剂" } ] }, { "text": "【预防】应对流行地区患者进行治疗,防止土地被含有虫卵的粪便污染,改善环境卫生,指导合理的积肥。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "虫卵" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "改善环境卫生" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "指导合理的积肥" } ] }, { "text": "结合生产,加强粪肥卫生管理和无害化措施。", "entities": [ { "start_idx": 5, "end_idx": 18, "type": "pro", "entity": "加强粪肥卫生管理和无害化措施" } ] }, { "text": "广泛展开卫生宣教,尽可能防止钩蚴侵入人体。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "广泛展开卫生宣教" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "钩蚴" } ] }, { "text": "四、病毒病毒性疾病的病原学诊断,对疾病的预防、控制、预后判断、治疗以及研究等方面均具有极为重要的意义,并可对病毒疫苗接种是否成功或有效进行准确监测。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "病毒" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "病毒性疾病" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "病毒疫苗" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "接种" } ] }, { "text": "例如单纯疱疹病毒脑炎用阿糖腺苷或阿昔洛韦(无环鸟苷)治疗,使病死率由80%以上降低到10%以下;严重巨细胞病毒感染以更昔洛韦(丙氧鸟苷)治疗有效率达到70%~80%;呼吸道合胞病毒肺炎以利巴韦林(病毒唑)雾化吸入治疗以及艾滋病用齐多夫定(zidovudine,AZT)治疗均取得一定的疗效。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "单纯疱疹病毒脑炎" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "阿糖腺苷" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "阿昔洛韦" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "无环鸟苷" }, { "start_idx": 48, "end_idx": 56, "type": "dis", "entity": "严重巨细胞病毒感染" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "更昔洛韦" }, { "start_idx": 63, "end_idx": 66, "type": "dru", "entity": "丙氧鸟苷" }, { "start_idx": 83, "end_idx": 91, "type": "dis", "entity": "呼吸道合胞病毒肺炎" }, { "start_idx": 93, "end_idx": 96, "type": "dru", "entity": "利巴韦林" }, { "start_idx": 98, "end_idx": 100, "type": "dru", "entity": "病毒唑" }, { "start_idx": 102, "end_idx": 105, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 110, "end_idx": 112, "type": "dis", "entity": "艾滋病" }, { "start_idx": 114, "end_idx": 117, "type": "dru", "entity": "齐多夫定" }, { "start_idx": 119, "end_idx": 128, "type": "dru", "entity": "zidovudine" }, { "start_idx": 130, "end_idx": 132, "type": "dru", "entity": "AZT" } ] }, { "text": "(一)采集和转运对于病毒学和病毒血清学检测标本采集和运送的基本要求是及时、准确、防污染、防干燥。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "病毒" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血清" } ] }, { "text": "标本的暂存和运送条件应因病毒而异,如肠道病毒可在-70℃下冻存较长时间,较远距离运送时可用干冰保持低温;而巨细胞病毒的标本则不应冷冻,应在4℃下暂存或运送。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 53, "end_idx": 57, "type": "mic", "entity": "巨细胞病毒" } ] }, { "text": "(二)实验室诊断方法1.病毒分离病毒分离技术至今被认为是诊断病毒性疾病的“黄金标准”。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "实验室诊断方法" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "病毒分离" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "病毒分离技术" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "病毒性疾病" } ] }, { "text": "这是因为通过该技术证实被检材料中存在活的、能够在体外复制的有传染性的病毒,其临床意义很大。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "病毒" } ] }, { "text": "将适当的临床标本接种于相应的生长良好的培养细胞上,经过一定时间(数日至数周),受感染的细胞出现具有一定特征的改变,可初步判断有无病毒生长以及大致是哪一类病毒。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "培养细胞" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "感染" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "细胞" }, { "start_idx": 64, "end_idx": 65, "type": "mic", "entity": "病毒" }, { "start_idx": 76, "end_idx": 77, "type": "mic", "entity": "病毒" } ] }, { "text": "但必须进行鉴定,即用特异性抗血清作中和试验或其他试验来确定该病毒属于何种病毒及哪个血清型或组。", "entities": [ { "start_idx": 9, "end_idx": 20, "type": "pro", "entity": "用特异性抗血清作中和试验" }, { "start_idx": 30, "end_idx": 31, "type": "mic", "entity": "病毒" }, { "start_idx": 36, "end_idx": 37, "type": "mic", "entity": "病毒" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "血清" } ] }, { "text": "病毒分离技术用于临床诊断的优点包括:特异性强;不易受到标本中一些因素,如呼吸道分泌物中的黏液和抗体等的干扰;如检测的各个环节掌握得好,其敏感性也较高;另外,病毒分离法能够检出病毒的种类相对多。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "病毒分离技术" }, { "start_idx": 36, "end_idx": 48, "type": "bod", "entity": "呼吸道分泌物中的黏液和抗体" }, { "start_idx": 78, "end_idx": 82, "type": "pro", "entity": "病毒分离法" }, { "start_idx": 87, "end_idx": 88, "type": "mic", "entity": "病毒" } ] }, { "text": "传统的病毒分离法的缺点有:检测需要的时间较长,在很多情况下只能做出回顾性诊断;耗费人力、物力较多,细胞培养技术上的要求比较严格;有不少病毒至今无法培养分离或培养分离十分困难,例如乙肝、丙肝病毒、某些型的肠道病毒、小DNA病毒等。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "传统的病毒分离法" }, { "start_idx": 49, "end_idx": 54, "type": "pro", "entity": "细胞培养技术" }, { "start_idx": 67, "end_idx": 68, "type": "mic", "entity": "病毒" }, { "start_idx": 73, "end_idx": 76, "type": "pro", "entity": "培养分离" }, { "start_idx": 78, "end_idx": 81, "type": "pro", "entity": "培养分离" }, { "start_idx": 89, "end_idx": 95, "type": "mic", "entity": "乙肝、丙肝病毒" }, { "start_idx": 101, "end_idx": 104, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 106, "end_idx": 111, "type": "mic", "entity": "小DNA病毒" } ] }, { "text": "近年来对病毒分离和鉴定技术进行了一些改进,从而大大缩短了检测时间。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "病毒分离和鉴定技术" } ] }, { "text": "例如对巨细胞病毒和流感病毒,将标本接种于细胞培养之后,经24~48小时的培养后将细胞用固定剂固定,再用荧光素或酶标记的病毒特异性单克隆抗体检测细胞内病毒抗原(对巨细胞病毒是检测其早期即刻抗原)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "流感病毒" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "细胞培养" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "培养" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "细胞" }, { "start_idx": 55, "end_idx": 55, "type": "dru", "entity": "酶" }, { "start_idx": 59, "end_idx": 70, "type": "pro", "entity": "病毒特异性单克隆抗体检测" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "细胞内" }, { "start_idx": 74, "end_idx": 77, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 80, "end_idx": 84, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 89, "end_idx": 94, "type": "mic", "entity": "早期即刻抗原" } ] }, { "text": "从病变部位,如体腔积液、活检组织、病损部位以及脑脊液、血液等标本中分离到病毒对其诊断意义很大,一般可以作出诊断。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "病变部位" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "体腔积液" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "活检组织" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "病损部位" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "脑脊液" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血液" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "分离" }, { "start_idx": 36, "end_idx": 37, "type": "mic", "entity": "病毒" } ] }, { "text": "但从鼻腔分泌物、粪便、尿中分离到某种病毒时,应结合临床审慎考虑分离出的病毒能否解释患者的临床疾病,这种情况下联合应用病毒分离与抗体检测方法对诊断的帮助更大。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "鼻腔分泌物" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "粪便" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "尿" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "分离" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "病毒" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "分离" }, { "start_idx": 35, "end_idx": 36, "type": "mic", "entity": "病毒" }, { "start_idx": 54, "end_idx": 68, "type": "pro", "entity": "联合应用病毒分离与抗体检测方法" } ] }, { "text": "对病毒分离来说,采样的部位和标本的种类十分重要。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "病毒分离" } ] }, { "text": "2.病毒的直接检查病毒的直接检查主要用电子显微镜来完成。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "病毒的直接检查" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "病毒的直接检查" }, { "start_idx": 19, "end_idx": 23, "type": "equ", "entity": "电子显微镜" } ] }, { "text": "一些病毒,如轮状病毒、甲肝病毒等的感染,可通过对标本的简单处理后进行负染,然后在电子显微镜下检查,根据病毒的形态特点来作出诊断。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "病毒" }, { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "甲肝病毒" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "感染" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "负染" }, { "start_idx": 40, "end_idx": 44, "type": "equ", "entity": "电子显微镜" }, { "start_idx": 51, "end_idx": 52, "type": "mic", "entity": "病毒" } ] }, { "text": "这一方法的优点是,相对简单、省时,可检出难于或无法培养分离的病毒。", "entities": [ { "start_idx": 18, "end_idx": 31, "type": "pro", "entity": "检出难于或无法培养分离的病毒" } ] }, { "text": "其缺点是必须有昂贵的电镜设备、经专门训练过的检测人员和要求标本中有较大量的病毒等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "equ", "entity": "电镜" }, { "start_idx": 37, "end_idx": 38, "type": "mic", "entity": "病毒" } ] }, { "text": "因为直接电镜检查只能根据病毒的形态来判断,无法作出血清型或分组的诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "直接电镜检查" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血清" } ] }, { "text": "因此这种方法不宜作为病毒性疾病的常规诊断方法。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "病毒性疾病" } ] }, { "text": "3.病毒抗原的检测病毒感染后一定时间内宿主的体液、分泌物、组织、细胞中存在病毒的抗原成分(可溶性抗原),可在感染的早期甚至潜伏期内就已存在并持续一定时间,直至完整病毒在体内消失后的一段时间。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "病毒抗原的检测" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "病毒感染" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "体液" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "分泌物" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "组织" }, { "start_idx": 37, "end_idx": 43, "type": "mic", "entity": "病毒的抗原成分" }, { "start_idx": 45, "end_idx": 49, "type": "mic", "entity": "可溶性抗原" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "感染" }, { "start_idx": 81, "end_idx": 82, "type": "mic", "entity": "病毒" } ] }, { "text": "通过检测这些抗原可以做到病毒感染的早期、快速诊断。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "抗原" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "病毒感染" } ] }, { "text": "对于培养分离比较困难或无法分离的病毒,抗原检测尤有重要的实用意义。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "培养分离" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "分离" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "病毒" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "抗原检测" } ] }, { "text": "病毒抗原检测分为以下两类:①检测组织、细胞内病毒抗原的方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 14, "end_idx": 25, "type": "pro", "entity": "检测组织、细胞内病毒抗原" } ] }, { "text": "一般用所谓免疫组织化学法,包括免疫荧光(IF)、免疫酶染(EIA)以及免疫电镜等技术检测。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "免疫组织化学法" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "IF" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "免疫酶染" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "EIA" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "免疫电镜" } ] }, { "text": "但有时其结果受取材的影响较大,如对下呼吸道感染用这类方法检测时,假如标本中柱状上皮细胞太少时无法作出诊断。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "下呼吸道感染" }, { "start_idx": 37, "end_idx": 42, "type": "ite", "entity": "柱状上皮细胞" } ] }, { "text": "②检测病毒可溶性抗原的方法。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "pro", "entity": "检测病毒可溶性抗原" } ] }, { "text": "较早期使用的技术有免疫扩散法、对流免疫电泳等。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "免疫扩散法" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "对流免疫电泳" } ] }, { "text": "敏感性较高的方法有血球凝集、乳胶凝集、酶联免疫吸附法(ELISA)、放射免疫法(RIA)等方法。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "血球凝集" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "乳胶凝集" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "酶联免疫吸附法" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "ELISA" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "放射免疫法" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "RIA" } ] }, { "text": "如果抗原检测结果为阴性病毒感染,须结合采样时机、体液种类及所用方法的敏感性等因素综合考虑。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "抗原检测" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "病毒感染" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "体液" } ] }, { "text": "此外抗原检测对某些病毒感染不能说明是急性感染、慢性感染或病毒携带状态,也就是说在一些情况下病毒抗原的存在不能说明当时的临床疾病是否确实是该病毒所致。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "抗原检测" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "病毒感染" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "急性感染" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "慢性感染" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "病毒" }, { "start_idx": 45, "end_idx": 48, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 69, "end_idx": 70, "type": "mic", "entity": "病毒" } ] }, { "text": "在这类情况下应密切结合临床资料以及其他有关的实验室检查,特别是抗体检测考虑诊断。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "验室检查" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "抗体检测" } ] }, { "text": "但对其他一些病毒如呼吸道合胞病毒、腺病毒、副流感病毒、流感病毒和轮状病毒等可以做出急性感染的诊断,因为这些病毒很少引起慢性感染或携带状态。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 15, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "腺病毒" }, { "start_idx": 21, "end_idx": 25, "type": "mic", "entity": "副流感病毒" }, { "start_idx": 27, "end_idx": 30, "type": "mic", "entity": "流感病毒" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "急性感染" }, { "start_idx": 53, "end_idx": 54, "type": "mic", "entity": "病毒" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "慢性感染" } ] }, { "text": "因此检测同一种病毒的不同抗原有着不同的意义。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "pro", "entity": "检测同一种病毒的不同抗原" } ] }, { "text": "如检测乙型肝炎病毒、EB病毒和艾滋病病毒的不同抗原各有一定的不同意义。", "entities": [ { "start_idx": 1, "end_idx": 24, "type": "pro", "entity": "检测乙型肝炎病毒、EB病毒和艾滋病病毒的不同抗原" } ] }, { "text": "病毒抗原检测的优点:相对简便,可做到早期、快速诊断,在很大程度上反映传染性,一般说来代价不高,以及可以检测无法培养分离的病毒。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 51, "end_idx": 61, "type": "pro", "entity": "检测无法培养分离的病毒" } ] }, { "text": "4.病毒核酸的检测不同的病毒或同一种病毒的不同型毒株的基因组都有其独特性,有别于其他生物、病毒或其他型的核苷酸序列。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "病毒核酸的检测" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "病毒" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "基因组" }, { "start_idx": 45, "end_idx": 46, "type": "mic", "entity": "病毒" }, { "start_idx": 52, "end_idx": 54, "type": "mic", "entity": "核苷酸" } ] }, { "text": "用于检测病毒核酸的技术主要是核酸探针杂交法和聚合酶链反应(PCR)技术。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "检测病毒核酸" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "核酸探针杂交法" }, { "start_idx": 22, "end_idx": 34, "type": "pro", "entity": "聚合酶链反应(PCR)技术" } ] }, { "text": "(1)核酸探针杂交技术:最常用的是斑点杂交法,具有特异性强,如阳性则反映传染性,一般不需要特殊仪器设备,用生物素或异羟基洋地黄毒苷原标记的探针杂交法需时较短,不需要用放射性同位素等特点,是比较实用的方法。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "核酸探针杂交技术" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "斑点杂交法" }, { "start_idx": 57, "end_idx": 65, "type": "dru", "entity": "异羟基洋地黄毒苷原" }, { "start_idx": 69, "end_idx": 73, "type": "pro", "entity": "探针杂交法" } ] }, { "text": "但目前这类方法应用不够普遍,可能因为有关试剂供应不足以及其敏感度不够高,要求标本中有比较大量的病毒核酸等有关。", "entities": [ { "start_idx": 47, "end_idx": 50, "type": "mic", "entity": "病毒核酸" } ] }, { "text": "(2)聚合酶链反应(PCR)技术:这是20世纪80年代中后期出现的分子生物学新技术。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "pro", "entity": "聚合酶链反应(PCR)技术" } ] }, { "text": "可以用于检测与宿主细胞基因组整合存在的病毒核酸。", "entities": [ { "start_idx": 4, "end_idx": 22, "type": "pro", "entity": "检测与宿主细胞基因组整合存在的病毒核酸" } ] }, { "text": "普通PCR属于定性检测,近几年已有人开发出定量PCR方法,但在技术上的难度较大。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "普通PCR" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "定量PCR" } ] }, { "text": "如果实验环境或用具被同样病毒、病毒核酸,特别是被以往扩增的产物污染时,容易造成假阳性结果。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "病毒核酸" } ] }, { "text": "如果采集标本的部位和种类合适,检出某种病毒的核酸即可诊断该病毒感染,并且可以认为有一定的传染性。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "病毒的核酸" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "病毒感染" } ] }, { "text": "但PCR检测结果不能说明患者所患的是急性还是慢性感染或是病毒携带状态。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "PCR检测" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "慢性感染" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "病毒携带状态" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "病毒" } ] }, { "text": "对某些病毒来讲,阳性结果还不能很好地解释临床疾病,甚至可能与临床疾病无关。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "病毒" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "阳性结果" } ] }, { "text": "其原因可能是因为PCR方法的敏感性极高,能够检出极微量的病毒核酸,而体内存在如此微量的病毒有时是不足以引起明显的疾病的。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "PCR方法" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "病毒核酸" }, { "start_idx": 43, "end_idx": 44, "type": "mic", "entity": "病毒" } ] }, { "text": "5.病毒特异性抗体的检测无论哪一类抗体,无论其所针对的是病毒的哪一类抗原,只要发现其滴度在进行性增高,一般可以诊断为该病毒的急性感染。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "病毒特异性抗体的检测" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "抗体" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "抗原" }, { "start_idx": 42, "end_idx": 49, "type": "sym", "entity": "滴度在进行性增高" }, { "start_idx": 59, "end_idx": 60, "type": "mic", "entity": "病毒" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "急性感染" } ] }, { "text": "标本一般用双份血清标本,但对中枢神经系统感染,脑脊液内抗体的检测可能更有诊断意义。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血清" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "中枢神经系统感染" }, { "start_idx": 23, "end_idx": 31, "type": "pro", "entity": "脑脊液内抗体的检测" } ] }, { "text": "在急性期的早期取一份血,在恢复期再取一次,两次采样之间间隔2~4周时间。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "pro", "entity": "急性期的早期取一份血" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "在恢复期再取一次" } ] }, { "text": "如恢复期抗体滴度达到急性期滴度的4倍或更高,可以诊断为急性感染,最大缺点是不能做早期诊断,而只能做回顾性诊断。", "entities": [ { "start_idx": 1, "end_idx": 20, "type": "sym", "entity": "恢复期抗体滴度达到急性期滴度的4倍或更高" }, { "start_idx": 4, "end_idx": 7, "type": "ite", "entity": "抗体滴度" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "滴度" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "急性感染" } ] }, { "text": "单份血清特异性IgG或总抗体的滴度比一般人群或同年龄组人群高出很多倍急性感染。", "entities": [ { "start_idx": 0, "end_idx": 33, "type": "sym", "entity": "单份血清特异性IgG或总抗体的滴度比一般人群或同年龄组人群高出很多倍" }, { "start_idx": 2, "end_idx": 16, "type": "ite", "entity": "血清特异性IgG或总抗体的滴度" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "急性感染" } ] }, { "text": "大多数病毒的急性原发性感染的早期体内便出现病毒特异性IgM抗体,如能检出某病毒的特异性IgM抗体,可以作出该病毒急性或近期感染的诊断。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "病毒" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "急性原发性感染" }, { "start_idx": 16, "end_idx": 30, "type": "sym", "entity": "体内便出现病毒特异性IgM抗体" }, { "start_idx": 21, "end_idx": 30, "type": "mic", "entity": "病毒特异性IgM抗体" }, { "start_idx": 33, "end_idx": 47, "type": "sym", "entity": "能检出某病毒的特异性IgM抗体" }, { "start_idx": 36, "end_idx": 47, "type": "mic", "entity": "某病毒的特异性IgM抗体" }, { "start_idx": 54, "end_idx": 62, "type": "dis", "entity": "病毒急性或近期感染" } ] }, { "text": "应用较多的是IF、EIA、ELISA和RIA等所谓快速诊断方法,检测特异性IgM抗体时容易受到类风湿因子(RF)的干扰而呈现假阳性结果。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "IF" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "EIA" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "ELISA" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "RIA" }, { "start_idx": 32, "end_idx": 41, "type": "pro", "entity": "检测特异性IgM抗体" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "类风湿因子" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "RF" } ] }, { "text": "如果检测病毒特异性IgM抗体的结果为阳性并且排除了类风湿因子的干扰,可以作出相应病毒急性或近期感染的诊断;如果结果为阴性,根据所用方法考虑是否有特异性IgG抗体或IgA抗体的影响。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "ite", "entity": "病毒特异性IgM抗体" }, { "start_idx": 4, "end_idx": 19, "type": "sym", "entity": "病毒特异性IgM抗体的结果为阳性" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "类风湿因子" }, { "start_idx": 38, "end_idx": 48, "type": "dis", "entity": "相应病毒急性或近期感染" }, { "start_idx": 72, "end_idx": 79, "type": "mic", "entity": "特异性IgG抗体" }, { "start_idx": 81, "end_idx": 85, "type": "mic", "entity": "IgA抗体" } ] }, { "text": "参考文献1.崔可忻.7岁以下儿童体格发育与营养评价.北京:人民卫生出版社,19922.刘湘云,林传家,薛沁冰.儿童保健学.第2版.南京:江苏科学技术出版社,20003.魏书珍,张秋业.儿童生长发育性疾病.北京:人民卫生出版社,19964.吴瑞萍,胡亚美,江载方,等.实用儿科学.第6版.北京:人民卫生出版社,1995:13-135.LevinMD,CareyWB,CrockerAC.Developmentalbehavioralpediatrics.3rded.Philadelphia:Saunders,19996.NeedlmanRD.GrowthandDevelopment,AssessmentofGrowthandDevelopmentalAssessment//JensonHB,BehrmanRE,KliegmanRM.NelsontextbookofPediatrics.16thed.Philadelphia:Saunders,20017.XuX,WangWP,GuoZP,etal.Secularchangeingrowthoverondecade(1980~1990)inShanghaiinfants.JournalofPediatricendocrinologyandmetabolism,2000,9:1603", "entities": [ { "start_idx": 92, "end_idx": 100, "type": "dis", "entity": "儿童生长发育性疾病" }, { "start_idx": 135, "end_idx": 136, "type": "dep", "entity": "儿科" } ] }, { "text": "第五节出血性疾病的诊断与鉴别诊断一、概述皮肤、黏膜自发性出血或轻微损伤后出血不易自行停止者称为出血倾向,由于创伤、溃疡、肿瘤坏死等损伤中、小血管引起的出血不属于此范畴。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "皮肤、黏膜自发性出血" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "皮肤" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "黏膜" }, { "start_idx": 32, "end_idx": 44, "type": "sym", "entity": "轻微损伤后出血不易自行停止" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "出血倾向" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "创伤" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "溃疡" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "肿瘤坏死" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "损伤" }, { "start_idx": 70, "end_idx": 77, "type": "sym", "entity": "小血管引起的出血" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "小血管" } ] }, { "text": "(一)血管的止血功能小动、静脉及微血管在止血过程中发挥着重要的作用。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血管" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "血" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "小动、静脉" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "微血管" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" } ] }, { "text": "具体表现在:①当血管受损发生出血时,机体立即通过神经和体液双重调控使该部位血管持续收缩达15~30分钟,此期间血流缓慢,有利于局部血栓的形成和止血;②由于血管内皮受损后胶原组织暴露,它促使血小板发生黏附、聚集和释放反应形成血小板栓子,又能激活凝血因子Ⅻ,启动内源性凝血;③损伤的血管壁释放组织因子,启动外源性凝血过程;④损伤的血管壁产生或释放的抗止血作用物质诸如前列腺素、组织纤溶酶原激活物(t-PA)和抗凝血酶Ⅲ(AT-Ⅲ)减少;而促凝血物如组织纤溶酶原激活物抑制剂(t-PA)、vWF等物质增多,能增强止血反应和加固止血血栓而止血。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "血管受损" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血管" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "出血" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "血" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "神经" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "体液" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "该部位血管" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "血流" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "局部血栓" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "血" }, { "start_idx": 77, "end_idx": 82, "type": "sym", "entity": "血管内皮受损" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "血管内皮" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "胶原组织" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "血小板" }, { "start_idx": 111, "end_idx": 115, "type": "bod", "entity": "血小板栓子" }, { "start_idx": 121, "end_idx": 125, "type": "bod", "entity": "凝血因子Ⅻ" }, { "start_idx": 133, "end_idx": 133, "type": "bod", "entity": "血" }, { "start_idx": 136, "end_idx": 137, "type": "dis", "entity": "损伤" }, { "start_idx": 139, "end_idx": 141, "type": "bod", "entity": "血管壁" }, { "start_idx": 144, "end_idx": 147, "type": "bod", "entity": "组织因子" }, { "start_idx": 155, "end_idx": 155, "type": "bod", "entity": "血" }, { "start_idx": 160, "end_idx": 165, "type": "bod", "entity": "损伤的血管壁" }, { "start_idx": 172, "end_idx": 178, "type": "bod", "entity": "抗止血作用物质" }, { "start_idx": 181, "end_idx": 184, "type": "bod", "entity": "前列腺素" }, { "start_idx": 186, "end_idx": 194, "type": "bod", "entity": "组织纤溶酶原激活物" }, { "start_idx": 196, "end_idx": 199, "type": "bod", "entity": "t-PA" }, { "start_idx": 202, "end_idx": 206, "type": "bod", "entity": "抗凝血酶Ⅲ" }, { "start_idx": 208, "end_idx": 211, "type": "bod", "entity": "AT-Ⅲ" }, { "start_idx": 217, "end_idx": 220, "type": "bod", "entity": "促凝血物" }, { "start_idx": 222, "end_idx": 233, "type": "bod", "entity": "组织纤溶酶原激活物抑制剂" }, { "start_idx": 235, "end_idx": 238, "type": "bod", "entity": "t-PA" }, { "start_idx": 241, "end_idx": 243, "type": "bod", "entity": "vWF" }, { "start_idx": 254, "end_idx": 254, "type": "bod", "entity": "血" }, { "start_idx": 260, "end_idx": 263, "type": "bod", "entity": "止血血栓" }, { "start_idx": 266, "end_idx": 266, "type": "bod", "entity": "血" } ] }, { "text": "由于血管止血功能异常引起的出血,临床上可分为遗传性和获得性两类。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "血管止血功能异常" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血管" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "出血" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" } ] }, { "text": "前者如遗传性毛细血管扩张症,系因小动、静脉及毛细血管壁变薄,仅有一层内皮组织构成,缺乏弹力纤维及肌层的血管壁失去舒缩功能,并因血流冲击成瘤状扩张,容易破裂出血。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "遗传性毛细血管扩张症" }, { "start_idx": 16, "end_idx": 28, "type": "sym", "entity": "小动、静脉及毛细血管壁变薄" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "小动、静脉" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "毛细血管壁" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "内皮组织" }, { "start_idx": 41, "end_idx": 59, "type": "sym", "entity": "缺乏弹力纤维及肌层的血管壁失去舒缩功能" }, { "start_idx": 41, "end_idx": 53, "type": "bod", "entity": "缺乏弹力纤维及肌层的血管壁" }, { "start_idx": 62, "end_idx": 71, "type": "sym", "entity": "因血流冲击成瘤状扩张" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "血流" }, { "start_idx": 73, "end_idx": 78, "type": "sym", "entity": "容易破裂出血" }, { "start_idx": 78, "end_idx": 78, "type": "bod", "entity": "血" } ] }, { "text": "获得性者有过敏性、感染性、中毒性紫癜,维生素C、维生素P缺乏症以及单纯性紫癜等。", "entities": [ { "start_idx": 5, "end_idx": 17, "type": "dis", "entity": "过敏性、感染性、中毒性紫癜" }, { "start_idx": 19, "end_idx": 30, "type": "dis", "entity": "维生素C、维生素P缺乏症" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "单纯性紫癜" } ] }, { "text": "(二)血小板的止血功能生理情况下血小板具有保护血管壁完整性、维持其正常通透性和防止血液外渗作用。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "血小板" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "血" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "血小板" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "血管壁" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "血液" } ] }, { "text": "当微小血管破损时,血管内皮下胶原和微纤维促使血小板GPⅠb以vWF为桥梁结合到胶原上去,实现了血小板的黏附。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "微小血管破损" }, { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "微小血管" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "血管内皮下胶原" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "微纤维" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "血小板" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "GPⅠb" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "vWF" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "胶原" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "血小板" } ] }, { "text": "血小板彼此间的黏附、集结作用称为聚集,在致聚剂如胶原、二磷酸腺苷(ADP)等诱导下,血小板表面形成了钙依赖性GPⅡb/Ⅲa复合物,血小板通过该复合物与纤维蛋白原的结合并以后者为桥梁完成了彼此间的聚集。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "致聚剂" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胶原" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "二磷酸腺苷" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "ADP" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "血小板表面" }, { "start_idx": 50, "end_idx": 63, "type": "bod", "entity": "钙依赖性GPⅡb/Ⅲa复合物" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "血小板" }, { "start_idx": 75, "end_idx": 79, "type": "bod", "entity": "纤维蛋白原" } ] }, { "text": "血小板在止血过程中能释放许多活性物质如5-羟色胺(5-HT)、ADP、钙、黏附蛋白等,能起到促进血管收缩、稳定聚集、参与和促进血液凝固作用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "血" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "活性物质" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "5-羟色胺" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "5-HT" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "ADP" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "钙" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "黏附蛋白" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "血管" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "血液" } ] }, { "text": "由于遗传或获得性的血小板数量或质量异常,最终导致血管通透性增加或者血小板栓子形成障碍而出血,这方面涉及的多种疾病参及本章第一、二节内容。", "entities": [ { "start_idx": 2, "end_idx": 18, "type": "sym", "entity": "遗传或获得性的血小板数量或质量异常" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "血小板" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "血管通透性增加" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "血管" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "血小板栓子形成障碍" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "血小板栓子" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "出血" } ] }, { "text": "(三)凝血因子的止血功能迄今所知人体共有凝血因子14个,其中12个为经典凝血因子,依次用罗马数字Ⅰ-表示(无因子Ⅵ);另有2个激肽系统的因子,即激肽释放酶原(PK)和相对分子量高的激肽原(HMWK)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "凝血因子" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "凝血因子" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "经典凝血因子" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "激肽系统" }, { "start_idx": 72, "end_idx": 77, "type": "bod", "entity": "激肽释放酶原" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "PK" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "激肽原" }, { "start_idx": 94, "end_idx": 97, "type": "bod", "entity": "HMWK" } ] }, { "text": "14个因子中只有因子Ⅳ为非蛋白,除因子Ⅲ外均存在于新鲜血浆中。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "14个因子" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "因子Ⅳ" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "蛋白" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "因子Ⅲ" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "新鲜血浆" } ] }, { "text": "生理情况下的凝血因子是以无活性的酶原形式存在于血液中,当血管破损发生出血时,即激活起始的凝血因子,后者又引起有规律的连锁性因子激活作用,最终生成纤维蛋白和导致血液凝固而止血。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "凝血因子" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "无活性的酶原" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血液" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "血管破损" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血管" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "出血" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "凝血因子" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "连锁性因子" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "血液" }, { "start_idx": 85, "end_idx": 85, "type": "bod", "entity": "血" } ] }, { "text": "整个凝血过程可分为凝血酶原酶形成、凝血酶形成和纤维蛋白形成三个阶段,这是一种生物放大的过程,也称瀑布学说具体(图10-10)。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "凝血酶原酶" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "凝血酶" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "纤维蛋白" } ] }, { "text": "图10-5凝血过程模式图注:PK:激肽释放酶原;KK:激肽释放酶;HMWK:相对分子量高的激肽原酶;PI:磷脂任何凝血因子遗传性缺陷或获得性异常均可致凝血障碍而出血,遗传性缺陷中最常见的为血友病,参见本章第三节。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "血" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "PK" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "激肽释放酶原" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "KK" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "激肽释放酶" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "HMWK" }, { "start_idx": 38, "end_idx": 48, "type": "bod", "entity": "相对分子量高的激肽原酶" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "PI" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "磷脂" }, { "start_idx": 57, "end_idx": 71, "type": "dis", "entity": "凝血因子遗传性缺陷或获得性异常" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "凝血障碍" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "出血" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "遗传性缺陷" }, { "start_idx": 94, "end_idx": 96, "type": "dis", "entity": "血友病" } ] }, { "text": "其次是凝血酶原缺乏症,纤维蛋白原缺乏症等。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "凝血酶原缺乏症" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "纤维蛋白原缺乏症" } ] }, { "text": "获得性凝血异常中,儿科以维生素K缺乏肝病的凝血因子合成减少较少见。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "获得性凝血异常" }, { "start_idx": 9, "end_idx": 10, "type": "dep", "entity": "儿科" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "维生素K缺乏肝病" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "凝血因子合成减少" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "凝血因子" } ] }, { "text": "(四)抗凝血及纤维蛋白溶解系统功能人体内除了凝血系统以外,尚存在一个功能上与之拮抗又与其保持动态平衡的抗凝血系统,它包括:①细胞抗凝:是指肝细胞及单核-巨噬细胞系统清除和摄取循环中已激活的凝血因子的作用;②体液抗凝:体液中存在抗凝血酶Ⅲ(AT-Ⅲ)、蛋白C(PC)、蛋白S(PS)等抗凝物质,能有效地灭活已被活化的凝血因子尤其是凝血酶;③纤维蛋白溶解系统:体内的纤维蛋白溶解酶原(纤溶酶原)可经因子Ⅻa、激肽释放酶、尿激酶、组织纤溶酶原激活物(tPA)等激活成有活性的纤溶酶,后者可使纤维蛋白(原)降解成纤维蛋白降解产物(FDP),这有助于将血液凝固限于出血部位,并在血管开始修复前清除掉凝块。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "抗凝血" }, { "start_idx": 7, "end_idx": 14, "type": "bod", "entity": "纤维蛋白溶解系统" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "凝血系统" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "抗凝血系统" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "细胞抗凝" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "肝细胞" }, { "start_idx": 73, "end_idx": 79, "type": "bod", "entity": "单核-巨噬细胞" }, { "start_idx": 90, "end_idx": 97, "type": "bod", "entity": "已激活的凝血因子" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "体液" }, { "start_idx": 108, "end_idx": 109, "type": "bod", "entity": "体液" }, { "start_idx": 113, "end_idx": 117, "type": "bod", "entity": "抗凝血酶Ⅲ" }, { "start_idx": 119, "end_idx": 122, "type": "bod", "entity": "AT-Ⅲ" }, { "start_idx": 125, "end_idx": 127, "type": "bod", "entity": "蛋白C" }, { "start_idx": 129, "end_idx": 130, "type": "bod", "entity": "PC" }, { "start_idx": 133, "end_idx": 135, "type": "bod", "entity": "蛋白S" }, { "start_idx": 137, "end_idx": 138, "type": "bod", "entity": "PS" }, { "start_idx": 141, "end_idx": 144, "type": "bod", "entity": "抗凝物质" }, { "start_idx": 157, "end_idx": 160, "type": "bod", "entity": "凝血因子" }, { "start_idx": 164, "end_idx": 166, "type": "bod", "entity": "凝血酶" }, { "start_idx": 169, "end_idx": 176, "type": "bod", "entity": "纤维蛋白溶解系统" }, { "start_idx": 181, "end_idx": 188, "type": "bod", "entity": "纤维蛋白溶解酶原" }, { "start_idx": 190, "end_idx": 193, "type": "bod", "entity": "纤溶酶原" }, { "start_idx": 197, "end_idx": 200, "type": "bod", "entity": "因子Ⅻa" }, { "start_idx": 202, "end_idx": 206, "type": "bod", "entity": "激肽释放酶" }, { "start_idx": 208, "end_idx": 210, "type": "bod", "entity": "尿激酶" }, { "start_idx": 212, "end_idx": 220, "type": "bod", "entity": "组织纤溶酶原激活物" }, { "start_idx": 222, "end_idx": 224, "type": "bod", "entity": "tPA" }, { "start_idx": 230, "end_idx": 236, "type": "bod", "entity": "有活性的纤溶酶" }, { "start_idx": 242, "end_idx": 248, "type": "bod", "entity": "纤维蛋白(原)" }, { "start_idx": 252, "end_idx": 259, "type": "bod", "entity": "纤维蛋白降解产物" }, { "start_idx": 261, "end_idx": 263, "type": "bod", "entity": "FDP" }, { "start_idx": 271, "end_idx": 272, "type": "bod", "entity": "血液" }, { "start_idx": 277, "end_idx": 278, "type": "sym", "entity": "出血" }, { "start_idx": 284, "end_idx": 285, "type": "bod", "entity": "血管" }, { "start_idx": 294, "end_idx": 295, "type": "bod", "entity": "凝块" } ] }, { "text": "血凝中还存在着tPA的抑制剂(tPAI)和α-2纤溶酶抑制剂,前者激活后能抑制tPA,后者则可中和从纤维蛋白播散到血浆中的纤溶酶。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血凝" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "tPA的抑制剂" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "tPAI" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "α-2纤溶酶抑制剂" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "tPA" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "血浆" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "纤溶酶" } ] }, { "text": "在恶性肿瘤、肝病、结缔组织病等疾患中产生的类肝素抗凝物质增多,可通过增加AT-Ⅲ的抗凝血酶活性引起出血。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肝病" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "类肝素抗凝物质" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "类肝素抗凝物质增多" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "AT-Ⅲ" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "抗凝血酶" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "出血" } ] }, { "text": "在创伤、产科及许多脏器的手术中损伤的组织可释放出大量tPA,使纤溶亢进而创面渗血不止。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dep", "entity": "产科" }, { "start_idx": 15, "end_idx": 28, "type": "sym", "entity": "损伤的组织可释放出大量tPA" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "组织" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "大量tPA" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "纤溶亢进" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "纤溶" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "创面渗血不止" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "血" } ] }, { "text": "二、儿科主要的抗结核药物(一)异烟肼异烟肼(INH或H)是主要的抗结核药物,仍是目前小儿化疗的首选药物,其特点是:①疗效高,INH口服后吸收迅速,1~2小时后血浓度可达5μg/ml,能在化疗前几天杀死病灶中结核菌群的90%。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dep", "entity": "儿科" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "抗结核药物" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "异烟肼" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "异烟肼" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "INH" }, { "start_idx": 26, "end_idx": 26, "type": "dru", "entity": "H" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "抗结核药物" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "化疗" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "INH" }, { "start_idx": 65, "end_idx": 66, "type": "pro", "entity": "口服" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "血" }, { "start_idx": 93, "end_idx": 94, "type": "pro", "entity": "化疗" }, { "start_idx": 103, "end_idx": 106, "type": "mic", "entity": "结核菌群" } ] }, { "text": "INH杀菌作用不受环境酸碱度影响。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "INH" }, { "start_idx": 4, "end_idx": 4, "type": "mic", "entity": "菌" } ] }, { "text": "②INH分子小,通透性强,可渗透到各种组织及体液中,特别是易于透过血-脑屏障,脑脊液浓度可达血浓度的近1/3。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "INH" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "各种组织" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "体液" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "血-脑屏障" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "脑脊液" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "血" } ] }, { "text": "脑膜有炎症时可达血浓度的90%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "脑膜有炎症" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脑膜" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "血浓度" } ] }, { "text": "因此INH对结脑的防治有特殊功效,成为小儿原发结核首选药物。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "INH" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "结脑" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "小儿原发结核" } ] }, { "text": "肺中浓度可与血浓度相似,又能渗透到干酪病灶中。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "血" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "干酪病灶" } ] }, { "text": "③对细胞内、外结核菌均有杀灭作用,对干酪病灶内代谢缓慢的持续存在结核菌亦有一定作用,因此是全杀菌药,是标准化疗方案与短程化疗方案中自始至终全程应用的药物。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "mic", "entity": "细胞内、外结核菌" }, { "start_idx": 18, "end_idx": 34, "type": "mic", "entity": "干酪病灶内代谢缓慢的持续存在结核菌" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "全杀菌药" }, { "start_idx": 51, "end_idx": 56, "type": "pro", "entity": "标准化疗方案" }, { "start_idx": 58, "end_idx": 63, "type": "pro", "entity": "短程化疗方案" } ] }, { "text": "主要毒性作用是肝功能损害和周围神经炎。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肝功能损害" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "周围神经炎" } ] }, { "text": "INH所致肝损害在儿童明显低于成人,有报告发生率为0.5%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "INH" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肝损害" } ] }, { "text": "INH对肝的毒性作用在剂量>10mg/kg/日时有所增加。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "INH" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肝" } ] }, { "text": "周围神经炎是由于该药竞争性抑制维生素B6代谢。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "周围神经炎" }, { "start_idx": 15, "end_idx": 25, "type": "bod", "entity": "维生素B6" } ] }, { "text": "典型的症状包括手脚麻木、疼痛维生素B6,这些症状是可逆的。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "手脚麻木、疼痛" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "手" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "脚" }, { "start_idx": 14, "end_idx": 24, "type": "dru", "entity": "维生素B6" } ] }, { "text": "⑤价格低廉,用药方便,口服,每天一次顿服,易执行。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "口服" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "顿服" } ] }, { "text": "(二)利福平利福平(RIF或R)为短程化疗主要药物,其特点:①杀菌作用发生最快,从药物接触结核菌到发生杀菌效力仅需1小时。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "利福平" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "利福平" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "RIF" }, { "start_idx": 14, "end_idx": 14, "type": "dru", "entity": "R" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "短程化疗" }, { "start_idx": 32, "end_idx": 32, "type": "mic", "entity": "菌" }, { "start_idx": 45, "end_idx": 47, "type": "mic", "entity": "结核菌" }, { "start_idx": 52, "end_idx": 52, "type": "mic", "entity": "菌" } ] }, { "text": "服药后1~2小时RFP在血中浓度可达MIC的30~100倍。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "RFP" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "血" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "MIC" } ] }, { "text": "RFP属低浓度抑菌,高浓度杀菌药。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "RFP" }, { "start_idx": 4, "end_idx": 15, "type": "dru", "entity": "低浓度抑菌,高浓度杀菌药" } ] }, { "text": "不但对细胞外结核菌且对细胞内休眠菌亦可杀灭,也是全杀菌药。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "mic", "entity": "细胞外结核菌" }, { "start_idx": 11, "end_idx": 16, "type": "mic", "entity": "细胞内休眠菌" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "全杀菌药" } ] }, { "text": "②口服后吸收分布良好,具有极好的穿透组织进入体液(包括中枢神经系统)的能力。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "口服" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "体液" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "其渗透入体腔量为血浓度的1/3。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "体腔" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "血浓度" } ] }, { "text": "脑脊液的浓度为血浓度的20%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "脑脊液的浓度" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "血浓度" } ] }, { "text": "但脑膜炎症时其在脑脊液浓度可增加4~5倍,故对结脑亦有强大治疗效果。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "脑膜炎症" }, { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "脑脊液浓度" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "结脑" } ] }, { "text": "③与INH及EB有协同作用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "INH" }, { "start_idx": 6, "end_idx": 7, "type": "dru", "entity": "EB" } ] }, { "text": "INH和RFP联用灭菌作用比任何其他联合用药均强,为短程化疗最佳联合。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "INH" }, { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "RFP" }, { "start_idx": 10, "end_idx": 10, "type": "mic", "entity": "菌" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "短程化疗" } ] }, { "text": "④主要副作用是肝损害,剂量大时毒副作用增多。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肝损害" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "毒副作用" } ] }, { "text": "与INH联合用药可增加肝脏毒性,儿童用INH+RFP时二者剂量最好各不超过10mg/(kg•d),RFP与PZA合用亦可增加肝损害机会。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "INH" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肝脏" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "INH" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "RFP" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "RFP" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "PZA" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "肝损害" } ] }, { "text": "其他不良反应包括过敏、血小板减少、白细胞减低,但都很少见。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "过敏" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "血小板减少" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "血小板" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "白细胞减低" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "白细胞" } ] }, { "text": "(三)吡嗪酰胺吡嗪酰胺(PZA或Z)属半杀菌药,其特点:①PZA作用受环境酸碱度影响大,当常规培养基pH7.0时PZA几乎对结核菌无作用;而当pH5.0~5.5时则对结核菌可发挥抑菌甚至杀菌作用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "吡嗪酰胺" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "吡嗪酰胺" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "PZA" }, { "start_idx": 16, "end_idx": 16, "type": "dru", "entity": "Z" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "半杀菌药" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "PZA" }, { "start_idx": 56, "end_idx": 58, "type": "dru", "entity": "PZA" }, { "start_idx": 62, "end_idx": 64, "type": "mic", "entity": "结核菌" }, { "start_idx": 83, "end_idx": 85, "type": "mic", "entity": "结核菌" }, { "start_idx": 90, "end_idx": 90, "type": "mic", "entity": "菌" }, { "start_idx": 94, "end_idx": 94, "type": "mic", "entity": "菌" } ] }, { "text": "②口服后吸收极好,对预防结核复发有重要意义。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "口服" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "结核" } ] }, { "text": "③PZA能渗透到很多组织及体液,包括脑脊液,有报道PZA脑脊液浓度可近似血浓度,故对治疗结脑有效。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "PZA" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "体液" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑脊液" }, { "start_idx": 25, "end_idx": 32, "type": "ite", "entity": "PZA脑脊液浓度" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "血浓度" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "结脑" } ] }, { "text": "20~30mg/(kg•d),疗程3个月,已证明有确切疗效且毒副作用大大减少;此外可见高尿酸血症,但真正痛风少见。", "entities": [ { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "高尿酸血症" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "痛风" } ] }, { "text": "⑤单一用药极易产生耐药,PZA与INH联合用可增强杀菌作用,目前INH+RFP+PZA为最强大灭菌组合。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "PZA" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "INH" }, { "start_idx": 26, "end_idx": 26, "type": "mic", "entity": "菌" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "INH" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "RFP" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "PZA" }, { "start_idx": 48, "end_idx": 48, "type": "mic", "entity": "菌" } ] }, { "text": "(四)链霉素链霉素(SM或S)为1944年生产的第一个抗结核药物,其特点:①SM在细胞外,pH中性和偏碱性环境中发挥作用,在pH>8时抗菌作用最强,对生长繁殖活跃的细胞外结核菌有杀菌作用,对细胞内菌无活性,故称为半杀菌药。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "链霉素" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "链霉素" }, { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "SM" }, { "start_idx": 13, "end_idx": 13, "type": "dru", "entity": "S" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "抗结核药物" }, { "start_idx": 38, "end_idx": 39, "type": "dru", "entity": "SM" }, { "start_idx": 68, "end_idx": 68, "type": "mic", "entity": "菌" }, { "start_idx": 75, "end_idx": 87, "type": "mic", "entity": "生长繁殖活跃的细胞外结核菌" }, { "start_idx": 90, "end_idx": 90, "type": "mic", "entity": "菌" }, { "start_idx": 95, "end_idx": 98, "type": "mic", "entity": "细胞内菌" }, { "start_idx": 106, "end_idx": 109, "type": "dru", "entity": "半杀菌药" } ] }, { "text": "②SM肌内注射后1~3小时可达高峰浓度40μg/ml。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "SM" }, { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "肌内注射" } ] }, { "text": "SM对新鲜渗出性病灶和空洞中结核菌抗菌作用最强,故对治疗小儿急性血行播散结核最为适宜。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "SM" }, { "start_idx": 3, "end_idx": 16, "type": "mic", "entity": "新鲜渗出性病灶和空洞中结核菌" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "小儿急性血行播散结核" } ] }, { "text": "③SM能渗入肺、肝、肾等脏器及浆膜腔,但不易通过血-脑屏障。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "SM" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肝" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肾" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脏器" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "浆膜腔" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "血-脑屏障" } ] }, { "text": "当脑膜有炎症时虽通透性可增加,但在脑脊液仍不足以达到有效的治疗浓度。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "脑膜" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "炎症" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "通透性可增加" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "脑脊液" } ] }, { "text": "SM不能进入巨噬细胞,又不能在酸性环境中起作用,故SM对巨噬细胞内休眠菌无能为力。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "SM" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "SM" }, { "start_idx": 28, "end_idx": 35, "type": "mic", "entity": "巨噬细胞内休眠菌" } ] }, { "text": "④SM毒副作用主要是听力障碍和肾毒性。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "SM" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "听力障碍" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肾毒性" } ] }, { "text": "但在应用时需进行听力监测,对家族中有药物性耳聋患儿应禁用。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "听力监测" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "药物性耳聋" } ] }, { "text": "⑤SM单用易产生耐药,一旦发现耐药应立即停用。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "SM" } ] }, { "text": "(五)乙胺丁醇乙胺丁醇(EB或B)为抑菌药。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "乙胺丁醇" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "乙胺丁醇" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "EB" }, { "start_idx": 15, "end_idx": 15, "type": "dru", "entity": "B" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "抑菌药" } ] }, { "text": "近年来在标准化疗中已取代PAS。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "PAS" } ] }, { "text": "②EB通透性较好。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "EB" } ] }, { "text": "在正常情况下难透过血-脑屏障,而当脑膜炎症时CSF浓度增高,可达血浓度20%~40%。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "血-脑屏障" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "脑膜炎症" }, { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "CSF浓度" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "血浓度" } ] }, { "text": "口服25mg/(kg•d)剂量时CSF浓度可达1~2μg/ml,达抑菌水平。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "CSF浓度" }, { "start_idx": 34, "end_idx": 34, "type": "mic", "entity": "菌" } ] }, { "text": "③EB常与INH、RFP等联合应用,可延缓二者耐药性产生,但不够有力,故不是短程化疗之主要药物。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "EB" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "INH" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "RFP" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "短程化疗" } ] }, { "text": "④主要副作用为球后视神经炎,视力减退,中心盲点和绿视能力丧失。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "球后视神经炎" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "视力减退" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "中心盲点" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "绿视能力丧失" } ] }, { "text": "(六)乙(丙)硫异烟胺1314TH及1321TH(ETH)乙(丙)硫异烟胺1314TH及1321TH(ETH)均为异烟肼衍生物,两药作用机制,吸收分布及用法均相似,只是1321TH副作用较轻。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "乙(丙)硫异烟胺" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "1314TH" }, { "start_idx": 18, "end_idx": 23, "type": "dru", "entity": "1321TH" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "ETH" }, { "start_idx": 29, "end_idx": 36, "type": "dru", "entity": "乙(丙)硫异烟胺" }, { "start_idx": 37, "end_idx": 42, "type": "dru", "entity": "1314TH" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "1321TH" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "ETH" }, { "start_idx": 57, "end_idx": 62, "type": "dru", "entity": "异烟肼衍生物" }, { "start_idx": 84, "end_idx": 89, "type": "dru", "entity": "1321TH" } ] }, { "text": "其特点:①口服吸收好;②吸收后广泛分布于组织及浆膜腔。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "口服" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "组织" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "浆膜腔" } ] }, { "text": "易渗透到脑脊液,可达血浓度的80%;③可用于耐药患者,疗效良好;④ETH副作用主要为胃肠道障碍及肝功损害。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "脑脊液" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "血浓度" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "ETH" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "胃肠道障碍" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "胃" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肠道" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "肝功损害" } ] }, { "text": "(七)其他药物包括氨基糖苷类药物和喹诺酮类药物。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dru", "entity": "氨基糖苷类药物" }, { "start_idx": 17, "end_idx": 22, "type": "dru", "entity": "喹诺酮类药物" } ] }, { "text": "卡那霉素和丁胺卡那霉素有明显的抗结核活性而用于耐链霉素结核病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "卡那霉素" }, { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "丁胺卡那霉素" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "结核" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "耐链霉素结核病" } ] }, { "text": "这些药物可肌内注射或静脉给药,为杀菌剂,与链霉素无交叉耐药性。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "肌内注射" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "静脉给药" }, { "start_idx": 17, "end_idx": 17, "type": "mic", "entity": "菌" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "链霉素" } ] }, { "text": "喹诺酮类药物具有明显的抗结核活性,常用于成人耐药性结核病。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "喹诺酮类药物" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "结核" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "成人耐药性结核病" } ] }, { "text": "但它们已有效地用于患有耐药结核杆菌感染又没有其他有效药物可用的患儿。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "耐药结核杆菌感染" } ] }, { "text": "第三节急性喉、气管、支气管炎急性喉气管支气管炎(acutelaryngotracheobronchitis,croup)为一常见的气道梗阻性疾病,多见于喉部急性感染以后,炎症急速下行蔓延至气管、支气管,甚至延及小支气管、毛细支气管。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "dis", "entity": "急性喉、气管、支气管炎" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "急性喉气管支气管炎" }, { "start_idx": 25, "end_idx": 53, "type": "dis", "entity": "acutelaryngotracheobronchitis" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "croup" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "气道梗阻性疾病" }, { "start_idx": 77, "end_idx": 82, "type": "dis", "entity": "喉部急性感染" }, { "start_idx": 86, "end_idx": 87, "type": "dis", "entity": "炎症" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "气管" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "支气管" }, { "start_idx": 106, "end_idx": 109, "type": "bod", "entity": "小支气管" }, { "start_idx": 111, "end_idx": 115, "type": "bod", "entity": "毛细支气管" } ] }, { "text": "病程中可有发热,但很少超过39℃。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" } ] }, { "text": "胸部X线检查可表现肺纹理改变、肺不张和肺气肿。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "胸部X线检查可表现肺纹理改变、肺不张和肺气肿" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肺" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肺" } ] }, { "text": "雾化吸入,稀化痰液,并及时吸出。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "稀化痰液" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "及时吸出" } ] }, { "text": "肾上腺素雾化吸入可暂时缓解症状,必要时重复使用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "雾化吸入" } ] }, { "text": "早期应用肾上腺皮质激素如口服或肌内注射地塞米松0.15~0.60mg/kg有助于减轻炎症水肿和纤毛上皮坏死,亦可雾化吸入地塞米松、布地奈德等表面皮质激素。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "口服" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "肌内注射" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "地塞米松" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "炎症" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "水肿" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "纤毛上皮坏死" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 60, "end_idx": 63, "type": "dru", "entity": "地塞米松" }, { "start_idx": 65, "end_idx": 68, "type": "dru", "entity": "布地奈德" }, { "start_idx": 70, "end_idx": 75, "type": "dru", "entity": "表面皮质激素" } ] }, { "text": "合并细菌感染者适当选用抗生素。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "细菌感染" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "抗生素" } ] }, { "text": "及时供氧,减少不必要的刺激,一般不宜使用镇静剂。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "及时供氧" }, { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "减少不必要的刺激" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "镇静剂" } ] }, { "text": "经上述治疗后,呼吸困难仍不缓解,缺氧继续加重者,应气管切开或鼻气管插管。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "缺氧" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "气管切开" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "鼻气管插管" } ] }, { "text": "四、头颅B超检查(一)诊断原理超声诊断是由超声探头把电转换成声波,发射出高频的超声束穿透体表,在组织中传播并产生反射、透射、折射、散射及衍射等;接收探头把这些反映组织特性的回波再转换成电信息,通过一系列放大、检波使显像管显示超声图像,或经计算程序得到各种超声系数。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "头颅B超检查" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "超声诊断" }, { "start_idx": 21, "end_idx": 24, "type": "equ", "entity": "超声探头" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "组织" }, { "start_idx": 72, "end_idx": 75, "type": "equ", "entity": "接收探头" }, { "start_idx": 107, "end_idx": 109, "type": "equ", "entity": "显像管" } ] }, { "text": "(二)检查方法显示技术分为A超、B超以及多普勒血流测定。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "A超" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "B超" }, { "start_idx": 20, "end_idx": 26, "type": "pro", "entity": "多普勒血流测定" } ] }, { "text": "在儿童期,应用技术比较成熟的是头颅B超检查(B-modesonography)。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "头颅B超检查" }, { "start_idx": 22, "end_idx": 37, "type": "pro", "entity": "B-modesonography" } ] }, { "text": "头颅B超是以前囟为透声窗,通过超声诊断原理将颅内信息展现在示波屏上。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "头颅B超" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "前囟" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "颅内" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "示波屏" } ] }, { "text": "(三)临床应用1.颅内出血B超可有助于诊断脑室内出血,而对硬脑膜下出血、蛛网膜下腔出血以及小脑内出血等分辨力较差。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "颅内出血" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "B超" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "脑室内出血" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "硬脑膜下出血" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "小脑内出血" } ] }, { "text": "2.新生儿缺血性缺氧性脑病B超能分辨缺氧性改变,能很好诊断脑室周围白质软化等其他脑实质改变B超能反映脑积水、囊腔形成以及室管膜下囊肿形成等改变,但不能仅根据影像学改变判断病变性质。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "新生儿缺血性缺氧性脑病" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "B超" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "缺氧性改变" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "脑室周围白质软化" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "脑室周围白质" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "脑实质" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "脑实质改变" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "B超" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "脑积水" }, { "start_idx": 54, "end_idx": 67, "type": "sym", "entity": "囊腔形成以及室管膜下囊肿形成" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "室管膜下" }, { "start_idx": 78, "end_idx": 80, "type": "pro", "entity": "影像学" } ] }, { "text": "第三节糖原累积病【概述】糖原累积病(glycogenstoragedisease,GSD)是一组由于先天性酶缺陷糖代谢障碍性疾病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "糖原累积病" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "糖原累积病" }, { "start_idx": 18, "end_idx": 39, "type": "dis", "entity": "glycogenstoragedisease" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "GSD" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "先天性酶" }, { "start_idx": 50, "end_idx": 55, "type": "sym", "entity": "先天性酶缺陷" }, { "start_idx": 56, "end_idx": 63, "type": "dis", "entity": "糖代谢障碍性疾病" } ] }, { "text": "这类疾病的共同生化特征是糖原代谢异常,多数类型可见到糖原在肝脏、肌肉等组织中贮积量增加肝脏病变肌肉组织受损糖原累积病多数属分解代谢上的缺陷,使糖原异常堆积。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "糖原代谢异常" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "糖原" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "糖原" }, { "start_idx": 26, "end_idx": 42, "type": "sym", "entity": "糖原在肝脏、肌肉等组织中贮积量增加" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肝脏" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肌肉" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "肝脏病变" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "肌肉组织" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "肌肉组织受损" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "糖原累积病" }, { "start_idx": 61, "end_idx": 68, "type": "sym", "entity": "分解代谢上的缺陷" }, { "start_idx": 71, "end_idx": 76, "type": "sym", "entity": "糖原异常堆积" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "糖原" } ] }, { "text": "【流行病学】糖原累积病的发病率较低,有报道为1/60000。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "糖原累积病" } ] }, { "text": "最常见的糖原累积病是Ia型,即VonGierke病,其发病率约1/100000。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "糖原累积病" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "VonGierke病" } ] }, { "text": "【病理生理和发病机制】糖原由无数α-D葡萄糖聚合而成,分子间由α-1,4-糖苷键相连,分支的键则以α-1,6-糖苷键相连,它的平均分子量在250万~450万之间。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "糖原" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "α-D葡萄糖" }, { "start_idx": 31, "end_idx": 39, "type": "bod", "entity": "α-1,4-糖苷键" }, { "start_idx": 49, "end_idx": 57, "type": "bod", "entity": "α-1,6-糖苷键" } ] }, { "text": "根据体内代谢的需要,糖原分子不断合成和分解,它们是在一组完全不同的催化酶作用下完成。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "糖原分子" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "催化酶" } ] }, { "text": "这些代谢步骤受许多生理上的条件所控制,如进食、饥饿、胰岛素、肾上腺素及胰高血糖素的分泌等,从而达到葡萄糖的内在稳定。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "胰岛素" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "胰高血糖素" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "葡萄糖合成糖原以磷酸化开始(图14-14),在肝脏由葡萄糖激酶催化,在肌肉则由己糖激酶催化,激酶的活性在饥饿时降低,进食时增高,磷酸化产生了6-磷酸葡萄糖,后者通过葡萄糖磷酸变位酶的作用转变成1-磷酸葡萄糖。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "糖原" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肝脏" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "葡萄糖激酶" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肌肉" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "己糖激酶" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "激酶" }, { "start_idx": 70, "end_idx": 76, "type": "bod", "entity": "6-磷酸葡萄糖" }, { "start_idx": 82, "end_idx": 89, "type": "bod", "entity": "葡萄糖磷酸变位酶" }, { "start_idx": 96, "end_idx": 102, "type": "bod", "entity": "1-磷酸葡萄糖" } ] }, { "text": "1-磷酸葡萄糖再通过尿苷二磷酸葡萄糖焦磷酸化酶转变成尿苷二磷酸葡萄糖。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "1-磷酸葡萄糖" }, { "start_idx": 10, "end_idx": 22, "type": "bod", "entity": "尿苷二磷酸葡萄糖焦磷酸化酶" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "尿苷二磷酸葡萄糖" } ] }, { "text": "后者中的葡萄糖残基通过糖原合成酶及淀粉-(1,4→1,6)转葡萄糖苷酶的作用,加入原有的糖原分子中,从而形成新的糖原分子。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "葡萄糖残基" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "糖原合成酶" }, { "start_idx": 17, "end_idx": 34, "type": "bod", "entity": "淀粉-(1,4→1,6)转葡萄糖苷酶" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "糖原分子" }, { "start_idx": 54, "end_idx": 59, "type": "bod", "entity": "新的糖原分子" } ] }, { "text": "其中糖原合成酶使糖原直键增长,而淀粉-(1,4→1,6)转葡萄糖苷酶使糖原产生分支。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "糖原合成酶" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "糖原" }, { "start_idx": 16, "end_idx": 33, "type": "bod", "entity": "淀粉-(1,4→1,6)转葡萄糖苷酶" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "糖原" } ] }, { "text": "糖原的分解由两个酶系统完成。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "糖原" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "酶系统" } ] }, { "text": "糖原分解成1-磷酸葡萄糖由磷酸化酶催化,1-磷酸葡萄糖经过葡萄糖磷酸变位酶的作用转变成6-磷酸葡萄糖,后者在葡萄糖-6-磷酸酶催化下,水解成葡萄糖。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "糖原" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "1-磷酸葡萄糖" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "磷酸化酶" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "1-磷酸葡萄糖" }, { "start_idx": 29, "end_idx": 36, "type": "bod", "entity": "葡萄糖磷酸变位酶" }, { "start_idx": 43, "end_idx": 49, "type": "bod", "entity": "6-磷酸葡萄糖" }, { "start_idx": 54, "end_idx": 62, "type": "bod", "entity": "葡萄糖-6-磷酸酶" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "上述磷酸化酶只能分解到糖原分支点之前4个葡萄糖残基,其剩余的葡萄糖残基通过脱支酶的作用,分解出葡萄糖。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "磷酸化酶" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "糖原" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "葡萄糖残基" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "葡萄糖残基" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "脱支酶" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "糖原分解的另一途径是通过存在于溶酶体中的α-1,4葡萄糖苷酶来完成。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "糖原" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "溶酶体" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "α-1,4葡萄糖苷酶" } ] }, { "text": "除GSDⅨb型为X连锁隐性遗传外,其余都是常染色体隐性遗传性疾病。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "X连锁隐性遗传" }, { "start_idx": 21, "end_idx": 31, "type": "dis", "entity": "常染色体隐性遗传性疾病" } ] }, { "text": "导致糖原累积病Ia型的致病基因G6Pase位于17号染色体长臂2区1带,约有12.5kb,包含5个外显子。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "糖原累积病Ia型" }, { "start_idx": 11, "end_idx": 20, "type": "bod", "entity": "致病基因G6Pase" }, { "start_idx": 23, "end_idx": 34, "type": "bod", "entity": "17号染色体长臂2区1带" } ] }, { "text": "G6Pase蛋白为细胞内质网膜蛋白,包含357个氨基酸。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "G6Pase蛋白" }, { "start_idx": 9, "end_idx": 16, "type": "bod", "entity": "细胞内质网膜蛋白" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "氨基酸" } ] }, { "text": "迄今为止,G6Pase基因编码区已发现100余种突变。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "bod", "entity": "G6Pase基因编码区" } ] }, { "text": "【临床表现】严重者在新生儿期即可出现严重的低血糖、酸中毒、呼吸困难和肝肿大低血糖抽搐。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "低血糖" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血糖" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "酸中毒" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肝" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "肝肿大" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "低血糖抽搐" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "血糖" } ] }, { "text": "轻者在幼儿期表现为生长落后、低血糖及肝大,易感染,也可出现高脂血症、高乳酸血症及高尿酸血症;由此将来可并发肾病或肾功能异常,早期表现为尿微量蛋白(尿试纸法测定尿微蛋白>2.5mg/mmolCr)出现。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "生长落后" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "低血糖" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "肝大" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肝" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "易感染" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "高脂血症" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "高乳酸血症" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "高尿酸血症" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "肾病" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "肾功能异常" }, { "start_idx": 67, "end_idx": 71, "type": "sym", "entity": "尿微量蛋白" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "尿" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "蛋白" }, { "start_idx": 73, "end_idx": 95, "type": "sym", "entity": "尿试纸法测定尿微蛋白>2.5mg/mmolCr" }, { "start_idx": 73, "end_idx": 76, "type": "pro", "entity": "尿试纸法" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "尿" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "蛋白" } ] }, { "text": "有些患者在年幼时即可出现骨质疏松。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "骨质疏松" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨质" } ] }, { "text": "图14-5糖原分解、合成的代谢途径(1)淀粉-(1,4→1,6)转葡萄糖苷酶(2)糖原合成酶(3)尿苷二磷酸葡萄糖焦磷酸化酶(4)磷酸葡萄糖变位酶(5)磷酸己糖异构酶(6)己糖激酶(7)葡萄糖-6-磷酸酶(8)淀粉-1,6-葡萄糖苷酶(脱支酶)(9)磷酸化酶(10)磷酸葡萄糖变位酶;糖(11)磷酸己糖异构酶(12)磷酸果糖激酶GSD目前分为11型。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "糖原" }, { "start_idx": 20, "end_idx": 37, "type": "bod", "entity": "淀粉-(1,4→1,6)转葡萄糖苷酶" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "糖原合成酶" }, { "start_idx": 49, "end_idx": 61, "type": "bod", "entity": "尿苷二磷酸葡萄糖焦磷酸化酶" }, { "start_idx": 65, "end_idx": 72, "type": "bod", "entity": "磷酸葡萄糖变位酶" }, { "start_idx": 76, "end_idx": 82, "type": "bod", "entity": "磷酸己糖异构酶" }, { "start_idx": 86, "end_idx": 89, "type": "bod", "entity": "己糖激酶" }, { "start_idx": 93, "end_idx": 101, "type": "bod", "entity": "葡萄糖-6-磷酸酶" }, { "start_idx": 105, "end_idx": 116, "type": "bod", "entity": "淀粉-1,6-葡萄糖苷酶" }, { "start_idx": 118, "end_idx": 120, "type": "bod", "entity": "脱支酶" }, { "start_idx": 125, "end_idx": 128, "type": "bod", "entity": "磷酸化酶" }, { "start_idx": 133, "end_idx": 140, "type": "bod", "entity": "磷酸葡萄糖变位酶" }, { "start_idx": 142, "end_idx": 142, "type": "bod", "entity": "糖" }, { "start_idx": 147, "end_idx": 153, "type": "bod", "entity": "磷酸己糖异构酶" }, { "start_idx": 158, "end_idx": 163, "type": "bod", "entity": "磷酸果糖激酶" }, { "start_idx": 164, "end_idx": 166, "type": "dis", "entity": "GSD" } ] }, { "text": "另加O型即糖原合成酶缺乏共12型,其各型病因、受累组织、起病时间,及临床表现各不相同:GSDO型(aglycogenosis)糖原合成酶缺陷。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "糖原合成酶" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "糖原合成酶缺乏" }, { "start_idx": 43, "end_idx": 69, "type": "dis", "entity": "GSDO型(aglycogenosis)糖原合成酶缺陷" } ] }, { "text": "受累组织为肝脏肝糖原缺乏肝糖原合成酶活性小于2%进食或补充葡萄糖后长时间高血糖。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "受累组织为肝脏肝糖原缺乏" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肝脏" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肝糖原" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "肝糖原合成酶" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "肝糖原合成酶活性小于2%" }, { "start_idx": 24, "end_idx": 38, "type": "sym", "entity": "进食或补充葡萄糖后长时间高血糖" } ] }, { "text": "反复低血糖抽搐可导致智能落后。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "反复低血糖抽搐" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血糖" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "智能落后" } ] }, { "text": "GSDⅠa型(VonGierke病)葡萄糖-6-磷酸酶缺陷。", "entities": [ { "start_idx": 0, "end_idx": 28, "type": "dis", "entity": "GSDⅠa型(VonGierke病)葡萄糖-6-磷酸酶缺陷" } ] }, { "text": "受累组织为肝、肾和小肠低血糖症状,呼吸困难,乳酸性酸中毒、酮血症,高脂血症、高尿酸血症,血小板功能障碍有出血倾向。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "受累组织为肝、肾和小肠" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肝" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肾" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "小肠" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "低血糖症状" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "呼吸" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "乳酸性酸中毒" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "酮血症" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "高脂血症" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "高尿酸血症" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "血小板功能障碍" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "有出血倾向" } ] }, { "text": "患儿呈娃娃脸,肝脏、肾脏肿大身材矮小智能发育正常。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "娃娃脸" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肝脏" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "肝脏、肾脏肿大" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肾" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "身材矮小" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "智能发育正常" } ] }, { "text": "半乳糖和果糖不能转变成葡萄糖并能导致酸中毒。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "半乳糖" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "半乳糖和果糖不能转变成葡萄糖" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "果糖" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "酸中毒" } ] }, { "text": "注射肾上腺素或胰高肾上腺素后对血糖浓度无改血糖浓度儿茶酚胺正常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "注射" }, { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "胰高肾上腺素" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "血糖浓度" }, { "start_idx": 21, "end_idx": 28, "type": "ite", "entity": "血糖浓度儿茶酚胺" } ] }, { "text": "GSDⅠb型葡萄糖-6-磷酸转位酶缺陷,受累组织和临床表现同GSDⅠ型,并且有中性粒细胞减少。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "dis", "entity": "GSDⅠb型葡萄糖-6-磷酸转位酶缺陷" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "中性粒细胞减少" } ] }, { "text": "肌张力低下,肌肉松弛,中度肝脏肿大,高脂血症育正常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肌张力" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肌肉" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "肌肉松弛" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "中度肝脏肿大" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肝脏" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "高脂血症" } ] }, { "text": "青少年型和成人型起病晚,以乏力,肌张力低下为主,可无心脏侵及。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "乏力" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "肌张力" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心脏" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "心脏侵及" } ] }, { "text": "GSDⅢ型淀粉-1,6-葡萄糖苷酶缺陷。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "dis", "entity": "GSDⅢ型淀粉-1,6-葡萄糖苷酶缺陷" } ] }, { "text": "受累组织为肝脏、Ⅰ型糖原累积病,有不同受累器官组合形式。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "受累组织为肝脏" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肝脏" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "Ⅰ型糖原累积病" } ] }, { "text": "无酮血症和高血脂,进食后肾上腺素或胰高痛风发作验有效应,智能发育正常,肝脏及心脏功能衰竭少见,尿儿茶酚胺浓度正常,预后好。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "酮血症" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "高血脂" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "进食后肾上腺素或胰高" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胰高" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "痛风发作" }, { "start_idx": 35, "end_idx": 43, "type": "sym", "entity": "肝脏及心脏功能衰竭" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "心脏功能衰竭" }, { "start_idx": 47, "end_idx": 53, "type": "ite", "entity": "尿儿茶酚胺浓度" } ] }, { "text": "受累组织主要为肝脏;心脏及肌肉糖原累积病。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肝脏" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肌肉" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "糖原累积病" } ] }, { "text": "症状与GSDⅦ型类似,智能发育正常,预后好。", "entities": [] }, { "text": "乳酸和尿酸水平正常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "乳酸" }, { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "尿酸" } ] }, { "text": "心肌和骨骼肌不受累。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心肌" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "骨骼肌" } ] }, { "text": "肝大和生长迟缓随着年龄增长而改善,一般在青春期症状消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "肝大" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肝" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "生长迟缓" } ] }, { "text": "GSDⅧ型酶缺陷不明,肝脏磷酸酶为无活性形式。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "GSDⅧ型酶缺陷" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "肝脏磷酸酶" }, { "start_idx": 11, "end_idx": 21, "type": "sym", "entity": "肝脏磷酸酶为无活性形式" } ] }, { "text": "肝、脑及骨骼肌正常。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肝" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "脑" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "骨骼肌" } ] }, { "text": "临床症状和体征:肝肿大、躯体运动失调,眼球震颤,逐渐出现神经系统退行性变化、痉挛,直至死亡。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肝" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "肝肿大" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "躯体" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "躯体运动失调" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "眼球" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "眼球震颤" }, { "start_idx": 24, "end_idx": 39, "type": "sym", "entity": "逐渐出现神经系统退行性变化、痉挛" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "神经系统" } ] }, { "text": "在疾病急性发作期尿儿茶酚胺排量增加。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "尿儿茶酚胺排量增加" }, { "start_idx": 8, "end_idx": 14, "type": "ite", "entity": "尿儿茶酚胺排量" } ] }, { "text": "GSDⅨ型肝脏磷酸化酶激酶缺陷(由4个不同的亚单位组成,包括PHKA2、PHKB、PHKG2及CALM1)。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "dis", "entity": "GSDⅨ型肝脏磷酸化酶激酶缺陷" } ] }, { "text": "临床症状和体征:肝脏明显肿大,脾脏不大。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肝脏" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "肝脏明显肿大" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脾脏" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "脾脏不大" } ] }, { "text": "无低血糖及酸中毒。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "低血糖" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "酸中毒" } ] }, { "text": "尿儿茶酚胺正常,注射肾上腺素或胰高血糖素血糖反应正常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "尿儿茶酚胺" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "胰高血糖素" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "血糖" } ] }, { "text": "GSDⅩ型肌肉磷酸甘油变位酶缺陷。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "dis", "entity": "GSDⅩ型肌肉磷酸甘油变位酶缺陷" } ] }, { "text": "GSDⅪ型乳酸脱氢酶A缺乏,受累组织为肝脏及肾脏。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "GSDⅪ型乳酸脱氢酶A缺乏" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肝脏" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肾脏" } ] }, { "text": "对肾上腺素和胰高血糖素无反应。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "对肾上腺素和胰高血糖素无反应" }, { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "胰高血糖素" } ] }, { "text": "注射胰高血糖素后尿环化3'5'-AMP排量增高。", "entities": [ { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "注射胰高血糖素后尿环化3'5'-AMP排量增高" }, { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "胰高血糖素" }, { "start_idx": 8, "end_idx": 18, "type": "ite", "entity": "尿环化3'5'-AMP" } ] }, { "text": "【实验室检查】1.生化异常包括低血糖、酮症酸中毒、乳酸血症、高尿酸血症及高脂血症,肝功能多数有改变。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "生化异常" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "低血糖" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "酮症酸中毒" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "乳酸血症" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "高尿酸血症" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "高脂血症" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "肝功能多数有改变" } ] }, { "text": "2.糖耐量试验呈现典型的糖尿病特征。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "糖耐量试验" }, { "start_idx": 2, "end_idx": 16, "type": "sym", "entity": "糖耐量试验呈现典型的糖尿病特征" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "糖尿病" } ] }, { "text": "3.肾上腺素试验皮下注射1∶1000肾上腺素0.02ml/kg,注射后0min、10min、20min、30min、40min、50min及60min分别测定血糖,正常者血糖上升40%~60%;患者血糖无明显上升。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "肾上腺素试验" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "皮下注射" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 79, "end_idx": 80, "type": "ite", "entity": "血糖" }, { "start_idx": 85, "end_idx": 86, "type": "ite", "entity": "血糖" }, { "start_idx": 99, "end_idx": 100, "type": "ite", "entity": "血糖" } ] }, { "text": "4.胰高血糖素试验肌注胰高血糖素30μg/kg(最大量1mg),于注射后0min、15min、30min、45min、60min、90min及120min取血测血糖。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "胰高血糖素试验" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "肌注" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "胰高血糖素" }, { "start_idx": 77, "end_idx": 81, "type": "pro", "entity": "取血测血糖" } ] }, { "text": "正常时在15~45min内血糖可升高1.5~2.8mmol/L,患者血糖升高不明显。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "血糖" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "血糖" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "血糖升高不明显" } ] }, { "text": "5.肝组织活体检查和酶活性测定肝组织糖原染色见糖原增多,但无法分型。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "肝组织活体检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "酶活性测定" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "肝组织糖原染色" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "肝组织糖原染色见糖原增多" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "糖原" } ] }, { "text": "特异性酶活性可降低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "特异性酶" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "特异性酶活性可降低" } ] }, { "text": "准确分型需进行酶学测定和基因诊断。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "酶学测定" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "基因诊断" } ] }, { "text": "【治疗】1.本病治疗首先应维持患者正常的血糖水平,防止低血糖,从而减轻临床症状。", "entities": [ { "start_idx": 13, "end_idx": 23, "type": "pro", "entity": "维持患者正常的血糖水平" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "低血糖" } ] }, { "text": "静脉给予葡萄糖0.5g/(kg•h),然后进行调整,以维持血糖在4~5mmol/L。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉给予" }, { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "血糖" } ] }, { "text": "服用时生玉米淀粉以1∶2比例与凉白开水混合(不要开水冲服),不宜加葡萄糖。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "pro", "entity": "服用时生玉米淀粉以1∶2比例与凉白开水混合" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "服用生玉米淀粉可防治低血糖和高乳酸血症。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "服用生玉米淀粉" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "低血糖" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "高乳酸血症" } ] }, { "text": "4.报道对严重肝损者肝移植可使Ⅰa型患者有正常的代谢平衡,有利于生长,改善生活质量。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "严重肝损" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "肝移植" } ] }, { "text": "但肝移植有较大风险。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "肝移植" } ] }, { "text": "5.对运动后骨骼肌疼痛者应避免剧烈运动。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "运动后骨骼肌疼痛" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "避免剧烈运动" } ] }, { "text": "一般来说,Ⅰ型糖原累积病较难处理;年龄越小、症状越重,其预后差,常因感染及酸中毒而使病情进展迅速。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "Ⅰ型糖原累积病" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "感染" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "酸中毒" } ] }, { "text": "有的患者并发心脏扩大,以后死于心力衰竭。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "心脏" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "国外研究曾随访调查43例Ⅰ型和Ⅲ型糖原累积病,发现51.8%Ⅰ型患者及25%Ⅲ型患者以后发生肝脏肿瘤,这些患者血清α-甲胎蛋白水平明显增高。", "entities": [ { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "Ⅰ型和Ⅲ型糖原累积病" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "肝脏肿瘤" }, { "start_idx": 55, "end_idx": 62, "type": "ite", "entity": "血清α-甲胎蛋白" }, { "start_idx": 55, "end_idx": 68, "type": "sym", "entity": "血清α-甲胎蛋白水平明显增高" } ] }, { "text": "家庭如需生育第二胎,可进行遗传咨询,或做产前DNA诊断。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "遗传咨询" }, { "start_idx": 20, "end_idx": 26, "type": "pro", "entity": "产前DNA诊断" } ] }, { "text": "第三节原发性肾病综合征肾病综合征(nephroticsyndrome,NS)简称肾病,系指由多种原因引起肾小球基底膜通透性增高导致大量蛋白丢失,从而出现低蛋白血症、高度水肿和高胆固醇血症的一组临床综合征。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "原发性肾病综合征" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 17, "end_idx": 33, "type": "dis", "entity": "nephroticsyndrome" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "NS" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "肾病" }, { "start_idx": 52, "end_idx": 62, "type": "sym", "entity": "肾小球基底膜通透性增高" }, { "start_idx": 52, "end_idx": 57, "type": "bod", "entity": "肾小球基底膜" }, { "start_idx": 65, "end_idx": 70, "type": "sym", "entity": "大量蛋白丢失" }, { "start_idx": 76, "end_idx": 80, "type": "sym", "entity": "低蛋白血症" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "高度水肿" }, { "start_idx": 87, "end_idx": 92, "type": "sym", "entity": "高胆固醇血症" } ] }, { "text": "我国各地区协作调查统计,原发性肾病综合征约占儿科泌尿系统住院病人的21%(1982年)和31%(1992年),是儿科最常见的肾脏疾病之一。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "原发性肾病综合征" }, { "start_idx": 22, "end_idx": 23, "type": "dep", "entity": "儿科" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 56, "end_idx": 57, "type": "dep", "entity": "儿科" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "肾脏疾病" } ] }, { "text": "【病因】肾病综合征按病因可分为原发性、继发性及先天性三种,原发性肾病综合征占90%以上,其次为各种继发性肾病综合征,先天性肾病综合征极为罕见。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "原发性肾病综合征" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "继发性肾病综合征" }, { "start_idx": 58, "end_idx": 65, "type": "dis", "entity": "先天性肾病综合征" } ] }, { "text": "(一)感染各种细菌(链球菌感染后肾炎及葡萄球菌感染后肾炎等)、病毒(HBV相关性肾炎、HIV相关性肾炎及HCV相关性肾炎)、寄生虫(疟疾、血吸虫及丝虫)、支原体、梅毒以及麻风等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "感染" }, { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "细菌" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "链球菌感染后肾炎" }, { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "葡萄球菌感染后肾炎" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "HBV相关性肾炎" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "HIV相关性肾炎" }, { "start_idx": 52, "end_idx": 59, "type": "dis", "entity": "HCV相关性肾炎" }, { "start_idx": 62, "end_idx": 64, "type": "mic", "entity": "寄生虫" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "疟疾" }, { "start_idx": 69, "end_idx": 71, "type": "mic", "entity": "血吸虫" }, { "start_idx": 73, "end_idx": 74, "type": "mic", "entity": "丝虫" }, { "start_idx": 77, "end_idx": 79, "type": "mic", "entity": "支原体" }, { "start_idx": 81, "end_idx": 82, "type": "mic", "entity": "梅毒" }, { "start_idx": 85, "end_idx": 86, "type": "mic", "entity": "麻风" } ] }, { "text": "(二)药物、中毒、过敏药物有含金属有机、无机物(有机汞及元素汞)、青霉胺、海洛因、非类固醇类抗炎药、丙磺舒、卡托普利、三甲双酮、甲妥因、高氯酸盐、抗蛇毒素及造影剂;中毒及过敏因素则有蜂蛰、蛇毒、花粉、血清及预防接种等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "中毒" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "过敏" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "青霉胺" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "海洛因" }, { "start_idx": 41, "end_idx": 48, "type": "dru", "entity": "非类固醇类抗炎药" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "丙磺舒" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "卡托普利" }, { "start_idx": 59, "end_idx": 62, "type": "dru", "entity": "三甲双酮" }, { "start_idx": 64, "end_idx": 66, "type": "dru", "entity": "甲妥因" }, { "start_idx": 68, "end_idx": 71, "type": "dru", "entity": "高氯酸盐" }, { "start_idx": 73, "end_idx": 76, "type": "dru", "entity": "抗蛇毒素" }, { "start_idx": 78, "end_idx": 80, "type": "dru", "entity": "造影剂" }, { "start_idx": 82, "end_idx": 83, "type": "dis", "entity": "中毒" }, { "start_idx": 85, "end_idx": 86, "type": "sym", "entity": "过敏" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "血清" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "预防接种" } ] }, { "text": "(三)全身性系统性疾病包括系统性红斑狼疮、过敏性、疱疹性皮炎、淀粉样变性、类肉瘤病、Sjögren综合征、类风湿性关节炎及混合性结缔组织病等。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "全身性系统性疾病" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "过敏性、疱疹性皮炎" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "淀粉样变性" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "类肉瘤病" }, { "start_idx": 42, "end_idx": 51, "type": "dis", "entity": "Sjögren综合征" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "类风湿性关节炎" }, { "start_idx": 61, "end_idx": 68, "type": "dis", "entity": "混合性结缔组织病" } ] }, { "text": "(四)肿瘤恶性肿瘤特别是淋巴细胞恶性肿瘤易诱发肾病综合征,包括霍奇金病、非霍奇金淋巴瘤、白血病、Wilm瘤、黑色素瘤、多发性骨髓瘤以及肺透明细胞癌等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "淋巴细胞恶性肿瘤" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "非霍奇金淋巴瘤" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "白血病" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "Wilm瘤" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "黑色素瘤" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "多发性骨髓瘤" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "肺透明细胞癌" } ] }, { "text": "(五)遗传性疾病Alport综合征、指甲-髌骨综合征、Fabry病、镰状红细胞贫血、胱氨酸病、Jenue综合征及抗胰蛋白酶缺乏等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "Alport综合征" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "指甲-髌骨综合征" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "Fabry病" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "镰状红细胞贫血" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "胱氨酸病" }, { "start_idx": 47, "end_idx": 54, "type": "dis", "entity": "Jenue综合征" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "抗胰蛋白酶缺乏" } ] }, { "text": "(六)代谢及内分泌疾病糖尿病、桥本甲状腺炎及淀粉样变性等。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "代谢及内分泌疾病" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "糖尿病" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "桥本甲状腺炎" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "淀粉样变性" } ] }, { "text": "(七)其他高血压、恶性肾小球硬化及肾移植慢性排斥反应等。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "高血压" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "恶性肾小球硬化" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "肾移植慢性排斥反应" } ] }, { "text": "【病理】尽管有些肾间质小管疾病累及肾小球后可出现大量蛋白并达到肾病综合征标准,但绝大多数原发或继发肾病综合征都是以肾小球病变为主,并可分别根据光镜下的肾小球病变而作病理分型,主要有5种病理类型:微小病变肾病(minimalchangenephropathy,MCN)、系膜增生性肾炎(mesangialproliferativeglomerulonephritis,MsPGN)、局灶节段性肾小球硬化(focalsegmentalglomerulosclerosis,FSGS)、膜性肾病(membraneousnephropathy,MN)和膜增生性肾炎(membranoproliferativeglomerulonephritis,MPGN)。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "肾间质小管疾病" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肾小球" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "大量蛋白" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 44, "end_idx": 53, "type": "dis", "entity": "原发或继发肾病综合征" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "肾小球病变" }, { "start_idx": 71, "end_idx": 72, "type": "equ", "entity": "光镜" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "肾小球病变" }, { "start_idx": 97, "end_idx": 102, "type": "dis", "entity": "微小病变肾病" }, { "start_idx": 104, "end_idx": 127, "type": "dis", "entity": "minimalchangenephropathy" }, { "start_idx": 129, "end_idx": 131, "type": "dis", "entity": "MCN" }, { "start_idx": 134, "end_idx": 140, "type": "dis", "entity": "系膜增生性肾炎" }, { "start_idx": 142, "end_idx": 181, "type": "dis", "entity": "mesangialproliferativeglomerulonephritis" }, { "start_idx": 183, "end_idx": 187, "type": "dis", "entity": "MsPGN" }, { "start_idx": 190, "end_idx": 199, "type": "dis", "entity": "局灶节段性肾小球硬化" }, { "start_idx": 201, "end_idx": 232, "type": "dis", "entity": "focalsegmentalglomerulosclerosis" }, { "start_idx": 234, "end_idx": 237, "type": "dis", "entity": "FSGS" }, { "start_idx": 240, "end_idx": 243, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 245, "end_idx": 266, "type": "dis", "entity": "membraneousnephropathy" }, { "start_idx": 268, "end_idx": 269, "type": "dis", "entity": "MN" }, { "start_idx": 272, "end_idx": 277, "type": "dis", "entity": "膜增生性肾炎" }, { "start_idx": 279, "end_idx": 317, "type": "dis", "entity": "membranoproliferativeglomerulonephritis" }, { "start_idx": 319, "end_idx": 322, "type": "dis", "entity": "MPGN" } ] }, { "text": "我国于1996年报告全国20家医院699例儿童肾病综合征肾活体组织检查检中MCN占18.7%,MsPGN占37.8%,FSGS为11.6%、MN为6.0%、MPGN为5.5%,余为轻微病变等其他类型。", "entities": [ { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "儿童肾病综合征" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "MCN" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "MsPGN" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "FSGS" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "MN" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "MPGN" }, { "start_idx": 90, "end_idx": 93, "type": "dis", "entity": "轻微病变" } ] }, { "text": "但这些比例受病人来源影响,且均为非选择性肾活体组织检查,因而难以准确反映其实际分布情况,国外有人对596例非选择性儿童肾病综合征病例做病理检查发现MCN占77.8%、MsPGN2.7%、FSGS6.7%、MN1.3%、MPGN6.7%,因此MCN为儿童肾病最主要的病理类型。", "entities": [ { "start_idx": 16, "end_idx": 26, "type": "pro", "entity": "非选择性肾活体组织检查" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "儿童肾病综合征" }, { "start_idx": 67, "end_idx": 70, "type": "pro", "entity": "病理检查" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "MCN" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "MsPGN" }, { "start_idx": 93, "end_idx": 96, "type": "dis", "entity": "FSGS" }, { "start_idx": 102, "end_idx": 103, "type": "dis", "entity": "MN" }, { "start_idx": 109, "end_idx": 112, "type": "dis", "entity": "MPGN" }, { "start_idx": 120, "end_idx": 122, "type": "dis", "entity": "MCN" }, { "start_idx": 124, "end_idx": 127, "type": "dis", "entity": "儿童肾病" } ] }, { "text": "【发病机制】本病的发病机制尚未完全明了,一般认为蛋白尿是由于肾小球细小血管壁电荷屏障和/或筛屏障的破坏所致。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "蛋白" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "尿" }, { "start_idx": 30, "end_idx": 50, "type": "sym", "entity": "肾小球细小血管壁电荷屏障和/或筛屏障的破坏" }, { "start_idx": 30, "end_idx": 37, "type": "bod", "entity": "肾小球细小血管壁" } ] }, { "text": "正常肾小球滤过膜带负电荷,电荷屏障由基底膜上的固定阴离子位点(主要为硫酸肝素多糖)及内皮、上皮细胞表面的多阴离子(主要为涎酸蛋白)所组成。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "肾小球滤过膜" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "电荷屏障" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "基底膜" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "硫酸肝素多糖" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "内皮、上皮细胞" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "多阴离子" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "涎酸蛋白" } ] }, { "text": "筛屏障则由滤过膜内侧的内皮细胞窗孔、基底膜及上皮细胞裂孔膜组成,其中基底膜起主要作用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "筛屏障" }, { "start_idx": 5, "end_idx": 20, "type": "bod", "entity": "滤过膜内侧的内皮细胞窗孔、基底膜" }, { "start_idx": 22, "end_idx": 28, "type": "bod", "entity": "上皮细胞裂孔膜" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "基底膜" } ] }, { "text": "非微小病变型肾病综合征通过免疫反应,激活补体及凝血、纤溶系统,以及基质金属蛋白酶而损伤基底膜,导致筛屏障的破坏,出现非选择性蛋白尿。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "非微小病变型肾病综合征" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "补体" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "凝血、纤溶系统" }, { "start_idx": 33, "end_idx": 39, "type": "bod", "entity": "基质金属蛋白酶" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "损伤基底膜" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "基底膜" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "导致筛屏障的破坏" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "筛屏障" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "非选择性蛋白尿" } ] }, { "text": "而且,其也可通过非免疫机制,如血压增高、血糖增高或由于基底膜结构缺陷而破坏筛屏障,出现蛋白尿。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "血压增高" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "血糖增高" }, { "start_idx": 27, "end_idx": 39, "type": "sym", "entity": "基底膜结构缺陷而破坏筛屏障" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "基底膜" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "出现蛋白尿" } ] }, { "text": "微小病变型肾病综合征可能与细胞免疫紊乱,特别是T细胞免疫功能紊乱有关,其依据在于:①MCN肾组织中无免疫球蛋白及补体沉积;②T细胞数降低,CD4/CD8比例失衡,Ts活性增高,淋巴细胞转化率降低,PHA皮试反应降低;③抑制T细胞的病毒感染可诱导本病缓解;④出现T细胞功能异常的疾病如霍奇金病可导致MCN;⑤抑制T细胞的皮质激素及免疫抑制剂可诱导本病缓解。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "微小病变型肾病综合征" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "细胞免疫紊乱" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "T细胞免疫功能紊乱" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "MCN" }, { "start_idx": 45, "end_idx": 59, "type": "sym", "entity": "肾组织中无免疫球蛋白及补体沉积" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "肾组织" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "补体" }, { "start_idx": 62, "end_idx": 67, "type": "sym", "entity": "T细胞数降低" }, { "start_idx": 62, "end_idx": 65, "type": "ite", "entity": "T细胞数" }, { "start_idx": 69, "end_idx": 79, "type": "sym", "entity": "CD4/CD8比例失衡" }, { "start_idx": 69, "end_idx": 75, "type": "ite", "entity": "CD4/CD8" }, { "start_idx": 81, "end_idx": 86, "type": "sym", "entity": "Ts活性增高" }, { "start_idx": 81, "end_idx": 82, "type": "ite", "entity": "Ts" }, { "start_idx": 88, "end_idx": 96, "type": "sym", "entity": "淋巴细胞转化率降低" }, { "start_idx": 88, "end_idx": 94, "type": "ite", "entity": "淋巴细胞转化率" }, { "start_idx": 98, "end_idx": 106, "type": "sym", "entity": "PHA皮试反应降低" }, { "start_idx": 98, "end_idx": 102, "type": "pro", "entity": "PHA皮试" }, { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "T细胞" }, { "start_idx": 115, "end_idx": 118, "type": "dis", "entity": "病毒感染" }, { "start_idx": 130, "end_idx": 139, "type": "dis", "entity": "T细胞功能异常的疾病" }, { "start_idx": 141, "end_idx": 144, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 148, "end_idx": 150, "type": "dis", "entity": "MCN" }, { "start_idx": 155, "end_idx": 157, "type": "bod", "entity": "T细胞" }, { "start_idx": 159, "end_idx": 162, "type": "dru", "entity": "皮质激素" }, { "start_idx": 164, "end_idx": 168, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "尽管肾病状态下血生化及内分泌改变也有可能诱导免疫抑制状态的产生,但这些改变主要见于MCN,而在非微小病变型肾病综合征中少见,说明这种免疫紊乱更可能是原因,而非肾病状态的结果。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肾病" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "血生化及内分泌改变" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "血生化" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "内分泌" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "免疫抑制状态" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "MCN" }, { "start_idx": 47, "end_idx": 57, "type": "dis", "entity": "非微小病变型肾病综合征" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "免疫紊乱" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "肾病" } ] }, { "text": "MCN免疫紊乱如何导致蛋白尿的产生?", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "MCN免疫紊乱" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "蛋白" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" } ] }, { "text": "现已发现:①淋巴细胞可产生一种29kd的多肽,其可导致肾小球滤过膜多阴离子减少,而出现蛋白尿;②刀豆素(ConA)刺激下的淋巴细胞可产生60~160kd的肾小球通透因子(glomerularpermeabilityfactor,GPF),GPF可直接引起蛋白尿;③淋巴细胞还可通过分泌12~18kd的可溶免疫反应因子(solubleimmuneresponsesuppressor,SIRS)而导致蛋白尿。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "29kd" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "多肽" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "肾小球滤过膜" }, { "start_idx": 27, "end_idx": 38, "type": "sym", "entity": "肾小球滤过膜多阴离子减少" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "出现蛋白尿" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "尿" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "刀豆素" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "ConA" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 77, "end_idx": 83, "type": "bod", "entity": "肾小球通透因子" }, { "start_idx": 85, "end_idx": 112, "type": "bod", "entity": "glomerularpermeabilityfactor" }, { "start_idx": 114, "end_idx": 116, "type": "bod", "entity": "GPF" }, { "start_idx": 119, "end_idx": 121, "type": "bod", "entity": "GPF" }, { "start_idx": 127, "end_idx": 129, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 129, "end_idx": 129, "type": "bod", "entity": "尿" }, { "start_idx": 132, "end_idx": 135, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 150, "end_idx": 157, "type": "bod", "entity": "可溶免疫反应因子" }, { "start_idx": 159, "end_idx": 189, "type": "bod", "entity": "solubleimmuneresponsesuppressor" }, { "start_idx": 191, "end_idx": 194, "type": "bod", "entity": "SIRS" }, { "start_idx": 199, "end_idx": 201, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 201, "end_idx": 201, "type": "bod", "entity": "尿" } ] }, { "text": "【病理生理】(一)大量蛋白尿为最根本的病理生理改变,也是导致本征其他三大特点的根本原因。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "蛋白" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" } ] }, { "text": "由于肾小球滤过膜受免疫或其他病因的损伤,电荷屏障或/和分子筛的屏障作用减弱,血浆蛋白大量漏入尿中。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "肾小球滤过膜" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "损伤" }, { "start_idx": 20, "end_idx": 36, "type": "sym", "entity": "电荷屏障或/和分子筛的屏障作用减弱" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "血浆蛋白大量漏入尿中" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "血浆蛋白" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "尿中" } ] }, { "text": "近年还注意到其他蛋白成分的丢失,及其造成的相应后果,如:①多种微量元素的载体蛋白,如转铁蛋白丢失致小细胞低色素性贫血,锌结合蛋白丢失致体内锌不足;②多种激素的结合蛋白,如25-羟骨化醇结合蛋白由尿中丢失致钙代谢紊乱,甲状腺素结合蛋白丢失导致T3、T4下降;③免疫球蛋白IgG、IgA及B因子、补体成分的丢失致抗感染力下降;④抗凝血酶Ⅲ、Ⅹ、Ⅺ因子及前列腺素结合蛋白丢失导致高凝及血栓形成。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "其他蛋白成分" }, { "start_idx": 29, "end_idx": 39, "type": "bod", "entity": "多种微量元素的载体蛋白" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 49, "end_idx": 57, "type": "dis", "entity": "小细胞低色素性贫血" }, { "start_idx": 59, "end_idx": 65, "type": "sym", "entity": "锌结合蛋白丢失" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "锌结合蛋白" }, { "start_idx": 67, "end_idx": 71, "type": "sym", "entity": "体内锌不足" }, { "start_idx": 69, "end_idx": 69, "type": "bod", "entity": "锌" }, { "start_idx": 74, "end_idx": 82, "type": "bod", "entity": "多种激素的结合蛋白" }, { "start_idx": 85, "end_idx": 100, "type": "sym", "entity": "25-羟骨化醇结合蛋白由尿中丢失" }, { "start_idx": 85, "end_idx": 95, "type": "bod", "entity": "25-羟骨化醇结合蛋白" }, { "start_idx": 102, "end_idx": 106, "type": "dis", "entity": "钙代谢紊乱" }, { "start_idx": 108, "end_idx": 117, "type": "sym", "entity": "甲状腺素结合蛋白丢失" }, { "start_idx": 108, "end_idx": 115, "type": "bod", "entity": "甲状腺素结合蛋白" }, { "start_idx": 120, "end_idx": 138, "type": "sym", "entity": "T3、T4下降" }, { "start_idx": 120, "end_idx": 127, "type": "bod", "entity": "T3" }, { "start_idx": 129, "end_idx": 136, "type": "bod", "entity": "T4" }, { "start_idx": 141, "end_idx": 164, "type": "sym", "entity": "免疫球蛋白IgG、IgA及B因子、补体成分的丢失" }, { "start_idx": 141, "end_idx": 148, "type": "bod", "entity": "免疫球蛋白IgG" }, { "start_idx": 150, "end_idx": 152, "type": "bod", "entity": "IgA" }, { "start_idx": 154, "end_idx": 156, "type": "bod", "entity": "B因子" }, { "start_idx": 158, "end_idx": 161, "type": "bod", "entity": "补体成分" }, { "start_idx": 166, "end_idx": 171, "type": "sym", "entity": "抗感染力下降" }, { "start_idx": 174, "end_idx": 195, "type": "sym", "entity": "抗凝血酶Ⅲ、Ⅹ、Ⅺ因子及前列腺素结合蛋白丢失" }, { "start_idx": 174, "end_idx": 178, "type": "bod", "entity": "抗凝血酶Ⅲ" }, { "start_idx": 180, "end_idx": 184, "type": "bod", "entity": "Ⅹ、Ⅺ因子" }, { "start_idx": 186, "end_idx": 193, "type": "bod", "entity": "前列腺素结合蛋白" }, { "start_idx": 198, "end_idx": 199, "type": "sym", "entity": "高凝" }, { "start_idx": 201, "end_idx": 204, "type": "sym", "entity": "血栓形成" }, { "start_idx": 201, "end_idx": 202, "type": "bod", "entity": "血栓" } ] }, { "text": "此外,肾小球上皮细胞及近端小管上皮细胞可胞饮白蛋白并对其进行降解,如果蛋白过载可导致肾小球上皮细胞及小管上皮细胞功能受损,这可能与疾病进展及治疗反应减低有关。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "肾小球上皮细胞" }, { "start_idx": 11, "end_idx": 18, "type": "bod", "entity": "近端小管上皮细胞" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "白蛋白" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "蛋白过载" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "蛋白" }, { "start_idx": 42, "end_idx": 59, "type": "sym", "entity": "肾小球上皮细胞及小管上皮细胞功能受损" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "肾小球上皮细胞" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "小管上皮细胞" } ] }, { "text": "(二)低白蛋白血症大量血浆白蛋白自尿中丢失是低白蛋白血症的主要原因;蛋白质分解的增加,为次要原因。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "低白蛋白血症" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "大量血浆白蛋白自尿中丢失" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "血浆白蛋白" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "尿" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "低白蛋白血症" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "蛋白质" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "蛋白质分解的增加" } ] }, { "text": "低白蛋白血症是病理生理改变中的关键环节,对机体内环境(尤其是渗透压和血容量)的稳定及多种物质代谢可产生多方面的影响。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "低白蛋白血症" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "渗透压" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "血容量" } ] }, { "text": "当血白蛋白低于25g/L时可出现水肿;同时因血容量减少,在并发大量体液丢失时极易诱发低血容量性休克。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "血白蛋白" }, { "start_idx": 1, "end_idx": 11, "type": "sym", "entity": "血白蛋白低于25g/L" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "水肿" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "血容量" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "血容量减少" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "大量体液丢失" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "体液" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "低血容量性休克" } ] }, { "text": "此外低白蛋白血症还可影响脂类代谢。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "低白蛋白血症" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脂类" } ] }, { "text": "(三)高胆固醇血症可能由于低蛋白血症致肝脏代偿性白蛋白合成增加,有些脂蛋白与白蛋白经共同合成途径而合成增加,再加上脂蛋白脂酶活力下降等因素而出现高脂血症。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "高胆固醇血症" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "低蛋白血症" }, { "start_idx": 19, "end_idx": 30, "type": "sym", "entity": "肝脏代偿性白蛋白合成增加" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肝脏" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "白蛋白" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "脂蛋白" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "白蛋白" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "脂蛋白脂酶" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "脂蛋白脂酶活力下降" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "高脂血症" } ] }, { "text": "一般血浆白蛋白<30g/L,即出现血胆固醇增高,如白蛋白进一步降低,则甘油三酯也增高。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "sym", "entity": "血浆白蛋白<30g/L" }, { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "血浆白蛋白" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "血胆固醇增高" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "血胆固醇" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "白蛋白进一步降低" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "白蛋白" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "甘油三酯也增高" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "甘油三酯" } ] }, { "text": "(四)水肿肾病综合征时水肿机制尚未完全阐明,可能机制:①由于血浆白蛋白下降,血浆胶体渗透压降低,血浆中水分由血管内转入组织间隙直接形成水肿;②水分外渗致血容量下降,通过容量和压力感受器使体内神经体液因子发生变化(如抗利尿激素、醛固酮及利钠因子等),引起水钠潴留而导致全身水肿;③低血容量使交感神经兴奋性增高,近端小管重吸收钠增多,加重水钠潴留;④其他肾内原因导致肾近曲小管回吸收钠增多。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "水肿" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "水肿" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "血浆白蛋白下降" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "血浆白蛋白" }, { "start_idx": 38, "end_idx": 46, "type": "sym", "entity": "血浆胶体渗透压降低" }, { "start_idx": 38, "end_idx": 44, "type": "ite", "entity": "血浆胶体渗透压" }, { "start_idx": 48, "end_idx": 62, "type": "sym", "entity": "血浆中水分由血管内转入组织间隙" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "血浆中水分" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "血管" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "组织间隙" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "水肿" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "水分外渗" }, { "start_idx": 76, "end_idx": 80, "type": "sym", "entity": "血容量下降" }, { "start_idx": 76, "end_idx": 78, "type": "ite", "entity": "血容量" }, { "start_idx": 84, "end_idx": 91, "type": "bod", "entity": "容量和压力感受器" }, { "start_idx": 93, "end_idx": 104, "type": "sym", "entity": "体内神经体液因子发生变化" }, { "start_idx": 93, "end_idx": 100, "type": "bod", "entity": "体内神经体液因子" }, { "start_idx": 107, "end_idx": 111, "type": "bod", "entity": "抗利尿激素" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "醛固酮" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "利钠因子" }, { "start_idx": 126, "end_idx": 129, "type": "sym", "entity": "水钠潴留" }, { "start_idx": 133, "end_idx": 136, "type": "sym", "entity": "全身水肿" }, { "start_idx": 133, "end_idx": 134, "type": "bod", "entity": "全身" }, { "start_idx": 139, "end_idx": 152, "type": "sym", "entity": "低血容量使交感神经兴奋性增高" }, { "start_idx": 144, "end_idx": 147, "type": "bod", "entity": "交感神经" }, { "start_idx": 154, "end_idx": 163, "type": "sym", "entity": "近端小管重吸收钠增多" }, { "start_idx": 154, "end_idx": 157, "type": "bod", "entity": "近端小管" }, { "start_idx": 165, "end_idx": 170, "type": "sym", "entity": "加重水钠潴留" }, { "start_idx": 175, "end_idx": 176, "type": "bod", "entity": "肾内" }, { "start_idx": 181, "end_idx": 191, "type": "sym", "entity": "肾近曲小管回吸收钠增多" }, { "start_idx": 181, "end_idx": 185, "type": "bod", "entity": "肾近曲小管" }, { "start_idx": 189, "end_idx": 189, "type": "bod", "entity": "钠" } ] }, { "text": "因此肾病综合征的水肿可能是上述诸多因素共同作用的结果,而且在不同的病人,不同病期也可能有所不同。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "水肿" } ] }, { "text": "【临床表现】(一)症状与体征1.起病多隐匿起病,诱因不明确,有诱因者往往为上呼吸道感染、肠炎、皮肤感染或各种过敏等。", "entities": [ { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "肠炎" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "过敏" } ] }, { "text": "2.发病年龄与病因有关,先天性肾病一般在生后不久(3~6个月内)发病;原发性肾病综合征可见于婴幼儿期、学龄前期及学龄期,其中微小病变多在2~5岁发病,而继发于结缔组织病的肾病综合征主要见于年长儿。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "先天性肾病" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "原发性肾病综合征" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "微小病变" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "3.水肿呈凹陷性,多见于颜面及下肢,严重者伴腹水、胸水及阴囊水肿。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "水肿呈凹陷性" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "多见于颜面及下肢" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "颜面" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "下肢" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "腹水" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "腹" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "胸水" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "胸" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "阴囊水肿" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "阴囊" } ] }, { "text": "单纯性肾病水肿尤剧,而许多肾炎性肾病往往水肿较轻。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "单纯性肾病" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "水肿尤剧" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肾炎性肾病" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "水肿较轻" } ] }, { "text": "4.蛋白尿大量蛋白尿是肾病综合征的必备条件,其标准为:①2周连续3次定性≥+++;②定量≥50~100mg/(kg•d);③国际小儿肾脏病学会(ISKDC)建议>40mg/(m2•h);④婴幼儿难以收集24小时尿,Mendoza建议任意一次尿蛋白/肌酐>2.0。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 28, "end_idx": 39, "type": "sym", "entity": "2周连续3次定性≥+++" }, { "start_idx": 42, "end_idx": 59, "type": "sym", "entity": "定量≥50~100mg/(kg•d)" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "小儿肾脏病" }, { "start_idx": 111, "end_idx": 111, "type": "bod", "entity": "尿" }, { "start_idx": 122, "end_idx": 135, "type": "sym", "entity": "任意一次尿蛋白/肌酐>2.0" }, { "start_idx": 126, "end_idx": 131, "type": "ite", "entity": "尿蛋白/肌酐" } ] }, { "text": "5.低白蛋白血症血浆白蛋白<30.0g/L,婴儿则<25.0g/L。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "低白蛋白血症" }, { "start_idx": 8, "end_idx": 20, "type": "sym", "entity": "血浆白蛋白<30.0g/L" }, { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "血浆白蛋白" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "婴儿则<25.0g/L" } ] }, { "text": "6.高脂血症主要为高胆固醇血症及高甘油三酯血症,血胆固醇≥5.7mmol/L,婴儿则≥5.2mmol/L,甘油三酯>1.2mmol/L。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "高脂血症" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "高胆固醇血症" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "高甘油三酯血症" }, { "start_idx": 24, "end_idx": 37, "type": "sym", "entity": "血胆固醇≥5.7mmol/L" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "血胆固醇" }, { "start_idx": 39, "end_idx": 51, "type": "sym", "entity": "婴儿则≥5.2mmol/L" }, { "start_idx": 53, "end_idx": 66, "type": "sym", "entity": "甘油三酯>1.2mmol/L" }, { "start_idx": 53, "end_idx": 56, "type": "ite", "entity": "甘油三酯" } ] }, { "text": "7.其他肾炎性肾病患儿还可有血尿甚至肉眼血尿、高血压或肾功能不全等表现。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "肾炎性肾病" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "血尿" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "尿" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "高血压" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "肾功能不全" } ] }, { "text": "(二)常见并发症1.感染是最常见的并发症及引起死亡的主要原因。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "感染" } ] }, { "text": "据1984年国际小儿肾脏病研究学会(ISKDC)统计,直接或间接因感染死亡者占肾病患儿死亡的70%。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "小儿肾脏病" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "感染" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "肾病" } ] }, { "text": "感染也常是病情反复和/或加重的诱因,并可影响激素的疗效。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "感染" }, { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "激素" } ] }, { "text": "本征易发生感染的原因有:①体液免疫功能低下(免疫球蛋白自尿中丢失、合成减少以及分解代谢增加);②常伴有细胞免疫功能和补体系统功能不足;③蛋白质营养不良及水肿致局部循环障碍;④常同时应用皮质激素及免疫抑制剂。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "感染" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "体液免疫功能低下" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "体液" }, { "start_idx": 22, "end_idx": 44, "type": "sym", "entity": "免疫球蛋白自尿中丢失、合成减少以及分解代谢增加" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 51, "end_idx": 65, "type": "sym", "entity": "细胞免疫功能和补体系统功能不足" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "细胞" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "补体系统" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "蛋白质营养不良" }, { "start_idx": 76, "end_idx": 77, "type": "sym", "entity": "水肿" }, { "start_idx": 79, "end_idx": 84, "type": "sym", "entity": "局部循环障碍" }, { "start_idx": 92, "end_idx": 95, "type": "dru", "entity": "皮质激素" }, { "start_idx": 97, "end_idx": 101, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "细菌性感染中既往以肺炎球菌感染为主,近年革兰阴性杆菌所致感染亦见增加(如大肠杆菌)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性感染" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "肺炎球菌感染" }, { "start_idx": 20, "end_idx": 25, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "感染" }, { "start_idx": 36, "end_idx": 39, "type": "mic", "entity": "大肠杆菌" } ] }, { "text": "2.高凝状态及血栓栓塞并发症肾病时体内凝血和纤溶系统可有如下变化:①纤维蛋白原增高;②血浆中第Ⅴ、Ⅶ凝血因子增加;③抗凝血酶Ⅲ下降;④血浆纤溶酶原活性下降;⑤血小板数量可增加,其黏附性和聚集力增高。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "高凝状态及血栓栓塞" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血栓" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肾病" }, { "start_idx": 19, "end_idx": 25, "type": "bod", "entity": "凝血和纤溶系统" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "纤维蛋白原增高" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "纤维蛋白原" }, { "start_idx": 43, "end_idx": 55, "type": "sym", "entity": "血浆中第Ⅴ、Ⅶ凝血因子增加" }, { "start_idx": 43, "end_idx": 53, "type": "ite", "entity": "血浆中第Ⅴ、Ⅶ凝血因子" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "抗凝血酶Ⅲ下降" }, { "start_idx": 58, "end_idx": 62, "type": "ite", "entity": "抗凝血酶Ⅲ" }, { "start_idx": 67, "end_idx": 76, "type": "sym", "entity": "血浆纤溶酶原活性下降" }, { "start_idx": 67, "end_idx": 72, "type": "ite", "entity": "血浆纤溶酶原" }, { "start_idx": 79, "end_idx": 97, "type": "sym", "entity": "血小板数量可增加,其黏附性和聚集力增高" }, { "start_idx": 79, "end_idx": 83, "type": "ite", "entity": "血小板数量" } ] }, { "text": "慢性的肾静脉血栓形成临床症状不明显,常仅为水肿加重及蛋白尿不缓解。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "慢性的肾静脉血栓形成" }, { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "肾静脉血栓" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "水肿加重" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "蛋白尿不缓解" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" } ] }, { "text": "除肾静脉外,其他部位的静脉或动脉也可发生此类并发症,如股静脉、股动脉、肺动脉、肠系膜动脉、冠状动脉和颅内动脉等,并引起相应症状。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肾静脉" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "静脉" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "动脉" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "股静脉" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "股动脉" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肺动脉" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "肠系膜动脉" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "颅内动脉" } ] }, { "text": "3.电解质紊乱主要为低钠血症、低钾血症及低钙血症。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "电解质紊乱" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "低钠血症" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "低钾血症" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "低钙血症" } ] }, { "text": "蛋白尿时钙与蛋白结合而丢失,维生素D结合蛋白丢失,肠吸收钙减低,服用激素的影响以及骨骼对甲状旁腺素调节作用的敏感性降低均可导致低钙血症,可出现低钙惊厥及骨质疏松。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "钙" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "蛋白" }, { "start_idx": 14, "end_idx": 23, "type": "sym", "entity": "维生素D结合蛋白丢失" }, { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "维生素D结合蛋白" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "肠吸收钙减低" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肠" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "钙" }, { "start_idx": 34, "end_idx": 35, "type": "dru", "entity": "激素" }, { "start_idx": 41, "end_idx": 58, "type": "sym", "entity": "骨骼对甲状旁腺素调节作用的敏感性降低" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "骨骼" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "甲状旁腺素" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "低钙血症" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "低钙惊厥" }, { "start_idx": 76, "end_idx": 79, "type": "sym", "entity": "骨质疏松" } ] }, { "text": "4.低血容量休克因血浆白蛋白低下、血浆胶体渗透降低,本征常有血容量不足,加上部分患儿长期不恰当忌盐,当有较急剧的体液丢失(如吐、泻、大剂量利尿应用及大量放腹水等)时即可出现程度不等的血容量不足乃至休克的症状,如烦躁不安、四肢湿冷、皮肤花斑纹、脉搏细速、心音低钝及血压下降测不出等表现。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "低血容量休克" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "血浆白蛋白低下" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "血浆白蛋白" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "血浆胶体渗透降低" }, { "start_idx": 17, "end_idx": 22, "type": "ite", "entity": "血浆胶体渗透" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "血容量不足" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "血容量" }, { "start_idx": 52, "end_idx": 59, "type": "sym", "entity": "较急剧的体液丢失" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "体液" }, { "start_idx": 62, "end_idx": 62, "type": "sym", "entity": "吐" }, { "start_idx": 64, "end_idx": 64, "type": "sym", "entity": "泻" }, { "start_idx": 66, "end_idx": 72, "type": "pro", "entity": "大剂量利尿应用" }, { "start_idx": 74, "end_idx": 78, "type": "pro", "entity": "大量放腹水" }, { "start_idx": 91, "end_idx": 95, "type": "sym", "entity": "血容量不足" }, { "start_idx": 91, "end_idx": 93, "type": "ite", "entity": "血容量" }, { "start_idx": 98, "end_idx": 99, "type": "dis", "entity": "休克" }, { "start_idx": 105, "end_idx": 108, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 110, "end_idx": 113, "type": "sym", "entity": "四肢湿冷" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "四肢" }, { "start_idx": 115, "end_idx": 116, "type": "bod", "entity": "皮肤" }, { "start_idx": 115, "end_idx": 119, "type": "sym", "entity": "皮肤花斑纹" }, { "start_idx": 121, "end_idx": 124, "type": "sym", "entity": "脉搏细速" }, { "start_idx": 121, "end_idx": 122, "type": "ite", "entity": "脉搏" }, { "start_idx": 126, "end_idx": 129, "type": "sym", "entity": "心音低钝" }, { "start_idx": 126, "end_idx": 127, "type": "ite", "entity": "心音" }, { "start_idx": 131, "end_idx": 137, "type": "sym", "entity": "血压下降测不出" }, { "start_idx": 131, "end_idx": 132, "type": "ite", "entity": "血压" } ] }, { "text": "5.急性肾衰竭起病时暂时性轻度氮质血症并不少见,病程中可发生急性肾衰竭。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "暂时性轻度氮质血症" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "6.肾小管功能障碍可表现为糖尿、氨基酸尿,以及从尿中丢失钾及磷,浓缩功能不足等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "肾小管功能障碍" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "糖尿" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "糖" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "尿" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "氨基酸尿" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "氨基酸" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "尿" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "尿中丢失钾及磷" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "尿" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "钾" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "磷" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "浓缩功能不足" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "浓缩功能" } ] }, { "text": "7.肾上腺皮质危象见于皮质激素突然撤减或感染应激时内源性皮质激素水平不足,表现为表情淡漠、呕吐、血压降低乃至休克。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "肾上腺皮质" }, { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "肾上腺皮质危象" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "皮质激素" }, { "start_idx": 20, "end_idx": 35, "type": "sym", "entity": "感染应激时内源性皮质激素水平不足" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "内源性皮质激素" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "表情淡漠" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呕吐" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "血压降低" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "血压" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "休克" } ] }, { "text": "8.其他如生长障碍,可能与蛋白丢失致营养不良,激素作用以及IGF及其结合蛋白失衡有关。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "生长障碍" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "蛋白丢失" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "蛋白" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "营养不良" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "激素" }, { "start_idx": 29, "end_idx": 39, "type": "sym", "entity": "IGF及其结合蛋白失衡" }, { "start_idx": 29, "end_idx": 37, "type": "bod", "entity": "IGF及其结合蛋白" } ] }, { "text": "动脉粥样硬化与长期高脂血症有关。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "动脉粥样硬化" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "高脂血症" } ] }, { "text": "【实验室检查】(一)尿液分析①尿常规:蛋白定性≥+++,肾炎性肾病可见血尿(离心尿红细胞>10个/HP);②尿C3及尿纤维蛋白原降解产物(FDP),肾炎性肾病时尿C3(+)、尿FDP增高;③尿蛋白电泳:单纯性肾病主要为白蛋白,肾炎性肾病时可出现大分子及小分子蛋白尿;④尿酶学:N-乙酰-β-葡萄糖氨基苷酶(NAG)升高见于大量蛋白尿时或病变影响肾小管功能时,尿溶菌酶升高反映肾小管吸收功能下降;⑤其他:视黄醛结合蛋白(RBP)、尿β2-微球蛋白、尿Kappa及Lamda轻链分析均是反映肾小管病变的指标,肾炎性肾病时可增高。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "尿液分析" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "尿常规" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "蛋白定性≥+++" }, { "start_idx": 19, "end_idx": 22, "type": "ite", "entity": "蛋白定性" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "肾炎性肾病" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "血尿" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "尿" }, { "start_idx": 38, "end_idx": 50, "type": "sym", "entity": "离心尿红细胞>10个/HP" }, { "start_idx": 38, "end_idx": 43, "type": "ite", "entity": "离心尿红细胞" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "尿C3" }, { "start_idx": 58, "end_idx": 67, "type": "ite", "entity": "尿纤维蛋白原降解产物" }, { "start_idx": 69, "end_idx": 71, "type": "ite", "entity": "FDP" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "肾炎性肾病" }, { "start_idx": 80, "end_idx": 82, "type": "ite", "entity": "尿C3" }, { "start_idx": 80, "end_idx": 85, "type": "sym", "entity": "尿C3(+)" }, { "start_idx": 87, "end_idx": 90, "type": "ite", "entity": "尿FDP" }, { "start_idx": 87, "end_idx": 92, "type": "sym", "entity": "尿FDP增高" }, { "start_idx": 95, "end_idx": 99, "type": "pro", "entity": "尿蛋白电泳" }, { "start_idx": 101, "end_idx": 105, "type": "dis", "entity": "单纯性肾病" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "白蛋白" }, { "start_idx": 113, "end_idx": 117, "type": "dis", "entity": "肾炎性肾病" }, { "start_idx": 120, "end_idx": 131, "type": "sym", "entity": "出现大分子及小分子蛋白尿" }, { "start_idx": 122, "end_idx": 131, "type": "bod", "entity": "大分子及小分子蛋白尿" }, { "start_idx": 134, "end_idx": 136, "type": "pro", "entity": "尿酶学" }, { "start_idx": 138, "end_idx": 158, "type": "sym", "entity": "N-乙酰-β-葡萄糖氨基苷酶(NAG)升高" }, { "start_idx": 138, "end_idx": 151, "type": "ite", "entity": "N-乙酰-β-葡萄糖氨基苷酶" }, { "start_idx": 153, "end_idx": 155, "type": "ite", "entity": "NAG" }, { "start_idx": 161, "end_idx": 165, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 165, "end_idx": 165, "type": "bod", "entity": "尿" }, { "start_idx": 168, "end_idx": 176, "type": "sym", "entity": "病变影响肾小管功能" }, { "start_idx": 172, "end_idx": 174, "type": "bod", "entity": "肾小管" }, { "start_idx": 179, "end_idx": 184, "type": "sym", "entity": "尿溶菌酶升高" }, { "start_idx": 179, "end_idx": 182, "type": "ite", "entity": "尿溶菌酶" }, { "start_idx": 187, "end_idx": 195, "type": "sym", "entity": "肾小管吸收功能下降" }, { "start_idx": 187, "end_idx": 189, "type": "bod", "entity": "肾小管" }, { "start_idx": 201, "end_idx": 207, "type": "ite", "entity": "视黄醛结合蛋白" }, { "start_idx": 209, "end_idx": 211, "type": "ite", "entity": "RBP" }, { "start_idx": 214, "end_idx": 227, "type": "ite", "entity": "尿β2-微球蛋白" }, { "start_idx": 229, "end_idx": 244, "type": "pro", "entity": "尿Kappa及Lamda轻链分析" }, { "start_idx": 249, "end_idx": 253, "type": "dis", "entity": "肾小管病变" }, { "start_idx": 258, "end_idx": 262, "type": "dis", "entity": "肾炎性肾病" } ] }, { "text": "(二)血生化总蛋白<30.0g/L、胆固醇>5.7mmol/L,甘油三酯>1.2mmol/L、LDL及VLDL增高,而HDL多下降。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "血生化" }, { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "总蛋白" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "总蛋白<30.0g/L" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "胆固醇" }, { "start_idx": 18, "end_idx": 30, "type": "sym", "entity": "胆固醇>5.7mmol/L" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "甘油三酯" }, { "start_idx": 32, "end_idx": 45, "type": "sym", "entity": "甘油三酯>1.2mmol/L" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "LDL" }, { "start_idx": 47, "end_idx": 56, "type": "sym", "entity": "LDL及VLDL增高" }, { "start_idx": 51, "end_idx": 54, "type": "ite", "entity": "VLDL" }, { "start_idx": 59, "end_idx": 61, "type": "ite", "entity": "HDL" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "HDL多下降" } ] }, { "text": "(三)血浆蛋白电泳白蛋白降低,α2及β升高,γ在单纯性肾病时降低,肾炎性肾病可正常或增高。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "血浆蛋白电泳" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "白蛋白降低" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "白蛋白" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "α2及β升高" }, { "start_idx": 15, "end_idx": 22, "type": "ite", "entity": "α2" }, { "start_idx": 24, "end_idx": 24, "type": "ite", "entity": "β" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "γ在单纯性肾病时降低" }, { "start_idx": 28, "end_idx": 28, "type": "ite", "entity": "γ" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "单纯性肾病" }, { "start_idx": 39, "end_idx": 49, "type": "sym", "entity": "肾炎性肾病可正常或增高" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "肾炎性肾病" } ] }, { "text": "(四)免疫学检查①血IgG降低,IgA降低,但IgM可升高;②补体一般正常,膜增生性肾炎可下降;③微小病变性肾病往往有细胞免疫功能降低表现如Ts活性增高、CD4/CD8降低等;④血清细胞因子水平各异,可表现为Th1细胞因子(如INF、IL2及IL12)降低,而Th2细胞因子(IL4、IL10及IL13)升高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "免疫学检查" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "血IgG降低" }, { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "血IgG" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "IgA降低" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "IgA" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "但IgM可升高" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "IgM" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "补体一般正常" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "补体" }, { "start_idx": 38, "end_idx": 46, "type": "sym", "entity": "膜增生性肾炎可下降" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "膜增生性肾炎" }, { "start_idx": 49, "end_idx": 55, "type": "dis", "entity": "微小病变性肾病" }, { "start_idx": 59, "end_idx": 68, "type": "sym", "entity": "细胞免疫功能降低表现" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "Ts活性增高" }, { "start_idx": 70, "end_idx": 71, "type": "ite", "entity": "Ts" }, { "start_idx": 77, "end_idx": 85, "type": "sym", "entity": "CD4/CD8降低" }, { "start_idx": 77, "end_idx": 83, "type": "ite", "entity": "CD4/CD8" }, { "start_idx": 89, "end_idx": 98, "type": "sym", "entity": "血清细胞因子水平各异" }, { "start_idx": 89, "end_idx": 94, "type": "ite", "entity": "血清细胞因子" }, { "start_idx": 104, "end_idx": 145, "type": "sym", "entity": "Th1细胞因子(如INF、IL2及IL12)降低" }, { "start_idx": 104, "end_idx": 116, "type": "ite", "entity": "Th1细胞因子" }, { "start_idx": 119, "end_idx": 121, "type": "ite", "entity": "INF" }, { "start_idx": 123, "end_idx": 131, "type": "ite", "entity": "IL2" }, { "start_idx": 133, "end_idx": 142, "type": "ite", "entity": "IL12" }, { "start_idx": 148, "end_idx": 195, "type": "sym", "entity": "Th2细胞因子(IL4、IL10及IL13)升高" }, { "start_idx": 148, "end_idx": 160, "type": "ite", "entity": "Th2细胞因子" }, { "start_idx": 162, "end_idx": 170, "type": "ite", "entity": "IL4" }, { "start_idx": 172, "end_idx": 181, "type": "ite", "entity": "IL10" }, { "start_idx": 183, "end_idx": 192, "type": "ite", "entity": "IL13" } ] }, { "text": "(五)血沉多明显增快,单纯性肾病时尤为显著,可>100mm/h。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "血沉" }, { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "血沉多明显增快" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "单纯性肾病" } ] }, { "text": "(六)血电解质及肾功能正常或出现低钠血症、低钾血症及低钙血症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血电解质" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "肾功能" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "低钠血症" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "低钾血症" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "低钙血症" } ] }, { "text": "肾功能一般正常,合并肾功能不全时可有BUN及Cr升高,内生肌酐廓清率下降。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肾功能" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "合并肾功能不全" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "BUN" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "BUN及Cr升高" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "Cr" }, { "start_idx": 27, "end_idx": 33, "type": "ite", "entity": "内生肌酐廓清率" }, { "start_idx": 27, "end_idx": 35, "type": "sym", "entity": "内生肌酐廓清率下降" } ] }, { "text": "(七)肾活体组织检查明确肾病综合征病理分型的主要依据。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "【诊断】2000年11月中华医学会儿科分会肾脏学组珠海会议制定的肾病综合征诊断及临床分型标准如下:(一)诊断标准大量蛋白尿[尿蛋白(+++)~(++++);1周内3次,24h尿蛋白定量≥50mg/kg];血浆白蛋白低于30g/L;血浆胆固醇高于5.7mmol/L;不同程度的水肿。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "dep", "entity": "儿科" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肾脏" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "尿" }, { "start_idx": 62, "end_idx": 64, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 62, "end_idx": 76, "type": "sym", "entity": "尿蛋白(+++)~(++++)" }, { "start_idx": 78, "end_idx": 99, "type": "sym", "entity": "1周内3次,24h尿蛋白定量≥50mg/kg" }, { "start_idx": 84, "end_idx": 91, "type": "ite", "entity": "24h尿蛋白定量" }, { "start_idx": 102, "end_idx": 106, "type": "ite", "entity": "血浆白蛋白" }, { "start_idx": 102, "end_idx": 113, "type": "sym", "entity": "血浆白蛋白低于30g/L" }, { "start_idx": 115, "end_idx": 119, "type": "ite", "entity": "血浆胆固醇" }, { "start_idx": 115, "end_idx": 130, "type": "sym", "entity": "血浆胆固醇高于5.7mmol/L" }, { "start_idx": 132, "end_idx": 138, "type": "sym", "entity": "不同程度的水肿" } ] }, { "text": "以上四项中以大量蛋白尿和低白蛋白血症为必要条件。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "尿" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "低白蛋白血症" } ] }, { "text": "(二)依临床表现分为两型1.单纯型NS(simpletypeNS)2.肾炎型NS(nephrotictypeNS)凡具有以下四项之一项或多项者属于肾炎型NS;①2周内分别3次以上离心尿检查RBC≥10个/HP,并证实为肾小球源性血尿者;②反复或持续高血压(学龄儿童≥130/90mmHg,学龄前儿童≥120/80mmHg)并除外使用糖皮质激素等原因所致;③肾功能不全,并排除由于血容量不足等所致;④持续低补体血症。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "单纯型NS" }, { "start_idx": 20, "end_idx": 31, "type": "dis", "entity": "simpletypeNS" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "肾炎型NS" }, { "start_idx": 41, "end_idx": 55, "type": "dis", "entity": "nephrotictypeNS" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "肾炎型NS" }, { "start_idx": 89, "end_idx": 93, "type": "pro", "entity": "离心尿检查" }, { "start_idx": 94, "end_idx": 96, "type": "ite", "entity": "RBC" }, { "start_idx": 94, "end_idx": 103, "type": "sym", "entity": "RBC≥10个/HP" }, { "start_idx": 109, "end_idx": 115, "type": "sym", "entity": "肾小球源性血尿" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "肾小球" }, { "start_idx": 115, "end_idx": 115, "type": "bod", "entity": "尿" }, { "start_idx": 119, "end_idx": 126, "type": "sym", "entity": "反复或持续高血压" }, { "start_idx": 128, "end_idx": 142, "type": "sym", "entity": "学龄儿童≥130/90mmHg" }, { "start_idx": 144, "end_idx": 159, "type": "sym", "entity": "学龄前儿童≥120/80mmHg" }, { "start_idx": 166, "end_idx": 170, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 178, "end_idx": 182, "type": "dis", "entity": "肾功能不全" }, { "start_idx": 189, "end_idx": 191, "type": "ite", "entity": "血容量" }, { "start_idx": 189, "end_idx": 193, "type": "sym", "entity": "血容量不足" }, { "start_idx": 199, "end_idx": 205, "type": "sym", "entity": "持续低补体血症" } ] }, { "text": "【治疗】(一)一般治疗1.休息与饮食高度水肿时宜卧床,病情稳定后可正常活动,但应避免剧烈活动。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "高度水肿" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "应避免剧烈活动" } ] }, { "text": "蛋白摄入量应适宜1~2g/(kg•d),并肾衰竭时宜低蛋白饮食<0.5g/(kg•d),并注意补充各种水溶性维生素及维生素D和钙、锌等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "蛋白" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "低蛋白饮食" }, { "start_idx": 45, "end_idx": 66, "type": "pro", "entity": "注意补充各种水溶性维生素及维生素D和钙、锌等" } ] }, { "text": "2.利尿轻度水肿可口服氢氯噻嗪(hydrochlorothiazide,DHCT)2mg/(kg•d),3次/d和保钾利尿剂如螺内酯(spironolactoneantisterone)2mg/(kg•d),重者可静脉注射呋塞米(furosemide,速尿)每次1~2mg/kg,NS患儿多有血容量不足,因此在应用呋塞米前可快速输注低分子右旋糖酐10ml/kg,较单用呋塞米利尿效果明显。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "利尿轻度水肿" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "口服" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "氢氯噻嗪" }, { "start_idx": 16, "end_idx": 34, "type": "dru", "entity": "hydrochlorothiazide" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "DHCT" }, { "start_idx": 57, "end_idx": 61, "type": "dru", "entity": "保钾利尿剂" }, { "start_idx": 63, "end_idx": 65, "type": "dru", "entity": "螺内酯" }, { "start_idx": 67, "end_idx": 91, "type": "dru", "entity": "spironolactoneantisterone" }, { "start_idx": 107, "end_idx": 110, "type": "pro", "entity": "静脉注射" }, { "start_idx": 111, "end_idx": 113, "type": "dru", "entity": "呋塞米" }, { "start_idx": 115, "end_idx": 124, "type": "dru", "entity": "furosemide" }, { "start_idx": 126, "end_idx": 127, "type": "dru", "entity": "速尿" }, { "start_idx": 140, "end_idx": 141, "type": "dis", "entity": "NS" }, { "start_idx": 146, "end_idx": 150, "type": "sym", "entity": "血容量不足" }, { "start_idx": 146, "end_idx": 146, "type": "bod", "entity": "血" }, { "start_idx": 157, "end_idx": 159, "type": "dru", "entity": "呋塞米" }, { "start_idx": 166, "end_idx": 172, "type": "dru", "entity": "低分子右旋糖酐" }, { "start_idx": 184, "end_idx": 186, "type": "dru", "entity": "呋塞米" } ] }, { "text": "3.抗凝肾病活动期多为高凝状态,可常规给予双嘧达莫(dipyridamole,潘生丁,persantin)5~8mg/(kg•d),3次/日及肝素每次1mg/kg,每日1~2次。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "抗凝肾病" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "高凝状态" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 26, "end_idx": 37, "type": "dru", "entity": "dipyridamole" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "潘生丁" }, { "start_idx": 43, "end_idx": 51, "type": "dru", "entity": "persantin" }, { "start_idx": 71, "end_idx": 72, "type": "dru", "entity": "肝素" } ] }, { "text": "还可选用尿激酶3万~6万U/d,一日2次,或华法林(warfarin),起始剂量为2.5mg,3次/d,以后维持在2.5~10mg/d,根据凝血酶原时间调整剂量。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "尿激酶" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "华法林" }, { "start_idx": 26, "end_idx": 33, "type": "dru", "entity": "warfarin" }, { "start_idx": 70, "end_idx": 75, "type": "ite", "entity": "凝血酶原时间" } ] }, { "text": "4.抗感染、降压以及各种并发症的治疗。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "抗感染" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "降压" } ] }, { "text": "(二)肾上腺皮质激素仍为治疗肾病综合征的首选药物。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "1.泼尼松(prednisone)口服治疗应用最广泛,适用初治病人。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "泼尼松" }, { "start_idx": 6, "end_idx": 15, "type": "dru", "entity": "prednisone" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "口服治疗" } ] }, { "text": "疗效判断:按上法治疗8周后判断疗效,如治疗8周后尿蛋白转阴为激素敏感,其中4周内转阴为高度敏感,8周后尿蛋白减少为+~++则为部分敏感;尿蛋白仍>+++为激素耐药;对激素敏感但需长期维持某一剂量的激素则为激素依赖;尿蛋白阴性,停药4周后又升至++以上为复发;未停用激素,尿蛋白由阴性转为++以上为反复;半年内复发或反复≥2次或1年内≥3次为频复发或频反复。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 30, "end_idx": 31, "type": "dru", "entity": "激素" }, { "start_idx": 51, "end_idx": 53, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 51, "end_idx": 60, "type": "sym", "entity": "尿蛋白减少为+~++" }, { "start_idx": 68, "end_idx": 70, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 68, "end_idx": 75, "type": "sym", "entity": "尿蛋白仍>+++" }, { "start_idx": 77, "end_idx": 78, "type": "dru", "entity": "激素" }, { "start_idx": 83, "end_idx": 84, "type": "dru", "entity": "激素" }, { "start_idx": 98, "end_idx": 99, "type": "dru", "entity": "激素" }, { "start_idx": 102, "end_idx": 103, "type": "dru", "entity": "激素" }, { "start_idx": 107, "end_idx": 109, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 132, "end_idx": 133, "type": "dru", "entity": "激素" }, { "start_idx": 135, "end_idx": 137, "type": "ite", "entity": "尿蛋白" } ] }, { "text": "激素耐药、依赖及频复发或频反复为难治性肾病。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "激素" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "激素耐药、依赖及频复发或频反复" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "难治性肾病" } ] }, { "text": "患儿对激素是否敏感与其类型有关。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "激素" } ] }, { "text": "据我国临床分型资料,单纯性病例78.9%呈完全效应;而肾炎型者为34.3%。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "肾炎" } ] }, { "text": "在病理组织类型方面,据ISKDC报告,471例小儿原发性肾病综合征呈现激素敏感者368例(78.1%)。", "entities": [ { "start_idx": 23, "end_idx": 32, "type": "dis", "entity": "小儿原发性肾病综合征" }, { "start_idx": 35, "end_idx": 36, "type": "dru", "entity": "激素" } ] }, { "text": "同时发现微小病变者中对激素敏感占93.1%、局灶节段硬化者中占29.7%、系膜增生者中占55.6%、膜增殖性肾炎者中仅占6.9%。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "微小病变" }, { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "激素" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "局灶节段硬化" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "系膜增生" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "膜增殖性肾炎" } ] }, { "text": "2.甲泼尼龙冲击治疗主要用于难治性肾病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "甲泼尼龙" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "冲击治疗" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "难治性肾病" } ] }, { "text": "剂量为20~30mg/(kg•d),总量<1.0g,加入10%葡萄糖100~250ml中静脉滴注,时间为1~2小时。", "entities": [ { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "如需冲击2个疗程则在第2疗程改为隔日静滴一次,连用3次。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "冲击" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "隔日静滴" } ] }, { "text": "3.其他肾上腺皮质激素也可用甲泼尼龙片剂(每片4mg,相当于泼尼松5mg)和曲安西龙(triamcinolone,阿赛松,每片4mg,相当于泼尼松5mg)取代泼尼松口服,且水钠潴留的不良反应要小,而治疗作用一样甚至更好,但价格较为昂贵。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "甲泼尼龙片剂" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "泼尼松" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "曲安西龙" }, { "start_idx": 43, "end_idx": 55, "type": "dru", "entity": "triamcinolone" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "阿赛松" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "泼尼松" }, { "start_idx": 79, "end_idx": 81, "type": "dru", "entity": "泼尼松" }, { "start_idx": 82, "end_idx": 83, "type": "pro", "entity": "口服" }, { "start_idx": 86, "end_idx": 94, "type": "sym", "entity": "水钠潴留的不良反应" }, { "start_idx": 86, "end_idx": 89, "type": "dis", "entity": "水钠潴留" } ] }, { "text": "对于反复的病人也可试用换激素剂型疗法,即以地塞米松取代泼尼松口服。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "换激素剂型疗法" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "地塞米松" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "泼尼松" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "口服" } ] }, { "text": "每一片地塞米松(0.75mg)换一片泼尼松(5mg),一般2~4周蛋白阴转后再换回泼尼松。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "地塞米松" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "泼尼松" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "蛋白" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "泼尼松" } ] }, { "text": "还可用地塞米松静脉冲击治疗,剂量每次1~2mg/kg,每日1次,连用3天为1疗程,疗效与甲泼尼龙相似,但副作用明显增加,易致高血压及水钠潴留,并且抑制垂体肾上腺轴的作用强,尽量少用。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "地塞米松静脉冲击治疗" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "甲泼尼龙" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "高血压" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "水钠潴留" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "垂体" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "肾上腺轴" } ] }, { "text": "此外曲安奈德(triamcinoloneacetonide,康宁克通A,kenacortA)每次0.6~1mg/kg,每1~2个月肌注一次,用于肾病皮质激素减完后的稳定期,可防复发。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "曲安奈德" }, { "start_idx": 7, "end_idx": 28, "type": "dru", "entity": "triamcinoloneacetonide" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "康宁克通A" }, { "start_idx": 36, "end_idx": 44, "type": "dru", "entity": "kenacortA" }, { "start_idx": 65, "end_idx": 66, "type": "pro", "entity": "肌注" }, { "start_idx": 72, "end_idx": 77, "type": "dru", "entity": "肾病皮质激素" } ] }, { "text": "长期服用皮质激素,可产生许多不良反应,如脂肪代谢紊乱,表现为水肿、体脂分布异常及库欣貌;蛋白质分解代谢增加出现氮负平衡、肌肉萎缩无力及伤口愈合不良;糖代谢紊乱可引起高血糖和糖尿;水电解质紊乱,出现水钠潴留和高血压;钙磷代谢紊乱发生高尿钙以及骨质稀疏;胃肠道可发生消化性溃疡,甚至穿孔;神经精神方面有欣快感、兴奋及失眠,严重时发生精神病和癫痫发作;由于抑制抗体形成易发生感染或隐性感染灶(如结核病)的活动和播散;长期用药还可发生白内障及股骨头无菌坏死。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "服用" }, { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "皮质激素" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "脂肪代谢紊乱" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "水肿、体脂分布异常及库欣貌" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "体脂" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "库欣貌" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "蛋白质" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "氮" }, { "start_idx": 55, "end_idx": 72, "type": "sym", "entity": "氮负平衡、肌肉萎缩无力及伤口愈合不良" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "肌肉萎缩" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "糖代谢紊乱" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "高血糖" }, { "start_idx": 86, "end_idx": 86, "type": "bod", "entity": "糖" }, { "start_idx": 87, "end_idx": 87, "type": "bod", "entity": "尿" }, { "start_idx": 89, "end_idx": 94, "type": "dis", "entity": "水电解质紊乱" }, { "start_idx": 98, "end_idx": 101, "type": "dis", "entity": "水钠潴留" }, { "start_idx": 103, "end_idx": 105, "type": "dis", "entity": "高血压" }, { "start_idx": 107, "end_idx": 112, "type": "dis", "entity": "钙磷代谢紊乱" }, { "start_idx": 115, "end_idx": 117, "type": "sym", "entity": "高尿钙" }, { "start_idx": 116, "end_idx": 116, "type": "bod", "entity": "尿" }, { "start_idx": 117, "end_idx": 117, "type": "bod", "entity": "钙" }, { "start_idx": 120, "end_idx": 123, "type": "dis", "entity": "骨质稀疏" }, { "start_idx": 125, "end_idx": 140, "type": "sym", "entity": "胃肠道可发生消化性溃疡,甚至穿孔" }, { "start_idx": 125, "end_idx": 127, "type": "bod", "entity": "胃肠道" }, { "start_idx": 131, "end_idx": 135, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 139, "end_idx": 140, "type": "dis", "entity": "穿孔" }, { "start_idx": 142, "end_idx": 171, "type": "sym", "entity": "神经精神方面有欣快感、兴奋及失眠,严重时发生精神病和癫痫发作" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "神经" }, { "start_idx": 164, "end_idx": 166, "type": "dis", "entity": "精神病" }, { "start_idx": 168, "end_idx": 171, "type": "dis", "entity": "癫痫发作" }, { "start_idx": 175, "end_idx": 203, "type": "sym", "entity": "抑制抗体形成易发生感染或隐性感染灶(如结核病)的活动和播散" }, { "start_idx": 175, "end_idx": 178, "type": "bod", "entity": "抑制抗体" }, { "start_idx": 184, "end_idx": 185, "type": "dis", "entity": "感染" }, { "start_idx": 187, "end_idx": 191, "type": "dis", "entity": "隐性感染灶" }, { "start_idx": 194, "end_idx": 196, "type": "dis", "entity": "结核病" }, { "start_idx": 205, "end_idx": 223, "type": "sym", "entity": "长期用药还可发生白内障及股骨头无菌坏死" }, { "start_idx": 213, "end_idx": 215, "type": "dis", "entity": "白内障" }, { "start_idx": 217, "end_idx": 223, "type": "dis", "entity": "股骨头无菌坏死" } ] }, { "text": "此外,如突然停药或遇手术、感染等应激状态时,内源性肾上腺皮质激素分泌不足,可产生肾上腺皮质功能不全甚至肾上腺危象表现,如恶心、呕吐、腹痛和休克。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "手术" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "内源性肾上腺皮质激素分泌不足" }, { "start_idx": 22, "end_idx": 31, "type": "bod", "entity": "内源性肾上腺皮质激素" }, { "start_idx": 40, "end_idx": 48, "type": "dis", "entity": "肾上腺皮质功能不全" }, { "start_idx": 51, "end_idx": 57, "type": "sym", "entity": "肾上腺危象表现" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "肾上腺" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "恶心" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "呕吐" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "腹痛" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "腹" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "休克" } ] }, { "text": "(三)免疫抑制剂适用于难治性肾病综合征。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "难治性肾病综合征" } ] }, { "text": "一般与中小剂量的皮质激素合用,有协同作用。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "皮质激素" } ] }, { "text": "常用的药物有:1.环磷酰胺(CTX)2~3mg/(kg•d)口服,疗程2~3个月,累积量不超过250mg/kg。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "CTX" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "口服" } ] }, { "text": "静脉冲击时,每次0.5~0.75mg/m2,每月1次,连用6次,必要时可追加2~4次,累积量一般150mg/kg。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉冲击" } ] }, { "text": "CTX主要不良反应有胃肠反应、血白细胞减少、脱发、出血性膀胱炎及性腺损害(主要为男孩),青春期应慎用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "CTX" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "胃肠反应" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "胃" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肠" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "血白细胞减少" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "血白细胞" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "脱发" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "发" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "出血性膀胱炎" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "性腺损害" } ] }, { "text": "2.其他包括苯丁酸氮芥(chlorambucil)0.2mg/(kg•d),总剂量<12~16mg;氮芥(chlormethine)0.1mg/(kg•d)静脉注射,4天为一疗程,1个月后可重复一疗程;环孢素A(ciclosporinA,CsA)5mg/(kg•d)口服,缓解后减量,可用6个月,维持全血CsA谷浓度在100~200ng/ml水平;6-硫鸟嘌呤(6-TG)1.5mg/(kg•d)口服,疗程一年。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "苯丁酸氮芥" }, { "start_idx": 12, "end_idx": 23, "type": "dru", "entity": "chlorambucil" }, { "start_idx": 50, "end_idx": 51, "type": "dru", "entity": "氮芥" }, { "start_idx": 53, "end_idx": 64, "type": "dru", "entity": "chlormethine" }, { "start_idx": 78, "end_idx": 81, "type": "pro", "entity": "静脉注射" }, { "start_idx": 101, "end_idx": 104, "type": "dru", "entity": "环孢素A" }, { "start_idx": 106, "end_idx": 117, "type": "dru", "entity": "ciclosporinA" }, { "start_idx": 119, "end_idx": 121, "type": "dru", "entity": "CsA" }, { "start_idx": 133, "end_idx": 134, "type": "pro", "entity": "口服" }, { "start_idx": 150, "end_idx": 157, "type": "ite", "entity": "全血CsA谷浓度" }, { "start_idx": 174, "end_idx": 179, "type": "dru", "entity": "6-硫鸟嘌呤" }, { "start_idx": 181, "end_idx": 184, "type": "dru", "entity": "6-TG" }, { "start_idx": 198, "end_idx": 199, "type": "pro", "entity": "口服" } ] }, { "text": "普乐可复(FK506)0.15mg/(kg•d),分2次口服,疗程3个月。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "普乐可复" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "口服" } ] }, { "text": "霉酚酸酯(MMF,骁悉),1~2g/d,分2次口服,疗程6个月以上,均有一定疗效。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "霉酚酸酯" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "MMF" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "骁悉" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "口服" } ] }, { "text": "(四)中医药可用中药抗凝、调节免疫并防止复发,常用雷公藤多甙片2mg/(kg•d),分3次口服,逐步减量至1mg/(kg•d),疗程3~5个月;川芎嗪4mg/(kg•d),有抗凝功效;保肾康100~150mg/次,每日3次口服;肾炎舒等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "中医药" }, { "start_idx": 8, "end_idx": 9, "type": "dru", "entity": "中药" }, { "start_idx": 25, "end_idx": 30, "type": "dru", "entity": "雷公藤多甙片" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "口服" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "川芎嗪" }, { "start_idx": 92, "end_idx": 94, "type": "dru", "entity": "保肾康" }, { "start_idx": 111, "end_idx": 112, "type": "pro", "entity": "口服" }, { "start_idx": 114, "end_idx": 116, "type": "dru", "entity": "肾炎舒" } ] }, { "text": "【预后】儿童肾病综合征的预后与原发病、病理类型及治疗反应密切相关。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "儿童肾病综合征" } ] }, { "text": "Habib等报告1~18年随访发展成慢性肾衰或者死亡者MCN为7%、FSGS为38%、MN为8%,以及MPGN为41%。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "慢性肾衰" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "MCN" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "FSGS" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "MN" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "MPGN" } ] }, { "text": "二、茶碱类药物虽然茶碱用于哮喘治疗已有半个多世纪,但它有治疗剂量的安全范围小,容易产生不良反应的缺点,随着β2受体激动剂的广泛使用,茶碱类药物的临床应用逐渐减少。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "茶碱" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "哮喘" }, { "start_idx": 54, "end_idx": 71, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 78, "end_idx": 82, "type": "dru", "entity": "茶碱类药物" } ] }, { "text": "近几年发现小剂量茶碱的抗炎和免疫调节作用,使人们对茶碱在治疗哮喘中的地位进行重新评估。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dru", "entity": "茶碱" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "茶碱" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "哮喘" } ] }, { "text": "尽管如此,茶碱类药物现在仍然只用于扩张支气管,而抗炎作用在临床中的价值尚需进一步评估。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "支气管" } ] }, { "text": "茶碱类药物主要通过直接扩张支气管平滑肌、促进纤毛运动以及增强膈肌的收缩力,以达到治疗哮喘的目的。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "支气管平滑肌" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "纤毛" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "膈肌" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "哮喘" } ] }, { "text": "在10~20μg/ml的血浓度范围内,有明显的支气管扩张效应,但超过20μg/ml可以出现不良反应。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "血浓度" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "支气管" } ] }, { "text": "由于不同人体之间氨茶碱的清除率差异比较大,加上有效治疗范围狭窄,应注意用药剂量的个体化和血浓度测定。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 44, "end_idx": 48, "type": "ite", "entity": "血浓度测定" } ] }, { "text": "(一)茶碱类药物常用制剂及使用方法1.氨茶碱氨茶碱(aminophylline)是茶碱与乙二胺形成的复盐,约含茶碱77%~83%,因而氨茶碱剂量相当于茶碱剂量/0.8。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 26, "end_idx": 38, "type": "dru", "entity": "aminophylline" }, { "start_idx": 41, "end_idx": 42, "type": "dru", "entity": "茶碱" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "乙二胺" }, { "start_idx": 50, "end_idx": 51, "type": "dru", "entity": "复盐" }, { "start_idx": 55, "end_idx": 56, "type": "dru", "entity": "茶碱" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 75, "end_idx": 76, "type": "dru", "entity": "茶碱" } ] }, { "text": "①负荷量法:首次给予氨茶碱4~6mg/kg(≤250mg),20~30分钟内静脉滴注;然后静脉连续输注维持静脉滴注剂量为(表8-8)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "负荷量法" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 45, "end_idx": 50, "type": "pro", "entity": "静脉连续输注" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "主要用于哮喘持续状态和未用茶碱类药物的患儿。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "哮喘持续状态" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "哮喘" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "茶碱类药物" } ] }, { "text": "一般给予负荷量后30分钟,应该测定茶碱血浓度,如血浓度<10μg/ml,按1mg/kg茶碱提高茶碱血浓度2μg/ml计算追加氨茶碱用药量,待血浓度达10μg/ml后再给予维持量;相反,如血浓度>20μg/ml,应等茶碱血浓度<20μg/ml后再给予维持量。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "给予负荷量" }, { "start_idx": 15, "end_idx": 21, "type": "ite", "entity": "测定茶碱血浓度" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "血浓度" }, { "start_idx": 43, "end_idx": 44, "type": "dru", "entity": "茶碱" }, { "start_idx": 47, "end_idx": 51, "type": "ite", "entity": "茶碱血浓度" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 70, "end_idx": 72, "type": "ite", "entity": "血浓度" }, { "start_idx": 93, "end_idx": 95, "type": "ite", "entity": "血浓度" }, { "start_idx": 107, "end_idx": 111, "type": "ite", "entity": "茶碱血浓度" } ] }, { "text": "对已经用氨茶碱的患儿,在给负荷量前应先测定茶碱血浓度,如血浓度<10μg/ml,按上述原则追加氨茶碱用药量,待血浓度达10μg/ml后再给予维持量。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "负荷量" }, { "start_idx": 19, "end_idx": 25, "type": "ite", "entity": "测定茶碱血浓度" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "血浓度" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 55, "end_idx": 57, "type": "ite", "entity": "血浓度" } ] }, { "text": "②普通法:每次2~4mg/kg,用25%~50%葡萄糖注射液20~40ml稀释后缓慢静脉注射(速度不得超过每分钟0.02mg/kg)或用5%葡萄糖注射液250ml稀释后静脉滴注,每6小时可以重复给原药量。", "entities": [ { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "葡萄糖注射液" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "静脉注射" }, { "start_idx": 70, "end_idx": 75, "type": "dru", "entity": "葡萄糖注射液" }, { "start_idx": 84, "end_idx": 87, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "主要用于已口服氨茶碱或不能进行血药浓度测定的患儿。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "口服" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 15, "end_idx": 20, "type": "ite", "entity": "血药浓度测定" } ] }, { "text": "③口服:每次4~6mg/kg,每6~8小时1次。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "口服" } ] }, { "text": "表8-18静脉输注氨茶碱的维持量2.二羟丙茶碱(喘定)二羟丙茶碱是茶碱的N-7位上接二羟丙基的中性衍生物,对胃的刺激性较小;该药心脏兴奋作用弱,因此适用于心动过速患儿。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "静脉输注" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "二羟丙茶碱" }, { "start_idx": 24, "end_idx": 25, "type": "dru", "entity": "喘定" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "二羟丙茶碱" }, { "start_idx": 33, "end_idx": 34, "type": "dru", "entity": "茶碱" }, { "start_idx": 53, "end_idx": 60, "type": "sym", "entity": "对胃的刺激性较小" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "胃" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "心脏" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "心动过速" } ] }, { "text": "口服:每次3~5mg/kg,每日3次。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" } ] }, { "text": "肌内注射:每次2.5~5mg/kg,每日1次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "肌内注射" } ] }, { "text": "静脉滴注:每次10~20mg/kg,每日1次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "3.茶碱的复方制剂(1)复方茶碱片:每片含茶碱25mg,盐酸麻黄碱10mg,非那西丁100mg,苯巴比妥10mg,氨基比林100mg,咖啡因1.5mg,柯柯碱25mg,颠茄浸膏2mg。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "茶碱的复方制剂" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "复方茶碱片" }, { "start_idx": 21, "end_idx": 22, "type": "dru", "entity": "茶碱" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "盐酸麻黄碱" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "非那西丁" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 57, "end_idx": 60, "type": "dru", "entity": "氨基比林" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "咖啡因" }, { "start_idx": 76, "end_idx": 78, "type": "dru", "entity": "柯柯碱" }, { "start_idx": 84, "end_idx": 87, "type": "dru", "entity": "颠茄浸膏" } ] }, { "text": "用法为每次1片,每日2次,口服。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "口服" } ] }, { "text": "(2)复方长效氨茶碱片:每片白色外层含氨茶碱100mg,氯苯那敏2mg,苯巴比妥15mg,氢氧化铝30mg;棕色内层含氨茶碱和茶碱各100mg。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "复方长效氨茶碱片" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "氯苯那敏" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "氢氧化铝" }, { "start_idx": 59, "end_idx": 61, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 63, "end_idx": 64, "type": "dru", "entity": "茶碱" } ] }, { "text": "外层在胃液内迅速崩解而呈速效;内层为缓释层,在肠液内缓慢崩解以维持药效。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胃液内" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肠液内" } ] }, { "text": "用法为每次1片,每日1~2次口服。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "口服" } ] }, { "text": "(3)止喘栓:每粒含氨茶碱0.2g,盐酸异丙嗪0.0125g,苯佐卡因0.023g;用法每次1粒,睡前塞入肛门。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "止喘栓" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "盐酸异丙嗪" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "苯佐卡因" }, { "start_idx": 49, "end_idx": 54, "type": "pro", "entity": "睡前塞入肛门" } ] }, { "text": "(4)阿斯美(强力阿司米通,Asmeton“strong”):每粒含盐酸甲氧那明(Methoxyphenamine,咳喘宁)12.5mg,那可丁7mg,氨茶碱25mg,氯苯那敏2mg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "阿斯美" }, { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "强力阿司米通" }, { "start_idx": 34, "end_idx": 39, "type": "dru", "entity": "盐酸甲氧那明" }, { "start_idx": 41, "end_idx": 56, "type": "dru", "entity": "Methoxyphenamine" }, { "start_idx": 58, "end_idx": 60, "type": "dru", "entity": "咳喘宁" }, { "start_idx": 69, "end_idx": 71, "type": "dru", "entity": "那可丁" }, { "start_idx": 76, "end_idx": 78, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 84, "end_idx": 87, "type": "dru", "entity": "氯苯那敏" } ] }, { "text": "4.茶碱缓释或控释剂(1)茶喘平(Theovent-LA):13~16岁每次125~250mg,6~8岁每次125mg,每12小时1次。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "茶碱缓释或控释剂" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "茶喘平" }, { "start_idx": 17, "end_idx": 27, "type": "dru", "entity": "Theovent-LA" } ] }, { "text": "剂量过大时可引起类似茶碱的副作用。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "茶碱" } ] }, { "text": "(2)舒弗美:3~6岁50mg,>6岁100mg,每日2次。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "舒弗美" } ] }, { "text": "(3)葆乐辉(优喘平Protheo):为长效无水茶碱控释片。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "葆乐辉" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "优喘平" }, { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "Protheo" }, { "start_idx": 20, "end_idx": 28, "type": "dru", "entity": "长效无水茶碱控释片" } ] }, { "text": "(二)茶碱类药物常见不良反应茶碱血浓度>25μg/ml,可以出现轻微的不良反应,包括恶心、呕吐、腹泻、易激惹;茶碱血浓度>35μg/ml,会出现较为严重的不良反应,如心律失常、低血压、突发性心跳停止和抽搐等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "茶碱血浓度" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "恶心" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呕吐" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "腹泻" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "腹" }, { "start_idx": 51, "end_idx": 53, "type": "sym", "entity": "易激惹" }, { "start_idx": 55, "end_idx": 59, "type": "ite", "entity": "茶碱血浓度" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "心律失常" }, { "start_idx": 88, "end_idx": 90, "type": "dis", "entity": "低血压" }, { "start_idx": 92, "end_idx": 98, "type": "dis", "entity": "突发性心跳停止" }, { "start_idx": 100, "end_idx": 101, "type": "sym", "entity": "抽搐" } ] }, { "text": "茶碱类药物治疗指数狭窄,干扰茶碱药代动力学的因素较多,个体差异大。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "茶碱类药物治疗" }, { "start_idx": 14, "end_idx": 15, "type": "dru", "entity": "茶碱" } ] }, { "text": "在使用茶碱类药物时要注意几个方面:①由于茶碱主要在肝脏代谢,因而严重的不良反应更容易出现在充血性心力衰竭、肝硬化和呼吸衰竭的患儿;②许多药物会干扰茶碱的代谢,引起茶碱血药浓度升高或降低,(表8-8);③个体差异大,治疗要强调个体化和测定血浓度;④不要超过每日总的茶碱剂量,(表8-8)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 20, "end_idx": 21, "type": "dru", "entity": "茶碱" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肝脏" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "肝硬化" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 73, "end_idx": 74, "type": "dru", "entity": "茶碱" }, { "start_idx": 81, "end_idx": 86, "type": "ite", "entity": "茶碱血药浓度" }, { "start_idx": 116, "end_idx": 120, "type": "ite", "entity": "测定血浓度" }, { "start_idx": 131, "end_idx": 132, "type": "dru", "entity": "茶碱" } ] }, { "text": "表8-19茶碱类药物的配伍及其临床意义表8-20达到治疗水平的平均每日茶碱总量", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "茶碱类药物" }, { "start_idx": 35, "end_idx": 36, "type": "dru", "entity": "茶碱" } ] }, { "text": "第九节输尿管膨出输尿管膨出亦称输尿管囊肿,是指膀胱内黏膜下输尿管末端的囊性扩张。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "输尿管膨出" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "输尿管膨出" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "输尿管囊肿" }, { "start_idx": 23, "end_idx": 38, "type": "sym", "entity": "膀胱内黏膜下输尿管末端的囊性扩张" }, { "start_idx": 23, "end_idx": 33, "type": "bod", "entity": "膀胱内黏膜下输尿管末端" } ] }, { "text": "膨出可开口于膀胱内,或异位开口于膀胱颈或更远端,多见于肾和输尿管重复畸形。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "膨出可开口于膀胱内" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "膀胱内" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "异位开口于膀胱颈或更远端" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "膀胱颈或更远端" }, { "start_idx": 27, "end_idx": 35, "type": "dis", "entity": "肾和输尿管重复畸形" } ] }, { "text": "其中60%~80%为异位型膨出,80%膨出来自重复肾的上肾部输尿管。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "异位型膨出" }, { "start_idx": 19, "end_idx": 32, "type": "sym", "entity": "膨出来自重复肾的上肾部输尿管" }, { "start_idx": 25, "end_idx": 32, "type": "bod", "entity": "肾的上肾部输尿管" } ] }, { "text": "【胚胎发生】输尿管膨出形成的原因尚不完全清楚。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "胚胎" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "输尿管膨出" } ] }, { "text": "多数意见是输尿管的Chwalle膜延迟破溃,导致输尿管管口狭窄及末端扩张。", "entities": [ { "start_idx": 5, "end_idx": 20, "type": "sym", "entity": "输尿管的Chwalle膜延迟破溃" }, { "start_idx": 5, "end_idx": 16, "type": "bod", "entity": "输尿管的Chwalle膜" }, { "start_idx": 24, "end_idx": 35, "type": "sym", "entity": "输尿管管口狭窄及末端扩张" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "输尿管管口" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "末端" } ] }, { "text": "【分型】输尿管膨出分为单纯型(原位型)及异位型两种。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "输尿管膨出" } ] }, { "text": "1.单纯型亦称原位型输尿管膨出,多见于成人及男性,膨出一般较小,位于正常位置的输尿管开口处,不阻塞膀胱颈部,常无症状,不易被发现,如不引起上尿路梗阻也不需处理。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "原位型输尿管膨出" }, { "start_idx": 25, "end_idx": 44, "type": "sym", "entity": "膨出一般较小,位于正常位置的输尿管开口处" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "输尿管开口处" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "阻塞膀胱颈部" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "膀胱颈部" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "上尿路梗阻" } ] }, { "text": "2.异位型此型占60%~80%,女性及小儿多见。", "entities": [] }, { "text": "由于对膀胱三角结构的破坏,常引起下肾部输尿管的反流。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "对膀胱三角结构的破坏" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "膀胱三角" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "下肾部输尿管的反流" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "下肾部输尿管" } ] }, { "text": "【临床表现】输尿管膨出可无任何症状,尤其是单纯型的,多在超声检查时被偶然发现。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "输尿管膨出" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "超声检查" } ] }, { "text": "异位型输尿管膨出位于膀胱基底部或延伸入尿道,可阻塞尿道内口,造成排尿困难及尿路感染。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "异位型输尿管膨出" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "膀胱基底部" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "尿道" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "阻塞尿道内口" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "尿道内口" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "排尿困难" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "尿" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "尿路感染" } ] }, { "text": "女孩常可见排尿时有肿块由尿道口脱出,为异位的输尿管膨出。", "entities": [ { "start_idx": 5, "end_idx": 16, "type": "sym", "entity": "排尿时有肿块由尿道口脱出" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "尿" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "尿道口" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "异位的输尿管膨出" } ] }, { "text": "如膨出内有结石形成则会出现血尿。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "膨出内有结石" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "血尿" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "血" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "尿" } ] }, { "text": "【诊断】静脉尿路造影(IVU)和排尿性膀胱尿道造影(VCUG)是主要的诊断方法。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "静脉尿路造影" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "IVU" }, { "start_idx": 16, "end_idx": 24, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "VCUG" } ] }, { "text": "如肾功能良好,由于膨出壁构成的低密度影,可以看到造影剂充盈入输尿管膨出部形成典型的蛇头样影,如内有结石,可有充盈缺损。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肾" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "膨出壁" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "低密度影" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "输尿管膨出部" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "蛇头样影" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "结石" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "充盈缺损" } ] }, { "text": "在VCUG中,可见无法充盈的输尿管膨出,也呈蛇头样。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "VCUG" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "输尿管膨出" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "呈蛇头样" } ] }, { "text": "异位输尿管膨出通常来自重复肾的上肾部,由于其功能差常不显影,而功能正常的下肾部被向下外侧推移,呈现出典型的“垂花”样(droopinglily)改变(图12-6)。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "异位输尿管膨出" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "重复肾的上肾部" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "功能差" }, { "start_idx": 31, "end_idx": 73, "type": "sym", "entity": "功能正常的下肾部被向下外侧推移,呈现出典型的“垂花”样(droopinglily)改变" }, { "start_idx": 31, "end_idx": 38, "type": "bod", "entity": "功能正常的下肾部" } ] }, { "text": "膀胱内输尿管膨出部无造影剂进入,形成充盈缺损,和VCUG的表现一样。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "膀胱内输尿管膨出部" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "充盈缺损" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "VCUG" } ] }, { "text": "但VCUG可发现膀胱输尿管反流情况。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "VCUG" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "有一点要引起注意,当膀胱内有大量造影剂进入时,反而因尿量的增加将膨出部压扁,充盈缺损现象反而消失。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "膀胱内" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "造影剂" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "尿量的增加" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "尿量" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "膨出部" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "膨出部压扁" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "充盈缺损现象" } ] }, { "text": "故要在膀胱充盈过程中观察输尿管膨出。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "膀胱" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "输尿管膨出" } ] }, { "text": "由于输尿管膨出内积有尿液,连同扩张的输尿管及肾脏可以在磁共振上得以显示。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "输尿管膨出内积有尿液" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "输尿管" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "尿液" }, { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "扩张的输尿管及肾脏" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "输尿管" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肾脏" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "磁共振" } ] }, { "text": "【治疗】输尿管膨出的治疗原则为解除梗阻,防止反流,保护肾功能。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "输尿管膨出" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "解除梗阻" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "防止反流" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "保护肾功能" } ] }, { "text": "无论单纯型或异位型输尿管膨出,无临床症状及并发症,肾功能良好者,均不需治疗。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "单纯型或异位型输尿管膨出" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肾" } ] }, { "text": "肾功能良好或尚可的单纯型或异位型输尿管膨出,如有临床症状并已造成输尿管及肾积水,均可先经膀胱镜进行膨出切开,使其引流通畅。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肾" }, { "start_idx": 9, "end_idx": 20, "type": "dis", "entity": "单纯型或异位型输尿管膨出" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "输尿管" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "输尿管及肾积水" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "肾" }, { "start_idx": 44, "end_idx": 46, "type": "pro", "entity": "膀胱镜" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "膨出切开" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "引流" } ] }, { "text": "单纯型膨出在其基底部做一横行切口,约0.5~0.8cm左右即可;异位型输尿管膨出则在其最下端纵行切开0.5~0.8cm。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "单纯型膨出" }, { "start_idx": 5, "end_idx": 30, "type": "pro", "entity": "在其基底部做一横行切口,约0.5~0.8cm左右即可" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "异位型输尿管膨出" }, { "start_idx": 41, "end_idx": 58, "type": "pro", "entity": "在其最下端纵行切开0.5~0.8cm" } ] }, { "text": "若膨出瘪缩,症状消失,肾功能好转,不需进一步治疗。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "膨出瘪缩" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肾" } ] }, { "text": "图12-6重复肾静脉尿路造影表现重复肾的上肾部,由于其功能差常不显影,而功能正常的下肾部被向下外侧推移,呈现出典型的“垂花”样改变(箭头所示)相应的肾功能严重受损或发育异常,应做肾及输尿管切除术。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "pro", "entity": "重复肾静脉尿路造影" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "重复肾的上肾部" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "功能差" }, { "start_idx": 36, "end_idx": 64, "type": "sym", "entity": "功能正常的下肾部被向下外侧推移,呈现出典型的“垂花”样改变" }, { "start_idx": 36, "end_idx": 43, "type": "bod", "entity": "功能正常的下肾部" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "肾" }, { "start_idx": 74, "end_idx": 85, "type": "sym", "entity": "肾功能严重受损或发育异常" }, { "start_idx": 89, "end_idx": 96, "type": "pro", "entity": "肾及输尿管切除术" } ] }, { "text": "如为重复畸形,做半肾切除。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "重复畸形" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "半肾切除" } ] }, { "text": "随着膨出瘪缩,绝大多数患儿症状可以消失,若仍有反复的尿路感染或排尿困难,再做膨出及输尿管残端切除。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "膨出瘪缩" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "尿路感染" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "排尿困难" }, { "start_idx": 38, "end_idx": 47, "type": "pro", "entity": "膨出及输尿管残端切除" } ] }, { "text": "三、轮状病毒疫苗轮状病毒(rotavirus,RV)是世界范围内引起婴幼儿腹泻的主要病原体。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "轮状病毒疫苗" }, { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 13, "end_idx": 21, "type": "mic", "entity": "rotavirus" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "RV" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "腹泻" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "腹" } ] }, { "text": "在全球范围内,每年因RV感染造成的死亡超过60万例。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "RV感染" } ] }, { "text": "WHO建议全球婴幼儿都应该接种轮状病毒疫苗。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "接种" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "轮状病毒疫苗" } ] }, { "text": "根据RV的组抗原蛋白(VP6)的抗原特异性差异,可将至今已发现的RV分为7个组(A~G),早期发现的RV为A组。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "mic", "entity": "RV的组抗原蛋白(VP6)的抗原" }, { "start_idx": 32, "end_idx": 33, "type": "mic", "entity": "RV" }, { "start_idx": 50, "end_idx": 51, "type": "mic", "entity": "RV" } ] }, { "text": "在人类,A组RV主要感染婴幼儿。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "mic", "entity": "A组RV" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "感染" } ] }, { "text": "RV外层蛋白VP7和VP4是主要的中和抗原。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "mic", "entity": "RV外层蛋白VP7和VP4" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "中和抗原" } ] }, { "text": "RV的基因分型是以VP7和VP4抗原性为基础的双重血清型(G/P型)分析系统,可将已发现的A组RV至少分为23种G型(VP7)和31种P型(VP4)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "RV的基因" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "VP7" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "VP4" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血清" }, { "start_idx": 45, "end_idx": 48, "type": "mic", "entity": "A组RV" }, { "start_idx": 59, "end_idx": 61, "type": "mic", "entity": "VP7" }, { "start_idx": 70, "end_idx": 72, "type": "mic", "entity": "VP4" } ] }, { "text": "目前国际上使用的轮状病毒疫苗有两种:Rotarix(比利时葛兰素史克公司研发)和RotaTeq(美国默克研究中心研发)轮状病毒疫苗,这两种疫苗对于预防严重轮状病毒腹泻高度有效,并且很安全。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "轮状病毒疫苗" }, { "start_idx": 18, "end_idx": 24, "type": "dru", "entity": "Rotarix" }, { "start_idx": 40, "end_idx": 46, "type": "dru", "entity": "RotaTeq" }, { "start_idx": 59, "end_idx": 64, "type": "dru", "entity": "轮状病毒疫苗" }, { "start_idx": 69, "end_idx": 70, "type": "dru", "entity": "疫苗" }, { "start_idx": 75, "end_idx": 82, "type": "dis", "entity": "严重轮状病毒腹泻" } ] }, { "text": "在拉丁美洲超过63000名婴幼儿参与了Rotarix的安全评价,结果证实该疫苗既不会引起发热,也与肠套叠无关。", "entities": [ { "start_idx": 19, "end_idx": 25, "type": "dru", "entity": "Rotarix" }, { "start_idx": 37, "end_idx": 38, "type": "dru", "entity": "疫苗" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "发热" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "肠套叠" } ] }, { "text": "而RotaTeq的临床安全实验是在在芬兰和美国进行的。", "entities": [ { "start_idx": 1, "end_idx": 14, "type": "pro", "entity": "RotaTeq的临床安全实验" } ] }, { "text": "一项超过70000名婴幼儿参加的临床实验表明,该疫苗不会引起发热和肠套叠。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "dru", "entity": "疫苗" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "发热" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "肠套叠" } ] }, { "text": "该疫苗的保护效率也较高,针对所有G1~G4型引起的RV肠胃炎的保护率达74.0%,对严重肠胃炎的保护率达98.0%,对住院治疗以及急诊病例的保护率达94.5%。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "疫苗" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "RV肠胃炎" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "严重肠胃炎" } ] }, { "text": "目前,具有代表性的可替代的RV候选疫苗也正在研发中,以期最终开发出新的疫苗,并进行推广使用。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "RV" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "疫苗" }, { "start_idx": 35, "end_idx": 36, "type": "dru", "entity": "疫苗" } ] }, { "text": "青少年由于面临生理上的巨大变化、学习上的压力、渴求与同伴交往等,他们经常会出现一些心理问题,因此,要求素质较高的教师给予心理疏导,同时培养他们人际交往技能和应付压力的能力。", "entities": [ { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "心理问题" }, { "start_idx": 51, "end_idx": 63, "type": "pro", "entity": "素质较高的教师给予心理疏导" }, { "start_idx": 67, "end_idx": 84, "type": "pro", "entity": "培养他们人际交往技能和应付压力的能力" } ] }, { "text": "第二节血小板功能异常性疾病血小板功能异常(qualitativeplateletdefects)引起的出血性疾病,是指血小板数量正常但血小板栓子形成缺陷导致的疾病,临床上共同的特点是出血时间延长和出血倾向。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "血小板功能异常性疾病" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "血小板功能异常" }, { "start_idx": 21, "end_idx": 46, "type": "dis", "entity": "qualitativeplateletdefects" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "血性疾病" }, { "start_idx": 59, "end_idx": 63, "type": "ite", "entity": "血小板数量" }, { "start_idx": 67, "end_idx": 75, "type": "sym", "entity": "血小板栓子形成缺陷" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "血小板栓子" }, { "start_idx": 91, "end_idx": 96, "type": "sym", "entity": "出血时间延长" }, { "start_idx": 91, "end_idx": 94, "type": "ite", "entity": "出血时间" }, { "start_idx": 98, "end_idx": 101, "type": "sym", "entity": "出血倾向" }, { "start_idx": 99, "end_idx": 99, "type": "bod", "entity": "血" } ] }, { "text": "出血倾向以皮肤黏膜为主,如皮肤瘀点、瘀斑,牙龈出血、黏膜出血等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "出血倾向" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "血" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "皮肤瘀点、瘀斑" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮肤" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "牙龈出血" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "黏膜出血" } ] }, { "text": "前者常发生于其他疾病中,如慢性肝、肾疾病,白血病、骨髓增生紊乱综合征、弥漫性血管内凝血(DIC)、病毒感染、甲状腺机能减退等。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "慢性肝、肾疾病" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "白血病" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "骨髓增生紊乱综合征" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "弥漫性血管内凝血" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "DIC" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "病毒感染" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "甲状腺机能减退" } ] }, { "text": "某些药物如阿司匹林、肝素、双嘧达莫等是临床常用的干扰血小板功能的药物。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "肝素" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 24, "end_idx": 33, "type": "dru", "entity": "干扰血小板功能的药物" } ] }, { "text": "本节将重点讨论先天性血小板功能异常性疾病。", "entities": [ { "start_idx": 7, "end_idx": 19, "type": "dis", "entity": "先天性血小板功能异常性疾病" } ] }, { "text": "先天性血小板功能缺陷性疾病可发生于血小板的黏附、聚集、释放各个阶段,也可见于血小板-凝血蛋白相互作用的缺陷。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "先天性血小板功能缺陷性疾病" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血小板" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "血小板" }, { "start_idx": 38, "end_idx": 52, "type": "sym", "entity": "血小板-凝血蛋白相互作用的缺陷" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "凝血蛋白" } ] }, { "text": "一、巨大血小板综合征巨大血小板综合征又称Bernard-Soulier综合征(Bernard-Souliersyndrome,BSS)以轻~中度的血小板减少,血小板体积增大,出血时间延长,凝血酶原消耗不良为特征,这是一种常染色体隐性遗传血小板黏附功能缺陷性出血疾病。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "巨大血小板综合征" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "巨大血小板综合征" }, { "start_idx": 20, "end_idx": 37, "type": "dis", "entity": "Bernard-Soulier综合征" }, { "start_idx": 39, "end_idx": 61, "type": "dis", "entity": "Bernard-Souliersyndrome" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "BSS" }, { "start_idx": 68, "end_idx": 77, "type": "sym", "entity": "轻~中度的血小板减少" }, { "start_idx": 73, "end_idx": 75, "type": "ite", "entity": "血小板" }, { "start_idx": 79, "end_idx": 85, "type": "sym", "entity": "血小板体积增大" }, { "start_idx": 79, "end_idx": 83, "type": "ite", "entity": "血小板体积" }, { "start_idx": 87, "end_idx": 92, "type": "sym", "entity": "出血时间延长" }, { "start_idx": 87, "end_idx": 90, "type": "ite", "entity": "出血时间" }, { "start_idx": 94, "end_idx": 97, "type": "ite", "entity": "凝血酶原" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "凝血酶原消耗" }, { "start_idx": 110, "end_idx": 131, "type": "dis", "entity": "常染色体隐性遗传血小板黏附功能缺陷性出血疾病" } ] }, { "text": "【发病机制】BSS的根本问题在于血小板膜糖蛋白GPⅠb-Ⅸ和GPV缺陷,聚丙烯酰胺电泳表明这实际上是一种缺乏,至今尚未发现GPⅠb-Ⅸ数量正常而功能异常的变异型。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "BSS" }, { "start_idx": 16, "end_idx": 34, "type": "sym", "entity": "血小板膜糖蛋白GPⅠb-Ⅸ和GPV缺陷" }, { "start_idx": 16, "end_idx": 32, "type": "bod", "entity": "血小板膜糖蛋白GPⅠb-Ⅸ和GPV" }, { "start_idx": 36, "end_idx": 42, "type": "pro", "entity": "聚丙烯酰胺电泳" }, { "start_idx": 61, "end_idx": 68, "type": "ite", "entity": "GPⅠb-Ⅸ数量" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "功能异常" } ] }, { "text": "血小板止血功能的第一步是黏附在受损的血管壁上,以异二聚体形式存在于血小板膜上的GPⅠb-Ⅸ是血小板的主要黏附受体。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "止血" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "受损的血管壁上" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "异二聚体" }, { "start_idx": 33, "end_idx": 44, "type": "bod", "entity": "血小板膜上的GPⅠb-Ⅸ" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "血小板" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "黏附受体" } ] }, { "text": "进一步的研究发现GPⅠb由α、β两条多肽链组成;其中α链含有血管性血友病因子(vonWillebrandFactor,vWF)的特异受体。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "GPⅠb" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "α、β两条多肽链" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "α链" }, { "start_idx": 30, "end_idx": 37, "type": "bod", "entity": "血管性血友病因子" }, { "start_idx": 39, "end_idx": 57, "type": "bod", "entity": "vonWillebrandFactor" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "vWF" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "受体" } ] }, { "text": "在血流的情况下GPⅠb缺乏的血小板不能与血管内皮下基质中的vWF相结合,因而就不能实现其黏附功能。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血流" }, { "start_idx": 7, "end_idx": 34, "type": "sym", "entity": "GPⅠb缺乏的血小板不能与血管内皮下基质中的vWF相结合" }, { "start_idx": 7, "end_idx": 16, "type": "bod", "entity": "GPⅠb缺乏的血小板" }, { "start_idx": 20, "end_idx": 31, "type": "bod", "entity": "血管内皮下基质中的vWF" } ] }, { "text": "GPV与GPⅠb-Ⅸ的关系尚不清楚,可能与膜的稳定性有关,同时它也是血小板膜上对凝血酶原敏感的蛋白,可能是导致凝血酶原消耗不良的原因。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "GPV" }, { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "GPⅠb-Ⅸ" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "膜" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "血小板膜上" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "蛋白" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 55, "end_idx": 62, "type": "sym", "entity": "凝血酶原消耗不良" } ] }, { "text": "血小板的体积变大,推测是由于GPⅠb-Ⅸ复合物缺乏,导致血小板膜与细胞骨架失去联系并变形而成。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "血小板的体积" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "血小板的体积变大" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "GPⅠb-Ⅸ复合物缺乏" }, { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "GPⅠb-Ⅸ复合物" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "血小板膜" }, { "start_idx": 28, "end_idx": 43, "type": "sym", "entity": "血小板膜与细胞骨架失去联系并变形" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "细胞骨架" } ] }, { "text": "所以BSS的出血表现是由于GPⅠb-Ⅸ复合物缺乏、血小板数减少、血小板增大而不易转运至受损血管壁的共同作用的结果。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "BSS" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "出血" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "血" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "GPⅠb-Ⅸ复合物缺乏" }, { "start_idx": 13, "end_idx": 21, "type": "bod", "entity": "GPⅠb-Ⅸ复合物" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "血小板" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "血小板数减少" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "血小板" }, { "start_idx": 32, "end_idx": 47, "type": "sym", "entity": "血小板增大而不易转运至受损血管壁" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "受损血管壁" } ] }, { "text": "另据遗传研究发现BSS是由于GPⅠbα和GPⅨ的点上突变所引起。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "BSS" }, { "start_idx": 14, "end_idx": 27, "type": "sym", "entity": "GPⅠbα和GPⅨ的点上突变" }, { "start_idx": 14, "end_idx": 25, "type": "bod", "entity": "GPⅠbα和GPⅨ的点上" } ] }, { "text": "【临床表现】杂合子可有血小板体积增大等生物学异常,但无出血症状。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "血小板" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "血小板体积增大" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "出血" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "血" } ] }, { "text": "纯合子多有中到重度的出血,以皮肤黏膜自发性出血为主,如瘀点、瘀斑、鼻出血、牙龈出血、胃肠道出血、月经过多等,重者也可血尿乃至颅内出血。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "中到重度的出血" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "皮肤黏膜自发性出血" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "瘀点" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "瘀斑" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "鼻出血" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "牙龈出血" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "胃肠道出血" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "月经过多" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "血尿" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "血" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "尿" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "颅内出血" } ] }, { "text": "【实验室检查】血小板数减少伴巨大血小板,出血时间延长、凝血酶原消耗不良、血小板黏附功能降低,瑞斯托霉素及人或牛的vWF不能诱导血小板聚集,低浓度的凝血酶诱导血小板聚集降低。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "血小板数" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "血小板数减少" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "巨大血小板" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "血小板" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "出血时间延长" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "出血时间" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "凝血酶原消耗不良" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "血小板" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "血小板黏附功能降低" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "瑞斯托霉素" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "血小板" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "凝血酶" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "血小板" }, { "start_idx": 78, "end_idx": 84, "type": "sym", "entity": "血小板聚集降低" } ] }, { "text": "如作膜糖蛋白测定,可发现血小板膜GPⅠb、Ⅸ及GPV降低或缺乏。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "ite", "entity": "膜糖蛋白测定" }, { "start_idx": 12, "end_idx": 30, "type": "sym", "entity": "血小板膜GPⅠb、Ⅸ及GPV降低或缺乏" }, { "start_idx": 12, "end_idx": 25, "type": "ite", "entity": "血小板膜GPⅠb、Ⅸ及GPV" } ] }, { "text": "【诊断】国内1981年制定、1986年修改的巨大血小板综合征诊断标准如下。", "entities": [ { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "巨大血小板综合征" } ] }, { "text": "(一)临床表现1.常染色体隐性遗传,男女均可患病。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "常染色体" } ] }, { "text": "2.轻度至中度皮肤、黏膜出血,女性月经过多。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "sym", "entity": "轻度至中度皮肤、黏膜出血" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "皮肤、黏膜出血" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "月经过多" } ] }, { "text": "3.肝脾不大。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肝" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "脾" } ] }, { "text": "(二)实验室检查1.血小板减少伴巨大血小板。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "血小板" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "血小板减少" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "巨大血小板" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "血小板" } ] }, { "text": "2.出血时间延长。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "出血时间延长" }, { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "出血时间" } ] }, { "text": "3.血小板聚集试验加瑞斯托霉素不聚集。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "血小板聚集试验" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "瑞斯托霉素" } ] }, { "text": "4.血小板玻珠滞留试验可减低。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "血小板玻珠滞留试验" } ] }, { "text": "6.vWF正常。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "vWF" } ] }, { "text": "7.血小板膜缺乏糖蛋白Ⅰb(GpⅠb)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "血小板膜" }, { "start_idx": 2, "end_idx": 18, "type": "sym", "entity": "血小板膜缺乏糖蛋白Ⅰb(GpⅠb)" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "糖蛋白Ⅰb" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "GpⅠb" } ] }, { "text": "8.排除继发性巨血小板症。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "继发性巨血小板症" } ] }, { "text": "40岁以后随体内肌肉组织的减少,含水量也下降,约为体重的45%~50%。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "体内肌肉组织" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "体内肌肉组织的减少" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "含水量也下降" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "含水量" } ] }, { "text": "体内水与蛋白质、碳水化合物或类脂相结合,形成胶体状态,主要分布在细胞内和细胞外。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "水" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "蛋白质" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "碳水化合物" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "类脂" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "细胞内" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "细胞外" } ] }, { "text": "其中,细胞内含水量占总水量的2/3,细胞外含水量约为1/3。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "细胞内" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "水" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "水" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "细胞外" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "水" } ] }, { "text": "各组织器官的含水量相差很大,以血液中最多,肌肉其次,脂肪组织中最少。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "组织器官" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "水" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "血液中" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肌肉" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "脂肪组织中" } ] }, { "text": "【水的平衡】水的来源不仅限于摄入的液体,还来自于固体食物中的水分以及食物氧化和组织细胞代谢所产生的水分(即内生水,混合饮食每100kcal热量产生的水为12g)。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "水" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "水" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "组织" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "细胞" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "水" } ] }, { "text": "水的排出主要通过肾脏,约占60%左右;其次由肺和皮肤,约占30%;正常情况下由消化道排出仅占10%以下。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "水" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肾脏" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "皮肤" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "消化道" } ] }, { "text": "【水的生理功能】①构成细胞和体液的重要组成部分,是保持每一个细胞外形和组成每一种体液不可缺少的物质。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "水" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "细胞" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "体液" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "细胞" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "体液" } ] }, { "text": "如血液中含水分高达83%,肌肉含水76%,骨骼含水22%,脂肪组织含水10%。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "血液中" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "水" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肌肉" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "水" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "骨骼" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "水" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "脂肪组织" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "水" } ] }, { "text": "不溶于水的蛋白质和脂肪分子可形成胶体或乳糜液,有利于营养素的消化、吸收和利用。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "蛋白质" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "脂肪分子" } ] }, { "text": "通过大小便、汗液以及呼吸等途径把代谢产物和有毒物质排出体外。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "大小便" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "汗液" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "代谢产物" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "有毒物质" } ] }, { "text": "水的比热大,可通过蒸发和出汗使皮肤散热,调节体温保持不变。", "entities": [ { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "汗" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" } ] }, { "text": "水可使皮肤滋润,眼泪、唾液、关节囊液和浆膜腔液则是相应器官的润滑剂。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "眼泪" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "唾液" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "关节囊液" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "浆膜腔液" } ] }, { "text": "婴儿新陈代谢旺盛,热量需要较多,但因肾脏浓缩功能尚未完善,因此所需的水分相对较多。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肾脏" } ] }, { "text": "假如婴幼儿每天摄入水量少于60ml/kg,即可出现脱水症状。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "脱水症状" } ] }, { "text": "第二节窦性心律失常窦性心律不齐是窦房结冲动发放的一种正常生理变异,与呼吸有关。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "窦性心律失常" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "窦性心律不齐" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "窦房结" } ] }, { "text": "患热性疾病或服用增加迷走张力的药物如地高辛时,窦性心律不齐加剧。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "热性疾病" }, { "start_idx": 8, "end_idx": 16, "type": "dru", "entity": "增加迷走张力的药物" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "地高辛" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "窦性心律不齐加剧" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "窦性心律不齐" } ] }, { "text": "运动时,窦性心律不齐通常消失。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "窦性心律不齐" } ] }, { "text": "某些全身性疾病如黏液水肿可出现窦性心动过缓,疾病控制后窦性心动过缓消失。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "黏液水肿" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "窦性心动过缓" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "心" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "窦性心动过缓" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "心" } ] }, { "text": "窦性心动过缓应与窦房及房室传导阻滞区别。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "窦性心动过缓" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "窦房及房室传导阻滞" } ] }, { "text": "窦性心动过缓患儿运动时心率可增加至100次/分以上,房室传导阻滞的患儿则不能。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "窦性心动过缓" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "心率" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "房室传导阻滞" } ] }, { "text": "低出生体重婴儿窦性心率变化很大。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "窦性心率变化很大" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "心" } ] }, { "text": "在这些婴儿窦性心动过缓多见,并可伴交界性逸搏。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "窦性心动过缓" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "心" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "交界性逸搏" } ] }, { "text": "房性期前收缩也很常见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房性期前收缩" } ] }, { "text": "这些心律的变化,尤其是心动过缓,在睡眠时更易出现,不伴症状,无需治疗。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "心律" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "心动过缓" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "心" } ] }, { "text": "游走心律是心脏的起搏点自窦房结至心房的任一部位周期性移动。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "心律" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心脏" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "窦房结" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心房" } ] }, { "text": "这种情况也可出现在患中枢神经系统疾患的患儿,如蛛网膜下腔出血。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "中枢神经系统疾患" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "蛛网膜下腔出血" } ] }, { "text": "第五节肺泡性蛋白沉积症肺泡性蛋白沉积症(pulmonaryalveolarproteinosis,PAP)以肺内有富含脂质的糖原染色阳性蛋白物质沉积并影响气体交换为特点。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肺泡性蛋白沉积症" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "肺泡性蛋白沉积症" }, { "start_idx": 20, "end_idx": 47, "type": "dis", "entity": "pulmonaryalveolarproteinosis" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "PAP" }, { "start_idx": 54, "end_idx": 80, "type": "sym", "entity": "肺内有富含脂质的糖原染色阳性蛋白物质沉积并影响气体交换" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肺内" }, { "start_idx": 57, "end_idx": 63, "type": "bod", "entity": "富含脂质的糖原" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "蛋白物质" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "气体" } ] }, { "text": "儿童PAP有两种类型:(一)先天性PAP常在出生后立即出现症状,并迅速出现呼吸衰竭,临床上与其他严重心肺疾病无法区别。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童PAP" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "先天性PAP" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "迅速出现呼吸衰竭" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "严重心肺疾病" } ] }, { "text": "其病因尚未明确,部分患儿与表面活性蛋白B(SP-B)遗传性缺乏有关。", "entities": [ { "start_idx": 13, "end_idx": 30, "type": "sym", "entity": "表面活性蛋白B(SP-B)遗传性缺乏" }, { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "表面活性蛋白B" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "SP-B" } ] }, { "text": "此外,粒细胞-巨噬细胞集落刺激因子(GM-CSF)可能与本病的发生有关。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "bod", "entity": "粒细胞-巨噬细胞集落刺激因子" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "GM-CSF" } ] }, { "text": "动物实验表明,GM-CSF及其受体缺陷鼠由于无法清除表面活性蛋白而发生PAP,应用GM-CSF则可缓解症状;人类研究中亦发现,一些PAP婴儿存在GM-CSF受体β亚单位表达缺陷。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "mic", "entity": "GM-CSF" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "活性蛋白" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "PAP" }, { "start_idx": 41, "end_idx": 46, "type": "dru", "entity": "GM-CSF" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "PAP" }, { "start_idx": 72, "end_idx": 87, "type": "dis", "entity": "GM-CSF受体β亚单位表达缺陷" } ] }, { "text": "(二)获得性或成人型PAP在儿童期较少见。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "获得性或成人型PAP" } ] }, { "text": "表现为呼吸困难、乏力、咳嗽、体重下降、胸痛、咯血等,晚期出现发绀、杵状指(趾)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "乏力" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "咳嗽" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "体重下降" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "胸痛" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "胸" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "咯血" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "血" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "发绀" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "杵状指(趾)" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "指" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "趾" } ] }, { "text": "某些患者开始时呈结节状阴影,从两下叶浸润,进展为全大叶实变。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "结节状阴影" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "两下叶浸润" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "两下叶" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "全大叶实变" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "全大叶" } ] }, { "text": "SP-B缺陷者几乎均在3个月内死亡。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "SP-B缺陷" } ] }, { "text": "获得性PAP可采用反复肺灌洗,亦可应用重组GM-CSF,后者具有较好的效果,可能成为代替肺灌洗的一种治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "获得性PAP" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "反复肺灌洗" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "重组GM-CSF" }, { "start_idx": 44, "end_idx": 46, "type": "pro", "entity": "肺灌洗" } ] }, { "text": "第十章免疫接种第一节我国儿童国家免疫规划利用疫苗(vaccine)来预防儿童感染性疾病是20世纪医学的重大贡献。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "免疫接种" }, { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "疫苗" }, { "start_idx": 25, "end_idx": 31, "type": "dru", "entity": "vaccine" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "该计划包括在全球范围内使用针对白喉、百日咳、破伤风、脊髓灰质炎、结核和麻疹6种疾病的疫苗。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "白喉" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "百日咳" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "破伤风" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "脊髓灰质炎" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "结核" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "麻疹" }, { "start_idx": 42, "end_idx": 43, "type": "dru", "entity": "疫苗" } ] }, { "text": "我国的儿童国家免疫规划包括国家免疫规划疫苗(表7-7)和省级增加的免疫规划疫苗两部分。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dru", "entity": "疫苗" }, { "start_idx": 37, "end_idx": 38, "type": "dru", "entity": "疫苗" } ] }, { "text": "省级人民政府在执行国家免疫规划时,根据本行政区域的传染病流行情况、人群免疫状况等因素,可以增加免费向公民提供的疫苗种类。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "传染病" }, { "start_idx": 55, "end_idx": 56, "type": "dru", "entity": "疫苗" } ] }, { "text": "2007年12月29日卫生部印发《扩大国家免疫规划实施方案》的通知(卫疾控发〔2007〕305号),我国扩大国家免疫规划实施方案,将乙型肝炎、结核病、脊髓灰质炎、百日咳、白喉、破伤风、麻疹、甲型肝炎、流行性脑脊髓膜炎、流行性乙型脑炎、风疹、流行性腮腺炎、流行性出血热、炭疽和钩端螺旋体病等15种疫苗可预防的传染病列入国家免疫规划。", "entities": [ { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "乙型肝炎" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "结核病" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "脊髓灰质炎" }, { "start_idx": 81, "end_idx": 83, "type": "dis", "entity": "百日咳" }, { "start_idx": 85, "end_idx": 86, "type": "dis", "entity": "白喉" }, { "start_idx": 88, "end_idx": 90, "type": "dis", "entity": "破伤风" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "麻疹" }, { "start_idx": 95, "end_idx": 98, "type": "dis", "entity": "甲型肝炎" }, { "start_idx": 100, "end_idx": 107, "type": "dis", "entity": "流行性脑脊髓膜炎" }, { "start_idx": 109, "end_idx": 115, "type": "dis", "entity": "流行性乙型脑炎" }, { "start_idx": 117, "end_idx": 118, "type": "dis", "entity": "风疹" }, { "start_idx": 120, "end_idx": 125, "type": "dis", "entity": "流行性腮腺炎" }, { "start_idx": 127, "end_idx": 132, "type": "dis", "entity": "流行性出血热" }, { "start_idx": 134, "end_idx": 135, "type": "dis", "entity": "炭疽" }, { "start_idx": 137, "end_idx": 142, "type": "dis", "entity": "钩端螺旋体病" }, { "start_idx": 147, "end_idx": 148, "type": "dru", "entity": "疫苗" } ] }, { "text": "表7-2我国儿童国家免疫规划疫苗及免疫时间表注:*加强免疫;**复种【使用规定】1.国家免疫规划疫苗常规免疫为:卡介苗接种1剂次;乙肝疫苗接种3剂次;脊灰疫苗口服4剂次,前3剂次为基础免疫,第4剂次为加强免疫;百白破疫苗接种5剂次,前3剂次为基础免疫,第4剂次为加强免疫;第5剂次使用白破疫苗加强免疫1剂次;麻疹疫苗接种2剂次,第2剂次为复种。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dru", "entity": "免疫规划疫苗" }, { "start_idx": 25, "end_idx": 30, "type": "dru", "entity": "" }, { "start_idx": 37, "end_idx": 42, "type": "dru", "entity": "" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "复种" }, { "start_idx": 62, "end_idx": 67, "type": "dru", "entity": "免疫规划疫苗" }, { "start_idx": 74, "end_idx": 76, "type": "dru", "entity": "卡介苗" }, { "start_idx": 77, "end_idx": 78, "type": "pro", "entity": "接种" }, { "start_idx": 83, "end_idx": 86, "type": "dru", "entity": "乙肝疫苗" }, { "start_idx": 87, "end_idx": 88, "type": "pro", "entity": "接种" }, { "start_idx": 93, "end_idx": 96, "type": "dru", "entity": "脊灰疫苗" }, { "start_idx": 97, "end_idx": 98, "type": "pro", "entity": "口服" }, { "start_idx": 123, "end_idx": 127, "type": "dru", "entity": "百白破疫苗" }, { "start_idx": 128, "end_idx": 129, "type": "pro", "entity": "接种" }, { "start_idx": 160, "end_idx": 163, "type": "dru", "entity": "白破疫苗" }, { "start_idx": 172, "end_idx": 175, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 176, "end_idx": 177, "type": "pro", "entity": "接种" }, { "start_idx": 187, "end_idx": 188, "type": "pro", "entity": "复种" } ] }, { "text": "3.免疫程序所列各种疫苗第1剂的接种时间为最小免疫起始月龄。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "疫苗" } ] }, { "text": "4.脊灰疫苗、百白破疫苗各剂次的间隔时间应≥28天。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "脊灰疫苗" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "百白破疫苗" } ] }, { "text": "5.乙肝疫苗第1剂在新生儿出生后24小时内尽早接种,第2剂在第1剂接种后1个月接种,第3剂在第1剂接种后6个月(6月龄)接种。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "乙肝疫苗" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "尽早接种" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "接种" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "接种" }, { "start_idx": 60, "end_idx": 61, "type": "pro", "entity": "接种" } ] }, { "text": "6.麻疹疫苗复种可使用含麻疹疫苗成分的其他联合疫苗,如麻疹风疹联合减毒活疫苗、麻疹腮腺炎风疹联合减毒活疫苗等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "复种" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "联合疫苗" }, { "start_idx": 27, "end_idx": 37, "type": "dru", "entity": "麻疹风疹联合减毒活疫苗" }, { "start_idx": 39, "end_idx": 52, "type": "dru", "entity": "麻疹腮腺炎风疹联合减毒活疫苗" } ] }, { "text": "7.如需同时接种两种以上国家免疫规划疫苗,应在不同部位接种,并严格按照第四章5.2要求进行接种。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dru", "entity": "疫苗" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "不同部位接种" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "接种" } ] }, { "text": "严禁将几种疫苗混合吸入1支注射器内接种。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "疫苗" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "接种" } ] }, { "text": "2种减毒活疫苗如未同时接种,应至少间隔4周再接种。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "减毒活疫苗" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "接种" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "接种" } ] }, { "text": "8.未完成基础免疫的14岁内儿童应尽早进行补种。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "补种" } ] }, { "text": "(2)未完成国家免疫规划疫苗免疫程序规定剂次的儿童,只需补种未完成的剂次。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "疫苗" } ] }, { "text": "(3)未完成百白破疫苗免疫程序的儿童,3月龄~6岁儿童使用百白破疫苗;7~11岁儿童使用白破联合疫苗;12岁以上儿童使用成人及青少年用白破联合疫苗。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "百白破疫苗" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "百白破疫苗" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "白破联合疫苗" }, { "start_idx": 67, "end_idx": 72, "type": "dru", "entity": "白破联合疫苗" } ] }, { "text": "(4)未完成脊灰疫苗免疫程序的儿童,4岁以下儿童未达到3剂次(含强化免疫等),应补种完成3剂次。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "脊灰疫苗" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "补种" } ] }, { "text": "(5)未完成麻疹疫苗免疫程序的儿童,未达到2剂次(含强化免疫等),应补种完成2剂次。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "补种" } ] }, { "text": "第四节麻疹麻疹(measles)是由麻疹病毒引起的急性出疹性呼吸道传染病,临床上具有发热、流涕、结合膜炎、咳嗽、麻疹黏膜斑和全身斑丘疹,疹退后糠麸样脱屑并留有色素沉着等特征。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "麻疹" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "麻疹" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "measles" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 25, "end_idx": 35, "type": "dis", "entity": "急性出疹性呼吸道传染病" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "发热" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "流涕" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "结合膜炎" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "咳嗽" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "麻疹黏膜斑" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "全身斑丘疹" }, { "start_idx": 68, "end_idx": 82, "type": "sym", "entity": "疹退后糠麸样脱屑并留有色素沉着" } ] }, { "text": "该病传染性极强,在广泛应用麻疹减毒活疫苗后,其典型周期性流行已得以控制,发病率和病死率大幅下降。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "dru", "entity": "麻疹减毒活疫苗" } ] }, { "text": "【病原和流行病学】麻疹病毒(measlesvirus)属副黏病毒科麻疹病毒属。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 14, "end_idx": 25, "type": "mic", "entity": "measlesvirus" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "副黏病毒" }, { "start_idx": 33, "end_idx": 36, "type": "mic", "entity": "麻疹病毒" } ] }, { "text": "H蛋白能与细胞受体结合并具血凝功能。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "H蛋白" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "细胞受体" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "血" } ] }, { "text": "F蛋白与病毒细胞融合和病毒溶血特性有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "F蛋白" }, { "start_idx": 4, "end_idx": 7, "type": "mic", "entity": "病毒细胞" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "溶血" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" } ] }, { "text": "M蛋白与病毒释出有关,与病毒RNA蛋白复合体结合后可抑制病毒转录。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "M蛋白" }, { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "病毒" }, { "start_idx": 12, "end_idx": 21, "type": "mic", "entity": "病毒RNA蛋白复合体" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒在人或猴原代肾细胞或EB病毒转化绢猴淋巴细胞系(B95-8)中生长良好;体外生存力弱,对热(56℃30分钟)、酸(pH<4.5)、紫外线和一般消毒剂均敏感。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肾细胞" }, { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "EB病毒" }, { "start_idx": 18, "end_idx": 24, "type": "mic", "entity": "绢猴淋巴细胞系" }, { "start_idx": 73, "end_idx": 75, "type": "dru", "entity": "消毒剂" } ] }, { "text": "患者在前驱期和出疹期眼结合膜、鼻咽分泌物、血和尿中存有病毒,通过呼吸道飞沫小滴或接触传播。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "出疹" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "眼结合膜" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 28, "type": "mic", "entity": "病毒" }, { "start_idx": 32, "end_idx": 38, "type": "bod", "entity": "呼吸道飞沫小滴" } ] }, { "text": "在应用麻疹疫苗前,麻疹呈周期性流行,易感者初感麻疹后发病率几乎为100%,全球每年发生麻疹约1亿~3亿例,死亡700万~800万例;我国1956~1965年间,麻疹报告发病率平均为766/10万;死亡率最高达39.7/10万。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "麻疹" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "麻疹" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "麻疹" }, { "start_idx": 80, "end_idx": 81, "type": "dis", "entity": "麻疹" } ] }, { "text": "广泛使用麻疹疫苗后,全国麻疹报告发病率自1987年以来一直控制在10/10万左右,死亡率则在0.1/10万以下;流行周期被打破或消失;流行形式主要为散在发病,但少数地区仍有周期性流行,大量易感染人群积累是其主要原因。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "麻疹" }, { "start_idx": 95, "end_idx": 96, "type": "dis", "entity": "感染" } ] }, { "text": "【发病机制和病理改变】病毒经鼻咽部,亦可能经结合膜侵入,在局部上皮细胞内增殖,而后播散到局部淋巴组织,在感染后2~3天形成第一次病毒血症,进而在局部和远处器官的单核-吞噬细胞系统内增殖,此时大量病毒到达皮肤和内脏,临床出现症状。", "entities": [ { "start_idx": 11, "end_idx": 26, "type": "sym", "entity": "病毒经鼻咽部,亦可能经结合膜侵入" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "结合膜" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "在局部上皮细胞内增殖" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "局部上皮细胞内" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "播散到局部淋巴组织" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "局部淋巴组织" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "感染" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "病毒血症" }, { "start_idx": 71, "end_idx": 91, "type": "sym", "entity": "在局部和远处器官的单核-吞噬细胞系统内增殖" }, { "start_idx": 72, "end_idx": 89, "type": "bod", "entity": "局部和远处器官的单核-吞噬细胞系统内" }, { "start_idx": 97, "end_idx": 98, "type": "mic", "entity": "病毒" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "皮肤" }, { "start_idx": 104, "end_idx": 105, "type": "bod", "entity": "内脏" } ] }, { "text": "至感染第15~17天,病毒血症逐渐消失,器官内病毒快速减少至消除。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "感染" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "病毒血症" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "器官内病毒" } ] }, { "text": "麻疹病毒直接损伤皮肤黏膜的血管内皮;CTL细胞杀伤病毒感染的靶细胞(上皮和内皮细胞、单核细胞和巨噬细胞),导致血管扩张和血浆渗漏;形成抗原抗体复合物,活化补体,造成血管内皮细胞损伤等参与麻疹的致病机制。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "麻疹病毒直接损伤皮肤黏膜的血管内皮" }, { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "血管内皮" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "CTL细胞" }, { "start_idx": 18, "end_idx": 51, "type": "sym", "entity": "CTL细胞杀伤病毒感染的靶细胞(上皮和内皮细胞、单核细胞和巨噬细胞)" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "病毒感染" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "靶细胞" }, { "start_idx": 34, "end_idx": 40, "type": "bod", "entity": "上皮和内皮细胞" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "单核细胞" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "血管扩张" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "血管" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "血浆渗漏" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "血浆" }, { "start_idx": 65, "end_idx": 73, "type": "sym", "entity": "形成抗原抗体复合物" }, { "start_idx": 67, "end_idx": 73, "type": "bod", "entity": "抗原抗体复合物" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "活化补体" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "补体" }, { "start_idx": 82, "end_idx": 89, "type": "dis", "entity": "血管内皮细胞损伤" }, { "start_idx": 93, "end_idx": 94, "type": "dis", "entity": "麻疹" } ] }, { "text": "由于细胞免疫功能降低,可致结核病性恶化和结核菌素试验假阴性。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "细胞免疫功能降低" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "结核病性恶化" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "结核病" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "结核菌素试验" } ] }, { "text": "广泛分布的多核巨细胞是麻疹的病理特征。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "多核巨细胞" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "麻疹" } ] }, { "text": "皮疹处见典型上皮合胞体巨大细胞,含核和胞质包涵体,并见角化不全、角化不良、海绵层细胞间水肿和细胞间水肿;表面血管扩张伴周围少量淋巴细胞与组织细胞麻疹黏膜斑(又称柯氏斑,Koplikspots)的病理改变与皮疹相似,多核巨细胞更多,水肿更严重,但炎症反应较轻。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "皮疹" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "皮" }, { "start_idx": 6, "end_idx": 14, "type": "bod", "entity": "上皮合胞体巨大细胞" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "核" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "胞质包涵体" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "角化不全" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "角化不良" }, { "start_idx": 37, "end_idx": 44, "type": "sym", "entity": "海绵层细胞间水肿" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "海绵层细胞间" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "细胞间水肿" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "细胞间" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "表面血管扩张" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "表面血管" }, { "start_idx": 59, "end_idx": 100, "type": "sym", "entity": "周围少量淋巴细胞与组织细胞麻疹黏膜斑(又称柯氏斑,Koplikspots)的病理改变" }, { "start_idx": 59, "end_idx": 66, "type": "bod", "entity": "周围少量淋巴细胞" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "组织细胞" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "麻疹黏膜斑" }, { "start_idx": 80, "end_idx": 82, "type": "dis", "entity": "柯氏斑" }, { "start_idx": 84, "end_idx": 94, "type": "dis", "entity": "Koplikspots" }, { "start_idx": 102, "end_idx": 103, "type": "sym", "entity": "皮疹" }, { "start_idx": 102, "end_idx": 102, "type": "bod", "entity": "皮" }, { "start_idx": 107, "end_idx": 113, "type": "sym", "entity": "多核巨细胞更多" }, { "start_idx": 107, "end_idx": 111, "type": "bod", "entity": "多核巨细胞" }, { "start_idx": 115, "end_idx": 119, "type": "sym", "entity": "水肿更严重" }, { "start_idx": 122, "end_idx": 127, "type": "sym", "entity": "炎症反应较轻" }, { "start_idx": 122, "end_idx": 123, "type": "dis", "entity": "炎症" } ] }, { "text": "【临床表现】(一)典型麻疹潜伏期一般为10~14天,被动免疫者可延至21~28天。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "麻疹" } ] }, { "text": "1.前驱期一般3~4天,有发热、结合膜炎(流血、流泪、畏光)、上感样表现(喷嚏、流涕、干咳)和柯氏斑(双侧颊黏膜见直径0.5~1mm大小的红色斑点,周围有红晕)。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "结合膜炎" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "流血" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "血" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "流泪" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "泪" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "畏光" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "上感样表现" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "喷嚏" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "流涕" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "涕" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "干咳" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "柯氏斑" }, { "start_idx": 51, "end_idx": 78, "type": "sym", "entity": "双侧颊黏膜见直径0.5~1mm大小的红色斑点,周围有红晕" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "双侧颊黏膜" } ] }, { "text": "2.出疹期在发热3~4天后出现皮疹,持续3~5天。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "sym", "entity": "疹" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "发热" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "皮疹" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "皮" } ] }, { "text": "皮疹先见于耳后发际,再自上而下延及躯干。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "皮疹先见于耳后发际,再自上而下延及躯干" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "皮" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "耳后发际" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "躯干" } ] }, { "text": "皮疹为玫瑰色斑丘疹,略高出皮面,疹间皮肤正常,可融合成片。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "皮疹为玫瑰色斑丘疹,略高出皮面" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "皮" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮面" }, { "start_idx": 16, "end_idx": 27, "type": "sym", "entity": "疹间皮肤正常,可融合成片" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" } ] }, { "text": "出疹时体温升高,咳嗽加剧,肺部可闻及少量啰音,颈淋巴结和肝脏可有轻度肿大。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "出疹时体温升高" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "咳嗽加剧" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "肺部可闻及少量啰音" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肺部" }, { "start_idx": 23, "end_idx": 35, "type": "sym", "entity": "颈淋巴结和肝脏可有轻度肿大" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "颈淋巴结" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肝脏" } ] }, { "text": "3.恢复期出疹3~5天后,皮疹按出疹顺序消退,疹退处有麦麸样脱屑并留有褐色色素沉着。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "出疹" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "皮" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "皮疹" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "出疹" }, { "start_idx": 23, "end_idx": 40, "type": "sym", "entity": "疹退处有麦麸样脱屑并留有褐色色素沉着" } ] }, { "text": "全身情况好转,体温下降,呼吸道症状很快消失。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "体温下降" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "呼吸道症状" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "呼吸道" } ] }, { "text": "(二)其他类型麻疹1.轻型麻疹见于有部分免疫者,如曾接种过麻疹疫苗、潜伏期接受被动免疫、6个月以下婴儿(有母体被动抗体)。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "麻疹" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "轻型麻疹" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "接种" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 53, "end_idx": 58, "type": "bod", "entity": "母体被动抗体" } ] }, { "text": "主要临床特点为潜伏期延长;前驱期短且症状轻微;常无麻疹黏膜斑;皮疹稀疏细小,消失快;可见脱屑,可不遗留色素斑;无并发症。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "潜伏期延长" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "前驱期短且症状轻微" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "麻疹黏膜斑" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "皮疹稀疏细小" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "皮" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "脱屑" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "遗留色素斑" } ] }, { "text": "2.重型麻疹见于病毒毒力过强和患者身体虚弱如重度营养不良或原有严重疾患时。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "重型麻疹" }, { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "患者身体虚弱" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "身体" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "重度营养不良" } ] }, { "text": "此型中毒症状重,起病即高热,持续在40~41℃,或体温不升。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "中毒症状重" }, { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "中毒" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "高热,持续在40~41℃" } ] }, { "text": "常见神经系统症状如嗜睡或谵妄、惊厥甚至昏迷;或心血管功能不全如气促、发绀、心率快、心音低钝;面色苍白、四肢冰凉、血压下降;皮疹常密集融合成片,或疹出不透,或出而骤退,或皮疹呈出血性伴黏膜和消化道出血无皮疹型麻疹见于免疫能力较强或白血病、恶性肿瘤等应用免疫抑制剂者。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "神经系统" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "嗜睡" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "谵妄" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "惊厥" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "昏迷" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "心血管功能不全" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "气促" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "发绀" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "心率快" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "心" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "心音低钝" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "心" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "面色苍白" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "面" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "四肢冰凉" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "四肢" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "血压下降" }, { "start_idx": 61, "end_idx": 69, "type": "sym", "entity": "皮疹常密集融合成片" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "皮" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "疹出不透" }, { "start_idx": 78, "end_idx": 81, "type": "sym", "entity": "出而骤退" }, { "start_idx": 84, "end_idx": 98, "type": "sym", "entity": "皮疹呈出血性伴黏膜和消化道出血" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "皮" }, { "start_idx": 88, "end_idx": 88, "type": "bod", "entity": "血" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "黏膜" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "消化道" }, { "start_idx": 98, "end_idx": 98, "type": "bod", "entity": "血" }, { "start_idx": 99, "end_idx": 104, "type": "dis", "entity": "无皮疹型麻疹" }, { "start_idx": 114, "end_idx": 116, "type": "dis", "entity": "白血病" }, { "start_idx": 118, "end_idx": 121, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 125, "end_idx": 129, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "病程中从无皮疹,可有柯氏斑,常以鼻咽部分泌物中找到多核巨细胞或血清学检查为诊断依据。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "皮疹" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "皮" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "柯氏斑" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "鼻咽部分泌物" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "多核巨细胞" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "血清学检查" } ] }, { "text": "4.异型麻疹见于接受过灭活麻疹疫苗或个别减毒活疫苗者,因缺乏F蛋白抗体,再感染麻疹野毒株后发生此型表现:前驱期短,无柯氏斑;出疹期发热和全身症状较重;出疹顺序为先四肢,后向躯干和面部发展;皮疹为多形性,有斑丘疹、荨麻疹、水疱和紫癜等;常伴腹痛和肌痛;易并发肺炎、肝炎和胸腔积液等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "异型麻疹" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "灭活麻疹疫苗" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "减毒活疫苗" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "F蛋白抗体" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "感染麻疹野毒株" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "前驱期短" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "无柯氏斑" }, { "start_idx": 62, "end_idx": 73, "type": "sym", "entity": "出疹期发热和全身症状较重" }, { "start_idx": 75, "end_idx": 92, "type": "sym", "entity": "出疹顺序为先四肢,后向躯干和面部发展" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "四肢" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "躯干" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "面部" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "皮疹为多形性" }, { "start_idx": 94, "end_idx": 94, "type": "bod", "entity": "皮" }, { "start_idx": 102, "end_idx": 104, "type": "dis", "entity": "斑丘疹" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "荨麻疹" }, { "start_idx": 110, "end_idx": 111, "type": "dis", "entity": "水疱" }, { "start_idx": 113, "end_idx": 114, "type": "dis", "entity": "紫癜" }, { "start_idx": 119, "end_idx": 120, "type": "sym", "entity": "腹痛" }, { "start_idx": 119, "end_idx": 119, "type": "bod", "entity": "腹" }, { "start_idx": 122, "end_idx": 123, "type": "sym", "entity": "肌痛" }, { "start_idx": 122, "end_idx": 122, "type": "bod", "entity": "肌" }, { "start_idx": 128, "end_idx": 129, "type": "dis", "entity": "肺炎" }, { "start_idx": 131, "end_idx": 132, "type": "dis", "entity": "肝炎" }, { "start_idx": 134, "end_idx": 137, "type": "dis", "entity": "胸腔积液" } ] }, { "text": "恢复期麻疹血凝抑制抗体滴度常大于1∶256。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "麻疹血凝抑制抗体" } ] }, { "text": "【实验室检查】(一)血常规白细胞总数减少,淋巴细胞分类相对增多。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "血常规" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "白细胞总数减少" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "白细胞" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "淋巴细胞分类相对增多" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "重型出血性皮疹患者可伴有血小板计数减少多核巨细胞检查于出疹前2天至出疹后1天,取患者鼻、咽、眼分泌物作涂片,瑞氏染色后直接镜检多核巨细胞。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "重型出血性皮疹" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "皮" }, { "start_idx": 12, "end_idx": 16, "type": "ite", "entity": "血小板计数" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "血小板计数减少" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "多核巨细胞检查" }, { "start_idx": 28, "end_idx": 28, "type": "sym", "entity": "疹" }, { "start_idx": 34, "end_idx": 34, "type": "sym", "entity": "疹" }, { "start_idx": 42, "end_idx": 49, "type": "bod", "entity": "鼻、咽、眼分泌物" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "瑞氏染色" }, { "start_idx": 61, "end_idx": 67, "type": "pro", "entity": "镜检多核巨细胞" } ] }, { "text": "(三)病原学检查1.病毒分离发热期取血、尿或鼻咽分泌物接种人或猴肾细胞,感染细胞融合,形成多核巨细胞,培养上清中含大量病毒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "病原学检查" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "病毒分离" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "接种" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肾细胞" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "感染细胞" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "多核巨细胞" }, { "start_idx": 59, "end_idx": 60, "type": "mic", "entity": "病毒" } ] }, { "text": "2.病毒抗原检测用免疫荧光法检测鼻咽分泌物或尿脱落细胞中麻疹病毒抗原,可早期快速诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "免疫荧光法检测" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "尿脱落细胞" }, { "start_idx": 28, "end_idx": 33, "type": "mic", "entity": "麻疹病毒抗原" } ] }, { "text": "3.特异性抗体测定用血凝抑制(HI)试验、补体结合试验(CF)或ELISA法检测急性期和恢复期双份血清,抗体滴度呈≥4倍增高有诊断意义。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "特异性抗体测定" }, { "start_idx": 10, "end_idx": 19, "type": "pro", "entity": "血凝抑制(HI)试验" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "补体结合试验" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "CF" }, { "start_idx": 32, "end_idx": 39, "type": "pro", "entity": "ELISA法检测" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "血清" }, { "start_idx": 52, "end_idx": 55, "type": "ite", "entity": "抗体滴度" } ] }, { "text": "ELISA法检测特异性IgM可诊断急性期感染,但注意可有假阳性。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "pro", "entity": "ELISA法检测特异性IgM" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "急性期感染" } ] }, { "text": "【并发症】(一)肺炎肺炎是最常见的并发症,也是引起麻疹死亡的主要原因。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肺炎" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肺炎" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "麻疹" } ] }, { "text": "麻疹肺炎有原发和继发两种。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "麻疹" }, { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肺炎" } ] }, { "text": "原发性肺炎为麻疹病毒所致全身疾患的一部分,随热退和皮疹出齐而消散。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "原发性肺炎" }, { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 22, "end_idx": 22, "type": "sym", "entity": "热" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "皮疹出齐" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "皮" } ] }, { "text": "继发性肺炎病原常见肺炎链球菌、流感杆菌、金黄色葡萄球菌或腺病毒等,多发生于出疹期。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "继发性肺炎" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "流感杆菌" }, { "start_idx": 20, "end_idx": 26, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "腺病毒" }, { "start_idx": 38, "end_idx": 38, "type": "sym", "entity": "疹" } ] }, { "text": "麻疹并发肺炎常较严重,胸腔并发症多,病死率也高。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "麻疹并发肺炎常较严重" }, { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "麻疹" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肺炎" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "胸腔并发症" } ] }, { "text": "(二)麻疹脑炎和亚急性硬化性全脑炎1.麻疹脑炎约占0.1%~0.2%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "麻疹" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "脑炎" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "亚急性硬化性全脑炎" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "麻疹" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "脑炎" } ] }, { "text": "多见于婴幼儿,发生于出疹后2~6天,也可见于前驱期或恢复期,病情与麻疹轻重无关。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "出疹" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "麻疹" } ] }, { "text": "临床表现和脑脊液变化与其他病毒性脑炎相似。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脑脊液" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "病毒性脑炎" } ] }, { "text": "2.亚急性硬化性全脑炎(subacutesclerosingpanencephalitis,SSPE)是一种罕见的致死性慢性进行性脑退行性病变,发病率约1/100万,主要见于年长儿童,在年幼时患过麻疹。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "亚急性硬化性全脑炎" }, { "start_idx": 12, "end_idx": 44, "type": "dis", "entity": "subacutesclerosingpanencephalitis" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "SSPE" }, { "start_idx": 57, "end_idx": 70, "type": "dis", "entity": "致死性慢性进行性脑退行性病变" }, { "start_idx": 98, "end_idx": 99, "type": "dis", "entity": "麻疹" } ] }, { "text": "先见智力和情绪改变,学习成绩忽然低下,不久发生阵挛性肌肉抽搐,遍及全身,最终呈去大脑强直状态。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "智力和情绪改变" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "学习成绩忽然低下" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "阵挛性肌肉抽搐,遍及全身" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肌肉" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "大脑强直状态" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "大脑" } ] }, { "text": "患者血清中麻疹病毒抗体滴度很高。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "sym", "entity": "血清中麻疹病毒抗体滴度很高" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 5, "end_idx": 12, "type": "ite", "entity": "麻疹病毒抗体滴度" } ] }, { "text": "在脑组织中用免疫荧光法检查证实有麻疹抗原或病毒存在。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "脑组织" }, { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "免疫荧光法检查" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "麻疹抗原" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "病毒" } ] }, { "text": "目前认为,麻疹病毒M蛋白缺失或功能缺陷是致病毒持续感染的主要原因。", "entities": [ { "start_idx": 5, "end_idx": 18, "type": "sym", "entity": "麻疹病毒M蛋白缺失或功能缺陷" }, { "start_idx": 5, "end_idx": 11, "type": "mic", "entity": "麻疹病毒M蛋白" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "感染" } ] }, { "text": "(三)营养障碍患病前营养状况较差、病程中持久高热、胃肠道功能紊乱以及护理不当、营养素供给不足的患者可出现营养障碍,如营养不良性水肿、维生素A缺乏性干眼症等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "营养障碍" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "营养状况较差" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "持久高热" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "胃肠道功能紊乱" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "营养素供给不足" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "营养素" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "营养障碍" }, { "start_idx": 58, "end_idx": 64, "type": "dis", "entity": "营养不良性水肿" }, { "start_idx": 66, "end_idx": 75, "type": "dis", "entity": "维生素A缺乏性干眼症" } ] }, { "text": "(四)结核病恶化患麻疹时机体免疫功能受到暂时性抑制,致使体内原来稳定状态的结核病灶重趋活动恶化,可发展为粟粒性肺结核或结核性脑膜炎。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "结核病恶化" }, { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "结核病" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "麻疹" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "免疫功能受到暂时性抑制" }, { "start_idx": 37, "end_idx": 46, "type": "sym", "entity": "结核病灶重趋活动恶化" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "结核病" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "粟粒性肺结核" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "结核性脑膜炎" } ] }, { "text": "【诊断和鉴别诊断】(一)诊断典型麻疹可借流行病学史诊断,各期典型表现如前驱期麻疹黏膜斑,出疹期出疹与发热的关系,出疹顺序和皮疹形态;恢复期疹退脱屑和色素沉着可以确立诊断,必要时辅以病原学检查,尤其是非典型麻疹者。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "麻疹" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "麻疹黏膜斑" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "出疹" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "发热" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "出疹" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "皮疹" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "皮" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "疹退脱屑" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "色素沉着" }, { "start_idx": 99, "end_idx": 103, "type": "dis", "entity": "非典型麻疹" } ] }, { "text": "(二)鉴别诊断发热、出疹在儿科常见,应根据流行病学、临床症状、发热与皮疹的关系、皮疹特征等,结合有关病原学检验结果进行鉴别诊断。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "出疹" }, { "start_idx": 13, "end_idx": 14, "type": "dep", "entity": "儿科" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "发热" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "皮疹" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "皮" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "皮疹" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "皮" }, { "start_idx": 50, "end_idx": 54, "type": "pro", "entity": "病原学检验" } ] }, { "text": "【预防】(一)控制传染源和切断传播途径对麻疹患者应做到早发现、早隔离、早治疗。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "控制传染源" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "切断传播途径" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "麻疹" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "早发现" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "早隔离" }, { "start_idx": 35, "end_idx": 37, "type": "pro", "entity": "早治疗" } ] }, { "text": "一般患者应隔离至出疹后6天,若并发肺炎则延至出疹后第10天。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "出疹" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肺炎" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "出疹" } ] }, { "text": "在麻疹流行期间,宣传教育易感者不到人群密集的场所去。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "麻疹" }, { "start_idx": 8, "end_idx": 24, "type": "pro", "entity": "宣传教育易感者不到人群密集的场所去" } ] }, { "text": "患者停留过的房间用紫外线照射消毒或通风半小时,患者衣物应在阳光下曝晒或用肥皂水清洗。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "pro", "entity": "患者停留过的房间用紫外线照射消毒或通风半小时" }, { "start_idx": 23, "end_idx": 40, "type": "pro", "entity": "患者衣物应在阳光下曝晒或用肥皂水清洗" } ] }, { "text": "(二)主动免疫对易感者应普遍接种麻疹减毒活疫苗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "主动免疫" }, { "start_idx": 12, "end_idx": 22, "type": "pro", "entity": "普遍接种麻疹减毒活疫苗" } ] }, { "text": "按照我国政府规定的儿童计划免疫程序,将8个月儿童定为初免对象,皮下注射麻疹减毒活疫苗0.2~0.25ml。", "entities": [ { "start_idx": 31, "end_idx": 34, "type": "equ", "entity": "皮下注射" }, { "start_idx": 35, "end_idx": 41, "type": "dru", "entity": "麻疹减毒活疫苗" } ] }, { "text": "在麻疹流行地区,可在接触麻疹的头2天内,对易感者进行应急接种,使机体在潜伏早期产生特异抗体,以防止发病或减轻症状。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "麻疹" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "麻疹" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "进行应急接种" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "特异抗体" } ] }, { "text": "由于大多数国家都存在未到初免年龄婴儿发病问题,而提早初免月龄则因婴儿胎传被动特异性抗体的存在会干扰麻疹疫苗免疫效果。", "entities": [ { "start_idx": 34, "end_idx": 42, "type": "bod", "entity": "胎传被动特异性抗体" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "麻疹疫苗" } ] }, { "text": "新一代麻疹疫苗的研究目标是提高免疫原性,使小于4月龄婴儿能早获免疫,安全有效,不产生异型麻疹,并可用其加强免疫。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "异型麻疹" } ] }, { "text": "目前研制的新型麻疹疫苗有以下几种:①合成肽疫苗及新型佐剂疫苗;②基因工程疫苗(载体疫苗);③亚单位疫苗。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "新型麻疹疫苗" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "合成肽疫苗" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "新型佐剂疫苗" }, { "start_idx": 32, "end_idx": 37, "type": "dru", "entity": "基因工程疫苗" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "载体疫苗" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "亚单位疫苗" } ] }, { "text": "(三)被动免疫对体弱有病和婴幼儿未接受过麻疹疫苗接种者,在接触麻疹后5天内予以肌内注射人丙种球蛋白0.25ml/kg可预防患病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "被动免疫" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "麻疹疫苗" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "麻疹" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "肌内注射" }, { "start_idx": 43, "end_idx": 48, "type": "dru", "entity": "人丙种球蛋白" } ] }, { "text": "接触麻疹5天后注射只能减轻症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "麻疹" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "注射" } ] }, { "text": "【治疗】主要为加强护理,防治并发症。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "加强护理" } ] }, { "text": "(一)护理包括给予足够水分和易消化富营养食物,居室保持适宜温湿度和空气新鲜;口、眼和皮肤经常清洗。", "entities": [ { "start_idx": 7, "end_idx": 21, "type": "pro", "entity": "给予足够水分和易消化富营养食物" }, { "start_idx": 23, "end_idx": 36, "type": "pro", "entity": "居室保持适宜温湿度和空气新鲜" }, { "start_idx": 38, "end_idx": 47, "type": "pro", "entity": "口、眼和皮肤经常清洗" } ] }, { "text": "(二)对症治疗高热时可温水灌肠或给予小量退热剂降温,切忌退热过猛引起虚脱。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "对症治疗" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "高热" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "温水灌肠" }, { "start_idx": 16, "end_idx": 24, "type": "pro", "entity": "给予小量退热剂降温" }, { "start_idx": 26, "end_idx": 35, "type": "pro", "entity": "切忌退热过猛引起虚脱" } ] }, { "text": "咳剧时给予镇咳祛痰剂。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "咳剧" }, { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "镇咳祛痰剂" } ] }, { "text": "(三)中医治疗中医认为麻疹属于“温热病”范围,前驱期治疗以辛凉透表为主;出疹期以清热解毒透疹为主;恢复期则养阴清余热、调理脾胃。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "中医治疗" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "麻疹" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "温热病" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "辛凉透表" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "出疹" }, { "start_idx": 40, "end_idx": 45, "type": "pro", "entity": "清热解毒透疹" }, { "start_idx": 53, "end_idx": 57, "type": "pro", "entity": "养阴清余热" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "调理脾胃" } ] }, { "text": "抗生素无预防并发症作用,故不宜滥用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "抗生素" } ] }, { "text": "总之,不管采用上述任何方法计算剂量,都必须与病儿具体情况相结合,才能得出比较确切的药物用量,如新生儿、小婴儿或营养不良儿因肝、肾功能较差,一般药物剂量宜偏小;用药目的、对象不同,剂量也不同;不同的剂量,其药理作用也有差异,这些都是儿科用药确定剂量应考虑的问题。", "entities": [ { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "营养不良" }, { "start_idx": 61, "end_idx": 67, "type": "sym", "entity": "肝、肾功能较差" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "肝" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "肾" }, { "start_idx": 115, "end_idx": 116, "type": "dep", "entity": "儿科" } ] }, { "text": "八、EB病毒肺炎3~5岁为感染高峰年龄。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "EB病毒肺炎" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" } ] }, { "text": "EB病毒感染后可累及全身各系统。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "EB病毒感染" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "全身各系统" } ] }, { "text": "在呼吸系统可表现为反复间质性肺炎、持续性咽峡炎等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "持续性咽峡炎" } ] }, { "text": "除一般肺炎的症状和体征外,可有时隐时现的咳嗽和反复发热,常伴有肝、脾和淋巴结肿大。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肺炎" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "时隐时现的咳嗽和反复发热" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肝" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "肝、脾和淋巴结肿大" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "脾" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "淋巴结" } ] }, { "text": "胸部X线检查以间质性病变为主。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "胸部X线检查以间质性病变为主" } ] }, { "text": "急性期外周血白细胞计数常明显增高,以淋巴细胞为主,并出现异常淋巴细胞。", "entities": [ { "start_idx": 0, "end_idx": 33, "type": "sym", "entity": "急性期外周血白细胞计数常明显增高,以淋巴细胞为主,并出现异常淋巴细胞" }, { "start_idx": 3, "end_idx": 10, "type": "ite", "entity": "外周血白细胞计数" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "淋巴细胞" }, { "start_idx": 28, "end_idx": 33, "type": "ite", "entity": "异常淋巴细胞" } ] }, { "text": "确诊常需依赖特异性抗体测定。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "特异性抗体测定" } ] }, { "text": "第六节气道异物气道异物是小儿常见危重急症。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "气道异物" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "气道异物" } ] }, { "text": "异物误吸入气道后,表现突然阵发性剧烈呛咳、憋气,继而呕吐及呼吸困难。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "异物误吸入气道" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "气道" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "突然阵发性剧烈呛咳、憋气" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "呕吐" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "其病情严重程度取决于异物大小、性质、嵌顿部位和气道阻塞程度,重者可造成窒息迅速死亡。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "气道" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "气道阻塞" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "窒息" } ] }, { "text": "(一)喉部异物(laryngealforeignbodies)患儿突然发生剧烈呛咳、憋气及发绀。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "喉部异物" }, { "start_idx": 8, "end_idx": 29, "type": "dis", "entity": "laryngealforeignbodies" }, { "start_idx": 33, "end_idx": 46, "type": "sym", "entity": "突然发生剧烈呛咳、憋气及发绀" } ] }, { "text": "(二)气管异物(trachealforeignbodies)可表现咳嗽、声音嘶哑、呼吸困难、青紫等,但以喘鸣、听诊有气管拍击声、触诊有气管撞击感为特点。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "气管异物" }, { "start_idx": 8, "end_idx": 28, "type": "dis", "entity": "trachealforeignbodies" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "咳嗽" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "青紫" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "喘鸣" }, { "start_idx": 55, "end_idx": 62, "type": "sym", "entity": "听诊有气管拍击声" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "听诊" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "触诊" }, { "start_idx": 64, "end_idx": 71, "type": "sym", "entity": "触诊有气管撞击感" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "气管" } ] }, { "text": "(三)支气管异物(bronchialforeignbodies)以右侧较为多见。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "支气管异物" }, { "start_idx": 9, "end_idx": 30, "type": "dis", "entity": "bronchialforeignbodies" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "右侧" } ] }, { "text": "初始症状与喉异物、气管异物相似。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "喉异物" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "气管异物" } ] }, { "text": "异物进入一侧支气管后,症状可暂时减轻,仅表现轻度喘鸣和咳嗽。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "异物进入一侧支气管" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "一侧支气管" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "轻度喘鸣和咳嗽" } ] }, { "text": "上述情况持续存在,极易继发感染。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" } ] }, { "text": "有些患儿无法追问到异物吸入史,因而对任何急性或慢性肺疾患者均应注意排除异物吸入,尤其是呼吸道感染治疗效果不佳、局部气道有阻塞征象、肺部体征多变者。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "异物吸入" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "急性或慢性肺疾" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "异物吸入" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 55, "end_idx": 61, "type": "sym", "entity": "局部气道有阻塞" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "局部气道" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "肺部" } ] }, { "text": "胸部X线检查诊断价值较大,对不透光异物,能直接确定异物的部位、大小或形状;对透光异物,则可根据呼吸道梗阻情况而做出判断,如局灶性肺气肿或肺不张。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "呼吸道梗阻" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "局灶性肺气肿" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "肺不张" } ] }, { "text": "此外应在透视下反复观察纵隔、横膈运动情况,如有纵隔移位与摆动,常提示支气管异物。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "透视" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "纵隔" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "横膈" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "纵隔移位与摆动" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "纵隔" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "支气管异物" } ] }, { "text": "1岁以内婴儿突然发生窒息伴失音时,应立即将患儿头部放低,用掌根叩打患儿背部(肩胛骨间)4次,以松动异物;继而患儿取仰卧位,用心肺复苏同样手法按压胸部4次(婴儿按压胸骨中部,年长儿按压部位略低);随后观察口腔内有无异物,并取出;意识不清者立即复苏。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "突然发生窒息" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "失音" }, { "start_idx": 18, "end_idx": 26, "type": "pro", "entity": "立即将患儿头部放低" }, { "start_idx": 28, "end_idx": 50, "type": "pro", "entity": "用掌根叩打患儿背部(肩胛骨间)4次,以松动异物" }, { "start_idx": 52, "end_idx": 95, "type": "pro", "entity": "继而患儿取仰卧位,用心肺复苏同样手法按压胸部4次(婴儿按压胸骨中部,年长儿按压部位略低)" }, { "start_idx": 97, "end_idx": 111, "type": "pro", "entity": "随后观察口腔内有无异物,并取出" }, { "start_idx": 113, "end_idx": 116, "type": "sym", "entity": "意识不清" }, { "start_idx": 118, "end_idx": 121, "type": "pro", "entity": "立即复苏" } ] }, { "text": "如不成功,可重复上述叩背、压胸、观察口腔、复苏环节。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "叩背" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "压胸" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "观察口腔" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "复苏" } ] }, { "text": "1岁以上患儿应平卧,从中腹部开始,向上、向内用力挤压6~10次;随后观察口腔内有无异物,并取出;意识不清者复苏。", "entities": [ { "start_idx": 6, "end_idx": 30, "type": "pro", "entity": "应平卧,从中腹部开始,向上、向内用力挤压6~10次" }, { "start_idx": 32, "end_idx": 46, "type": "pro", "entity": "随后观察口腔内有无异物,并取出" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "意识不清" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "复苏" } ] }, { "text": "如不成功,可重复以上压腹、观察口腔、复苏步骤。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "压腹" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "观察口腔" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "复苏" } ] }, { "text": "如患儿能咳嗽和呼吸,无需上述操作。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "咳嗽" } ] }, { "text": "应尽快进行硬质支气管镜检查和治疗,延迟处理可增加感染机会。", "entities": [ { "start_idx": 1, "end_idx": 15, "type": "pro", "entity": "尽快进行硬质支气管镜检查和治疗" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "感染" } ] }, { "text": "纤维支气管镜对深部或上叶支气管异物及深部植物性残渣有较好的诊断和治疗效果。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "深部或上叶支气管异物" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "深部植物性残渣" } ] }, { "text": "异物取出前不宜应用肺部物理治疗及支气管扩张剂,以免引起异物移位至大气道引起窒息。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "肺部物理治疗" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "支气管扩张剂" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "异物移位至大气道" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "大气道" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "窒息" } ] }, { "text": "参考文献1.胡亚美,江载芳.实用儿科学(上册).第7版.北京:人民卫生出版社,20022.WaldoE.Nelson等主编.陈荣华等主译.尼尔逊儿科学(上册).第15版.北京:世界图书出版社,19993.顾玉成.小儿恙虫病115例临床分析.医学信息,2010,23(6):1934-19344.毕晶,秦维娜.小儿地方性斑疹伤寒235例分析.河北医药,2008,30(6):8715.中华人民共和国卫生部.人粒细胞无形体病预防控制技术指南(试行).20086.张丽娟,徐建国.当代立克次体学与立克次体病.长春:吉林大学出版社,2010:235", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dep", "entity": "儿科" }, { "start_idx": 72, "end_idx": 73, "type": "dep", "entity": "儿科" }, { "start_idx": 106, "end_idx": 110, "type": "dis", "entity": "小儿恙虫病" }, { "start_idx": 154, "end_idx": 162, "type": "dis", "entity": "小儿地方性斑疹伤寒" }, { "start_idx": 202, "end_idx": 209, "type": "dis", "entity": "人粒细胞无形体病" }, { "start_idx": 239, "end_idx": 242, "type": "mic", "entity": "立克次体" }, { "start_idx": 245, "end_idx": 249, "type": "dis", "entity": "立克次体病" } ] }, { "text": "第七章心功能衰竭见第九篇第十一章。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "心功能衰竭" } ] }, { "text": "第二节心电图检查一、儿童发育过程中的心电图变化围生期心脏生理学和室腔的优势发生了显著变化。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心电图检查" }, { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "心电图" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心脏" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "室腔" } ] }, { "text": "这些变化可在新生儿期心电图的演变中得到体现。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "心电图" } ] }, { "text": "由于在足月胎儿肺体循环的血管阻力几乎相等,心脏的宫内做功可造成左右心室的容积几乎相等。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "肺体循环的血管" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心脏" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "左右心室" } ] }, { "text": "出生后由于胎盘循环停止,体循环血管阻力上升,肺循环因肺的膨胀而血管阻力下降。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "胎盘循环" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "体循环血管" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "体循环血管阻力上升" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺循环" }, { "start_idx": 22, "end_idx": 36, "type": "sym", "entity": "肺循环因肺的膨胀而血管阻力下降" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肺" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "血管" } ] }, { "text": "这些变化反映在心电图上则表现为右室壁开始变薄。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "心电图" }, { "start_idx": 7, "end_idx": 21, "type": "sym", "entity": "心电图上则表现为右室壁开始变薄" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "右室壁" } ] }, { "text": "心电图主要通过QRS及T波形态学特征的变化来显示这些解剖及血流动力学特征的改变。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "解剖" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "血流" } ] }, { "text": "在儿科患者,除了标准12导心电图外,还应加做V3R或V4R导联,后者对右室肥厚的评估极其重要。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dep", "entity": "儿科" }, { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "标准12导心电图" }, { "start_idx": 20, "end_idx": 42, "type": "pro", "entity": "加做V3R或V4R导联" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "右室" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "右室肥厚" } ] }, { "text": "在出生后最初几天,电轴右偏、右胸前导联(V3R或V4R及V1)R波高耸、T波直立为正常现象。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "电轴右偏" }, { "start_idx": 14, "end_idx": 57, "type": "sym", "entity": "右胸前导联(V3R或V4R及V1)R波高耸、T波直立" } ] }, { "text": "随着肺血管阻力的下降,右胸前导联T波变负。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺血管" }, { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "肺血管阻力的下降" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "右胸前导联T波变负" } ] }, { "text": "如V3R、V4R或V1导联T波直立持续超过1周,即使无QRS电压标准,也应诊断为右室肥厚或劳损。", "entities": [ { "start_idx": 1, "end_idx": 40, "type": "sym", "entity": "V3R、V4R或V1导联T波直立持续超过1周" }, { "start_idx": 58, "end_idx": 64, "type": "dis", "entity": "右室肥厚或劳损" } ] }, { "text": "这一现象代表了儿科及成人心电图最重要但又微妙的差异之一。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dep", "entity": "儿科" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "心电图" } ] }, { "text": "成人心脏科医师在解读儿科心电图时常在这方面出错。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dep", "entity": "心脏科" }, { "start_idx": 10, "end_idx": 11, "type": "dep", "entity": "儿科" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "心电图" } ] }, { "text": "右胸导联R/S之比>1,并可维持这种状态数月到数年,因为整个婴儿期右室保持相对肥厚。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "右胸导联R/S之比>1,并可维持这种状态数月到数年" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "右室保持相对肥厚" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "右室" } ] }, { "text": "不过在生命最初几天内反映左室电势的R波主导迅速变得明显。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "左室" } ] }, { "text": "以后的几年中,QRS轴逐渐左偏,右室电势慢慢消退。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "QRS轴逐渐左偏" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "右室电势慢慢消退" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "右室" } ] }, { "text": "右室变薄、左室增长的过程主要表现在右胸前导联QRS-T波群。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "右室" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "右室变薄" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "左室" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "左室增长" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "右胸前导联QRS-T波群" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "右胸" } ] }, { "text": "因此,只有理解了成人期前不同年龄心室的正常发育生理学,才能正确地诊断儿童的左室或右室肥大。", "entities": [ { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "左室或右室肥大" } ] }, { "text": "随着左室电势的逐渐主导,心电图逐渐演变为年长儿及成人的图形特征。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "左室" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "心电图" } ] }, { "text": "心室肥大可导致胸导联R波及S波电压增加。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心室肥大" }, { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "胸导联R波及S波电压增加" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "胸" } ] }, { "text": "这些波的幅度受下列因素影响:特殊电极与心脏表面的贴近度,通过心室的电激动顺序的不同可致不同程度电势变化及心肌的肥厚程度。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "心脏表面" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心室" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "心肌" } ] }, { "text": "由于儿童的胸壁相对较薄,诊断心室肥厚不能单凭电压变化。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胸壁" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "胸壁相对较薄" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "心室肥厚" } ] }, { "text": "生后第一周诊断病理性右室肥厚是困难的,因为在这一时期存在着生理性右室肥厚。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "病理性右室肥厚" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "生理性右室肥厚" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "右室" } ] }, { "text": "为此,需系列跟踪心电图变化,以确定过了这一时期后是否仍存在电轴显著右偏,异常的右心前导联电势或T波,或二者均存在。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "心电图" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "电轴显著右偏" }, { "start_idx": 36, "end_idx": 48, "type": "sym", "entity": "异常的右心前导联电势或T波" } ] }, { "text": "相反,在新生儿出现成人心电图图形应诊断左室扩大。", "entities": [ { "start_idx": 4, "end_idx": 15, "type": "sym", "entity": "新生儿出现成人心电图图形" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "左室扩大" } ] }, { "text": "由于早产儿肺小动脉肌性中层发育不全,肺血管阻力低,其心电图可表现得较足月儿更“成熟”。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "早产儿" }, { "start_idx": 5, "end_idx": 16, "type": "dis", "entity": "肺小动脉肌性中层发育不全" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肺血管" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "肺血管阻力低" }, { "start_idx": 26, "end_idx": 41, "type": "sym", "entity": "心电图可表现得较足月儿更“成熟”" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "心电图" } ] }, { "text": "有些早产儿可表现为所有心前导联低电压。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "早产儿" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "所有心前导联低电压" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "心" } ] }, { "text": "第三节心腔及大血管的压力和阻力及其临床意义血流从心脏进入主动脉到达外周并从外周经静脉回到心脏主要是由于压力的作用。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "心腔" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "大血管" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血流" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心脏" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "主动脉" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "静脉" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "心脏" } ] }, { "text": "要使血流向前流动,近端的压力必须高于远端的压力。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血流" } ] }, { "text": "血流在循环系统的流动在体循环靠体循环压力,在肺循环则靠肺动脉的压力。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血流" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "循环系统" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "体循环" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "体循环" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺循环" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺动脉" } ] }, { "text": "血管内的压力依赖于在血管管内流动的血流及血管对血流的阻力。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血管内" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "血管管内" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血流" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "血管" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血流" } ] }, { "text": "三者的相互关系可由Poiseuille公式表示:压力(mmHg)=血流量(升/分)×阻力(Wood单位)80达因/秒/厘米5=1Wood单位心输出量增大或和体循环阻力增加均可导致体循环血压的升高,在绝大多数的高血压患者,心输出量正常,血压升高的主要原因是直接与体循环阻力升高有关。", "entities": [ { "start_idx": 79, "end_idx": 86, "type": "sym", "entity": "单位心输出量增大" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "心" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "体循环" }, { "start_idx": 89, "end_idx": 95, "type": "sym", "entity": "体循环阻力增加" }, { "start_idx": 100, "end_idx": 107, "type": "sym", "entity": "体循环血压的升高" }, { "start_idx": 100, "end_idx": 102, "type": "bod", "entity": "体循环" }, { "start_idx": 115, "end_idx": 117, "type": "dis", "entity": "高血压" }, { "start_idx": 121, "end_idx": 121, "type": "bod", "entity": "心" }, { "start_idx": 128, "end_idx": 131, "type": "sym", "entity": "血压升高" }, { "start_idx": 128, "end_idx": 129, "type": "ite", "entity": "血压" }, { "start_idx": 141, "end_idx": 147, "type": "sym", "entity": "体循环阻力升高" }, { "start_idx": 141, "end_idx": 143, "type": "bod", "entity": "体循环" } ] }, { "text": "如果将上述公式应用到肺循环,该公式表达为:肺动脉压力=肺动脉血流×肺血管阻力(PVR)肺动脉压力上升(肺动脉高压)可与左向右分流肺血流增加及肺阻力上升有关。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺循环" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "肺动脉压力" }, { "start_idx": 27, "end_idx": 31, "type": "ite", "entity": "肺动脉血流" }, { "start_idx": 33, "end_idx": 37, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "PVR" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "肺动脉压力上升" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "肺动脉" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "左向右分流" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "肺血流" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "肺血流增加" }, { "start_idx": 70, "end_idx": 74, "type": "sym", "entity": "肺阻力上升" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "肺" } ] }, { "text": "当肺动脉高压高压由肺血流血流增加所致时称为动力性高压,同样,如果肺动脉高压高压是由于肺阻力上升所致则称为梗阻性高压,肺高压导致肺动脉肌层肥厚继而出现内膜纤维化形成非可逆性改变称为肺血管梗阻性疾病。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "高压" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺血流" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "肺血流血流增加" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血流" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "动力性高压" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "高压" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "肺阻力上升" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肺" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "梗阻性高压" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "肺高压" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "肺动脉肌层" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "肺动脉肌层肥厚" }, { "start_idx": 74, "end_idx": 78, "type": "sym", "entity": "内膜纤维化" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "内膜" }, { "start_idx": 81, "end_idx": 86, "type": "sym", "entity": "非可逆性改变" }, { "start_idx": 89, "end_idx": 96, "type": "dis", "entity": "肺血管梗阻性疾病" } ] }, { "text": "此时,患者的非可逆性变化使其不能手术或治疗。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "非可逆性变化" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "手术" } ] }, { "text": "同样肺血管的非可逆性变化是艾森门格综合征(Eisenmengersyndrome)的基本病理基础,表现为严重的肺高压合并心房、大血管水平的右向左分流。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺血管" }, { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "肺血管的非可逆性变化" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "艾森门格综合征" }, { "start_idx": 21, "end_idx": 39, "type": "dis", "entity": "Eisenmengersyndrome" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "严重的肺高压" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "肺" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "心房" }, { "start_idx": 60, "end_idx": 73, "type": "sym", "entity": "心房、大血管水平的右向左分流" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "大血管" } ] }, { "text": "在临床上,可有方法区分动力型和器质性肺高压。", "entities": [ { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "动力型和器质性肺高压" } ] }, { "text": "动力型肺高压常表现为大量左向右分流伴有肺动脉高压,而器质性肺动脉高压常表现为少量的左向右分流伴有肺动脉高压。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "动力型肺高压" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "大量左向右分流" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "器质性肺动脉高压" }, { "start_idx": 38, "end_idx": 45, "type": "sym", "entity": "少量的左向右分流" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "肺血管阻塞性疾病时,右向左分流开始出现,表现为艾森门格综合征。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "肺血管阻塞性疾病" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "右向左分流" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "艾森门格综合征" } ] }, { "text": "由于正常情况下肺动脉顺应性良好,可接纳三倍正常血流量而不出现肺高压,因此,有时大量的左向右分流并不合并肺高压。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺动脉" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "血" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "肺高压" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "大量的左向右分流" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "肺高压" } ] }, { "text": "通常情况下,左侧心腔的压力高于右侧,但两侧心房间的心房间的压差较小,而在收缩期两侧心室间心室间的压差显著,同样在大血管水平无论在收缩期或者舒张期均有明显的压差。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "左侧心腔" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "右侧" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "两侧心房间的" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "心房间的" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "两侧心室间" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "心室间的" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "大血管" } ] }, { "text": "左右心房间的交通导致两侧心房压力趋于平衡,因此,房间隔缺损时的左向右分流是在较小的压差下进行的(后者取决于左右心室的顺应性),故杂音不明显。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "左右心房间" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "两侧心房" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "左向右分流" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "左右心室" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "杂音" } ] }, { "text": "右心室在舒张期接纳额外的血流,导致右心室舒张期容量负荷过重。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血流" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "右心室" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "右心室舒张期容量负荷过重" } ] }, { "text": "在室间隔缺损,缺损的大小决定有多少左心室压力被传递到右心室。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "缺损" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "左心室" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右心室" } ] }, { "text": "在大型的室间隔缺损时两侧心室的压力几乎相等;在小型的室间隔缺损时,为全收缩期的杂音、而在大型的室间隔缺损时只表现为收缩期杂音。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "两侧心室" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "全收缩期的杂音" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "收缩期杂音" } ] }, { "text": "在非常大型的室间隔缺损时,左向右分流量取决于肺血管的阻力,由于右心室是在收缩期接受来自左心室的分流,此时右心室腔已较小,分流血流直接进入肺动脉,右心室只起到管道的作用,因此,室间隔缺损并不导致右心室容量负荷的增加,而左心室却在舒张期接纳额外的血流而出现容量负荷过重。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "左向右分流" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺血管" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心室" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "左心室" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "右心室腔" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "右心室腔已较小" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "血流" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "肺动脉" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "右心室" }, { "start_idx": 87, "end_idx": 91, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "右心室" }, { "start_idx": 96, "end_idx": 105, "type": "sym", "entity": "右心室容量负荷的增加" }, { "start_idx": 108, "end_idx": 110, "type": "bod", "entity": "左心室" }, { "start_idx": 108, "end_idx": 131, "type": "sym", "entity": "左心室却在舒张期接纳额外的血流而出现容量负荷过重" }, { "start_idx": 121, "end_idx": 122, "type": "bod", "entity": "血流" } ] }, { "text": "在动脉导管未闭,无论在舒张期和收缩期,主动脉压力均高于肺动脉,在整个心动周期中均有血流从主动脉向肺动脉分流,导致连续性杂音。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "主动脉" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺动脉" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "心" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "血流" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "主动脉" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "肺动脉" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "连续性杂音" } ] }, { "text": "分流的血流在舒张期由左心室接纳导致左心室容量负荷过重。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血流" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "左心室" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "左心室" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "左心室容量负荷过重" } ] }, { "text": "同样,动脉导管的大小是主动脉向肺动脉血液分流量的决定因素之一。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "动脉导管" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "主动脉" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺动脉" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血液" } ] }, { "text": "在大型的动脉导管未闭时,主肺动脉的压力相仿,分流量取决于肺阻力,此时可仅有收缩期杂音。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "主肺动脉" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "收缩期杂音" } ] }, { "text": "分流量不依赖非阻力而依赖两侧压力阶差的分流称为强制性分流,可见于左室右房分流及从乏氏窦向右心室或右房的分流时。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "分流量不依赖非阻力而依赖两侧压力阶差的分流" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "强制性分流" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "左室" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "左室右房分流" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "右房" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "乏氏窦" }, { "start_idx": 40, "end_idx": 52, "type": "sym", "entity": "乏氏窦向右心室或右房的分流" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "右心室" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "右房" } ] }, { "text": "第二节高频振荡通气(一)高频振荡通气通气的原理高频振荡通气(highfrequencyoscillation,HFO)具有小潮气量、高频率、高平均气道压(MAP)通气的特点。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "高频振荡通气" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "高频振荡通气" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "高频振荡通气" }, { "start_idx": 30, "end_idx": 53, "type": "pro", "entity": "highfrequencyoscillation" }, { "start_idx": 55, "end_idx": 57, "type": "pro", "entity": "HFO" } ] }, { "text": "也有将气流阻断技术(interruptor)应用以产生高频气流,但由于不能产生主动的呼气相供气,因此不是正式的HFO设备。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "气流阻断技术" }, { "start_idx": 10, "end_idx": 20, "type": "pro", "entity": "interruptor" }, { "start_idx": 55, "end_idx": 59, "type": "equ", "entity": "HFO设备" } ] }, { "text": "HFO作用机制包括不对称气流束、强化弥散、肺内气体振荡和近端肺泡通气,患儿通气的气体量可以在低于或接近无效腔量的水平。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "HFO" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "不对称气流束" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "强化弥散" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "肺内气体振荡" }, { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "近端肺泡通气" } ] }, { "text": "由于HFO高频率效应,实际产生的是接近连续气流的高频切换气流。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "HFO" } ] }, { "text": "HFO通气量取决于振幅、气管插管的大小及呼吸系统的阻力和顺应性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "HFO" }, { "start_idx": 12, "end_idx": 15, "type": "equ", "entity": "气管插管" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "呼吸系统" } ] }, { "text": "基于吸呼比(I∶E)为1∶1时会出现肺泡呼气末正压的假设,有些振荡器I:E可调。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺泡" } ] }, { "text": "(二)HFO的使用HFO通常使用的频率在10~15Hz,小婴儿用较高的频率,大婴儿用较低的频率,目前常用于新生儿和婴幼儿,体重最大在10kg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "HFO" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "HFO" } ] }, { "text": "HFO通过调节PEEP水平获得MAP。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "HFO" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "MAP" } ] }, { "text": "振幅设置应能看到胸壁振动,目的是既消除肺不张又不使肺过度膨胀。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胸壁" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "肺不张" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "肺过度膨胀" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肺" } ] }, { "text": "肺膨胀程度可通过胸部X线检查来评估。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "胸部X线检查" } ] }, { "text": "振幅和频率调节可以影响二氧化碳的排出效率,PEEP(MAP)调节可以影响氧和水平。", "entities": [ { "start_idx": 21, "end_idx": 31, "type": "pro", "entity": "PEEP(MAP)调节" } ] }, { "text": "一般治疗危重呼吸衰竭小儿时,MAP达到20~30cmH2O,振幅达到30cmH2O以上,不会对心率、血压产生不良影响。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "危重呼吸衰竭" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "MAP" }, { "start_idx": 69, "end_idx": 70, "type": "ite", "entity": "心率" }, { "start_idx": 72, "end_idx": 73, "type": "ite", "entity": "血压" } ] }, { "text": "HFO效果良好时,上机应用的最初2~3小时,可见气道分泌物增多,经气道吸引清洁后会逐渐减少。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "HFO" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "气道分泌物增多" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "气道" } ] }, { "text": "当吸入氧浓度小于0.3时,平均气道压可逐渐下调至10cmH2O以下,可以转为IPPV模式继续治疗。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "平均气道压" }, { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "平均气道压可逐渐下调" }, { "start_idx": 49, "end_idx": 54, "type": "pro", "entity": "IPPV模式" } ] }, { "text": "如在儿童标准型急性淋巴细胞白血病中,假设不采用全颅放疗作为脑膜白血病预防不会影响其5年无病生存率。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "儿童标准型急性淋巴细胞白血病" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "全颅放疗" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "脑膜白血病" } ] }, { "text": "由于儿童肿瘤发病率较低,病人数积累慢,因此应尽量限制方案目的的范围,以保证结果的可靠性。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "Ⅰ期研究需较严格的条件,常用的限制性条件为:①常规治疗失败,但各脏器功能正常,以保证正确评估药物的毒性;②与上次常规治疗有一定时间的间距,以避免原药物的叠加作用;③实体瘤病人需正常的骨髓造血功能,以正确评估药物的骨髓抑制作用,并需与白血病病人分组实验;④通常没有必要限制组织学分型。", "entities": [ { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "实体瘤" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "骨髓" }, { "start_idx": 94, "end_idx": 94, "type": "bod", "entity": "血" }, { "start_idx": 106, "end_idx": 107, "type": "bod", "entity": "骨髓" }, { "start_idx": 116, "end_idx": 118, "type": "dis", "entity": "白血病" } ] }, { "text": "Ⅱ期研究常用的限制性条件为:①无治疗史者为最理想,以避免前期治疗所致的获得性耐药或耐药消减;②由于儿童肿瘤在初诊时均有治愈可能性,因此以常规方案失败者为对象更容易使人接受;③可用于已知常规方案预后不良者,如大年龄、晚期神经母细胞瘤,此时可在常规治疗方案前采用Ⅱ期临床方案作“窗口治疗”;④注意病理分型,因有效性可能与此有关;⑤存在可检测的肿瘤负荷,以估计药物的效果。", "entities": [ { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 103, "end_idx": 114, "type": "dis", "entity": "大年龄、晚期神经母细胞瘤" }, { "start_idx": 137, "end_idx": 140, "type": "pro", "entity": "窗口治疗" }, { "start_idx": 166, "end_idx": 172, "type": "pro", "entity": "检测的肿瘤负荷" } ] }, { "text": "(四)设计设计部分应简明地说明研究的结构、交代达到研究目的的方法学,至少要包括病人的分配、对结果评估的标准(即研究终点,endpoints),如在前面举例的急性淋巴细胞白血病,终点应为复发。", "entities": [ { "start_idx": 78, "end_idx": 86, "type": "dis", "entity": "急性淋巴细胞白血病" } ] }, { "text": "病人在诱导缓解、巩固治疗、强化治疗、维持治疗至8个月时随机分配进入全颅放疗和不放疗组,其余全身治疗相同,长期随访观察至复发为终点。", "entities": [ { "start_idx": 3, "end_idx": 21, "type": "pro", "entity": "诱导缓解、巩固治疗、强化治疗、维持治疗" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "全颅放疗" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "放疗" } ] }, { "text": "1.Ⅰ期临床研究实验方案设计在儿童肿瘤中,Ⅰ期方案设计标准是3例实体瘤和3例白血病为一个研究组,采用同样的实验药物剂量,观察药物毒性。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "实体瘤" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "白血病" } ] }, { "text": "假如毒性反应为血液学表现,实体瘤组应终止,白血病组可继续进行剂量升级直至出现非血液学的DLT,此时的前一剂量即是安全剂量。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血液" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "实体瘤" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "白血病" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "血液" } ] }, { "text": "4.治疗计划治疗计划在方案中应明确、完整,如外科手术、放疗、化疗、支持疗法、对毒副反应的处理规则等,以保证治疗的一致性。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "外科手术" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "放疗" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "化疗" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "支持疗法" }, { "start_idx": 38, "end_idx": 45, "type": "pro", "entity": "对毒副反应的处理" } ] }, { "text": "对于长期生存率高的肿瘤,如急性淋巴细胞白血病,以无病生存时间为终点也很有意义。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "急性淋巴细胞白血病" } ] }, { "text": "其他附加性研究终点有有关生命质量如第二肿瘤、生长发育紊乱、精神心理损害等。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "第二肿瘤" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "生长发育紊乱" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "精神心理损害" } ] }, { "text": "第十三章烧烫伤【概述】烧烫伤是指单纯由热水、蒸汽、火焰、电击等高温所造成的热烧伤。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "热烧伤" } ] }, { "text": "烧烫伤是儿科临床常见的意外之一。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 4, "end_idx": 5, "type": "dep", "entity": "儿科" } ] }, { "text": "小儿烧烫伤的特点:小儿皮肤薄,即使接触温度不很高的热物,也可导致烧烫伤;对热的回避反射不够迅速,与热物接触时间较久,与成人相比更容易发生较深的损伤;小儿总血量与皮肤面积的比值较成人小得多,所以同样面积的烧烫伤和皮肤液体的渗出,对小儿的影响就大得多,也更易发生休克;烧烫伤的渗出以及其后饮食失调也容易发生脱水、酸中毒;对感染的抵抗力差,败血症与毒血症也较多;小儿不能照顾自己,大小便污染创面的机会较多。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小儿烧烫伤" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "皮肤" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 68, "end_idx": 72, "type": "sym", "entity": "较深的损伤" }, { "start_idx": 77, "end_idx": 77, "type": "bod", "entity": "血" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "皮肤" }, { "start_idx": 101, "end_idx": 103, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 105, "end_idx": 111, "type": "sym", "entity": "皮肤液体的渗出" }, { "start_idx": 105, "end_idx": 108, "type": "bod", "entity": "皮肤液体" }, { "start_idx": 129, "end_idx": 130, "type": "dis", "entity": "休克" }, { "start_idx": 132, "end_idx": 134, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 136, "end_idx": 137, "type": "sym", "entity": "渗出" }, { "start_idx": 142, "end_idx": 145, "type": "sym", "entity": "饮食失调" }, { "start_idx": 151, "end_idx": 152, "type": "sym", "entity": "脱水" }, { "start_idx": 154, "end_idx": 156, "type": "dis", "entity": "酸中毒" }, { "start_idx": 158, "end_idx": 165, "type": "sym", "entity": "对感染的抵抗力差" }, { "start_idx": 159, "end_idx": 160, "type": "dis", "entity": "感染" }, { "start_idx": 167, "end_idx": 169, "type": "dis", "entity": "败血症" }, { "start_idx": 171, "end_idx": 173, "type": "dis", "entity": "毒血症" }, { "start_idx": 187, "end_idx": 198, "type": "sym", "entity": "大小便污染创面的机会较多" }, { "start_idx": 187, "end_idx": 189, "type": "bod", "entity": "大小便" }, { "start_idx": 192, "end_idx": 193, "type": "bod", "entity": "创面" } ] }, { "text": "【病因及发病机制】烧烫伤与所接触的高温热源性质有关。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "烧烫伤" } ] }, { "text": "小儿常见的烧烫伤病因有:(一)皮肤接触高温物体或高温液体多见于3岁以下、具有行走等初步活动能力的幼儿,接触如尚未冷却饭菜、开水、烫锅、电热杯等所致。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" } ] }, { "text": "烫伤部位与正常组织边界较为清楚。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "烫伤部位与正常组织边界较为清楚" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "烫伤部位" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "正常组织" } ] }, { "text": "(二)接触明火多见于2岁以上小儿,在缺乏防护情况下接触炉火、火柴、易燃物突然燃烧后烫伤,其受伤边界通常不很清楚。", "entities": [ { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "烫伤" }, { "start_idx": 45, "end_idx": 54, "type": "sym", "entity": "受伤边界通常不很清楚" } ] }, { "text": "(三)爆炸时烧伤较少,多见于烟花爆竹意外。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "烧伤" } ] }, { "text": "受伤部位烫伤程度较均匀。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "受伤部位烫伤程度较均匀" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "受伤部位" } ] }, { "text": "小儿烧烫伤的程度取决于受伤的方式和烫伤面积。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "受伤" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "烫伤" } ] }, { "text": "1.局部变化皮肤遇高热后(超过60℃),组织内蛋白即可发生凝固,以致细胞坏死。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 37, "type": "sym", "entity": "组织内蛋白即可发生凝固,以致细胞坏死" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "组织内蛋白" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "细胞坏死" } ] }, { "text": "局部反应依热物的温度、接触的时间以及受伤皮肤厚薄而异。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "受伤皮肤" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" } ] }, { "text": "按局部组织坏死的深度,小儿烧烫伤分为三度。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "局部组织坏死" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "小儿烧烫伤" } ] }, { "text": "但由于小儿皮肤很薄,分度有困难。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "皮肤" } ] }, { "text": "2.全身变化严重烧烫伤者可出现休克。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "严重烧烫伤" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "休克" } ] }, { "text": "早期出现休克多由于疼痛和精神刺激引起,一般为暂时性,不严重。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "休克" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "疼痛" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "精神刺激" } ] }, { "text": "而继发性休克是因为毛细血管渗出增加,使组织水肿和创面大量渗液、血浆损耗、血液浓缩和循环血量减低,继而出现组织缺氧、血压下降、脉搏低弱、低血钠与酸中毒、少尿或无尿等。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "继发性休克" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "毛细血管渗出增加" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "毛细血管" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "组织水肿" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "组织" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "创面大量渗液" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "创面" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "血浆" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "血浆损耗" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血液" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "血液浓缩" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "循环血量减低" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "血" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "组织缺氧" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "组织" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "血压" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "血压下降" }, { "start_idx": 62, "end_idx": 63, "type": "ite", "entity": "脉搏" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "脉搏低弱" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "低血钠" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "酸中毒" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "少尿" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "尿" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "无尿" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "尿" } ] }, { "text": "烧烫伤后6~8小时内液体渗出最快,并于36~48小时达最高峰,通常超过淋巴回流的能力,以后逐渐减慢。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "液体渗出" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "淋巴" } ] }, { "text": "【临床表现及诊断】诊治烧烫伤时应注意详细了解受伤面积、深度,对特殊部位如五官、关节、面部等,精确计算烧烫伤面积和估计深度,有助于判断损伤的严重性,估计预后,有利于治疗,同时也是液体补给的依据。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "受伤" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "五官" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "关节" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "面部" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "损伤" }, { "start_idx": 88, "end_idx": 91, "type": "pro", "entity": "液体补给" } ] }, { "text": "烧烫伤面积和深度的测算如下:(一)烧烫伤面积的计算1.手掌法伤员自己五指并拢时的手掌面积,相当于全身体表面积的1%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "手掌" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "五指" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "手掌" }, { "start_idx": 48, "end_idx": 53, "type": "ite", "entity": "全身体表面积" } ] }, { "text": "此方法精确度稍差,常用于急诊室估计小面积的烧烫伤或用于估计小范围的三度烧烫伤。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "三度烧烫伤" } ] }, { "text": "2.体表面积计算法参照小儿身体各部位在不同年龄时的面积百分率进行计算(表6-6)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "体表面积" } ] }, { "text": "但小儿年龄越小,头部的比例越大,下肢比例越小。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "头部" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "下肢" } ] }, { "text": "随着年龄的增大,小儿头部与下肢的比例逐渐与成人相接近。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "头部" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "下肢" } ] }, { "text": "可参照下列公式校正:小儿头部面积(%)=9+(12-年龄)小儿两下肢面积(%)=41-(12-年龄)亦可参照表6-6及图6-8。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "头部面积" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "下肢面积" } ] }, { "text": "表6-17小儿烧烫伤参考面积速查表(二)烧烫伤深度的估计临床通常采用“三度四分”法(表6-6)进行评定。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "小儿烧烫伤" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 34, "end_idx": 40, "type": "ite", "entity": "“三度四分”法" } ] }, { "text": "故在治疗48小时后应再重新核实修正;手掌、足底皮肤厚,早期易将二度误为三度,而婴幼儿皮肤很薄,易将三度误为二度,需加以注意。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "手掌" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "足底皮肤" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "皮肤" } ] }, { "text": "图6-8烧烫伤面积估计(三)烧烫伤的分类1.轻度总面积在10%以下,无三度的烧烫伤。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "烧烫伤" } ] }, { "text": "2.中度总面积在11%~20%之间,或三度在5%以下,或头面部、手、足、会阴部的二度烧烫伤。", "entities": [ { "start_idx": 28, "end_idx": 44, "type": "dis", "entity": "头面部、手、足、会阴部的二度烧烫伤" } ] }, { "text": "3.重度总面积在21%~50%之间,或三度在5%~15%之间,或合并有呼吸道灼伤,伴大面积软组织损伤、骨折、肾衰竭。", "entities": [ { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "呼吸道灼伤" }, { "start_idx": 42, "end_idx": 49, "type": "dis", "entity": "大面积软组织损伤" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "骨折" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "由于小儿发育尚未成熟,对休克、感染等抵抗力较差。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "休克" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "感染" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "抵抗力较差" } ] }, { "text": "尤其2岁以下婴儿免疫力低下,更为危险,所以对小儿烧烫伤的严重程度应有足够的认识。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "免疫力低下" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "小儿烧烫伤" } ] }, { "text": "【治疗】主要包括早期处理、输液治疗、创面的处理、败血症的防治以及特殊部位烧烫伤的处理。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "早期处理" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "输液治疗" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "创面的处理" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "败血症" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "烧烫伤" } ] }, { "text": "(一)早期处理小面积烧烫伤,治疗重点在创面本身。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "早期处理小面积烧烫伤" }, { "start_idx": 14, "end_idx": 22, "type": "pro", "entity": "治疗重点在创面本身" } ] }, { "text": "对怀疑存在中度以上的或伴有头面部、会阴部烧烫伤的患儿,均应尽可能收住院治疗。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "头面部、会阴部烧烫伤" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "住院治疗" } ] }, { "text": "边输液,边估计烧烫伤面积和深度,测体重和预算休克期输液量(可先输入电解质液如林格乳酸钠液或葡萄糖盐水);并进行血生化检测。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "pro", "entity": "边输液,边估计烧烫伤面积和深度" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "测体重" }, { "start_idx": 20, "end_idx": 27, "type": "pro", "entity": "预算休克期输液量" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "输入" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "电解质液" }, { "start_idx": 38, "end_idx": 43, "type": "dru", "entity": "林格乳酸钠液" }, { "start_idx": 45, "end_idx": 49, "type": "dru", "entity": "葡萄糖盐水" }, { "start_idx": 55, "end_idx": 59, "type": "ite", "entity": "血生化检测" } ] }, { "text": "对于大面积的或会阴部烧烫伤,还应留置导尿管,并测定每小时尿量和尿比重,预防破伤风和感染。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "大面积的或会阴部烧烫伤" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "留置导尿管" }, { "start_idx": 23, "end_idx": 33, "type": "ite", "entity": "测定每小时尿量和尿比重" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "破伤风" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "感染" } ] }, { "text": "表6-18烧烫伤深度估计(二)输液方法输液总量按“累积损失量+生理需要量”计算。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "输液" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "输液" }, { "start_idx": 25, "end_idx": 29, "type": "ite", "entity": "累积损失量" }, { "start_idx": 31, "end_idx": 35, "type": "ite", "entity": "生理需要量" } ] }, { "text": "累积损失量是每1%体表烧烫伤面积每千克体重2~4ml,即累积损失量=每1%烧烫伤面积×每千克体重×2~4ml。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "累积损失量" }, { "start_idx": 9, "end_idx": 15, "type": "ite", "entity": "体表烧烫伤面积" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "体重" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "累积损失量" }, { "start_idx": 37, "end_idx": 41, "type": "ite", "entity": "烧烫伤面积" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "体重" } ] }, { "text": "选择溶液时,胶体液和晶体液的比例应根据烧烫伤的深度和面积决定。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "胶体液" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "晶体液" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "烧烫伤" } ] }, { "text": "中、小面积浅度烫伤胶体和晶体液的比例为1∶1~1∶3,胶体液可采用右旋糖酐或代血浆,晶体可选用生理盐水或平衡液(2份生理盐水+1份1.25%碳酸氢钠溶液)。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "中、小面积浅度烫伤" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "胶体" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "晶体液" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "胶体液" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "右旋糖酐" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "代血浆" }, { "start_idx": 42, "end_idx": 43, "type": "dru", "entity": "晶体" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "生理盐水" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "平衡液" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "生理盐水" }, { "start_idx": 70, "end_idx": 75, "type": "dru", "entity": "碳酸氢钠溶液" } ] }, { "text": "大面积深度烧烫伤者胶体和晶体液的比例按1∶1计算,其中胶体应以血浆为主。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "大面积深度烧烫伤" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "胶体" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "晶体液" }, { "start_idx": 27, "end_idx": 28, "type": "dru", "entity": "胶体" }, { "start_idx": 31, "end_idx": 32, "type": "dru", "entity": "血浆" } ] }, { "text": "此外,由于三度烧烫伤红细胞破坏及损失较多,治疗中应注意输注全血。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "三度烧烫伤" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "红细胞" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "红细胞破坏及损失较多" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "输注全血" } ] }, { "text": "全血与血浆的比例可按1∶2计算。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "全血" }, { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "血浆" } ] }, { "text": "每天生理需要量可按1800ml/m2体表面积计算或按婴儿100~150ml/kg、儿童70~100ml/kg计算。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "ite", "entity": "每天生理需要量" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "体表面积" } ] }, { "text": "烧烫伤患儿应按以下的输液量和速度进行。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "烧烫伤" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "输液" } ] }, { "text": "第一个24小时液量为:①第一个8小时:输液量=1/2累积损失量+1/3每天生理需要量;②第二个8小时:输液量=1/4累积损失量+1/3每天生理需要量;③第三个8小时:输液量=1/4累积损失量+1/3每天生理需要量。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "输液" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "累积损失量" }, { "start_idx": 35, "end_idx": 41, "type": "ite", "entity": "每天生理需要量" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "输液" }, { "start_idx": 58, "end_idx": 62, "type": "ite", "entity": "累积损失量" }, { "start_idx": 67, "end_idx": 73, "type": "ite", "entity": "每天生理需要量" }, { "start_idx": 83, "end_idx": 84, "type": "pro", "entity": "输液" }, { "start_idx": 90, "end_idx": 94, "type": "ite", "entity": "累积损失量" }, { "start_idx": 99, "end_idx": 105, "type": "ite", "entity": "每天生理需要量" } ] }, { "text": "第二个24小时全天输液量为:首日累积损失量×0.5+每天生理需要量。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "输液" }, { "start_idx": 14, "end_idx": 20, "type": "ite", "entity": "首日累积损失量" }, { "start_idx": 26, "end_idx": 32, "type": "ite", "entity": "每天生理需要量" } ] }, { "text": "在无休克和灌注不良情况下,补液可按24小时内均匀输注方式进行。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "休克" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "灌注不良" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "补液" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "均匀输注" } ] }, { "text": "48小时以后的输液应根据具体情况而定,如无异常情况可按第二个24小时液量给予。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "输液" } ] }, { "text": "输液时注意点:①实际烧烫伤面积计算时,一度者一般不予计算在内;②50%以上的大面积烧烫伤者以不超过50%面积的输液量为原则,时间计算是以受伤的时间算起;③胶体液、晶体液须交替输注,不要在短时间内集中输入某一种液体;④如有休克,则应加大胶体和晶体溶液输注量;⑤头面部及特重度者,输液量应酌情增加。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "输液" }, { "start_idx": 8, "end_idx": 14, "type": "ite", "entity": "实际烧烫伤面积" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "大面积烧烫伤" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "输液" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "受伤" }, { "start_idx": 77, "end_idx": 79, "type": "dru", "entity": "胶体液" }, { "start_idx": 81, "end_idx": 83, "type": "dru", "entity": "晶体液" }, { "start_idx": 85, "end_idx": 88, "type": "pro", "entity": "交替输注" }, { "start_idx": 90, "end_idx": 105, "type": "pro", "entity": "不要在短时间内集中输入某一种液体" }, { "start_idx": 110, "end_idx": 111, "type": "dis", "entity": "休克" }, { "start_idx": 115, "end_idx": 126, "type": "pro", "entity": "加大胶体和晶体溶液输注量" }, { "start_idx": 129, "end_idx": 131, "type": "bod", "entity": "头面部" }, { "start_idx": 138, "end_idx": 145, "type": "pro", "entity": "输液量应酌情增加" } ] }, { "text": "以下是临床输液治疗的理想目标:尿量在1~1.5ml/(kg•h)以上(不同年龄的小儿每天尿量见表6-6),尿比重在1.015~1.020之间;安静无烦躁,精神状态良好。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "输液治疗" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "尿量" }, { "start_idx": 44, "end_idx": 45, "type": "ite", "entity": "尿量" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "尿比重" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "烦躁" } ] }, { "text": "心率在每分钟140次以下;周围循环灌注良好;收缩血压维持在年龄×2+10.64Pa(80mmHg)以上,中心静脉压在0.78~1.17kPa(8~12cmH2O)范围。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "心率" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "周围循环" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "收缩血压" }, { "start_idx": 52, "end_idx": 56, "type": "ite", "entity": "中心静脉压" } ] }, { "text": "表6-19不同年龄小儿的尿量(三)创面的处理四肢、躯干部位的烧烫伤可采用包扎法。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "尿量" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "创面的处理" }, { "start_idx": 22, "end_idx": 32, "type": "dis", "entity": "四肢、躯干部位的烧烫伤" }, { "start_idx": 36, "end_idx": 38, "type": "pro", "entity": "包扎法" } ] }, { "text": "头面部、会阴部因受进食、呼吸、及两便排泄的影响,在渗出量较多时可采用暴露法。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "头面部" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "会阴部" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "两便" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "渗出量较多" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "渗出量" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "暴露法" } ] }, { "text": "深度烧烫伤者的创面应早期切痂,以期减少瘢痕增生而造成的畸形和功能障碍。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "深度烧烫伤" }, { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "创面应早期切痂" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "瘢痕增生" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "畸形" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "功能障碍" } ] }, { "text": "(四)败血症的防治烧烫伤感染以金黄色葡萄球菌及铜绿假单胞菌最多。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "败血症" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "烧烫伤感染" }, { "start_idx": 15, "end_idx": 21, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 23, "end_idx": 28, "type": "mic", "entity": "铜绿假单胞菌" } ] }, { "text": "感染的最大危害期是早期水肿回收期(48小时后)的毒血症或败血症以及Ⅲ度烧烫伤脱痂期(2~3周时)的败血症,局部组织坏死,使浅度变为深创面不能如期愈合。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "感染" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "水肿" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "毒血症" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "败血症" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "Ⅲ度烧烫伤" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "败血症" }, { "start_idx": 53, "end_idx": 58, "type": "dis", "entity": "局部组织坏死" }, { "start_idx": 61, "end_idx": 73, "type": "sym", "entity": "浅度变为深创面不能如期愈合" } ] }, { "text": "(五)特殊部位的处理头面部烧烫伤由于渗出多,水肿严重,创面宜采用暴露法,并加强护理,注意呼吸、进食、五官分泌物等变化。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "特殊部位的处理" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "头面部烧烫伤" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "渗出多" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "水肿严重" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "创面" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "暴露法" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "加强护理" }, { "start_idx": 42, "end_idx": 57, "type": "pro", "entity": "注意呼吸、进食、五官分泌物等变化" } ] }, { "text": "手部烧烫伤后易形成瘢痕挛缩,常造成畸形和功能障碍,因此在初次清创后以包扎为宜,并放置功能位;同时,尽量抬高患肢以减少水肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "手部烧烫伤" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "瘢痕挛缩" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "畸形" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "功能障碍" }, { "start_idx": 27, "end_idx": 37, "type": "pro", "entity": "在初次清创后以包扎为宜" }, { "start_idx": 40, "end_idx": 44, "type": "pro", "entity": "放置功能位" }, { "start_idx": 49, "end_idx": 59, "type": "pro", "entity": "尽量抬高患肢以减少水肿" } ] }, { "text": "会阴部烧烫伤除采用暴露法外,还应加强局部护理和清洁,避免尿、粪污染创面。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "会阴部烧烫伤" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "暴露法" }, { "start_idx": 16, "end_idx": 24, "type": "pro", "entity": "加强局部护理和清洁" }, { "start_idx": 26, "end_idx": 34, "type": "pro", "entity": "避免尿、粪污染创面" } ] }, { "text": "对于明确三度烧烫伤部分,创面宜早期切痂,移植大片自体皮,并在创面愈合后及早开始功能锻炼及理疗。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "三度烧烫伤" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "创面宜早期切痂" }, { "start_idx": 20, "end_idx": 26, "type": "pro", "entity": "移植大片自体皮" }, { "start_idx": 29, "end_idx": 45, "type": "pro", "entity": "在创面愈合后及早开始功能锻炼及理疗" } ] }, { "text": "第五节室间隔完整型肺动脉闭锁室间隔完整型肺动脉闭锁指右心室流出道完全梗阻,但室间隔完整。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "室间隔完整型肺动脉闭锁" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "室间隔完整型肺动脉闭锁" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "右心室流出道" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "右心室流出道完全梗阻" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "室间隔" } ] }, { "text": "根据右心室与肺动脉的连接情况分两类:第一类,右心室流出道存在,肺动脉瓣组织发育畸形为无空洞的隔膜;第二类,右心室流出道漏斗腔消失呈肌性闭锁。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "右心室" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺动脉" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "右心室流出道" }, { "start_idx": 31, "end_idx": 40, "type": "dis", "entity": "肺动脉瓣组织发育畸形" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "无空洞的隔膜" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "隔膜" }, { "start_idx": 53, "end_idx": 63, "type": "sym", "entity": "右心室流出道漏斗腔消失" }, { "start_idx": 53, "end_idx": 61, "type": "bod", "entity": "右心室流出道漏斗腔" }, { "start_idx": 65, "end_idx": 68, "type": "dis", "entity": "肌性闭锁" } ] }, { "text": "以上分类有重要的临床意义,据近期报道用激光和射频消融技术在闭锁瓣膜上打孔,对第一类有较好疗效,但对肌性闭锁仍缺乏理想的治疗方法。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "激光" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "射频消融技术" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "闭锁瓣膜上" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "肌性闭锁" } ] }, { "text": "【病理解剖】右心室大小不一,但均有不同程度发育不良,室壁心肌肥厚,心室腔狭小。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "病理解剖" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心室" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "右心室大小不一" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "发育不良" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "室壁心肌肥厚" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "室壁心肌" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "心室腔狭小" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "心室腔" } ] }, { "text": "Ball等根据形态学将右室分为三部分(流入部、小梁部、流出部),此分类与手术方式有密切关系。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "右室" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "流入部" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "小梁部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "流出部" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "手术" } ] }, { "text": "从性质上判断,三尖瓣瓣环直径Z指数(三尖瓣瓣环直径与体表面积成正相关)与右心室腔大小有关,直径越接近正常,右心室发育越完善。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "三尖瓣瓣环" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "三尖瓣瓣环" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "右心室腔" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "右心室" } ] }, { "text": "本病常伴三尖瓣的异常,瓣膜装置小,瓣叶发育不良及Ebstein畸形常导致严重的三尖瓣反流及右心室增大;三尖瓣狭窄则导致右室肥厚。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "三尖瓣的异常" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "瓣膜装置小" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "瓣膜" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "瓣叶发育不良" }, { "start_idx": 24, "end_idx": 32, "type": "dis", "entity": "Ebstein畸形" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "三尖瓣反流" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "右心室增大" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "右心室" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "三尖瓣狭窄" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "右室肥厚" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "右室" } ] }, { "text": "心房水平的右向左分流为患儿生存所必需,因此必须保持卵圆孔开放或人为造成房间隔缺损。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心房" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "心房水平的右向左分流" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "卵圆孔关闭是导致本病早产儿死亡的常见原因。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "卵圆孔关闭" } ] }, { "text": "肺血流由狭长扭曲的动脉导管供应,肺动脉干常发育良好,多数右室漏斗部正常的患者,肺动脉瓣发育好,但相互融合,而右心室发育不良、漏斗部狭窄或闭锁者,肺动脉瓣发育极差,肺动脉分支常闭锁闭锁,少数病例肺动脉血流由双侧动脉导管及主肺动脉侧支供应。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺血流" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "动脉导管" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "肺动脉干" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "右室漏斗部" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "右心室发育不良" }, { "start_idx": 62, "end_idx": 69, "type": "dis", "entity": "漏斗部狭窄或闭锁" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 72, "end_idx": 79, "type": "sym", "entity": "肺动脉瓣发育极差" }, { "start_idx": 81, "end_idx": 88, "type": "sym", "entity": "肺动脉分支常闭锁" }, { "start_idx": 81, "end_idx": 85, "type": "bod", "entity": "肺动脉分支" }, { "start_idx": 87, "end_idx": 88, "type": "dis", "entity": "闭锁" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "闭锁" }, { "start_idx": 96, "end_idx": 100, "type": "bod", "entity": "肺动脉血流" }, { "start_idx": 96, "end_idx": 116, "type": "sym", "entity": "肺动脉血流由双侧动脉导管及主肺动脉侧支供应" }, { "start_idx": 102, "end_idx": 107, "type": "bod", "entity": "双侧动脉导管" }, { "start_idx": 109, "end_idx": 114, "type": "bod", "entity": "主肺动脉侧支" } ] }, { "text": "本病常伴冠状动脉异常,合并右心室冠状动脉瘘者约45%,这对于确定治疗方法有重要意义。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "冠状动脉异常" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "右心室冠状动脉瘘" } ] }, { "text": "右心室发育不良者常合并这种心室冠脉连接。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "右心室发育不良" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "心室冠脉" } ] }, { "text": "由于右心室高压,收缩期湍流致冠状主动脉内膜损伤,肌层发育不良,弹性纤维组织增生,最终导致其狭窄、关闭,如右心室压力降低,右心室冠状动脉瘘即可减轻。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "右心室" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "右心室高压" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "冠状主动脉内膜损伤" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "肌层发育不良" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "弹性纤维组织增生" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "狭窄" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "关闭" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "右心室" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "右心室压力降低" }, { "start_idx": 60, "end_idx": 67, "type": "dis", "entity": "右心室冠状动脉瘘" } ] }, { "text": "右心室依赖性冠脉循环存在时,主动脉舒张期压力不足以将血液射入冠状动脉,且此时常伴冠状动脉异常,包括冠状动脉近端缺如、冠状动脉狭窄或中断及左冠脉或右冠脉与右心室之间的冠状动脉心室瘘。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "冠脉循环" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "主动脉" }, { "start_idx": 14, "end_idx": 33, "type": "sym", "entity": "主动脉舒张期压力不足以将血液射入冠状动脉" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血液" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "冠状动脉异常" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "冠状动脉近端缺如" }, { "start_idx": 58, "end_idx": 66, "type": "dis", "entity": "冠状动脉狭窄或中断" }, { "start_idx": 68, "end_idx": 88, "type": "dis", "entity": "左冠脉或右冠脉与右心室之间的冠状动脉心室瘘" } ] }, { "text": "因此,尽管收缩期高压力的右心室将少量血液经血管窦射入冠状动脉,对于维持心肌灌注仍非常重要。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "右心室" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血液" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "血管窦" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "维持心肌灌注" } ] }, { "text": "此时如建立右心室肺动脉连续性循环,将使右心室压力降低,导致心肌局部缺血和梗死。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "右心室" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺动脉" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "右心室压力降低" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心室" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "心肌局部缺血和梗死" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "心肌局部" } ] }, { "text": "存在右心室冠状动脉连接者约10%发生右心室依赖型冠脉循环。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "右心室冠状动脉" }, { "start_idx": 18, "end_idx": 27, "type": "sym", "entity": "右心室依赖型冠脉循环" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "右心室" } ] }, { "text": "【病理生理】本病存在心房水平右向左分流,常伴卵圆孔未闭。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "心房水平右向左分流" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心房" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "卵圆孔未闭" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "卵圆孔" } ] }, { "text": "右心室收缩期压力超过体循环压力,右心室在收缩期形成的高压血流,使胚胎时期的右心室-冠脉通道持续开放,冠脉血液通过发育不良的三尖瓣进入右心室,如有三尖瓣关闭不全,血液反流入右心房;如无三尖瓣反流则通过右心室冠状动脉瘘进入冠脉循环。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "右心室收缩期压力超过体循环压力" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 16, "end_idx": 29, "type": "sym", "entity": "右心室在收缩期形成的高压血流" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "右心室" }, { "start_idx": 32, "end_idx": 48, "type": "sym", "entity": "胚胎时期的右心室-冠脉通道持续开放" }, { "start_idx": 37, "end_idx": 44, "type": "bod", "entity": "右心室-冠脉通道" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "冠脉血液" }, { "start_idx": 56, "end_idx": 68, "type": "sym", "entity": "发育不良的三尖瓣进入右心室" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "右心室" }, { "start_idx": 72, "end_idx": 78, "type": "dis", "entity": "三尖瓣关闭不全" }, { "start_idx": 80, "end_idx": 87, "type": "sym", "entity": "血液反流入右心房" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "血液" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "右心房" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "三尖瓣反流" }, { "start_idx": 99, "end_idx": 112, "type": "sym", "entity": "右心室冠状动脉瘘进入冠脉循环" }, { "start_idx": 99, "end_idx": 106, "type": "dis", "entity": "右心室冠状动脉瘘" } ] }, { "text": "手术可使右心室与肺动脉干保持血流畅通,右心室压力低于体循环压力环压力,此时若伴右心室依赖性冠脉循环,可造成心肌局部缺血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "右心室" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "肺动脉干" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" }, { "start_idx": 19, "end_idx": 30, "type": "sym", "entity": "右心室压力低于体循环压力" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心室" }, { "start_idx": 38, "end_idx": 48, "type": "sym", "entity": "伴右心室依赖性冠脉循环" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "右心室" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "冠脉" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "心肌局部缺血" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "心肌局部" } ] }, { "text": "体循环氧合作用依赖于肺血流量,尽管肺血流量增加可减轻发绀,但大的动脉导管未闭可导致充血性心力衰竭。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "肺血流量" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "肺血流量" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发绀" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "动脉导管" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "充血性心力衰竭" } ] }, { "text": "【临床表现】出生后数小时内出现中心性发绀,随着动脉导管功能性关闭,发绀逐渐加重。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "中心性发绀" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "动脉导管功能性关闭" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "动脉导管" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "发绀逐渐加重" } ] }, { "text": "高度扩张的右心室压迫肺脏使之不同程度的发育不良导致呼吸困难、呼吸急促,体格检查可见心尖向左侧移位,心尖搏动呈抬举样,第一、二心音呈单一音,因三尖瓣关闭不全,胸骨左下缘可闻及全收缩期杂音,胸骨左上缘闻及动脉导管未闭所产生的短促、柔和的收缩期喷射性杂音,静脉输注前列腺素E2、E1后导管杂音增强心房间通道狭小及低心排血量发绀明显,伴有脉搏减弱,肝脏肿大。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "高度扩张的右心室压迫肺脏" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "右心室" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肺脏" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "不同程度的发育不良" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 35, "end_idx": 67, "type": "sym", "entity": "体格检查可见心尖向左侧移位,心尖搏动呈抬举样,第一、二心音呈单一音" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "体格检查" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "心尖" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "心尖" }, { "start_idx": 70, "end_idx": 76, "type": "dis", "entity": "三尖瓣关闭不全" }, { "start_idx": 78, "end_idx": 91, "type": "sym", "entity": "胸骨左下缘可闻及全收缩期杂音" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "胸骨左下缘" }, { "start_idx": 93, "end_idx": 123, "type": "sym", "entity": "胸骨左上缘闻及动脉导管未闭所产生的短促、柔和的收缩期喷射性杂音" }, { "start_idx": 93, "end_idx": 97, "type": "bod", "entity": "胸骨左上缘" }, { "start_idx": 100, "end_idx": 103, "type": "bod", "entity": "动脉导管" }, { "start_idx": 125, "end_idx": 128, "type": "pro", "entity": "静脉输注" }, { "start_idx": 129, "end_idx": 137, "type": "dru", "entity": "前列腺素E2、E1" }, { "start_idx": 139, "end_idx": 144, "type": "sym", "entity": "导管杂音增强" }, { "start_idx": 139, "end_idx": 140, "type": "bod", "entity": "导管" }, { "start_idx": 145, "end_idx": 151, "type": "sym", "entity": "心房间通道狭小" }, { "start_idx": 145, "end_idx": 149, "type": "bod", "entity": "心房间通道" }, { "start_idx": 153, "end_idx": 161, "type": "sym", "entity": "低心排血量发绀明显" }, { "start_idx": 153, "end_idx": 154, "type": "bod", "entity": "低心" }, { "start_idx": 156, "end_idx": 156, "type": "bod", "entity": "血" }, { "start_idx": 165, "end_idx": 168, "type": "sym", "entity": "脉搏减弱" }, { "start_idx": 170, "end_idx": 173, "type": "sym", "entity": "肝脏肿大" }, { "start_idx": 170, "end_idx": 171, "type": "bod", "entity": "肝脏" } ] }, { "text": "【辅助检查】(一)胸部X线心影增大,重度三尖瓣关闭不全者增大明显,心影占据大部分胸腔,肺野清晰,肺血管纹理减少。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "胸部X线心影增大" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "胸部X线" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "心" }, { "start_idx": 18, "end_idx": 31, "type": "sym", "entity": "重度三尖瓣关闭不全者增大明显" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "重度三尖瓣关闭不全" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "心影占据大部分胸腔" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "心" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "胸腔" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "肺野清晰" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肺" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "肺血管纹理减少" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "肺血管" } ] }, { "text": "(二)心电图特征性表现为电轴左偏,左心室占优势,右心室低电压,右心房增大,乃因三尖瓣闭锁及左心室双入口所致。", "entities": [ { "start_idx": 3, "end_idx": 35, "type": "sym", "entity": "心电图特征性表现为电轴左偏,左心室占优势,右心室低电压,右心房增大" }, { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "心电图" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "左心室" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "右心室" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心房" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "左心室双入口" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "左心室" } ] }, { "text": "(三)超声心动图剑突下切面,二维超声及脉冲多普勒可充分显示双心房间异常交通,胸骨旁及心尖四腔切面可显示三尖瓣大小、形态学改变及反流严重程度。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "超声心动图剑突下切面" }, { "start_idx": 14, "end_idx": 36, "type": "sym", "entity": "二维超声及脉冲多普勒可充分显示双心房间异常交通" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "二维超声" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "脉冲多普勒" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "双心房间" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "胸骨旁" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "心尖四腔切面" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "反流严重" } ] }, { "text": "经剑下冠状、矢状切面,可根据右心室发育不良程度将右心室分为三部分。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "bod", "entity": "剑下冠状、矢状切面" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "右心室发育不良" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "右心室" } ] }, { "text": "由于技术上和本病右心室解剖结构上的原因,测量右心室容积临床意义不大。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "右心室" }, { "start_idx": 20, "end_idx": 26, "type": "pro", "entity": "测量右心室容积" } ] }, { "text": "较常用的方法为根据体表面积计算出三尖瓣瓣环直径的正常值,再通过Z指数来判断右心室大小。", "entities": [ { "start_idx": 7, "end_idx": 26, "type": "pro", "entity": "根据体表面积计算出三尖瓣瓣环直径的正常值" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "Z指数" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "右心室" } ] }, { "text": "右心室流出道肌性闭锁者漏斗腔完全消失,右心室与主肺动脉干之间形成分隔,经胸骨旁大动脉短轴切面显示最佳(图9-9)。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "右心室流出道肌性闭锁者漏斗腔完全消失" }, { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "右心室流出道肌性闭锁" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "漏斗腔" }, { "start_idx": 19, "end_idx": 33, "type": "sym", "entity": "右心室与主肺动脉干之间形成分隔" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心室" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "主肺动脉干" }, { "start_idx": 36, "end_idx": 45, "type": "bod", "entity": "胸骨旁大动脉短轴切面" } ] }, { "text": "肺动脉瓣闭锁者,瓣膜相互融合,漏斗部末端形成盲端,右心室收缩漏斗部末端形成盲端,在二维超声不易与肺动脉狭窄脉冲多普勒脉冲多普勒和彩色多普勒可看到湍流通过狭窄的肺动脉瓣口,可伴或不伴反流。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "肺动脉瓣闭锁" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "瓣膜相互融合" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "瓣膜" }, { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "漏斗部末端形成盲端" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "漏斗部末端" }, { "start_idx": 25, "end_idx": 38, "type": "sym", "entity": "右心室收缩漏斗部末端形成盲端" }, { "start_idx": 25, "end_idx": 34, "type": "bod", "entity": "右心室收缩漏斗部末端" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "二维超声" }, { "start_idx": 48, "end_idx": 57, "type": "pro", "entity": "肺动脉狭窄脉冲多普勒" }, { "start_idx": 58, "end_idx": 62, "type": "pro", "entity": "脉冲多普勒" }, { "start_idx": 64, "end_idx": 68, "type": "pro", "entity": "彩色多普勒" }, { "start_idx": 72, "end_idx": 83, "type": "sym", "entity": "湍流通过狭窄的肺动脉瓣口" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "肺动脉瓣口" }, { "start_idx": 90, "end_idx": 91, "type": "sym", "entity": "反流" } ] }, { "text": "动脉导管走向常较垂直,在胸骨上切面更易看到。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "动脉导管" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "胸骨上切面" } ] }, { "text": "图9-32超声心动图显示:完整的肺动脉瓣将右心室(RV)和肺动脉(PA)分开二维超声不易判断右心室肥大时有无右心室冠脉交通,彩色多普勒可探查窦状隙血流,这种窦状血流常出现于右心室腔小,心室内压较高的患儿。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "超声心动图" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "右心室" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "RV" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肺动脉" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "PA" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "二维超声" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "右心室肥大" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "右心室" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "右心室冠脉交通" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "右心室" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "冠脉" }, { "start_idx": 62, "end_idx": 74, "type": "pro", "entity": "彩色多普勒可探查窦状隙血流" }, { "start_idx": 78, "end_idx": 81, "type": "sym", "entity": "窦状血流" }, { "start_idx": 80, "end_idx": 80, "type": "bod", "entity": "血" }, { "start_idx": 86, "end_idx": 90, "type": "sym", "entity": "右心室腔小" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "右心室" }, { "start_idx": 92, "end_idx": 97, "type": "sym", "entity": "心室内压较高" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "心室" } ] }, { "text": "心血管造影是确立诊断的重要依据。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "心血管造影" } ] }, { "text": "(四)心导管手术或经导管右心室减压术前应先行心导管和心血管造影以判断有无冠状动脉狭窄或中断。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心导管手术" }, { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "经导管右心室减压术" }, { "start_idx": 22, "end_idx": 30, "type": "pro", "entity": "心导管和心血管造影" }, { "start_idx": 36, "end_idx": 44, "type": "dis", "entity": "冠状动脉狭窄或中断" } ] }, { "text": "血流动力学显示右心室舒张期压力等于或大于体循环压力。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血流" }, { "start_idx": 7, "end_idx": 24, "type": "sym", "entity": "右心室舒张期压力等于或大于体循环压力" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "右心室" } ] }, { "text": "低于体循环压力者少见,常见于由三尖瓣发育不良、Ebstein畸形和右心室狭小所致的严重三尖瓣反流。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "低于体循环压力" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "三尖瓣发育不良" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "Ebstein畸形" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "右心室" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "右心室狭小" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "三尖瓣反流" } ] }, { "text": "右心室收缩末期压力升高,顺应性降低。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "右心室收缩末期压力升高,顺应性降低" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" } ] }, { "text": "因存在非限制性的心房间交通,左右平均动脉压相近。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心房" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "动脉压" } ] }, { "text": "右心室造影正侧位片可显示三尖瓣活动功能、大小及右室形态,以及是否存在右心房冠状动脉交通。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "右心室造影正侧位片" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "右室" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "右心房冠状动脉交通" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "右心房" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "如无右心室冠状动脉交通存在,则无右心室依赖性冠脉循环,反之,并不能说明存在右心室依赖性冠脉循环。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "右心室冠状动脉" }, { "start_idx": 2, "end_idx": 12, "type": "sym", "entity": "右心室冠状动脉交通存在" }, { "start_idx": 16, "end_idx": 25, "type": "sym", "entity": "右心室依赖性冠脉循环" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "右心室" }, { "start_idx": 37, "end_idx": 46, "type": "sym", "entity": "右心室依赖性冠脉循环" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "右心室" } ] }, { "text": "顺行的右心室依赖性冠脉循环球囊封闭术或经逆行升主动脉造影可以证实有无冠脉狭窄或中断,某些患者需用冠状动脉造影才能明确冠脉的走行(图9-9)。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "sym", "entity": "右心室依赖性冠脉循环" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "右心室" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "球囊封闭术" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "主动脉造影" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "冠脉狭窄或中断" }, { "start_idx": 48, "end_idx": 62, "type": "pro", "entity": "冠状动脉造影才能明确冠脉的走行" } ] }, { "text": "【治疗】静脉输注前列腺素E1、E2保持动脉导管持续开放。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "静脉输注" }, { "start_idx": 8, "end_idx": 16, "type": "dru", "entity": "前列腺素E1、E2" }, { "start_idx": 17, "end_idx": 26, "type": "pro", "entity": "保持动脉导管持续开放" } ] }, { "text": "代谢性酸中毒患儿及严重低氧血症的新生儿可静脉滴注碳酸氢盐,正压通气及肌肉松弛有助于使高危新生儿病情的稳定。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "低氧血症" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "碳酸氢盐" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "正压通气" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "肌肉松弛" } ] }, { "text": "球囊房隔成形术并非必须采用,对于准备建立右心室肺动脉持续通道的者不适用。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "球囊房隔成形术" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "右心室" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺动脉" } ] }, { "text": "太大的心房间通道将会减少右心室充盈及右心室减压后顺行的肺动脉血流量,故球囊房间隔成形术仅用于少见的严重的限制性房隔所致的心搏出量过低和严重的低氧血症。", "entities": [ { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "太大的心房间通道将会减少右心室充盈及右心室减压后顺行的肺动脉血流量" }, { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "心房间通道" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "右心室" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "右心室" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "肺动脉血" }, { "start_idx": 35, "end_idx": 42, "type": "pro", "entity": "球囊房间隔成形术" }, { "start_idx": 49, "end_idx": 65, "type": "sym", "entity": "严重的限制性房隔所致的心搏出量过低" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "房隔" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "心" }, { "start_idx": 67, "end_idx": 73, "type": "sym", "entity": "严重的低氧血症" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "低氧血症" } ] }, { "text": "冠状动脉异常者禁忌做右心室减压术。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "冠状动脉异常" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "右心室减压术" } ] }, { "text": "术前行心导管检查时,球囊房隔成形术应同时进行。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心导管检查" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "球囊房隔成形术" } ] }, { "text": "当右心室非常小或冠脉灌注依靠体循环右室压差维持时,可行单心室修补术;若有右心室冠脉通道,即使右心室依赖性冠脉循环被排除,由于三尖瓣闭锁,狭小的右心室可伴血栓形成,实行Fonton术前或术中均应考虑上述问题。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "右心室非常小" }, { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "右心室" }, { "start_idx": 8, "end_idx": 22, "type": "sym", "entity": "冠脉灌注依靠体循环右室压差维持" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "冠脉" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "右室" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "单心室修补术" }, { "start_idx": 36, "end_idx": 42, "type": "bod", "entity": "右心室冠脉通道" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "右心室" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "冠脉" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 68, "end_idx": 79, "type": "sym", "entity": "狭小的右心室可伴血栓形成" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "右心室" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "血" }, { "start_idx": 83, "end_idx": 89, "type": "pro", "entity": "Fonton术" } ] }, { "text": "当然,最好是能够建立两心室循环。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "两心室" } ] }, { "text": "两心室循环就是要建立右心室与肺动脉的持续血流,多项数据表明,右心室减压后右室腔和三尖瓣环可继续发育,这可能与右心室压力负荷减压后肌性肥厚逐渐消退有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "两心室" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "右心室" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺动脉" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "右心室" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "右室腔" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "三尖瓣环" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "右心室压力负荷" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "右心室" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "肌性肥厚" } ] }, { "text": "相应地,右心室顺应性和舒张期血流充盈改善,通过两心房间的右向左分流减少。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "右心室" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "两心房" } ] }, { "text": "相反,如果右心室流出道没有开放,患者仅做体肺动脉分流姑息手术,右心室似乎不会持续发育,两心室循环术也难成功。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "右心室流出道没有开放" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "右心室" }, { "start_idx": 20, "end_idx": 29, "type": "pro", "entity": "体肺动脉分流姑息手术" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "右心室似乎不会持续发育" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心室" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "两心室循环术" } ] }, { "text": "打开肺动脉瓣膜及跨瓣补片可扩大流出道,如右心室发育好,在打开右心室流出道之后,向前的肺动脉血流通常是足够的。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "肺动脉瓣膜" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "跨瓣补片" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "右心室" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "右心室" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肺动脉" } ] }, { "text": "动脉导管可在手术中被结扎或等待术后自然关闭。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "动脉导管" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "手术" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "结扎" } ] }, { "text": "少数患者若左侧导管未结扎,大量的分流可导致严重的心力衰竭和体循环减少,此时需行二期动脉导管结扎术。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "左侧导管" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "结扎" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "严重的心力衰竭" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "体循环减少" }, { "start_idx": 41, "end_idx": 47, "type": "pro", "entity": "动脉导管结扎术" } ] }, { "text": "如右心室较小,顺应性差,则需在术后数天到2~3周内连续静脉输入前列腺素E1、E2保持动脉导管持续开放,为右心室持续发育和顺应性改善争取时间。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "右心室较小,顺应性差" }, { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "右心室" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "静脉输入" }, { "start_idx": 31, "end_idx": 39, "type": "dru", "entity": "前列腺素E1、E2" }, { "start_idx": 40, "end_idx": 49, "type": "pro", "entity": "保持动脉导管持续开放" }, { "start_idx": 51, "end_idx": 64, "type": "pro", "entity": "为右心室持续发育和顺应性改善" } ] }, { "text": "连续静脉输入前列腺素E2~3周后,如肺循环仍依赖导管供血,便需行改良Blalock-Taussing分流术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "静脉输入" }, { "start_idx": 6, "end_idx": 13, "type": "dru", "entity": "前列腺素E2~3" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "导管" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" }, { "start_idx": 32, "end_idx": 52, "type": "pro", "entity": "改良Blalock-Taussing分流术" } ] }, { "text": "某些医院,体肺动脉分流术常同时作肺动脉瓣膜切开术。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "体肺动脉分流术" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "肺动脉瓣膜切开术" } ] }, { "text": "这时结扎动脉导管可能有利于肺动脉瓣膜切开术肺动脉充血。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "结扎动脉导管" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "肺动脉瓣膜切开术" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "肺动脉充血" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺动脉" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" } ] }, { "text": "Rosenthal等使用激光和射频消融进行肺动脉瓣打孔,用具有坚硬末端的冠脉导丝穿透闭锁的肺动脉瓣,并已成功应用于临床(图9-34)。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "Rosenthal" }, { "start_idx": 10, "end_idx": 26, "type": "pro", "entity": "使用激光和射频消融进行肺动脉瓣打孔" }, { "start_idx": 28, "end_idx": 48, "type": "pro", "entity": "用具有坚硬末端的冠脉导丝穿透闭锁的肺动脉瓣" } ] }, { "text": "瓣膜被打孔后再用一个较大的球囊撑开。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "瓣膜" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "打孔" }, { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "球囊" } ] }, { "text": "Alui等比较了以射频消融辅助的瓣膜切开术和球囊扩张术及Blalock-Taussing分流术术后情况表明,经导管治疗更有效、更安全。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "Alui" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "射频消融" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "瓣膜切开术" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "球囊扩张术" }, { "start_idx": 28, "end_idx": 46, "type": "pro", "entity": "Blalock-Taussing分流术" } ] }, { "text": "经导管治疗21例成功19例,1例院内死亡,2例出院后死亡。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "equ", "entity": "导管" } ] }, { "text": "16例存活者,12例成功建立了双心室循环,7例不需要持续治疗。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "建立了双心室循环" } ] }, { "text": "相反,手术4例患者,3例院内死亡,1例出院后4个月死亡,所有的存活者均需持续治疗,最终存活8例中,7例成功建立了双心室循环。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "双心室循环" } ] }, { "text": "图9-33右心室造影正位片A.和侧位片;B.显示:右心室发育不良伴肺动脉瓣闭锁不良伴肺动脉瓣闭锁,另一患者右心室造影正位片;C.显示:存在心室-冠脉窦隙交通不论如何建立右心室肺动脉持续血流,如有参与的肺动脉流出道狭窄狭窄均需进一步治疗。", "entities": [ { "start_idx": 5, "end_idx": 18, "type": "pro", "entity": "右心室造影正位片A.和侧位片" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "右心室发育不良" }, { "start_idx": 33, "end_idx": 40, "type": "dis", "entity": "肺动脉瓣闭锁不良" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "肺动脉瓣闭锁" }, { "start_idx": 53, "end_idx": 60, "type": "pro", "entity": "右心室造影正位片" }, { "start_idx": 67, "end_idx": 77, "type": "sym", "entity": "存在心室-冠脉窦隙交通" }, { "start_idx": 69, "end_idx": 75, "type": "bod", "entity": "心室-冠脉窦隙" }, { "start_idx": 82, "end_idx": 93, "type": "pro", "entity": "建立右心室肺动脉持续血流" }, { "start_idx": 100, "end_idx": 107, "type": "dis", "entity": "肺动脉流出道狭窄" } ] }, { "text": "此外,无论已进行了导管治疗还是手术治疗,如果患者存在严重的低氧血症,还需行体肺动脉分流术以增加肺动脉血流。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "导管治疗" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "手术治疗" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "低氧血症" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "体肺动脉分流术" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "肺动脉血流" } ] }, { "text": "重复进行球囊肺动脉瓣环切开术可有效缓解部分流出道的梗阻,或用补片扩大肺动脉以减轻梗阻。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "pro", "entity": "进行球囊肺动脉瓣环切开术" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "部分流出道的梗阻" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "部分流出道" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "补片" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "肺动脉" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "梗阻" } ] }, { "text": "这些患者中的一部分人将永久获得双心室循环,同时关闭心房间通道和体肺动脉分流。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "双心室循环" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "心房间通道" }, { "start_idx": 25, "end_idx": 36, "type": "sym", "entity": "心房间通道和体肺动脉分流" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "体肺动脉" } ] }, { "text": "心房间的交通可能会自动闭合也可能需更进一步的关闭治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "心房间" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "关闭治疗" } ] }, { "text": "当右心室的功能不足以维持肺循环时,用暂时的球囊封闭心房间的交通以保证心输出量和右心室压力,这一步是十分必要的。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "右心室" }, { "start_idx": 1, "end_idx": 14, "type": "sym", "entity": "右心室的功能不足以维持肺循环" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肺循环" }, { "start_idx": 17, "end_idx": 30, "type": "pro", "entity": "用暂时的球囊封闭心房间的交通" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "心" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "右心室" } ] }, { "text": "对于开放流出道后右心室很小的患者,可采用双向肺动脉连接,完成一个半心室的修补。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "开放流出道" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "右心室" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "右心室很小" }, { "start_idx": 18, "end_idx": 26, "type": "pro", "entity": "采用双向肺动脉连接" }, { "start_idx": 28, "end_idx": 37, "type": "pro", "entity": "完成一个半心室的修补" } ] }, { "text": "右心室依赖性冠状动脉循环者应进行右心室减压或血栓成形术。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "右心室依赖性冠状动脉循环" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "冠状动脉循环" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "进行右心室减压" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "血栓成形术" } ] }, { "text": "球囊房隔成形术也可考虑。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "球囊房隔成形术" } ] }, { "text": "初期手术可以是体肺动脉分流,以保证体循环的氧分压。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "手术" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "体肺动脉分流" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "体循环" } ] }, { "text": "对于大多数此类患者,单心房修补是最后的措施。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "单心房修补" } ] }, { "text": "然而右心室血流造成冠脉持续高压,将会导致心肌纤维化和心力衰竭。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "右心室血流" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "冠脉" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "冠脉持续高压" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "心肌纤维化" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心肌" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "Fontan分流术可为将来的心脏移植做好准备。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "Fontan分流术" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "心脏移植" } ] }, { "text": "少部分三尖瓣严重发育不良或Ebstein畸形患者,右心大而壁薄,压力低。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "三尖瓣严重发育不良" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "Ebstein畸形" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "右心大而壁薄,压力低" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "右心" } ] }, { "text": "暂时缓解方法包括体肺动脉分流术,改良Fontan分流术和心脏移植。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "暂时缓解方法" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "体肺动脉分流术" }, { "start_idx": 16, "end_idx": 26, "type": "pro", "entity": "改良Fontan分流术" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "心脏移植" } ] }, { "text": "先天性心脏外科医生协会前瞻性研究表明,在1987年到1990年的71例新生儿中,行手术瓣膜切开加或不加体、肺动脉分流术及跨环补片(RVOT),或仅做体、肺动脉分流术,1月存活率81%,4年为64%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 5, "end_idx": 6, "type": "dep", "entity": "外科" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "手术" }, { "start_idx": 43, "end_idx": 51, "type": "pro", "entity": "瓣膜切开加或不加体" }, { "start_idx": 53, "end_idx": 58, "type": "pro", "entity": "肺动脉分流术" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "跨环补片" }, { "start_idx": 65, "end_idx": 68, "type": "pro", "entity": "RVOT" }, { "start_idx": 73, "end_idx": 81, "type": "pro", "entity": "做体、肺动脉分流术" } ] }, { "text": "出生体重低和右心室依赖性冠脉分流是造成死亡的危险因素。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "体重低" }, { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "体重" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心室" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "右心室依赖性冠脉分流" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "冠脉" } ] }, { "text": "仅在最初治疗为瓣膜切开术或跨膜补片术时,较小的Z指数为危险因素,最初治疗为分流术时,则不构成危险因素。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "瓣膜切开术" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "跨膜补片术" }, { "start_idx": 37, "end_idx": 39, "type": "pro", "entity": "分流术" } ] }, { "text": "图9-34以球囊扩张术建立右心室和肺动脉持续分流A、B.用球囊将瓣膜孔逐渐扩大;C、D.行激光瓣膜打孔术和气囊瓣膜成形术后,重复右心室造影显示右心室和肺动脉干间已成功地连接近年来,Jahangire等从Boston报道了在术后生存方面的重大进展。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "球囊扩张术" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "右心室" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肺动脉" }, { "start_idx": 28, "end_idx": 38, "type": "pro", "entity": "用球囊将瓣膜孔逐渐扩大" }, { "start_idx": 45, "end_idx": 51, "type": "pro", "entity": "激光瓣膜打孔术" }, { "start_idx": 53, "end_idx": 59, "type": "pro", "entity": "气囊瓣膜成形术" }, { "start_idx": 64, "end_idx": 68, "type": "pro", "entity": "右心室造影" }, { "start_idx": 71, "end_idx": 79, "type": "bod", "entity": "右心室和肺动脉干间" } ] }, { "text": "他们将病人分层次,根据右心室大小和是否伴有右心室依赖性冠脉循环,接受单独的、部分的双心室或全部的双心室修补,全部存活率为98%,并积累了许多经导管治疗的经验。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "右心室" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "右心室" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "右心室依赖性冠脉循环" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "冠脉循环" }, { "start_idx": 34, "end_idx": 52, "type": "pro", "entity": "单独的、部分的双心室或全部的双心室修补" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "导管治疗" } ] }, { "text": "目前使用激光或射频消融辅助瓣膜切开术及球囊扩张术被认为是一种具有确切意义的治疗方法。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "激光" }, { "start_idx": 7, "end_idx": 17, "type": "pro", "entity": "射频消融辅助瓣膜切开术" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "球囊扩张术" } ] }, { "text": "第二章不同年龄儿童的生长发育第一节新生儿的生长发育新生儿(newborn)是胎儿的延续,为了做好优生优育,新生儿是儿科医师的重点对象。", "entities": [ { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "胎儿" }, { "start_idx": 57, "end_idx": 58, "type": "dep", "entity": "儿科" } ] }, { "text": "根据世界卫生组织的建议以及我国有关新生儿会议的规定,新生儿期的标准为:自出生脐带结扎开始到生后28天内。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "脐带结扎" } ] }, { "text": "一、新生儿分类根据新生儿的成熟度和胎龄及出生体重的关系,新生儿分类如下:(一)按胎龄分类胎龄(gestationalage)自母亲末次月经第1天算起,到分娩为止,一般为40周。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "出生体重" } ] }, { "text": "胎龄满37周(259天)到不满42周(293天)出生者称足月新生儿(fullterminfant),亦称成熟儿,其出生体重绝大多数大于2500g。", "entities": [ { "start_idx": 57, "end_idx": 60, "type": "ite", "entity": "出生体重" } ] }, { "text": "胎龄未满37周者,不论体重超过或不足2500g,均称早产儿(preterminfant),亦称未成熟儿(prematureinfant)。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "胎龄未满37周" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "体重" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "早产儿" }, { "start_idx": 30, "end_idx": 42, "type": "dis", "entity": "preterminfant" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "未成熟儿" }, { "start_idx": 52, "end_idx": 66, "type": "dis", "entity": "prematureinfant" } ] }, { "text": "满42周或以上出生者,不论体重多少,均称过期产儿(postterminfant)。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "满42周或以上出生" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "体重" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "过期产儿" }, { "start_idx": 25, "end_idx": 38, "type": "dis", "entity": "postterminfant" } ] }, { "text": "(二)按出生体重分类不论胎龄如何,凡出生体重(生后1小时内测量)在2500g以下者,均称低出生体重儿(lowbirthweightinfant)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "ite", "entity": "出生体重" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "出生体重" }, { "start_idx": 18, "end_idx": 39, "type": "sym", "entity": "出生体重(生后1小时内测量)在2500g以下" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "低出生体重儿" }, { "start_idx": 51, "end_idx": 70, "type": "dis", "entity": "lowbirthweightinfant" } ] }, { "text": "凡出生体重低于1500g者称极低出生体重儿(verylowweightinfant);凡出生体重小于该胎龄正常体重第10百分位数者,称小于胎龄儿(smallforgestationalageinfant,SGA)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "出生体重" }, { "start_idx": 1, "end_idx": 11, "type": "sym", "entity": "出生体重低于1500g" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "低出生体重儿" }, { "start_idx": 22, "end_idx": 40, "type": "dis", "entity": "verylowweightinfant" }, { "start_idx": 44, "end_idx": 47, "type": "ite", "entity": "出生体重" }, { "start_idx": 44, "end_idx": 63, "type": "sym", "entity": "出生体重小于该胎龄正常体重第10百分位数" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "小于胎龄儿" }, { "start_idx": 74, "end_idx": 100, "type": "dis", "entity": "mallforgestationalageinfant" }, { "start_idx": 102, "end_idx": 104, "type": "dis", "entity": "SGA" } ] }, { "text": "出生体重在同龄体重第10至90百分位之间者称适于胎龄儿(appropriateforgestationalageinfant,AGA)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "出生体重" }, { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "出生体重在同龄体重第10至90百分位之间" } ] }, { "text": "出生体重在第90百分位以上者称大于胎龄儿(largeforgestationalageinfant,LGA)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "出生体重" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "出生体重在第90百分位以上" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "大于胎龄儿" }, { "start_idx": 21, "end_idx": 48, "type": "dis", "entity": "largeforgestationalageinfant" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "LGA" } ] }, { "text": "这种分类法,将新生儿体重与胎龄联合反映出来,提示了其内在的涵义。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "体重" } ] }, { "text": "第十三章脑脓肿脑脓肿(brainabscess)是中枢神经系统局灶性化脓感染相对常见的类型之一,特别是社会经济状况欠佳的人群,仍然是一个严重问题。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 11, "end_idx": 22, "type": "dis", "entity": "brainabscess" }, { "start_idx": 25, "end_idx": 37, "type": "dis", "entity": "中枢神经系统局灶性化脓感染" } ] }, { "text": "脑脓肿中1/4发生于儿童,发病高峰为4~7岁。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "随着影像诊断技术的进步,临床对这类局灶感染的认识越来越深入。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "影像诊断技术" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "局灶感染" } ] }, { "text": "而诊断或治疗不当会导致严重的不良后果,甚至死亡。", "entities": [] }, { "text": "【病因】大多数微生物(如细菌、真菌或寄生虫)均可引起中枢神经系统局灶性化脓性感染。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "细菌" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "真菌" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "寄生虫" }, { "start_idx": 26, "end_idx": 39, "type": "dis", "entity": "中枢神经系统局灶性化脓性感染" } ] }, { "text": "多数脑脓肿为混合性感染。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "混合性感染" } ] }, { "text": "链球菌和革兰阴性细菌,例如枸橼酸杆菌、沙门菌、沙雷菌属、变形杆菌、肠菌属和脆弱类杆菌属等,是引起新生儿脑脓肿的常见细菌。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "链球菌" }, { "start_idx": 4, "end_idx": 9, "type": "mic", "entity": "革兰阴性细菌" }, { "start_idx": 13, "end_idx": 17, "type": "mic", "entity": "枸橼酸杆菌" }, { "start_idx": 19, "end_idx": 21, "type": "mic", "entity": "沙门菌" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "沙雷菌属" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 33, "end_idx": 35, "type": "mic", "entity": "肠菌属" }, { "start_idx": 37, "end_idx": 41, "type": "mic", "entity": "脆弱类杆菌" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "新生儿脑脓肿" }, { "start_idx": 57, "end_idx": 58, "type": "mic", "entity": "细菌" } ] }, { "text": "新生儿B组溶血性链球菌和枸橼酸杆菌脑膜炎时伴发脑脓肿的可能性非常高,故对于治疗不顺利的病例一定要常规进行CT、MRI或B超检查,以除外脑脓肿。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "dis", "entity": "新生儿B组溶血性链球菌和枸橼酸杆菌脑膜炎" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "CT" }, { "start_idx": 55, "end_idx": 57, "type": "pro", "entity": "MRI" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "B超" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "不同部位和类型的脑脓肿病原体有所不同。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "头颅创伤引起的脑脓肿常见的病原是金黄色葡萄球菌和链球菌。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "头颅创伤" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 16, "end_idx": 22, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "链球菌" } ] }, { "text": "中耳乳突炎并发的颞叶脑脓肿,以及隐源性脑内小脓肿(直径在1~1.5cm以下,常见于顶叶),常见病原包括厌氧菌、需氧链球菌和肠杆菌。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "中耳乳突炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "颞叶脑脓肿" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "隐源性脑内小脓肿" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "顶叶" }, { "start_idx": 51, "end_idx": 53, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 55, "end_idx": 59, "type": "mic", "entity": "需氧链球菌" }, { "start_idx": 61, "end_idx": 63, "type": "mic", "entity": "肠杆菌" } ] }, { "text": "先天性青紫型心脏病、心内膜炎、化脓性血栓性静脉炎、败血症以及骨髓炎等血行播散引起的脑脓肿大多沿大脑中动脉分布,致病菌包括微需氧链球菌、厌氧菌及金黄色葡萄球菌等。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "先天性青紫型心脏病" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "化脓性血栓性静脉炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "败血症" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "骨髓炎" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "血" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "大脑中动脉" }, { "start_idx": 60, "end_idx": 65, "type": "mic", "entity": "微需氧链球菌" }, { "start_idx": 67, "end_idx": 69, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 71, "end_idx": 77, "type": "mic", "entity": "金黄色葡萄球菌" } ] }, { "text": "【发病机制】脑脓肿的形成按其机制,可分为血行播散、邻近感染灶蔓延和隐源性感染几类。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "血" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "邻近感染灶" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "隐源性感染" } ] }, { "text": "(一)血行播散是儿童脑脓肿的常见原因。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "儿童脑脓肿" } ] }, { "text": "心、肺及皮肤等部位的感染灶均可通过血循环波及脑部。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "心" }, { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "心、肺及皮肤等部位的感染灶均可通过血循环波及脑部" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肺" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血循环" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "脑部" } ] }, { "text": "青紫型先天性心脏病常伴血液浓缩,易发生血栓或脓栓,是小儿血源性脑脓肿的最常见诱因,尤以法洛四联症引起的多见。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "青紫型先天性心脏病" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "血液浓缩" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血液" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "血栓" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "脓栓" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "小儿血源性脑脓肿" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "感染性心内膜炎患儿也易于发生血源性脑脓肿。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "血源性脑脓肿" } ] }, { "text": "慢性化脓性肺部疾病,如肺脓肿、脓胸和支气管扩张症也是重要的诱因。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "慢性化脓性肺部疾病" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肺脓肿" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "脓胸" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "支气管扩张症" } ] }, { "text": "菌血症的严重程度和持续时间是是否发生脑脓肿的重要因素。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "菌血症" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "脑脓肿可作为外周化脓性感染(如骨髓炎、牙齿、皮肤及消化道等)引起的菌血症或败血症的转移灶出现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "外周化脓性感染" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "骨髓炎" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "牙齿" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "皮肤" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "消化道" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "菌血症" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "败血症" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "转移灶" } ] }, { "text": "隐源性脑脓肿找不到原发感染灶,实际上也多属于血源性。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "隐源性脑脓肿" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "原发感染灶" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "血" } ] }, { "text": "(二)邻近组织感染灶的直接蔓延邻近感染灶(常见如中耳、鼻窦、眼眶和头面皮肤)的蔓延是脑脓肿的第二个常见诱因。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "邻近组织感染灶" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "邻近组织" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "邻近感染灶" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "中耳" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "鼻窦" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "眼眶" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "头面皮肤" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "中耳、乳突炎和鼻窦感染是邻近蔓延的最常见感染部位,以耳源性脑脓肿尤为多见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "中耳、乳突炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "鼻窦感染" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "感染" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "耳源性脑脓肿" } ] }, { "text": "大多数病例的邻近感染蔓延是通过早已存在的解剖通道蔓延,但也可通过血栓性静脉炎或骨髓炎扩散。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "邻近感染蔓延" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "解剖" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "血栓性静脉炎" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "骨髓炎" } ] }, { "text": "细菌性脑膜炎患者在发生严重的组织损伤时也可能导致脑脓肿的形成。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "细菌性脑膜炎" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "严重的组织损伤" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "脑部手术或脑室内引流偶可并发脑脓肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "脑部手术" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "脑室内引流" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "头颅穿通伤,因骨碎片或异物进入脑部疾病可引起局部感染疾病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "头颅穿通伤" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "骨碎片或异物进入脑部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脑部" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "局部感染疾病" } ] }, { "text": "(三)隐源性感染实质上是血源性脑脓肿的隐匿型,原发感染灶不明显,机体抵抗力弱时,脑实质内隐伏的细菌逐渐发展为脑脓肿。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "隐源性感染" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "血源性脑脓肿" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "原发感染灶" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "机体抵抗力弱" }, { "start_idx": 40, "end_idx": 48, "type": "mic", "entity": "脑实质内隐伏的细菌" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "成人脑脓肿以邻近组织感染灶的直接蔓延为主,尤以耳源性最多见,约占2/3。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "成人脑脓肿" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "邻近组织感染灶" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "邻近组织" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "耳" } ] }, { "text": "继发于慢性化脓性中耳炎及乳突炎。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "慢性化脓性中耳炎" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "乳突炎" } ] }, { "text": "脓肿多见于额叶前部或底部。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "脓肿" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "额叶前部" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "底部" } ] }, { "text": "血源性脑脓肿约占脑脓肿的1/4。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "血源性脑脓肿" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "多由于身体其他部位感染,细菌栓子经动脉血行播散到脑内而形成脑脓肿。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "感染" }, { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "细菌栓子" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "动脉" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "脑内" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "脑脓肿多分布于大脑中动脉供应区、额叶及顶叶,有的为多发性小脓肿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 7, "end_idx": 14, "type": "bod", "entity": "大脑中动脉供应区" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "额叶" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "顶叶" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "多发性小脓肿" } ] }, { "text": "外伤也是成人脑脓肿常见原因。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "外伤" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "成人脑脓肿" } ] }, { "text": "多继发于开放性脑损伤。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "开放性脑损伤" } ] }, { "text": "在婴幼儿由于对感染的局限能力差,脓肿常较大而缺乏完整的包膜。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "对感染的局限能力差" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "感染" }, { "start_idx": 16, "end_idx": 28, "type": "sym", "entity": "脓肿常较大而缺乏完整的包膜" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "包膜" } ] }, { "text": "【临床表现】疾病脑脓肿临床症状受许多因素影响。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "通常额叶或顶叶脓肿可长时间无症状,只有在脓肿增大产生明显占位效应或波及关键脑功能区(如感觉及运动皮质)时才会出现症状和体征。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "额叶或顶叶脓肿" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "脓肿增大" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "明显占位效应" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "脑功能区" }, { "start_idx": 43, "end_idx": 49, "type": "bod", "entity": "感觉及运动皮质" } ] }, { "text": "致病菌的致病力和宿主机体的免疫状态也可影响脑脓肿临床表现的急缓和轻重。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "致病菌" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "脑内小脓肿,即直径在1~1.5cm以下的脑脓肿,常见于顶叶,临床表现大多轻微。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "脑内小脓肿" }, { "start_idx": 7, "end_idx": 22, "type": "sym", "entity": "直径在1~1.5cm以下的脑脓肿" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "脑" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "顶叶" } ] }, { "text": "多数病例以局灶性感觉或运动性癫痫发作起病,个别可有颅内压增高表现,局灶性体征少见。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "运动性癫痫" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "【辅助检查】(一)常规检查血常规检查对中枢神经系统局灶性化脓性感染的诊断通常无特殊意义。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "血常规检查" }, { "start_idx": 19, "end_idx": 32, "type": "dis", "entity": "中枢神经系统局灶性化脓性感染" } ] }, { "text": "大约50%的脑脓肿患儿外周血白细胞轻度增多,伴发脑膜炎的患者白细胞明显增高(>20×109/L),可有核左移(杆状核超过7%)。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 11, "end_idx": 16, "type": "ite", "entity": "外周血白细胞" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "外周血白细胞轻度增多" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 30, "end_idx": 53, "type": "sym", "entity": "白细胞明显增高(>20×109/L)" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "白细胞" }, { "start_idx": 57, "end_idx": 59, "type": "sym", "entity": "核左移" }, { "start_idx": 57, "end_idx": 57, "type": "ite", "entity": "核" }, { "start_idx": 61, "end_idx": 67, "type": "sym", "entity": "杆状核超过7%" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "杆状核" } ] }, { "text": "血培养阳性率较低(约10%),但如阳性则对诊断有特异性意义。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "血培养" } ] }, { "text": "(二)脑脊液检查稳定期脑脓肿脑脊液多无明显异常,可有蛋白轻度升高,白细胞稍高或正常,糖轻度降低,压力多数升高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "稳定期脑脓肿" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "脑脊液" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "蛋白轻度升高" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "蛋白" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "白细胞稍高" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "白细胞" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "糖轻度降低" }, { "start_idx": 42, "end_idx": 42, "type": "ite", "entity": "糖" }, { "start_idx": 48, "end_idx": 53, "type": "sym", "entity": "压力多数升高" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "压力" } ] }, { "text": "在病程早期,特别是并发脑膜炎症明显者,脑脊液可有显著异常。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "脑膜炎症" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "脑脊液可有显著异常" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "脑脊液" } ] }, { "text": "由于脑脓肿大多并发颅内压增高,腰椎穿刺引起的并发症明显增加;因此不应将腰椎穿刺列为脑脓肿的常规检查。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "如临床怀疑脑脓肿,应首先行神经影像学检查确诊。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "神经影像学检查" } ] }, { "text": "在除外颅内压增高之前,禁忌腰椎穿刺。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "腰椎穿刺" } ] }, { "text": "脑脊液培养阳性率不高,在同时存在脑膜炎或脑脓肿破溃至蛛网膜下腔时培养的阳性率增高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "脑脊液培养" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "破溃至蛛网膜下腔" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "蛛网膜下腔" } ] }, { "text": "(三)神经影像学检查CT和MRI是诊断脑脓肿的首选检查。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "神经影像学检查" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "CT" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "MRI" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "随着CT和MRI的应用,脑脓肿的死亡率下降了90%。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "CT" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "MRI" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "一般脑脓肿的典型CT表现是:①脓腔呈圆形或类圆形低密度区;②脓肿壁可呈等密度或稍高密度环状影,增强扫描呈环状强化,壁厚一般5~6mm;③脓肿周围脑组织水肿,呈广泛低密度区,多表现为不规则指状或树叶状;④脓肿较大者见占位效应。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "CT" }, { "start_idx": 15, "end_idx": 27, "type": "sym", "entity": "脓腔呈圆形或类圆形低密度区" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脓腔" }, { "start_idx": 30, "end_idx": 45, "type": "sym", "entity": "脓肿壁可呈等密度或稍高密度环状影" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "脓肿壁" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "增强扫描呈环状强化" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "增强扫描" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "壁" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "壁厚一般5~6mm" }, { "start_idx": 68, "end_idx": 76, "type": "sym", "entity": "脓肿周围脑组织水肿" }, { "start_idx": 68, "end_idx": 74, "type": "bod", "entity": "脓肿周围脑组织" }, { "start_idx": 78, "end_idx": 84, "type": "sym", "entity": "呈广泛低密度区" }, { "start_idx": 87, "end_idx": 98, "type": "sym", "entity": "表现为不规则指状或树叶状" }, { "start_idx": 101, "end_idx": 104, "type": "sym", "entity": "脓肿较大" }, { "start_idx": 107, "end_idx": 110, "type": "sym", "entity": "占位效应" } ] }, { "text": "脓肿直径一般为2~5cm。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "脓肿" } ] }, { "text": "值得注意的是尽管上述表现可高度怀疑脑脓肿,但其他病变(如肿瘤、肉芽肿,吸收中的血肿或梗死)也可有类似的CT表现。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "肿瘤" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "肉芽肿" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "吸收中的血肿" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "血" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "梗死" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "CT" } ] }, { "text": "此外,CT异常一般在出现临床症状后数天表现,病初CT正常并不能排除脑脓肿,对高度怀疑者应复查。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "CT" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "CT异常" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "CT" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "MRI比CT更敏感,更特异,病变可更早被检出,有些CT检测不到的微小病灶MRI亦可清晰显示,并可准确地鉴别脑脊液和脓液,可协助判断脓肿破裂。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "MRI" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "CT" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "CT" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "微小病灶" }, { "start_idx": 36, "end_idx": 38, "type": "pro", "entity": "MRI" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "脑脊液" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "脓液" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "脓肿破裂" } ] }, { "text": "【诊断与鉴别诊断】如患儿有外周化脓性病灶,特别是中耳炎、乳突炎、皮肤感染或败血症,或有青紫型先天性心脏病或感染性心内膜炎,或有开放性颅脑损伤等病史,一旦出现中枢神经系统症候,即应考虑脑脓肿的可能性,及时进行CT或MRI检查可明确诊断。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "外周化脓性病灶" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "中耳炎" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "乳突炎" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "败血症" }, { "start_idx": 43, "end_idx": 51, "type": "dis", "entity": "青紫型先天性心脏病" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 63, "end_idx": 69, "type": "dis", "entity": "开放性颅脑损伤" }, { "start_idx": 78, "end_idx": 83, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 78, "end_idx": 85, "type": "sym", "entity": "中枢神经系统症候" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 103, "end_idx": 110, "type": "pro", "entity": "CT或MRI检查" } ] }, { "text": "脑内小脓肿的诊断要点是:①隐匿起病,多无明确感染史;②无明显感染中毒症状;③以局灶性癫痫发作为首发及主要症状,常无明显局灶体征;④脑脊液化验多属正常,或仅有压力或蛋白轻度升高;⑤CT平扫脓腔显示不清,脓腔与周围脑水肿界限模糊,表现为2~5cm大小的不规则低密度区,CT值5~27H。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "脑内小脓肿" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "感染" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "感染" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "中毒" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "局灶性癫痫" }, { "start_idx": 65, "end_idx": 69, "type": "ite", "entity": "脑脊液化验" }, { "start_idx": 78, "end_idx": 86, "type": "sym", "entity": "压力或蛋白轻度升高" }, { "start_idx": 78, "end_idx": 79, "type": "ite", "entity": "压力" }, { "start_idx": 81, "end_idx": 82, "type": "ite", "entity": "蛋白" }, { "start_idx": 89, "end_idx": 92, "type": "pro", "entity": "CT平扫" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "脓腔" }, { "start_idx": 100, "end_idx": 111, "type": "sym", "entity": "脓腔与周围脑水肿界限模糊" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "脓腔" }, { "start_idx": 105, "end_idx": 105, "type": "bod", "entity": "脑" }, { "start_idx": 116, "end_idx": 130, "type": "sym", "entity": "2~5cm大小的不规则低密度区" }, { "start_idx": 132, "end_idx": 133, "type": "equ", "entity": "CT" } ] }, { "text": "增强扫描后呈团块状强化,少数呈环状,强化影直径<1.5cm,多数居于低密度区周边;⑥多数位于幕上近皮层区,以顶叶最为多见,大多为单发。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "增强扫描后呈团块状强化" }, { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "增强扫描" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "呈环状" }, { "start_idx": 18, "end_idx": 28, "type": "sym", "entity": "强化影直径<1.5cm" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "居于低密度区周边" }, { "start_idx": 44, "end_idx": 65, "type": "sym", "entity": "位于幕上近皮层区,以顶叶最为多见,大多为单发" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "幕上近皮层区" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "顶叶" } ] }, { "text": "需要与脑脓肿鉴别的疾病很多,包括感染性和非感染性两类疾病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 16, "end_idx": 27, "type": "dis", "entity": "感染性和非感染性两类疾病" } ] }, { "text": "结核性脑膜炎、结核瘤或结核性脓肿。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "结核瘤" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "结核性脓肿" } ] }, { "text": "偶见结核瘤液化形成脓肿,此时很难与脑脓肿鉴别。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "结核瘤" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "液化" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "脓肿" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "应与脑脓肿鉴别的非感染性疾病包括脑血管意外、静脉窦血栓以及中枢神经系统肿瘤等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "非感染性疾病" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "脑血管意外" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "静脉窦血栓" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "中枢神经系统肿瘤" } ] }, { "text": "【治疗】脑脓肿的治疗包括内科或外科疗法,确诊后应尽快决定治疗方案。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "内科或外科疗法" } ] }, { "text": "(一)内科治疗单纯内科治疗的适应证包括:①病情稳定,无严重颅压增高的体征;②脓肿大小在2~3cm以内;③病程在2周以内,CT或MRI检查提示脓肿包膜尚未形成;④多发性脓肿;⑤有手术禁忌证,如脓肿深在或位于危险区,或患儿身体状况不适合手术等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "内科治疗" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "单纯内科治疗" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "严重颅压增高" }, { "start_idx": 38, "end_idx": 49, "type": "sym", "entity": "脓肿大小在2~3cm以内" }, { "start_idx": 60, "end_idx": 67, "type": "pro", "entity": "CT或MRI检查" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "脓肿包膜" }, { "start_idx": 80, "end_idx": 84, "type": "sym", "entity": "多发性脓肿" }, { "start_idx": 88, "end_idx": 89, "type": "pro", "entity": "手术" }, { "start_idx": 95, "end_idx": 104, "type": "sym", "entity": "脓肿深在或位于危险区" }, { "start_idx": 116, "end_idx": 117, "type": "pro", "entity": "手术" } ] }, { "text": "内科治疗系指以抗生素应用为核心,包括对症治疗、支持治疗和病情监护等措施在内的综合性疗法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "内科治疗" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "抗生素应用" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "对症治疗" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "支持治疗" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "病情监护" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "综合性疗法" } ] }, { "text": "治疗原则与其他类型的中枢神经系统感染相同,以下重点介绍抗生素的应用。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "中枢神经系统感染" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "抗生素" } ] }, { "text": "治疗脑脓肿的抗生素选择主要依据可能的致病菌及其对所采用的抗生素是否敏感,以及抗生素在感染部位是否能达到有效浓度等因素。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "抗生素" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "致病菌" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "抗生素" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "抗生素" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "感染" } ] }, { "text": "既往青霉素(或氨苄西林)加氯霉素或甲硝唑常用于治疗与青紫型先天性心脏病、中耳炎及鼻窦炎有关的脑脓肿。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "青霉素" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "加氯霉素" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "青紫型先天性心脏病" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "中耳炎" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "在新生儿,由于肺炎球菌感染很少见,建议首选头孢三嗪加氨苄西林。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "肺炎球菌感染" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "头孢三嗪" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "如为单发性脓肿,经外科完全切除或引流效果较好,大多数病例经3~4周治疗即可。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "单发性脓肿" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "外科完全切除" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "引流" } ] }, { "text": "(二)外科治疗对不符合上述单纯内科治疗标准的患者应进行外科治疗以取得尽可能好的结果。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "外科治疗" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "单纯内科治疗" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "外科治疗" } ] }, { "text": "外科治疗常用两种方法:脑立体定向穿刺抽脓或脓肿切除。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "外科治疗" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "脑立体定向穿刺抽脓" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "脓肿切除" } ] }, { "text": "在CT引导下穿刺抽脓一般安全、准确、快速且有效,并发症和死亡率低。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "pro", "entity": "CT引导下穿刺抽脓" } ] }, { "text": "引流脓液病原学检查可快速明确致病菌并进行药敏试验,从而避免经验选用抗生素的潜在危险。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "引流脓液病原学检查" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "致病菌" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "药敏试验" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "抗生素" } ] }, { "text": "缺点是某些病例需要反复吸脓,这样会造成更多的组织损伤和出血。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "反复吸脓" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "组织损伤" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "出血" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "血" } ] }, { "text": "手术切除脑脓肿的适应证如下:①真菌或蠕虫脓肿,病人对药物治疗无效;②后颅窝脓肿;③多腔性脓肿;④穿刺吸脓效果不佳。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "手术切除" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "真菌或蠕虫脓肿" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "药物治疗" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "后颅窝脓肿" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "多腔性脓肿" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "穿刺吸脓" } ] }, { "text": "虽然脑脓肿最经典的治疗是单纯的抗生素治疗或外科手术切除,但临床有很多选择,应根据脓肿的部位、大小、分期、囊壁厚度及全身情况等综合考虑,确定最适宜的治疗方案。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "外科手术切除" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "脓肿" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "囊壁" } ] }, { "text": "在外科治疗方面,多数专家认为手术切除治疗较穿刺和引流术的平均死亡率和并发症(尤其是继发性癫痫)明显降低。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "外科治疗" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "手术切除治疗" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "穿刺" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "引流术" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "继发性癫痫" } ] }, { "text": "对于一般状况良好,能安全地度过脑脓肿的脑炎期、化脓期和包膜形成早期者,主张行显微外科切除术,包括那些位于功能区和多发的脑脓肿患儿。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "脑炎" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "化脓" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "包膜" }, { "start_idx": 38, "end_idx": 44, "type": "pro", "entity": "显微外科切除术" }, { "start_idx": 50, "end_idx": 61, "type": "dis", "entity": "位于功能区和多发的脑脓肿" } ] }, { "text": "【预后】由于早期诊断和治疗水平的提高,儿童脑脓肿的死亡率由既往的30%下降至5%~15%。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "儿童脑脓肿" } ] }, { "text": "大约2/3的脑脓肿患者可完全恢复而不留后遗症,存活者中10%~30%并发癫痫发作。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "癫痫发作" } ] }, { "text": "其他神经后遗症包括偏瘫、脑神经麻痹(5%~10%)、脑积水、智力或行为异常等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "神经后遗症" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "偏瘫" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "脑神经麻痹" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "脑积水" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "智力或行为异常" } ] }, { "text": "第三节酸碱平衡正常儿童血pH与成人一样,均为7.4,但其范围稍宽,即7.35~7.45。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "血pH" } ] }, { "text": "人体调节pH在较稳定的水平取决于两个机制:①理化或缓冲机制,作为保护过多的酸或碱丢失;②生理机制,主要为肾脏和肺直接作用于缓冲机制,使其非常有效地发挥作用。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "pH" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "酸" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "碱" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "肾脏" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "肺" } ] }, { "text": "血液及其他体液的缓冲系统主要包括两个方面:碳酸、碳酸氢盐系统和非碳酸氢盐系统。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "体液" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "缓冲系统" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "碳酸、碳酸氢盐系统" }, { "start_idx": 31, "end_idx": 37, "type": "bod", "entity": "非碳酸氢盐系统" } ] }, { "text": "在血液非碳酸氢盐系统,主要为血红蛋白、有机及无机磷,血浆蛋白占较少部分。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "bod", "entity": "血液非碳酸氢盐系统" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "有机及无机磷" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "血浆蛋白" } ] }, { "text": "在间质液几乎无非碳酸氢盐缓冲系统。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "间质液" }, { "start_idx": 7, "end_idx": 15, "type": "bod", "entity": "非碳酸氢盐缓冲系统" } ] }, { "text": "在细胞内液,碳酸、碳酸氢盐及非碳酸盐缓冲系统均起作用,后者主要由有机磷蛋白及其他成分组成。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "细胞内液" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "碳酸" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "非碳酸盐缓冲系统" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "有机磷蛋白" } ] }, { "text": "酸碱平衡是指正常体液保持一定的H+浓度。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "体液" }, { "start_idx": 15, "end_idx": 24, "type": "ite", "entity": "H+浓度" } ] }, { "text": "机体在代谢过程中不断产生酸性和碱性物质,必须通过体内缓冲系统以及肺、肾的调节作用使体液pH维持在7.40(7.35~7.45),以保证机体的正常代谢和生理功能。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "酸性和碱性物质" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "体内缓冲系统" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "肺" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肾" }, { "start_idx": 41, "end_idx": 44, "type": "ite", "entity": "体液pH" } ] }, { "text": "细胞外液的pH主要取决于血液中最重要的一对缓冲物质,即和H2CO3两者含量的比值。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "细胞外液" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "pH" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血液" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "缓冲物质" }, { "start_idx": 28, "end_idx": 35, "type": "bod", "entity": "H2" }, { "start_idx": 36, "end_idx": 44, "type": "bod", "entity": "CO3" } ] }, { "text": "正常和H2CO3比值保持在20∶1。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "H2" }, { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "CO3" } ] }, { "text": "当某种因素促使两者比值发生改变或体内代偿功能不全时,体液pH即发生改变,超出7.35~7.45的正常范围,出现酸碱平衡紊乱。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "两者比值发生改变" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "体内代偿功能不全" }, { "start_idx": 26, "end_idx": 29, "type": "ite", "entity": "体液pH" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "酸碱平衡紊乱" } ] }, { "text": "肺通过排出或保留CO2来调节血液中碳酸的浓度,肾负责排酸保碱,肺的调节作用较肾为快,但两者的功能均有一定限度。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 16, "type": "bod", "entity": "CO2" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "血液" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "碳酸" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肾" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "酸" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "碱" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "肺" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "肾" } ] }, { "text": "当肺呼吸功能障碍使CO2排出过少或过多、使血浆中H2CO3的量增加或减少所引起的酸碱平衡紊乱,称为呼吸性酸中毒或碱中毒。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "肺呼吸功能障碍" }, { "start_idx": 9, "end_idx": 17, "type": "bod", "entity": "CO2" }, { "start_idx": 9, "end_idx": 24, "type": "sym", "entity": "CO2排出过少或过多" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血浆" }, { "start_idx": 30, "end_idx": 53, "type": "sym", "entity": "H2CO3的量增加或减少" }, { "start_idx": 30, "end_idx": 37, "type": "bod", "entity": "H2" }, { "start_idx": 38, "end_idx": 46, "type": "bod", "entity": "CO3" }, { "start_idx": 58, "end_idx": 63, "type": "dis", "entity": "酸碱平衡紊乱" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "碱中毒" } ] }, { "text": "若因代谢紊乱使血浆中H2CO3的量增加或减少而引起的酸碱平衡紊乱,则称为代谢性酸中毒或碱中毒。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "代谢紊乱" }, { "start_idx": 7, "end_idx": 33, "type": "sym", "entity": "血浆中H2CO3的量增加或减少" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血浆" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "H2" }, { "start_idx": 18, "end_idx": 26, "type": "bod", "entity": "CO3" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "酸碱平衡紊乱" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "碱中毒" } ] }, { "text": "出现酸碱平衡紊乱后,机体可通过肺、肾调节使/H2CO3的比值维持在20∶1,即pH维持在正常范围内,称为代偿性代谢性(或呼吸性)酸中毒(或碱中毒);如果/H2CO3的比值不能维持在20∶1,即pH低于或高于正常范围,则称为失代偿性代谢性(或呼吸性)酸中毒(或碱中毒)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "酸碱平衡紊乱" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肺" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肾" }, { "start_idx": 22, "end_idx": 41, "type": "ite", "entity": "H2CO3的比值" }, { "start_idx": 51, "end_idx": 52, "type": "ite", "entity": "pH" }, { "start_idx": 64, "end_idx": 84, "type": "dis", "entity": "代偿性代谢性(或呼吸性)酸中毒(或碱中毒)" }, { "start_idx": 88, "end_idx": 117, "type": "sym", "entity": "/H2CO3的比值不能维持在20∶1" }, { "start_idx": 89, "end_idx": 108, "type": "ite", "entity": "H2CO3的比值" }, { "start_idx": 120, "end_idx": 130, "type": "sym", "entity": "pH低于或高于正常范围" }, { "start_idx": 120, "end_idx": 121, "type": "ite", "entity": "pH" }, { "start_idx": 135, "end_idx": 155, "type": "dis", "entity": "失代偿性代谢性(或呼吸性)酸中毒(或碱中毒" } ] }, { "text": "常见的酸碱失衡为单纯型(呼吸性酸中毒、呼吸性碱中毒、代谢性酸中毒、代谢性碱中毒);有时亦出现混合型。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "酸碱失衡" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "呼吸性碱中毒" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "代谢性碱中毒" } ] }, { "text": "一、代谢性酸中毒所有代谢性酸中毒(metabolicacidosis)都有下列两种可能之一:①细胞外液酸产生过多细胞外液碳酸氢盐的丢失。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 17, "end_idx": 33, "type": "dis", "entity": "metabolicacidosis" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "细胞外液" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "细胞外液酸产生过多" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "酸" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "细胞外液" }, { "start_idx": 56, "end_idx": 66, "type": "sym", "entity": "细胞外液碳酸氢盐的丢失" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "碳酸氢盐" } ] }, { "text": "前者常见有酮症酸中毒,肾衰时磷酸、硫酸及组织低氧时产生的乳酸增多。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "酮症酸中毒" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肾衰" }, { "start_idx": 14, "end_idx": 31, "type": "sym", "entity": "磷酸、硫酸及组织低氧时产生的乳酸增多" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "磷酸" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "硫酸" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "组织低氧时产生的乳酸" } ] }, { "text": "后者代谢性酸中毒是由于碳酸氢盐从肾脏或小肠液的丢失,常发生于腹泻、小肠瘘管的引流等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 11, "end_idx": 24, "type": "sym", "entity": "碳酸氢盐从肾脏或小肠液的丢失" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肾脏" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "小肠液" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "腹泻" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "腹" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "小肠瘘管的引流" } ] }, { "text": "腹泻大便常呈酸性,这是由于小肠液在肠道经细菌发酵作用,产生有机酸,后者与碱性肠液中和,使最终大便仍以酸性为主。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹泻" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "腹" }, { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "大便常呈酸性" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "大便" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "小肠液" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "肠道" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "细菌" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "有机酸" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "碱性肠液" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "大便" }, { "start_idx": 46, "end_idx": 53, "type": "sym", "entity": "大便仍以酸性为主" } ] }, { "text": "在霍乱病人,由于短期内大量肠液产生,大便呈碱性。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "霍乱" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "短期内大量肠液产生" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肠液" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "大便" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "大便呈碱性" } ] }, { "text": "代谢性酸中毒时主要的缓冲是碳酸氢盐,也可通过呼吸代偿使PaCO2降低,但通过呼吸代偿很少能使血液pH完全达到正常。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 27, "end_idx": 39, "type": "sym", "entity": "PaCO2降低" }, { "start_idx": 27, "end_idx": 37, "type": "bod", "entity": "PaCO2" }, { "start_idx": 52, "end_idx": 55, "type": "ite", "entity": "血液pH" } ] }, { "text": "呼吸代偿只是改善pH的下降(部分代偿),完全代偿取决于肾脏酸化尿液,使血碳酸氢盐水平达到正常,再通过呼吸的重新调节,最终才能使血酸碱平衡达到正常。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "pH" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肾脏" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "尿液" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "血碳酸氢盐" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "血" } ] }, { "text": "在诊断单纯或混合性酸中毒时阴离子间隙(aniongap,AG)常有很大的帮助。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "单纯或混合性酸中毒" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 19, "end_idx": 26, "type": "ite", "entity": "aniongap" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "AG" } ] }, { "text": "测得的阳离子为钠和钾,可测得的阴离子为氯和碳酸氢根。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "阳离子" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "钠" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "钾" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "阴离子" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "氯" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "碳酸氢根" } ] }, { "text": "因钾离子浓度相对较低,在计算阴离子间隙时常忽略不计。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "ite", "entity": "钾离子浓度" }, { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "阴离子间隙" } ] }, { "text": "阴离子间隙=Na+-(Cl-+),正常为12mmol/L(范围:8~16mmol/L)由于阴离子蛋白、硫酸根和其他常规不测定的阴离子的存在,正常阴离子间隙为(12±4)mmol/L。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "Na+" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "Cl-" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "阴离子蛋白" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "硫酸根" }, { "start_idx": 69, "end_idx": 77, "type": "bod", "entity": "常规不测定的阴离子" }, { "start_idx": 84, "end_idx": 88, "type": "ite", "entity": "阴离子间隙" } ] }, { "text": "AG的增加几乎总是由于代谢性酸中毒所致。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "AG的增加" }, { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "AG" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "但是,不是所有的代谢性酸中毒均有AG增高。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "AG增高" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "AG" } ] }, { "text": "AG增高见于代谢性酸中毒伴有常规不测定的阴离子如乳酸、酮体等增加。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "AG增高" }, { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "AG" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "常规不测定的阴离子" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "乳酸" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "酮体" } ] }, { "text": "代谢性酸中毒不伴有常规不测定的阴离子增高时AG不增高,称为高氯性代谢性酸中毒。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "常规不测定的阴离子增高" }, { "start_idx": 9, "end_idx": 17, "type": "bod", "entity": "常规不测定的阴离子" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "AG不增高" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "AG" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "高氯性代谢性酸中毒" } ] }, { "text": "当代谢性酸中毒由肾小管酸中毒或大便碳酸氢盐丢失引起时,阴离子间隙可以正常。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "肾小管酸中毒" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "大便" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "大便碳酸氢盐丢失" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 27, "end_idx": 31, "type": "ite", "entity": "阴离子间隙" } ] }, { "text": "当血浆碳酸氢根水平降低时,氯离子作为伴随钠在肾小管重吸收的主要阴离子,其吸收率增加了。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "血浆碳酸氢根水平降低" }, { "start_idx": 1, "end_idx": 6, "type": "ite", "entity": "血浆碳酸氢根" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "氯离子" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "钠" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肾小管" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "阴离子" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "吸收率增加" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "吸收率" } ] }, { "text": "由于酸中毒时碳酸氢根浓度降低、血浆氯增高,使总阴离子保持不变。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "酸中毒" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "碳酸氢根浓度降低" }, { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "碳酸氢根" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "血浆氯增高" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "血浆氯" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "总阴离子保持不变" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "总阴离子" } ] }, { "text": "肾功能衰竭时血磷、硫等有机阴离子的增加;糖尿病人的酮症酸中毒、乳酸性酸中毒、高血糖非酮症性昏迷、未定名的有机酸血症、氨代谢障碍等均可使阴离子间隙增加。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肾功能衰竭" }, { "start_idx": 6, "end_idx": 18, "type": "sym", "entity": "血磷、硫等有机阴离子的增加" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "血磷" }, { "start_idx": 9, "end_idx": 9, "type": "ite", "entity": "硫" }, { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "有机阴离子" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "糖尿病" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "酮症酸中毒" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "乳酸性酸中毒" }, { "start_idx": 38, "end_idx": 46, "type": "dis", "entity": "高血糖非酮症性昏迷" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "氨代谢障碍" }, { "start_idx": 67, "end_idx": 73, "type": "sym", "entity": "阴离子间隙增加" }, { "start_idx": 67, "end_idx": 71, "type": "ite", "entity": "阴离子间隙" } ] }, { "text": "阴离子间隙增加也见于大量青霉素应用后、水杨酸中毒等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "阴离子间隙增加" }, { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "大量青霉素应用" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "水杨酸中毒" } ] }, { "text": "可见于肾病综合征,此时血清白蛋白降低,而白蛋白在pH7.4时属阴离子;多发性骨髓瘤时由于阴离子蛋白的产生增加,也可使阴离子间隙降低。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "血清白蛋白降低" }, { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "血清白蛋白" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "白蛋白" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "pH" }, { "start_idx": 31, "end_idx": 33, "type": "ite", "entity": "阴离子" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "多发性骨髓瘤" }, { "start_idx": 44, "end_idx": 53, "type": "sym", "entity": "阴离子蛋白的产生增加" }, { "start_idx": 44, "end_idx": 48, "type": "ite", "entity": "阴离子蛋白" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "阴离子间隙降低" }, { "start_idx": 58, "end_idx": 62, "type": "ite", "entity": "阴离子间隙" } ] }, { "text": "阴离子间隙增加及正常阴离子间隙代谢性酸中毒原因见表2-2。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "阴离子间隙增加" }, { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 10, "end_idx": 14, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "表2-7阴离子间隙增加及正常阴离子间隙代谢性酸中毒原因", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "阴离子间隙增加" }, { "start_idx": 4, "end_idx": 8, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "阴离子间隙" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "第五节肺动脉狭窄右心室向肺循环泵血的出路发生梗阻最常见的原因是肺动脉瓣狭窄。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 8, "end_idx": 23, "type": "sym", "entity": "右心室向肺循环泵血的出路发生梗阻" }, { "start_idx": 8, "end_idx": 19, "type": "bod", "entity": "右心室向肺循环泵血的出路" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "梗阻" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "肺动脉瓣狭窄" } ] }, { "text": "肺动脉狭窄(pulmonarystenosis)(伴或不伴其他病变)的发生率约占所有先天性心脏病患儿的25%~30%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 6, "end_idx": 22, "type": "dis", "entity": "pulmonarystenosis" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "本节主要讨论肺动脉瓣狭窄。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "肺动脉瓣狭窄" } ] }, { "text": "一、肺动脉瓣狭窄肺动脉瓣狭窄占所有右室流出道梗阻性病变的80%~90%。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 17, "end_idx": 26, "type": "dis", "entity": "右室流出道梗阻性病变" } ] }, { "text": "1761年JohnBaptistMorgagni首先描述了肺动脉瓣狭窄,最初认为该病罕见,但随着诊断能力的提高,目前发现肺动脉瓣狭窄约占先天性心脏病患儿的8%~10%。", "entities": [ { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "Campbell发现在肺动脉瓣狭窄患儿的同胞中心脏病的发生率为2.1%,通常为肺动脉瓣狭窄或法洛四联症。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "心脏病" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "先天性心脏病第二自然史的研究发现在449个肺动脉瓣狭窄患儿的1356个同胞中明确或可能患有先天性心脏病的百分率分别为1.1%和2.1%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "【病理】肺动脉瓣的三个瓣缘互相融合,形成圆顶样或漏斗样的结构,仅有中央的小孔可通,全身循环的血流必须通过此一隘口。", "entities": [ { "start_idx": 4, "end_idx": 16, "type": "sym", "entity": "肺动脉瓣的三个瓣缘互相融合" }, { "start_idx": 4, "end_idx": 12, "type": "bod", "entity": "肺动脉瓣的三个瓣缘" }, { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "形成圆顶样或漏斗样的结构" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "圆顶样或漏斗样的结构" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "仅有中央的小孔可通" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "中央的小孔" }, { "start_idx": 41, "end_idx": 55, "type": "sym", "entity": "全身循环的血流必须通过此一隘口" }, { "start_idx": 41, "end_idx": 47, "type": "bod", "entity": "全身循环的血流" } ] }, { "text": "在肺动脉面上可见三瓣融合的嵴线向肺动脉壁放射,瓣叶可短缩、增厚和僵直,有时仅有两瓣,年长儿和成年患者于狭窄的瓣口可有疣状的赘生物或钙化。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "在肺动脉面上可见三瓣融合的嵴线向肺动脉壁放射" }, { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "肺动脉面上" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "三瓣融合的嵴线" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "肺动脉壁" }, { "start_idx": 23, "end_idx": 33, "type": "sym", "entity": "瓣叶可短缩、增厚和僵直" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "瓣叶" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "有时仅有两瓣" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "两瓣" }, { "start_idx": 50, "end_idx": 66, "type": "sym", "entity": "于狭窄的瓣口可有疣状的赘生物或钙化" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "瓣口" } ] }, { "text": "严重病例瓣口直径可仅1~2mm。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "严重病例瓣口直径可仅1~2mm" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "瓣口" } ] }, { "text": "中度狭窄者于瓣叶联合有部分粘连,瓣叶的中心部仍能启闭自如。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "中度狭窄" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "瓣叶联合有部分粘连" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "瓣叶联合" }, { "start_idx": 16, "end_idx": 27, "type": "sym", "entity": "瓣叶的中心部仍能启闭自如" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "瓣叶的中心部" } ] }, { "text": "偶有病例瓣叶并无粘连,只因瓣叶特厚,启闭不灵活,瓣环可能亦偏小,致使右心室泵出受阻,称肺动脉瓣发育不良,往往有家族性。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "瓣叶" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "粘连" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "瓣叶特厚" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "瓣叶" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "启闭不灵活" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "瓣环可能亦偏小" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "瓣环" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "右心室泵出受阻" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "右心室" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "肺动脉瓣发育不良" } ] }, { "text": "Noonan综合征大多有此病变。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "Noonan综合征" } ] }, { "text": "肥厚在圆锥部尤著,使右心室流出道狭窄。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "肥厚在圆锥部尤著" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "圆锥部" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "右心室流出道狭窄" } ] }, { "text": "右心房有继发的增大,已闭的卵圆孔可能因此被撑开,或者器质性房间隔缺损的伴发,右心房压如超过左心房,可产生右向左的分流而引起中央性青紫。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "右心房有继发的增大" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心房" }, { "start_idx": 10, "end_idx": 22, "type": "sym", "entity": "已闭的卵圆孔可能因此被撑开" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "已闭的卵圆孔" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "器质性房间隔缺损" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "右心房压如超过左心房" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "右心房压" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "左心房" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "右向左的分流" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "中央性青紫" } ] }, { "text": "肺动脉主干常扩张,内壁常有“射流”现象。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肺动脉主干" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "肺动脉主干常扩张" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "内壁常有“射流”现象" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "内壁" } ] }, { "text": "扩张自瓣环以上开始,有时可延伸到左肺动脉。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "扩张自瓣环以上开始,有时可延伸到左肺动脉" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "瓣环" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "左肺动脉" } ] }, { "text": "扩张的程度与狭窄的严重性并不成比例,轻度的狭窄可能有明显的肺动脉扩张,而极严重的狭窄可以没有扩张。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "扩张" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "狭窄" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "轻度的狭窄" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "明显的肺动脉扩张" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肺动脉" }, { "start_idx": 36, "end_idx": 47, "type": "sym", "entity": "极严重的狭窄可以没有扩张" } ] }, { "text": "有人提出狭窄后扩张与肺动脉本身的缺陷可能亦有关。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "狭窄后扩张" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "肺动脉本身的缺陷" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺动脉" } ] }, { "text": "【病理生理】右心室向肺动脉射血遇到瓣口狭窄的困阻,右心室必须提高收缩压方能向肺动脉泵血,其收缩压提高的程度与狭窄的严重性成比例。", "entities": [ { "start_idx": 6, "end_idx": 23, "type": "sym", "entity": "右心室向肺动脉射血遇到瓣口狭窄的困阻" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心室" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺动脉" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "瓣口" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "右心室" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "提高收缩压" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "收缩压" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "肺动脉" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "血" }, { "start_idx": 45, "end_idx": 62, "type": "sym", "entity": "收缩压提高的程度与狭窄的严重性成比例" }, { "start_idx": 45, "end_idx": 47, "type": "ite", "entity": "收缩压" } ] }, { "text": "因室间隔无缺损,所以严重狭窄时右心室的压力高度可以超过左心室。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "室间隔" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "缺损" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "严重狭窄" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "右心室的压力高度可以超过左心室" }, { "start_idx": 15, "end_idx": 22, "type": "ite", "entity": "右心室的压力高度" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "左心室" } ] }, { "text": "右心室的血流进肺虽有困难,但无室缺的旁路可走,所以全身所有静脉血仍必须完全进肺。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "右心室的血流进肺虽有困难,但无室缺的旁路可走" }, { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "右心室的血" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肺" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "室缺的旁路" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "全身所有静脉血" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "肺" } ] }, { "text": "但如狭窄严重,右心室壁极度增厚使心肌供血不足,可导致右心衰竭。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "狭窄严重" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "右心室壁极度增厚" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "右心室壁" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "心肌供血不足" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心肌" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "右心衰竭" } ] }, { "text": "在胎内,肺动脉瓣狭窄使右心室的心肌肥厚,右心室输出量仍可维持正常,对胎儿循环无多大影响;如狭窄很重,右心室输出量大减,腔静脉血回右房后大多通过卵圆孔或房间隔缺损流入左心房、左心室,而右心室则偏小。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "右心室的心肌肥厚" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "右心室的心肌" }, { "start_idx": 20, "end_idx": 25, "type": "ite", "entity": "右心室输出量" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "狭窄很重" }, { "start_idx": 50, "end_idx": 57, "type": "sym", "entity": "右心室输出量大减" }, { "start_idx": 50, "end_idx": 55, "type": "ite", "entity": "右心室输出量" }, { "start_idx": 59, "end_idx": 88, "type": "sym", "entity": "腔静脉血回右房后大多通过卵圆孔或房间隔缺损流入左心房、左心室" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "腔静脉血" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "右房" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "左心房" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "左心室" }, { "start_idx": 91, "end_idx": 96, "type": "sym", "entity": "右心室则偏小" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "右心室" } ] }, { "text": "临床上有一少见的肺动脉狭窄类型为右心室先天发育不良,三尖瓣也偏小,往往伴有大房间隔缺损,于是产生大量右向左分流,左心室偏大,青紫明显。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "右心室先天发育不良" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "三尖瓣也偏小" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "大房间隔缺损" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "大量右向左分流" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "左心室偏大" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "左心室" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "青紫明显" } ] }, { "text": "大多数患轻、中度肺动脉瓣狭窄的婴儿与儿童生长发育正常,因此体肺循环血流量随年龄而增长。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "轻、中度肺动脉瓣狭窄" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "体肺循环血流量随年龄而增长" }, { "start_idx": 29, "end_idx": 35, "type": "ite", "entity": "体肺循环血流量" } ] }, { "text": "如狭窄的肺动脉瓣不能相应生长,右心室收缩压必须明显增加以维持心输出量。", "entities": [ { "start_idx": 1, "end_idx": 13, "type": "sym", "entity": "狭窄的肺动脉瓣不能相应生长" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "右心室收缩压必须明显增加" }, { "start_idx": 15, "end_idx": 20, "type": "ite", "entity": "右心室收缩压" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "心输出量" } ] }, { "text": "此外,由于婴儿的正常静态心率高于年长儿,随着心率的下降,每搏量将相应增加,因而越过狭窄瓣膜的收缩期血流也将相应增加。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "静态心率" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "心率的下降" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "心率" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "每搏量将相应增加" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "每搏量" }, { "start_idx": 39, "end_idx": 56, "type": "sym", "entity": "越过狭窄瓣膜的收缩期血流也将相应增加" }, { "start_idx": 39, "end_idx": 50, "type": "bod", "entity": "越过狭窄瓣膜的收缩期血流" } ] }, { "text": "【临床表现】轻度狭窄可完全无症状;中度狭窄在2~3岁内无症状,但年长后劳力时即感易疲及气促;严重狭窄者中度体力劳动亦可呼吸困难和力不从心,有的平素活动一如常人,但一次体力活动时突有昏厥甚至猝死。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "轻度狭窄" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "中度狭窄" }, { "start_idx": 32, "end_idx": 44, "type": "sym", "entity": "年长后劳力时即感易疲及气促" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "严重狭窄" }, { "start_idx": 51, "end_idx": 67, "type": "sym", "entity": "中度体力劳动亦可呼吸困难和力不从心" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "突有昏厥" }, { "start_idx": 94, "end_idx": 95, "type": "dis", "entity": "猝死" } ] }, { "text": "如房间隔完整无缺,严重的狭窄可产生周围性青紫。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "房间隔" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "严重的狭窄" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "周围性青紫" } ] }, { "text": "在婴幼期,心导管检查时右心房的a波有时很高,但在颈静脉却看不见明显的搏动。", "entities": [ { "start_idx": 5, "end_idx": 20, "type": "sym", "entity": "心导管检查时右心房的a波有时很高" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "心导管检查" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "右心房" }, { "start_idx": 24, "end_idx": 35, "type": "sym", "entity": "颈静脉却看不见明显的搏动" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "颈静脉" } ] }, { "text": "体征方面心前区可较饱满,但明显突出者很少。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "心前区可较饱满" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "心前区" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "明显突出" } ] }, { "text": "肺动脉干虽扩张,但在胸骨左缘第二肋间摸不到搏动。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "肺动脉干虽扩张" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肺动脉干" }, { "start_idx": 10, "end_idx": 22, "type": "sym", "entity": "胸骨左缘第二肋间摸不到搏动" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "胸骨左缘第二肋间" } ] }, { "text": "右心室如有衰竭而扩张,在心前区有广泛的搏动,甚至可延伸到腋前线。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "右心室如有衰竭而扩张" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "衰竭" }, { "start_idx": 12, "end_idx": 30, "type": "sym", "entity": "心前区有广泛的搏动,甚至可延伸到腋前线" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "心前区" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "腋前线" } ] }, { "text": "收缩期震颤在胸骨左缘第二、三肋间可以摸到,杂音很响者震颤可波及胸骨柄上窝及胸骨左缘下部;心力衰竭时震颤减弱甚至消失,新生儿患者亦可无震颤。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "收缩期震颤在胸骨左缘第二、三肋间可以摸到" }, { "start_idx": 6, "end_idx": 15, "type": "bod", "entity": "胸骨左缘第二、三肋间" }, { "start_idx": 21, "end_idx": 42, "type": "sym", "entity": "杂音很响者震颤可波及胸骨柄上窝及胸骨左缘下部" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "胸骨柄上窝" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "胸骨左缘下部" }, { "start_idx": 44, "end_idx": 56, "type": "sym", "entity": "心力衰竭时震颤减弱甚至消失" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "震颤" } ] }, { "text": "听诊时胸骨左缘上部有洪亮的喷射性收缩杂音,此杂音为本病的主要体征,响度往往达4/6级以上;因通过狭窄处流进肺动脉分支散开很广,所以杂音向左上胸、心前区、颈部、腋下及背面传导。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" }, { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "听诊时胸骨左缘上部有洪亮的喷射性收缩杂音" }, { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "胸骨左缘上部" }, { "start_idx": 33, "end_idx": 43, "type": "sym", "entity": "响度往往达4/6级以上" }, { "start_idx": 46, "end_idx": 61, "type": "sym", "entity": "通过狭窄处流进肺动脉分支散开很广" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "狭窄处" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "肺动脉分支" }, { "start_idx": 65, "end_idx": 85, "type": "sym", "entity": "杂音向左上胸、心前区、颈部、腋下及背面传导" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "左上胸" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "心前区" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "颈部" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "腋下" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "背面" } ] }, { "text": "轻度狭窄时杂音短促,振峰不超过收缩中期;严重狭窄时,渐强振动延时很长,甚至主动脉的关闭音亦可被杂音掩盖。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "轻度狭窄" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "杂音短促" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "振峰不超过收缩中期" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "严重狭窄" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "渐强振动延时很长" }, { "start_idx": 37, "end_idx": 50, "type": "sym", "entity": "主动脉的关闭音亦可被杂音掩盖" }, { "start_idx": 37, "end_idx": 43, "type": "ite", "entity": "主动脉的关闭音" } ] }, { "text": "第一心音正常,轻度和中度狭窄者可听到收缩早期(于Q波后约0.08秒)喀喇音,但如狭窄严重,有喀喇音者减少;狭窄越重,喀喇音越早,甚至与第一音相重,使第一音呈金属样的声响。", "entities": [ { "start_idx": 7, "end_idx": 36, "type": "sym", "entity": "轻度和中度狭窄者可听到收缩早期(于Q波后约0.08秒)喀喇音" }, { "start_idx": 39, "end_idx": 51, "type": "sym", "entity": "如狭窄严重,有喀喇音者减少" }, { "start_idx": 53, "end_idx": 71, "type": "sym", "entity": "狭窄越重,喀喇音越早,甚至与第一音相重" }, { "start_idx": 74, "end_idx": 83, "type": "sym", "entity": "第一音呈金属样的声响" } ] }, { "text": "喀喇音的来源系由于增厚但仍具弹性的瓣膜在开始收缩时突然绷紧所致。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "喀喇音" }, { "start_idx": 9, "end_idx": 28, "type": "sym", "entity": "增厚但仍具弹性的瓣膜在开始收缩时突然绷紧" }, { "start_idx": 9, "end_idx": 18, "type": "bod", "entity": "增厚但仍具弹性的瓣膜" } ] }, { "text": "第二心音分裂,分裂程度与狭窄严重性成比例,重者可达0.14秒,但肺动脉瓣关闭音很轻甚至听不到。", "entities": [ { "start_idx": 0, "end_idx": 29, "type": "sym", "entity": "第二心音分裂,分裂程度与狭窄严重性成比例,重者可达0.14秒" }, { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "第二心音" }, { "start_idx": 32, "end_idx": 45, "type": "sym", "entity": "肺动脉瓣关闭音很轻甚至听不到" }, { "start_idx": 32, "end_idx": 38, "type": "ite", "entity": "肺动脉瓣关闭音" } ] }, { "text": "狭窄后的肺动脉扩张为本病特征性的改变,有时扩张延伸到左肺动脉,但在婴儿期扩张多不明显。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "狭窄后的肺动脉扩张" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肺动脉" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "扩张延伸到左肺动脉" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "婴儿期扩张多不明显" } ] }, { "text": "心电图将显示右心房扩大、P波高耸。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "心电图将显示右心房扩大、P波高耸" }, { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心房" } ] }, { "text": "心电图还可显示右心室肥大电轴右偏,其程度依赖于狭窄的严重程度。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "心电图还可显示右心室肥大电轴右偏" }, { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "右心室" }, { "start_idx": 17, "end_idx": 29, "type": "sym", "entity": "其程度依赖于狭窄的严重程度" } ] }, { "text": "右胸前导联将显示R波高耸,狭窄严重时出现T波倒置、ST段压低。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "右胸前导联将显示R波高耸" }, { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "右胸前导联" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "狭窄严重" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "T波倒置" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "ST段压低" } ] }, { "text": "多普勒超声可检查心房水平有无分流,更重要的是还可较可靠地估测肺动脉瓣狭窄的严重程度。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "多普勒超声" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心房" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "肺动脉瓣狭窄" } ] }, { "text": "【自然病程】一些中度肺动脉瓣狭窄患儿右心室收缩压可多年不变,提示瓣口随体格生长而相应生长。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "中度肺动脉瓣狭窄" }, { "start_idx": 18, "end_idx": 23, "type": "ite", "entity": "右心室收缩压" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "瓣口" } ] }, { "text": "不过有些患儿右心室压力显著增加,出现这种情况要么是瓣口生长不够,要么发生漏斗部继发性狭窄,或二者同时出现。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心室" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "右心室压力显著增加" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "瓣口" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "瓣口生长不够" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "漏斗部继发性狭窄" } ] }, { "text": "出现这种情况时,右心室舒张末压最终将增高,并发生右心衰竭。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "ite", "entity": "右心室舒张末压" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "右心室舒张末压最终将增高" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "右心衰竭" } ] }, { "text": "与右心衰竭发生有关的另一因素是心肌灌注不足。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "右心衰竭" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心肌" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "心肌灌注不足" } ] }, { "text": "轻度肺动脉瓣狭窄患儿右心室收缩压轻度升高,右心室心输出量及右心室心肌无明显影响。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "轻度肺动脉瓣狭窄" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "右心室收缩压" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "右心室收缩压轻度升高" }, { "start_idx": 21, "end_idx": 27, "type": "ite", "entity": "右心室心输出量" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "右心室心肌" } ] }, { "text": "在大多数情况下,随着患儿的生长,右心室收缩压几无明显升高,可有轻度右心室肥厚。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "右心室收缩压" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "轻度右心室肥厚" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "右心室" } ] }, { "text": "【治疗】严重肺动脉瓣狭窄(右心室收缩压超过体循环压力)患儿应接受球囊瓣膜成形术,如无该术适应证,则应接受外科瓣膜切开术。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "严重肺动脉瓣狭窄" }, { "start_idx": 13, "end_idx": 18, "type": "ite", "entity": "右心室收缩压" }, { "start_idx": 13, "end_idx": 25, "type": "sym", "entity": "右心室收缩压超过体循环压力" }, { "start_idx": 32, "end_idx": 38, "type": "pro", "entity": "球囊瓣膜成形术" }, { "start_idx": 52, "end_idx": 58, "type": "pro", "entity": "外科瓣膜切开术" } ] }, { "text": "大多数严重肺动脉瓣狭窄患儿漏斗部也狭窄,有关外科瓣膜切开时是否肥厚的肌肉应一同切除目前尚有争议。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "严重肺动脉瓣狭窄" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "漏斗部也狭窄" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "漏斗部" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "外科瓣膜切开" }, { "start_idx": 31, "end_idx": 40, "type": "pro", "entity": "肥厚的肌肉应一同切除" } ] }, { "text": "一般认为,仅肺动脉瓣下局限性明显的肥厚部可经肺动脉切除,流出道弥漫性肥厚可不作处理。", "entities": [ { "start_idx": 6, "end_idx": 26, "type": "pro", "entity": "肺动脉瓣下局限性明显的肥厚部可经肺动脉切除" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "流出道弥漫性肥厚" } ] }, { "text": "在大多数患儿,一旦肺动脉瓣狭窄解除,漏斗部肥厚将消退。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "漏斗部肥厚" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "漏斗部" } ] }, { "text": "肺动脉瓣狭窄较轻(右室收缩压低于体循环收缩压)患儿的手术标准目前尚未确定,不过,一般相信如右室收缩压超过50mmHg,则有可能导致心肌损害。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "右室收缩压" }, { "start_idx": 9, "end_idx": 21, "type": "sym", "entity": "右室收缩压低于体循环收缩压" }, { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "体循环收缩压" }, { "start_idx": 45, "end_idx": 49, "type": "ite", "entity": "右室收缩压" }, { "start_idx": 45, "end_idx": 57, "type": "sym", "entity": "右室收缩压超过50mmHg" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "心肌损害" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "心肌" } ] }, { "text": "因此,对这些超过上述压力的患儿可推荐狭窄解除。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "狭窄解除" } ] }, { "text": "球囊瓣膜成形术是大多数患儿的首选治疗方法,这一方法甚至可用于婴儿(有关肺动脉瓣狭窄球囊瓣膜成形术可参见本篇第三章第八节经导管介入治疗)。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "球囊瓣膜成形术" }, { "start_idx": 35, "end_idx": 47, "type": "pro", "entity": "肺动脉瓣狭窄球囊瓣膜成形术" }, { "start_idx": 59, "end_idx": 65, "type": "pro", "entity": "经导管介入治疗" } ] }, { "text": "如针对该术的指征不适合或其他原因,则外科瓣膜切除也不失为简单有效的方法。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "外科瓣膜切除" } ] }, { "text": "细菌性肺炎@第二节细菌性肺炎一、肺炎链球菌肺炎肺炎链球菌常引起以肺大叶或肺节段为单位的炎症,但在年幼儿童,由于免疫功能尚不成熟,病菌沿支气管播散形成以小气道周围实变为特征的病变(支气管肺炎)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "肺炎链球菌肺炎" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺大叶" }, { "start_idx": 32, "end_idx": 44, "type": "sym", "entity": "肺大叶或肺节段为单位的炎症" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "肺节段" }, { "start_idx": 64, "end_idx": 65, "type": "mic", "entity": "病菌" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "支气管" }, { "start_idx": 75, "end_idx": 79, "type": "bod", "entity": "小气道周围" }, { "start_idx": 75, "end_idx": 81, "type": "sym", "entity": "小气道周围实变" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "支气管肺炎" } ] }, { "text": "细菌性肺炎@年长儿童肺炎链球菌肺炎(pneumococcalpneumonia)的临床表现与成人相似。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 16, "type": "dis", "entity": "年长儿童肺炎链球菌肺炎" }, { "start_idx": 18, "end_idx": 38, "type": "dis", "entity": "pneumococcalpneumonia" } ] }, { "text": "细菌性肺炎@可先有短暂轻微的上呼吸道感染症状,继而寒战、高热,伴烦躁或嗜睡、干咳、气急、发绀及鼻扇、锁骨上、肋间隙及肋弓下凹陷等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 9, "end_idx": 21, "type": "sym", "entity": "短暂轻微的上呼吸道感染症状" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "寒战" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "高热" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "烦躁" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "嗜睡" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "干咳" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "气急" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "发绀" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "鼻扇" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "鼻" }, { "start_idx": 50, "end_idx": 62, "type": "sym", "entity": "锁骨上、肋间隙及肋弓下凹陷" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "锁骨上" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "肋间隙" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "肋弓下" } ] }, { "text": "细菌性肺炎@可伴有铁锈色痰。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "铁锈色痰" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "痰" } ] }, { "text": "细菌性肺炎@早期常缺乏体征,多在2~3天后出现肺部实变体征。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "肺部实变体征" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺部" } ] }, { "text": "细菌性肺炎@重症患儿可并发感染性休克、中毒脑病、脑水肿甚至脑疝。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "感染性休克" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "中毒脑病" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "脑水肿" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "脑疝" } ] }, { "text": "细菌性肺炎@婴儿肺炎链球菌肺炎的临床表现多变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 14, "type": "dis", "entity": "婴儿肺炎链球菌肺炎" } ] }, { "text": "细菌性肺炎@常先有鼻塞、厌食等先驱症状,数天后突然发热、烦躁不安、呼吸困难、发绀,伴气急、心动过速、三凹征等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "鼻塞" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "鼻" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "厌食" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "突然发热" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "呼吸" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "发绀" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "气急" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "心动过速" }, { "start_idx": 50, "end_idx": 52, "type": "sym", "entity": "三凹征" } ] }, { "text": "细菌性肺炎@体格检查常无特征性,实变区域可表现叩诊浊音、管性呼吸音,有时可闻啰音。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "体格检查" }, { "start_idx": 16, "end_idx": 32, "type": "sym", "entity": "实变区域可表现叩诊浊音、管性呼吸音" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "叩诊" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "可闻啰音" } ] }, { "text": "细菌性肺炎@肺部体征在整个病程中变化较少,但恢复期湿啰音增多。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肺部" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "恢复期湿啰音增多" } ] }, { "text": "细菌性肺炎@外周血白细胞计数常增高,达15×109~40×109/L,以中性粒细胞为主。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 13, "type": "ite", "entity": "外周血白细胞计数" }, { "start_idx": 6, "end_idx": 45, "type": "sym", "entity": "外周血白细胞计数常增高,达15×109~40×109/L" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "以中性粒细胞为主" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "细菌性肺炎@多数患儿鼻咽分泌物中可培养出肺炎链球菌,但其致病意义无法肯定。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "鼻咽分泌物中可培养出肺炎链球菌" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "细菌性肺炎@如能在抗生素应用前进行血培养或胸水培养,具有一定的诊断意义。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "抗生素应用" }, { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "血培养" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "胸水培养" } ] }, { "text": "细菌性肺炎@X线改变与临床过程不一定平行,实变病灶出现较肺部体征早,但在临床缓解后数周仍未完全消散。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 19, "type": "sym", "entity": "X线改变与临床过程不一定平行" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "X线" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "实变病灶出现较肺部体征早" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肺部" } ] }, { "text": "细菌性肺炎@可有胸膜反应伴渗出。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "胸膜反应" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胸膜" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "渗出" } ] }, { "text": "细菌性肺炎@肺炎链球菌肺炎患儿10%~30%存在菌血症,但由于抗生素的早期应用,国内血培养阳性率甚低。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "肺炎链球菌肺炎" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "菌血症" }, { "start_idx": 31, "end_idx": 38, "type": "pro", "entity": "抗生素的早期应用" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "血培养" } ] }, { "text": "细菌性肺炎@血清学方法,如测定患儿血清、尿液或唾液中的肺炎链球菌抗原可协助诊断,但也有研究者认为此法无法区别肺炎链球菌的感染和定植。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "血清学方法" }, { "start_idx": 13, "end_idx": 33, "type": "ite", "entity": "测定患儿血清、尿液或唾液中的肺炎链球菌抗原" }, { "start_idx": 54, "end_idx": 58, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "感染" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "定植" } ] }, { "text": "细菌性肺炎@最近有报道通过测定血清Pneumolysin抗体,或含有针对肺炎链球菌种特异荚膜多糖、型特异荚膜多糖复合物、蛋白抗原Pneumolysin抗体的循环免疫复合物进行诊断,但在婴儿,其敏感性尚嫌不足。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 13, "end_idx": 29, "type": "ite", "entity": "测定血清Pneumolysin抗体" }, { "start_idx": 36, "end_idx": 47, "type": "mic", "entity": "肺炎链球菌种特异荚膜多糖" }, { "start_idx": 49, "end_idx": 58, "type": "mic", "entity": "型特异荚膜多糖复合物" }, { "start_idx": 60, "end_idx": 76, "type": "mic", "entity": "蛋白抗原Pneumolysin抗体" }, { "start_idx": 78, "end_idx": 84, "type": "bod", "entity": "循环免疫复合物" } ] }, { "text": "细菌性肺炎@亦可通过聚合酶链反应检测胸水或血中的肺炎链球菌DNA协助诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "聚合酶链反应" }, { "start_idx": 16, "end_idx": 31, "type": "pro", "entity": "检测胸水或血中的肺炎链球菌DNA" } ] }, { "text": "细菌性肺炎@此外,年长儿右下叶肺炎常由于刺激横膈引起腹痛,需与急性阑尾炎鉴别。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "年长儿右下叶肺炎" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "刺激横膈" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "横膈" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "腹痛" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "腹" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "急性阑尾炎" } ] }, { "text": "细菌性肺炎@肺炎链球菌耐药性问题已引起普遍关注。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 10, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "细菌性肺炎@对青霉素敏感株仍可选用青霉素G10万U/(kg•d)治疗,但青霉素低度耐药株(MIC2.0~4.0μg/ml)应加大青霉素剂量至10万~30万U/(kg•d),以上治疗无效、病情危重或高度耐药者(MIC>4.0μg/ml)应选用第三代头孢霉素,如头孢噻肟、头孢曲松或万古霉素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 6, "end_idx": 12, "type": "mic", "entity": "对青霉素敏感株" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "青霉素G" }, { "start_idx": 36, "end_idx": 43, "type": "mic", "entity": "青霉素低度耐药株" }, { "start_idx": 62, "end_idx": 68, "type": "pro", "entity": "加大青霉素剂量" }, { "start_idx": 120, "end_idx": 126, "type": "dru", "entity": "第三代头孢霉素" }, { "start_idx": 129, "end_idx": 132, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 134, "end_idx": 137, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 139, "end_idx": 142, "type": "dru", "entity": "万古霉素" } ] }, { "text": "第二节幼儿期(1~3岁)的膳食特点【幼儿期的营养需要】此期生长速度较1岁以前有所缓慢,但仍是脑和机体各个器官、系统生长发育较为迅速的时期。", "entities": [ { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "脑" }, { "start_idx": 48, "end_idx": 56, "type": "bod", "entity": "机体各个器官、系统" } ] }, { "text": "婴儿期丰满的皮下脂肪开始逐渐减少,肌肉开始逐渐发达起来,20个乳牙约在2.5岁出齐,咀嚼和消化能力明显加强。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "皮下脂肪" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肌肉" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "乳牙" } ] }, { "text": "【幼儿期的膳食特点】幼儿的咀嚼和消化功能较婴儿期成熟,但乳牙尚未出全,还在陆续萌出,胃肠消化吸收功能仍不完善,而其饮食正从乳类为主过渡到以谷类为主食,再配加鱼、肉、蔬菜等混合饮食。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "乳牙" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "胃肠" } ] }, { "text": "③由于幼儿期的胃容量和消化酶的分泌有限,但需要量相对较大,因而,进食次数要多于成人,每天5次,即3餐加上午、下午点心各1次。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "胃容量" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "消化酶" } ] }, { "text": "④培养良好的饮食习惯能使小儿保持正常的食欲,特别要注意避免小儿养成挑食、偏食和多吃零食的不良饮食习惯,防止营养素不均衡而造成单个营养素缺乏性疾病的发生。", "entities": [ { "start_idx": 62, "end_idx": 71, "type": "dis", "entity": "单个营养素缺乏性疾病" } ] }, { "text": "随着小儿乳牙的逐渐长出和消化能力的增强,可适当调整烹饪方式,并在蔬菜的选择上放宽范围,这对增进幼儿的咀嚼能力、锻炼胃肠道功能、获得全面而均衡的营养非常有益。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "乳牙" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "胃肠道" } ] }, { "text": "但需要注意饮食安全,当咀嚼能力还未完善时,要禁止给予整粒葡萄、圣女果、果冻、花生、各种豆类、话梅和含有鱼刺、碎骨的食物,避免不慎进入气管引起窒息以及刺伤咽部和食管。", "entities": [ { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "气管" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "窒息" }, { "start_idx": 74, "end_idx": 80, "type": "sym", "entity": "刺伤咽部和食管" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "咽部" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "食管" } ] }, { "text": "另外,幼儿期易患龋齿,这虽然与牙齿的本身健康有关,但与牙齿的保健意识和方法也有很大关系。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "龋齿" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "牙齿" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "牙齿" } ] }, { "text": "甜又黏的食物对牙齿构成的威胁最大。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "牙齿" } ] }, { "text": "三、产前诊断对象产前诊断适应对象主要有如下几种情况:1.夫妇之一有染色体异常(数目或结构),或生育过染色体病患儿的孕妇,特别是表型正常,而具有染色体异常的携带者。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "产前诊断" }, { "start_idx": 33, "end_idx": 44, "type": "dis", "entity": "染色体异常(数目或结构)" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "染色体病" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "染色体异常" } ] }, { "text": "2.夫妇之一明确为某种单基因病患者,或曾生育过某一单基因病患儿的孕妇。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "单基因病" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "单基因病" } ] }, { "text": "3.夫妇之一有神经管畸形或生育过开放性神经管畸形儿的孕妇。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "神经管畸形" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "开放性神经管畸形儿" } ] }, { "text": "5.羊水过多的孕妇。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "羊水过多" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "羊水" } ] }, { "text": "8.具有染色体断裂综合征家系的孕妇。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "染色体断裂综合征" } ] }, { "text": "三、尿液沉渣显微镜检查尿液沉渣显微镜检查可检出各种细胞成分、管型以及结晶,采用离心尿可提高检出率。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "尿液沉渣显微镜检查" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "尿液沉渣显微镜检查" }, { "start_idx": 25, "end_idx": 28, "type": "ite", "entity": "细胞成分" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "管型" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "结晶" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "离心尿" } ] }, { "text": "方法为:10ml新鲜尿,1500转。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "尿" } ] }, { "text": "分速度离心沉淀5分钟后,弃上清留沉渣0.2ml,混匀后置载玻片上镜检。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "分速度离心沉淀5分钟后" }, { "start_idx": 12, "end_idx": 22, "type": "pro", "entity": "弃上清留沉渣0.2ml" }, { "start_idx": 24, "end_idx": 33, "type": "pro", "entity": "混匀后置载玻片上镜检" } ] }, { "text": "正常小儿尿液中红细胞<2个/高倍视野(HP),白细胞<5个/HP,一般无管型。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "尿液" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "红细胞" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "白细胞" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "管型" } ] }, { "text": "1.红细胞尿离心检查正常值为0~1个/HP,不离心者正常值为0或偶见。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "红细胞尿离心检查" } ] }, { "text": "红细胞≥3个/HP即为血尿,但≤50个/HP,一般为镜下血尿,>50个/HP多为肉眼血尿。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "红细胞≥3个/HP" }, { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "红细胞" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "≤50个/HP" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "镜下血尿" }, { "start_idx": 26, "end_idx": 26, "type": "equ", "entity": "镜" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "血" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": ">50个/HP" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "血" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "尿" } ] }, { "text": "通过观察红细胞形态可区分肾小球性及非肾小球性血尿,前者严重变形的红细胞(包括体积大而染色浅、穿孔、环状、芽胞及残骸5种)占30%以上,由此可与后者区别。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "观察红细胞形态" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "肾小球性及非肾小球性血尿" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肾小球" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肾小球" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "血" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "严重变形的红细胞" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "红细胞" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "体积大而染色浅" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "穿孔" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "环状" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "芽胞" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "残骸" } ] }, { "text": "红细胞形态的观察方法有活体不染色观察、涂片染色后镜检、相差显微镜镜检以及扫描电镜检查等几种方法,现以相差显微镜不染色直接观察最常用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "红细胞" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "活体不染色观察" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "涂片染色后镜检" }, { "start_idx": 27, "end_idx": 33, "type": "pro", "entity": "相差显微镜镜检" }, { "start_idx": 36, "end_idx": 41, "type": "pro", "entity": "扫描电镜检查" }, { "start_idx": 50, "end_idx": 61, "type": "pro", "entity": "相差显微镜不染色直接观察" } ] }, { "text": "此外,也可作尿三杯试验来鉴别血尿的来源,如初始血尿说明病变在尿道,终末血尿说明病变在膀胱颈部及三角区,全程血尿者其病变在膀胱以上。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "尿三杯试验" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "血尿" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "初始血尿" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "血" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "病变在尿道" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "尿道" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "终末血尿" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "血" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "尿" }, { "start_idx": 39, "end_idx": 49, "type": "sym", "entity": "病变在膀胱颈部及三角区" }, { "start_idx": 42, "end_idx": 49, "type": "bod", "entity": "膀胱颈部及三角区" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "全程血尿" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "血" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "尿" }, { "start_idx": 57, "end_idx": 63, "type": "sym", "entity": "病变在膀胱以上" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "膀胱以上" } ] }, { "text": "血尿的原因为:①肾脏实质损害:如同时存在500mg/d以上的蛋白尿及管型说明多来自肾脏,可见于各种急慢性肾炎、肾结核以及肾静脉血栓等;②来自肾脏以下的泌尿系:如泌尿系感染、出血性膀胱炎、结石和肿瘤等;③肾外疾病:各种出凝血疾病以及心功能不全等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "血尿" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "尿" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "肾脏实质损害" }, { "start_idx": 20, "end_idx": 35, "type": "sym", "entity": "500mg/d以上的蛋白尿及管型" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "蛋白" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "尿" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肾脏" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "急慢性肾炎" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "肾结核" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "肾静脉血栓" }, { "start_idx": 70, "end_idx": 77, "type": "bod", "entity": "肾脏以下的泌尿系" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 86, "end_idx": 91, "type": "dis", "entity": "出血性膀胱炎" }, { "start_idx": 93, "end_idx": 94, "type": "dis", "entity": "结石" }, { "start_idx": 96, "end_idx": 97, "type": "dis", "entity": "肿瘤" }, { "start_idx": 101, "end_idx": 104, "type": "dis", "entity": "肾外疾病" }, { "start_idx": 108, "end_idx": 112, "type": "dis", "entity": "出凝血疾病" }, { "start_idx": 115, "end_idx": 119, "type": "dis", "entity": "心功能不全" } ] }, { "text": "此外,剧烈运动、肾下垂和高钙尿症也可出现血尿。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肾下垂" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "高钙尿症" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "血尿" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "尿" } ] }, { "text": "2.白细胞离心法正常值为2~3个/HP以下,直接涂片法为<0~1/HP。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "白细胞离心法" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "直接涂片法" } ] }, { "text": "白细胞>5个/HP则有意义。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "白细胞" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "白细胞>5个/HP" } ] }, { "text": "可见于泌尿系感染、急性肾炎、狼疮性肾炎、坏死性血管炎以及间质性肾炎等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "狼疮性肾炎" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "坏死性血管炎" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "间质性肾炎" } ] }, { "text": "3.上皮细胞上皮细胞可来自肾小管、肾盂、输尿管、膀胱、尿道及阴道。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肾小管" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肾盂" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "输尿管" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "膀胱" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "尿道" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "阴道" } ] }, { "text": "但只有肾小管上皮细胞有诊断意义。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "肾小管上皮细胞" } ] }, { "text": "正常小儿尿中不应见到小圆形上皮细胞(肾小管细胞)。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "小圆形上皮细胞" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "肾小管细胞" } ] }, { "text": "如出现小圆形细胞说明肾实质损害,见于急性肾小管坏死、肾炎和肾盂肾炎。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "出现小圆形细胞" }, { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "小圆形细胞" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肾实质损害" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "急性肾小管坏死" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "肾炎" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "4.管型为蛋白质在肾小管内凝固而成,圆柱状,边缘不规则。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "管型" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "蛋白质" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肾小管" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "圆柱状" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "边缘不规则" } ] }, { "text": "所有管型主要以Tamm-Horsfall蛋白为基质组成。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "管型" }, { "start_idx": 7, "end_idx": 21, "type": "bod", "entity": "Tamm-Horsfall蛋白" } ] }, { "text": "Tamm-Horsfall蛋白在酸性尿、电解质高浓度尿、肾小管淤滞或伴蛋白尿时溶解度降低。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "bod", "entity": "Tamm-Horsfall蛋白" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "酸性尿" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "尿" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "电解质高浓度尿" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "尿" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "肾小管淤滞" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "蛋白" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "尿" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "溶解度降低" } ] }, { "text": "由于原尿在集合管处最浓缩且呈酸性,故管型主要在此节段形成。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "原尿" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "集合管处" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "管型" } ] }, { "text": "少尿时易形成管型;相反,稀释尿(尿比重<1.004)及尿pH>6.5时极难见到管型。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "少尿" }, { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "尿" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "管型" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "稀释尿" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "尿" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "尿比重<1.004" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "尿比重" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "尿pH>6.5" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "尿pH" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "管型" } ] }, { "text": "透明管型主要由Tamm-Horsfall蛋白组成,还有少量白蛋白及氯化物,正常时无,偶尔见到时不一定表示肾实质受损,可见于一过性蛋白尿疾病和发热、直立性蛋白尿以及尿少时如脱水或有效血容量下降或伴用利尿剂后,在急性肾炎和肾病综合征时的细胞管型其特征是随着细胞排出增加而形成,故管型的内容对诊断有重要价值,如红细胞管型多见于肾小球肾炎或血管炎;白细胞管型多见于肾脏感染性疾患如肾盂肾炎;小圆形上皮细胞管型可见于急性肾小管坏死和小管间质性疾病,也可见于急性肾炎及肾病时。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "透明管型" }, { "start_idx": 7, "end_idx": 21, "type": "bod", "entity": "Tamm-Horsfall蛋白" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "少量白蛋白" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "氯化物" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "肾实质受损" }, { "start_idx": 61, "end_idx": 68, "type": "dis", "entity": "一过性蛋白尿疾病" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "发热" }, { "start_idx": 73, "end_idx": 78, "type": "dis", "entity": "直立性蛋白尿" }, { "start_idx": 81, "end_idx": 82, "type": "sym", "entity": "尿少" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "尿" }, { "start_idx": 85, "end_idx": 86, "type": "sym", "entity": "脱水" }, { "start_idx": 88, "end_idx": 94, "type": "sym", "entity": "有效血容量下降" }, { "start_idx": 88, "end_idx": 92, "type": "ite", "entity": "有效血容量" }, { "start_idx": 98, "end_idx": 100, "type": "dru", "entity": "利尿剂" }, { "start_idx": 104, "end_idx": 107, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 109, "end_idx": 113, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 116, "end_idx": 119, "type": "ite", "entity": "细胞管型" }, { "start_idx": 126, "end_idx": 127, "type": "bod", "entity": "细胞" }, { "start_idx": 137, "end_idx": 138, "type": "ite", "entity": "管型" }, { "start_idx": 152, "end_idx": 156, "type": "sym", "entity": "红细胞管型" }, { "start_idx": 152, "end_idx": 154, "type": "bod", "entity": "红细胞" }, { "start_idx": 160, "end_idx": 164, "type": "dis", "entity": "肾小球肾炎" }, { "start_idx": 166, "end_idx": 168, "type": "dis", "entity": "血管炎" }, { "start_idx": 170, "end_idx": 174, "type": "sym", "entity": "白细胞管型" }, { "start_idx": 170, "end_idx": 172, "type": "bod", "entity": "白细胞" }, { "start_idx": 178, "end_idx": 184, "type": "dis", "entity": "肾脏感染性疾患" }, { "start_idx": 186, "end_idx": 189, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 191, "end_idx": 199, "type": "sym", "entity": "小圆形上皮细胞管型" }, { "start_idx": 191, "end_idx": 197, "type": "bod", "entity": "小圆形上皮细胞" }, { "start_idx": 203, "end_idx": 209, "type": "dis", "entity": "急性肾小管坏死" }, { "start_idx": 211, "end_idx": 217, "type": "dis", "entity": "小管间质性疾病" }, { "start_idx": 223, "end_idx": 226, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 228, "end_idx": 229, "type": "dis", "entity": "肾病" } ] }, { "text": "脂肪管型可见于有中~重度蛋白尿的肾脏病如肾病时;颗粒管型代表褪变的细胞管型或蛋白质凝集而成,见于多种肾脏疾病;蜡样管型是由细胞管型转化为颗粒管型后再形成的,见于尿流十分缓慢时,主要见于进行性肾衰竭时;巨大管型较其他管型均大,似颗粒或蜡样管型外观,见于急、慢性肾衰竭时,因而也叫肾衰竭管型。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "脂肪管型" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "有中~重度蛋白尿" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "尿" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肾脏病" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肾病" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "颗粒管型" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "细胞管型" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "细胞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "蛋白质" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "肾脏疾病" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "蜡样管型" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "细胞管型" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "细胞" }, { "start_idx": 68, "end_idx": 71, "type": "sym", "entity": "颗粒管型" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "尿流" }, { "start_idx": 92, "end_idx": 97, "type": "dis", "entity": "进行性肾衰竭" }, { "start_idx": 100, "end_idx": 103, "type": "sym", "entity": "巨大管型" }, { "start_idx": 105, "end_idx": 108, "type": "sym", "entity": "其他管型" }, { "start_idx": 113, "end_idx": 119, "type": "sym", "entity": "颗粒或蜡样管型" }, { "start_idx": 125, "end_idx": 131, "type": "dis", "entity": "急、慢性肾衰竭" }, { "start_idx": 138, "end_idx": 142, "type": "sym", "entity": "肾衰竭管型" }, { "start_idx": 138, "end_idx": 140, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "5.结晶尿尿中出现结晶取决于尿液酸度、温度、胶体状态以及该结晶在尿中的浓度。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "结晶尿" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "尿中出现结晶" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "尿液" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "结晶" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "尿" } ] }, { "text": "胱氨酸结晶(六角形、扁平和双折光)为胱氨酸尿症的特征,其他尿酸以及草酸盐结晶等可能与结石有关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "胱氨酸结晶" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "六角形" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "扁平" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "双折光" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "胱氨酸尿症" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "尿酸以及草酸盐结晶" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "结石" } ] }, { "text": "尿中如出现磺胺结晶时应及时停药,多饮水并给碱性药,以免引起尿闭等不良后果。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "尿中如出现磺胺结晶" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "尿中" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "磺胺" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "及时停药" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "多饮水" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "给碱性药" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "尿闭" } ] }, { "text": "二、结肠镜检查结肠的许多疾病常隐匿于肠腔,纤维(电子)结肠镜是诊断大肠内病变最有效、可靠和简便的检查方法,不仅可澄清钡剂灌肠X线检查有疑问的病变处,而且能在病变处取病理活检确定病变的性质。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "结肠镜检查" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "结肠" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肠腔" }, { "start_idx": 21, "end_idx": 29, "type": "equ", "entity": "纤维(电子)结肠镜" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "大肠内病变" }, { "start_idx": 58, "end_idx": 65, "type": "pro", "entity": "钡剂灌肠X线检查" }, { "start_idx": 77, "end_idx": 85, "type": "pro", "entity": "在病变处取病理活检" } ] }, { "text": "儿科结肠镜的开展明显提高了结肠病变的发现率与确诊率。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "儿科结肠镜" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "结肠病变" } ] }, { "text": "(一)结肠镜检查(colonoscopy)的适应证和禁忌证1.适应证(1)便血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "结肠镜检查" }, { "start_idx": 9, "end_idx": 19, "type": "pro", "entity": "colonoscopy" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "便血" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "便" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "血" } ] }, { "text": "(2)不能解释的慢性腹泻。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "不能解释的慢性腹泻" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "腹" } ] }, { "text": "(3)炎症性肠病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "炎症性肠病" } ] }, { "text": "(4)恶变的监视:家族性多发息肉。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "恶变" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "家族性多发息肉" } ] }, { "text": "(5)肠放射学检查异常但不能定性者。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "肠放射学检查" }, { "start_idx": 3, "end_idx": 15, "type": "sym", "entity": "肠放射学检查异常但不能定性" } ] }, { "text": "(6)结肠异物、息肉摘除、乙状结肠扭转的减压与复原不明原因的腹水、腹痛及腹部肿块。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "结肠异物" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "结肠" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "息肉摘除" }, { "start_idx": 13, "end_idx": 24, "type": "pro", "entity": "乙状结肠扭转的减压与复原" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "不明原因的腹水" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "腹" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "腹痛" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "腹" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "腹部肿块" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "腹部" } ] }, { "text": "(8)大便习惯的改变。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "大便习惯的改变" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "大便" } ] }, { "text": "2.禁忌证(1)严重的心肺疾患无法耐受内镜检查或处于休克危重状态者。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "严重的心肺疾患" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "内镜检查" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "休克危重状态" } ] }, { "text": "(2)疑有肠穿孔和腹膜炎或疑有腹腔内有广泛粘连者。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "腹腔内有广泛粘连" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "腹腔内" } ] }, { "text": "(3)大肠炎症疾病的急性活动期,急性憩室炎等。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "大肠炎症疾病的急性活动期" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "急性憩室炎" } ] }, { "text": "(4)严重的坏死性结肠炎,中毒性巨结肠,疼痛的肛门病变以及极不合作者。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "严重的坏死性结肠炎" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "中毒性巨结肠" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "疼痛" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "肛门病变" } ] }, { "text": "(5)患出血性疾病(必要时,检查时不做活体组织检查和息肉切除)。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "息肉切除" } ] }, { "text": "(二)结肠镜检查与操作技术1.了解病情如做过结肠造影者应阅读X线片及报告单,估计病变部位与性质,并注意有无检查禁忌证。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "结肠镜检查" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "结肠造影者" }, { "start_idx": 30, "end_idx": 32, "type": "pro", "entity": "X线片" } ] }, { "text": "2.肠道准备根据患儿年龄和病情及当地条件选用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "肠道准备" } ] }, { "text": "(1)灌肠法:检查前3天吃无渣半流质;检查前1天吃流质;检查日当天禁食。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "灌肠法" }, { "start_idx": 7, "end_idx": 17, "type": "pro", "entity": "检查前3天吃无渣半流质" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "检查前1天吃流质" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "检查日当天禁食" } ] }, { "text": "术前1天晚及术前2小时分别用开塞露1~2只通便,术前1小时用温生理盐水清洁灌肠。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "开塞露" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "通便" }, { "start_idx": 24, "end_idx": 38, "type": "pro", "entity": "术前1小时用温生理盐水清洁灌肠" } ] }, { "text": "(2)泻药法:口服便塞停片5~30mg,术前1天晚顿服或术前3天分次口服,术前1天流质,一般不需灌肠;或术前一天下午4点起番泻叶通便2次小婴儿或紧张不安者术前30分钟可肌内注射地西泮0.1~0.3mg/kg口服10%水合氯醛0.5ml/kg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "泻药法" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "口服" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "便塞停片" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "顿服" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "口服" }, { "start_idx": 37, "end_idx": 42, "type": "pro", "entity": "术前1天流质" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "灌肠" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "番泻叶" }, { "start_idx": 84, "end_idx": 87, "type": "pro", "entity": "肌内注射" }, { "start_idx": 88, "end_idx": 90, "type": "dru", "entity": "地西泮" }, { "start_idx": 103, "end_idx": 104, "type": "pro", "entity": "口服" }, { "start_idx": 105, "end_idx": 111, "type": "dru", "entity": "10%水合氯醛" } ] }, { "text": "也可用全身麻醉,氯胺酮4~6mg/kg肌内注射,但需麻醉医师在场活体组织检查的患儿术后进半流质饮食一日,未做活体组织检查的患儿,术后无特殊处理。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "全身麻醉" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "氯胺酮" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "肌内注射" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 41, "end_idx": 50, "type": "pro", "entity": "术后进半流质饮食一日" }, { "start_idx": 54, "end_idx": 59, "type": "pro", "entity": "活体组织检查" } ] }, { "text": "5.操作方法先作直肠指检了解有无异常,注意肛门有无痔疮、肛裂。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "直肠指检" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肛门" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "痔疮" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "肛裂" } ] }, { "text": "助手在肛门口及结肠镜管外周涂上润滑剂,然后用示指将镜头轻轻压入肛门,插入直肠后注入少量气体,使肠腔较充盈,在术者示意下,循肠腔进镜,反复抽气,变换体位,并可根据光运行轨迹了解肠腔管走行,直至回盲部。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "pro", "entity": "在肛门口及结肠镜管外周涂上润滑剂" }, { "start_idx": 21, "end_idx": 32, "type": "pro", "entity": "用示指将镜头轻轻压入肛门" }, { "start_idx": 34, "end_idx": 44, "type": "pro", "entity": "插入直肠后注入少量气体" }, { "start_idx": 46, "end_idx": 51, "type": "pro", "entity": "使肠腔较充盈" }, { "start_idx": 60, "end_idx": 64, "type": "pro", "entity": "循肠腔进镜" }, { "start_idx": 66, "end_idx": 69, "type": "pro", "entity": "反复抽气" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "变换体位" }, { "start_idx": 78, "end_idx": 91, "type": "pro", "entity": "根据光运行轨迹了解肠腔管走行" }, { "start_idx": 93, "end_idx": 97, "type": "pro", "entity": "直至回盲部" } ] }, { "text": "然后慢慢退镜,同时注意观察黏膜形态、色泽、血管及有无异常,如发现病灶,便可在退镜时取活检。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "慢慢退镜" }, { "start_idx": 7, "end_idx": 27, "type": "pro", "entity": "同时注意观察黏膜形态、色泽、血管及有无异常" }, { "start_idx": 29, "end_idx": 43, "type": "pro", "entity": "如发现病灶,便可在退镜时取活检" } ] }, { "text": "6.操作注意事项进镜过程中要少注气,插镜、退镜应遵循退退进进原则。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "pro", "entity": "进镜过程中要少注气" }, { "start_idx": 18, "end_idx": 31, "type": "pro", "entity": "插镜、退镜应遵循退退进进原则" } ] }, { "text": "当粪渣过多影响视野,肠腔狭窄不能通过,广泛糜烂溃疡出血而进镜困难,腹痛难忍不合作者,皆应终止进镜,切勿强行插入。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "粪渣过多影响视野" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "肠腔狭窄不能通过" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肠腔" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "广泛糜烂溃疡出血而进镜困难" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "血" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "腹痛难忍不合作" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "腹" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "终止进镜" } ] }, { "text": "(三)结肠病变的内镜诊断1.溃疡性结肠炎溃疡性结肠炎病变多累及直肠和乙状结肠,早期黏膜血管充血,纹理模糊,呈弥漫性红斑,表面粗糙不平。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "结肠病变" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "内镜诊断" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "溃疡性结肠炎" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "溃疡性结肠炎" }, { "start_idx": 26, "end_idx": 37, "type": "sym", "entity": "病变多累及直肠和乙状结肠" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "直肠" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "乙状结肠" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "早期黏膜血管充血" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "黏膜血管" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "血" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "纹理模糊" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "呈弥漫性红斑" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "表面粗糙不平" } ] }, { "text": "病变进展则黏膜易脆,颜色暗红,有接触性或自发性出血,脓性分泌物散布其上。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "病变进展则黏膜易脆" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "黏膜" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "颜色暗红" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "有接触性或自发性出血" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "血" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "脓性分泌物散布其上" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "脓性分泌物" } ] }, { "text": "重者大量糜烂附以斑斑血迹。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "重者大量糜烂附以斑斑血迹" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "血" } ] }, { "text": "当病变呈慢性时可见许多浅表溃疡,大小不一,重者呈地图样分布,表面有黄色分泌物,四周黏液明显充血、水肿。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "病变呈慢性" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "可见许多浅表溃疡" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "大小不一" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "重者呈地图样分布" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "表面有黄色分泌物" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "黄色分泌物" }, { "start_idx": 39, "end_idx": 49, "type": "sym", "entity": "四周黏液明显充血、水肿" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "四周黏液" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "血" } ] }, { "text": "2.克罗恩病克罗恩病直肠通常是正常的,病变多累及回肠和回盲部,分布呈节段性黏液组织下淋巴组织,黏膜病变相对较轻。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "克罗恩病" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "克罗恩病" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "直肠" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "病变多累及回肠和回盲部" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "回肠" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "回盲部" }, { "start_idx": 31, "end_idx": 45, "type": "sym", "entity": "分布呈节段性黏液组织下淋巴组织" }, { "start_idx": 37, "end_idx": 45, "type": "bod", "entity": "黏液组织下淋巴组织" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "黏膜病变相对较轻" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "黏膜" } ] }, { "text": "多见纵行小溃疡,直径1~3mm不等,深1~5mm,边缘呈穿凿状,基底水肿或瘢痕状,溃疡间的黏膜正常或苍白,增生呈铺路石子样,并有肠狭窄和假息肉样的病变。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "多见纵行小溃疡" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "直径1~3mm不等" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "深1~5mm" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "边缘呈穿凿状" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "基底水肿或瘢痕状" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "基底" }, { "start_idx": 41, "end_idx": 51, "type": "sym", "entity": "溃疡间的黏膜正常或苍白" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "溃疡间的黏膜" }, { "start_idx": 53, "end_idx": 60, "type": "sym", "entity": "增生呈铺路石子样" }, { "start_idx": 62, "end_idx": 74, "type": "sym", "entity": "并有肠狭窄和假息肉样的病变" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "肠" } ] }, { "text": "若遇密集性大小均匀的扁平结节性病变呈红色或淡灰色,且成片状,边界清晰,很像皮肤的结节病。", "entities": [ { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "若遇密集性大小均匀的扁平结节性病变呈红色或淡灰色" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "且成片状" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "边界清晰" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "很像皮肤的结节病" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "皮肤" } ] }, { "text": "3.肠息肉(intestinalpolyp)息肉可发生在肠道的任何部位,儿童息肉多发部位是直肠与乙状结肠,呈单个或多发。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肠息肉" }, { "start_idx": 6, "end_idx": 20, "type": "dis", "entity": "intestinalpolyp" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "息肉" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肠道" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "儿童息肉" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "直肠" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "乙状结肠" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "呈单个或多发" } ] }, { "text": "单发息肉形态小如米粒,大如核桃,表面有光滑、充血、糜烂及分叶状,基底宽窄不一,高出黏膜表面。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "单发息肉形态小如米粒" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "大如核桃" }, { "start_idx": 16, "end_idx": 30, "type": "sym", "entity": "表面有光滑、充血、糜烂及分叶状" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "血" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "基底宽窄不一" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "基底" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "高出黏膜表面" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "黏膜表面" } ] }, { "text": "多发性息肉可成为串珠状排列,有的呈葡萄状,大小不一,黏膜肿胀,息肉与息肉之间的黏膜呈炎症改变或正常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "多发性息肉" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "可成为串珠状排列" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "有的呈葡萄状" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "大小不一" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "黏膜肿胀" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "黏膜" }, { "start_idx": 31, "end_idx": 48, "type": "sym", "entity": "息肉与息肉之间的黏膜呈炎症改变或正常" }, { "start_idx": 31, "end_idx": 40, "type": "bod", "entity": "息肉与息肉之间的黏膜" } ] }, { "text": "第二章原发性免疫缺陷病免疫缺陷病(immunodeficiencydiseases,ID)是指免疫系统的器官(如胸腺),免疫活性细胞(如淋巴细胞和吞噬细胞)及免疫活性分子(免疫球蛋白、淋巴因子、补体分子和细胞膜表面分子)发生缺陷引起某种免疫反应能力缺失或降低,导致机体防御能力普遍或部分下降的一组临床综合征。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "原发性免疫缺陷病" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "免疫缺陷病" }, { "start_idx": 17, "end_idx": 40, "type": "dis", "entity": "immunodeficiencydiseases" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "ID" }, { "start_idx": 47, "end_idx": 53, "type": "bod", "entity": "免疫系统的器官" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "胸腺" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "免疫活性细胞" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 79, "end_idx": 84, "type": "bod", "entity": "免疫活性分子" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "淋巴因子" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "补体分子" }, { "start_idx": 102, "end_idx": 108, "type": "bod", "entity": "细胞膜表面分子" }, { "start_idx": 110, "end_idx": 113, "type": "sym", "entity": "发生缺陷" }, { "start_idx": 116, "end_idx": 128, "type": "sym", "entity": "某种免疫反应能力缺失或降低" }, { "start_idx": 132, "end_idx": 144, "type": "sym", "entity": "机体防御能力普遍或部分下降" } ] }, { "text": "因先天遗传因素,如先天性基因突变(mutation)、缺失(deletion)等所致免疫功能缺陷病称为原发性免疫缺陷病(primaryimmunodeficiencydiseases,PID),若因后天因素(理化因素、感染因素、营养因素、疾病因素、生理发育不成熟以及老年退化等)所致免疫缺陷称为继发性免疫缺陷病(secondaryimmunodeficiencydisease,SID)。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "先天性基因突变" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "先天性基因" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "mutation" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "缺失" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "deletion" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "免疫功能缺陷病" }, { "start_idx": 51, "end_idx": 58, "type": "dis", "entity": "原发性免疫缺陷病" }, { "start_idx": 60, "end_idx": 90, "type": "dis", "entity": "primaryimmunodeficiencydiseases" }, { "start_idx": 92, "end_idx": 94, "type": "dis", "entity": "PID" }, { "start_idx": 141, "end_idx": 144, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 147, "end_idx": 154, "type": "dis", "entity": "继发性免疫缺陷病" }, { "start_idx": 156, "end_idx": 187, "type": "dis", "entity": "secondaryimmunodeficiencydisease" }, { "start_idx": 189, "end_idx": 191, "type": "dis", "entity": "SID" } ] }, { "text": "SID可能也受基因影响,但其影响程度较PID小,且为多基因共同作用结果。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "SID" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "基因" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "PID" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "多基因" } ] }, { "text": "因而SID往往表现轻度免疫功能缺陷,且常为可逆性变异,及时去除不利因素之后,有望恢复正常免疫功能。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "SID" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "轻度免疫功能缺陷" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "可逆性变异" } ] }, { "text": "【发病率】自1952年Bruton报告首例先天性无丙种球蛋白血症(congenitalagammaglobulinemia)以来,全球报道的PID病例已愈万例,但其总发病率尚无确切资料。", "entities": [ { "start_idx": 21, "end_idx": 31, "type": "dis", "entity": "先天性无丙种球蛋白血症" }, { "start_idx": 33, "end_idx": 60, "type": "dis", "entity": "congenitalagammaglobulinemia" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "PID" } ] }, { "text": "以活产婴的发病数计算,1981年日本和1983年澳洲的报道约为1/10000,但未包括无症状性IgA缺乏症。", "entities": [ { "start_idx": 43, "end_idx": 52, "type": "dis", "entity": "无症状性IgA缺乏症" } ] }, { "text": "香港特区于1990年前未报告原发性免疫缺陷病,但在开展此病研究之后,仅仅10余年间,在600多万人口中已发现100多例不同类型PID,表明PID在我国人群中并不少见,若按PID总发病率>1/8000计,我国每年大约2500万新生儿中,至少有3000例PID。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "原发性免疫缺陷病" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "PID" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "PID" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "PID" }, { "start_idx": 125, "end_idx": 127, "type": "dis", "entity": "PID" } ] }, { "text": "这不包括IgA缺乏症或正在研究、调查之中的甘露聚糖结合蛋白(MBP)基因缺陷病人。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "IgA缺乏症" }, { "start_idx": 21, "end_idx": 37, "type": "dis", "entity": "甘露聚糖结合蛋白(MBP)基因缺陷" } ] }, { "text": "根据日本和瑞士的资料(1988年),PID中单纯抗体缺陷占50%,其中包括一些T细胞辅助功能缺乏而致B细胞产生抗体能力下降;细胞免疫缺陷占10%,联合免疫缺陷(SCID)占20%;吞噬细胞及中性粒细胞缺陷占18%,补体缺陷占2%。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "PID" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "单纯抗体缺陷" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "T细胞辅助功能缺乏" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "T细胞" }, { "start_idx": 50, "end_idx": 60, "type": "sym", "entity": "B细胞产生抗体能力下降" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "B细胞" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "抗体" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "细胞免疫缺陷" }, { "start_idx": 73, "end_idx": 78, "type": "dis", "entity": "联合免疫缺陷" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "SCID" }, { "start_idx": 90, "end_idx": 101, "type": "dis", "entity": "吞噬细胞及中性粒细胞缺陷" }, { "start_idx": 107, "end_idx": 110, "type": "dis", "entity": "补体缺陷" } ] }, { "text": "因T细胞功能低下,不能提供辅助B细胞合成、分泌免疫球蛋白的信息(包括细胞因子和细胞表面分子介导的信息传递),也同样发生不同程度的抗体产生减少,因而在全部PID病人中,约80%PID病例存在免疫球蛋白和(或)抗体缺陷。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "T细胞" }, { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "T细胞功能低下" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "B细胞" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "细胞因子" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "细胞表面分子" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "抗体" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "抗体产生减少" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "PID" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "PID" }, { "start_idx": 94, "end_idx": 106, "type": "sym", "entity": "免疫球蛋白和(或)抗体缺陷" }, { "start_idx": 94, "end_idx": 98, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "抗体" } ] }, { "text": "【分类、病因及临床特征】1971年WHO组织专家委员会制定了PID命名原则:即按综合征的发病机制,病理生理改变,特别是遗传学特征来命名,废除传统的人名和地名命名方式。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "PID" } ] }, { "text": "1971年WHO专家委员会按新的命名原则进行了首次全球统一分类,随着对各种PID认识的逐渐深入,每隔2~3年又将分类进行重新审订。", "entities": [ { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "PID" } ] }, { "text": "下面列举的PID是1997年第7次修订稿中统一使用的疾病分类名称。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "PID" } ] }, { "text": "(一)联合免疫缺陷病(表17-17)表17-3联合免疫缺陷病续表注:↓下降,→正常,↘逐渐下降,XL:X连锁遗传,AR:常染色体隐性遗传,ADA:腺苷脱氨酶,RAG:重组活化基因联合免疫缺陷病(combinedimmunodeficiency)该组疾病中T和B细胞均可能有明显缺陷,临床表现为婴儿期严重致死性感染,细胞免疫和抗体反应均缺陷外周血淋巴细胞减少T淋巴细胞为主。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "联合免疫缺陷病" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "联合免疫缺陷病" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "X连锁" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "常染色体" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "ADA" }, { "start_idx": 73, "end_idx": 77, "type": "bod", "entity": "腺苷脱氨酶" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "RAG" }, { "start_idx": 83, "end_idx": 88, "type": "bod", "entity": "重组活化基因" }, { "start_idx": 89, "end_idx": 95, "type": "dis", "entity": "联合免疫缺陷病" }, { "start_idx": 97, "end_idx": 120, "type": "dis", "entity": "combinedimmunodeficiency" }, { "start_idx": 127, "end_idx": 131, "type": "bod", "entity": "T和B细胞" }, { "start_idx": 127, "end_idx": 139, "type": "sym", "entity": "T和B细胞均可能有明显缺陷" }, { "start_idx": 146, "end_idx": 155, "type": "dis", "entity": "婴儿期严重致死性感染" }, { "start_idx": 157, "end_idx": 158, "type": "bod", "entity": "细胞" }, { "start_idx": 157, "end_idx": 168, "type": "sym", "entity": "细胞免疫和抗体反应均缺陷" }, { "start_idx": 162, "end_idx": 163, "type": "bod", "entity": "抗体" }, { "start_idx": 169, "end_idx": 177, "type": "sym", "entity": "外周血淋巴细胞减少" }, { "start_idx": 169, "end_idx": 175, "type": "bod", "entity": "外周血淋巴细胞" }, { "start_idx": 178, "end_idx": 182, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "1.严重联合免疫缺陷(severecombinedimmunodeficiency,SCID)(1)T细胞缺陷B细胞正常SCID(T-B+SCID):①X连锁T-B+SCID病因为定位于Xq13.1上的白细胞介素-2受体γ链(IL-2Rγ)基因突变导致SCID,且是SCID中较常见的病种。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "严重联合免疫缺陷" }, { "start_idx": 11, "end_idx": 40, "type": "dis", "entity": "severecombinedimmunodeficiency" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "SCID" }, { "start_idx": 50, "end_idx": 63, "type": "dis", "entity": "T细胞缺陷B细胞正常SCID" }, { "start_idx": 65, "end_idx": 84, "type": "dis", "entity": "T-B+SCID" }, { "start_idx": 88, "end_idx": 98, "type": "dis", "entity": "X连锁T-B+SCID" }, { "start_idx": 105, "end_idx": 135, "type": "sym", "entity": "Xq13.1上的白细胞介素-2受体γ链(IL-2Rγ)基因突变" }, { "start_idx": 105, "end_idx": 133, "type": "bod", "entity": "Xq13.1上的白细胞介素-2受体γ链(IL-2Rγ)基因" }, { "start_idx": 138, "end_idx": 141, "type": "dis", "entity": "SCID" }, { "start_idx": 145, "end_idx": 148, "type": "dis", "entity": "SCID" } ] }, { "text": "近期发现IL-2Rr也是IL-4、IL-7、IL-9和IL-15受体的共同γ链(γc),因此又称IL-2Rγc。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "IL-2Rr" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "IL-4" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "IL-7" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "IL-9" }, { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "IL-15受体" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "共同γ链" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "γc" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "IL-2Rγc" } ] }, { "text": "临床表现为早期反复、严重真菌、细菌和病毒感染及发生移植物抗宿主反应(GVHR)。", "entities": [ { "start_idx": 5, "end_idx": 21, "type": "sym", "entity": "早期反复、严重真菌、细菌和病毒感染" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "真菌" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "细菌" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "病毒感染" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "移植物抗宿主反应" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "GVHR" } ] }, { "text": "外周血T细胞缺乏或明显减少B细胞可正常或增高但血清IgM、IgA及IgG水平低淋巴细胞增殖活性低骨髓移植在本病的成功率可达90%,转rc基因治疗尚处于试验阶段。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "外周血T细胞缺乏或明显减少" }, { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "血T细胞" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "B细胞" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "B细胞可正常或增高" }, { "start_idx": 22, "end_idx": 38, "type": "sym", "entity": "但血清IgM、IgA及IgG水平低" }, { "start_idx": 23, "end_idx": 27, "type": "ite", "entity": "血清IgM" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "IgA" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "IgG" }, { "start_idx": 39, "end_idx": 42, "type": "ite", "entity": "淋巴细胞" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "淋巴细胞增殖活性低" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 65, "end_idx": 71, "type": "pro", "entity": "转rc基因治疗" } ] }, { "text": "②常染色体T-B+SCID是由于细胞内激酶Jak3基因突变。", "entities": [ { "start_idx": 1, "end_idx": 24, "type": "dis", "entity": "常染色体T-B+SCID" }, { "start_idx": 28, "end_idx": 38, "type": "bod", "entity": "细胞内激酶Jak3基因" }, { "start_idx": 28, "end_idx": 40, "type": "sym", "entity": "细胞内激酶Jak3基因突变" } ] }, { "text": "免疫学改变与临床表现同T-B+SCID。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "免疫学改变" }, { "start_idx": 11, "end_idx": 30, "type": "dis", "entity": "T-B+SCID" } ] }, { "text": "(2)T和B细胞均缺如SCID(T-B-SCID):①RAG-1/RAG-2缺陷致T-B-SCID为重组活化基因-1(RAG-1)或RAG-2突变所致,婴儿期即发病,外周血T和B细胞计数均明显减少但NK活性正常或升高11p13编码VDJ基因重组酶的RAG1/RAG2突变,使T细胞受体(TCR)和B细胞表面免疫球蛋白(SIg)的VDJ结构重组发生障碍,患者外周血T、B细胞均减少,患儿在生后2~3个月即发生严重的复发性感染。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "T和B细胞均缺如SCID" }, { "start_idx": 16, "end_idx": 35, "type": "dis", "entity": "T-B-SCID" }, { "start_idx": 39, "end_idx": 51, "type": "sym", "entity": "RAG-1/RAG-2缺陷" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "RAG-1" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "RAG-2" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "T-B-SCID" }, { "start_idx": 62, "end_idx": 84, "type": "sym", "entity": "重组活化基因-1(RAG-1)或RAG-2突变" }, { "start_idx": 62, "end_idx": 69, "type": "bod", "entity": "重组活化基因-1" }, { "start_idx": 71, "end_idx": 75, "type": "bod", "entity": "RAG-1" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "RAG-2" }, { "start_idx": 95, "end_idx": 109, "type": "sym", "entity": "外周血T和B细胞计数均明显减少" }, { "start_idx": 95, "end_idx": 104, "type": "ite", "entity": "外周血T和B细胞计数" }, { "start_idx": 111, "end_idx": 119, "type": "sym", "entity": "NK活性正常或升高" }, { "start_idx": 111, "end_idx": 112, "type": "bod", "entity": "NK" }, { "start_idx": 120, "end_idx": 146, "type": "sym", "entity": "11p13编码VDJ基因重组酶的RAG1/RAG2突变" }, { "start_idx": 120, "end_idx": 134, "type": "bod", "entity": "11p13编码VDJ基因重组酶" }, { "start_idx": 136, "end_idx": 144, "type": "bod", "entity": "RAG1/RAG2" }, { "start_idx": 149, "end_idx": 186, "type": "sym", "entity": "T细胞受体(TCR)和B细胞表面免疫球蛋白(SIg)的VDJ结构重组发生障碍" }, { "start_idx": 149, "end_idx": 153, "type": "bod", "entity": "T细胞受体" }, { "start_idx": 155, "end_idx": 157, "type": "bod", "entity": "TCR" }, { "start_idx": 160, "end_idx": 169, "type": "bod", "entity": "B细胞表面免疫球蛋白" }, { "start_idx": 171, "end_idx": 173, "type": "bod", "entity": "SIg" }, { "start_idx": 176, "end_idx": 178, "type": "bod", "entity": "VDJ" }, { "start_idx": 190, "end_idx": 200, "type": "sym", "entity": "外周血T、B细胞均减少" }, { "start_idx": 190, "end_idx": 197, "type": "ite", "entity": "外周血T、B细胞" }, { "start_idx": 218, "end_idx": 222, "type": "dis", "entity": "复发性感染" } ] }, { "text": "②腺苷脱氨酶(ADA)缺陷为ADA基因突变,ADA缺乏引起嘌呤旁路和甲基化旁路的中间代谢产物脱氧三磷酸腺苷(dATP)和S-腺苷同型半亮氨酸(S-adenosylhomocysteine)堆积,它们具有细胞毒效应,抑制T、B细胞增殖和分化。", "entities": [ { "start_idx": 1, "end_idx": 12, "type": "sym", "entity": "腺苷脱氨酶(ADA)缺陷" }, { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "腺苷脱氨酶" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "ADA" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "ADA基因突变" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "ADA基因" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "ADA缺乏" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "ADA" }, { "start_idx": 29, "end_idx": 95, "type": "sym", "entity": "嘌呤旁路和甲基化旁路的中间代谢产物脱氧三磷酸腺苷(dATP)和S-腺苷同型半亮氨酸(S-adenosylhomocysteine)堆积" }, { "start_idx": 29, "end_idx": 52, "type": "bod", "entity": "嘌呤旁路和甲基化旁路的中间代谢产物脱氧三磷酸腺苷" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "dATP" }, { "start_idx": 60, "end_idx": 69, "type": "bod", "entity": "S-腺苷同型半亮氨酸" }, { "start_idx": 71, "end_idx": 92, "type": "bod", "entity": "S-adenosylhomocysteine" }, { "start_idx": 101, "end_idx": 105, "type": "sym", "entity": "细胞毒效应" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "细胞" }, { "start_idx": 107, "end_idx": 118, "type": "sym", "entity": "抑制T、B细胞增殖和分化" }, { "start_idx": 109, "end_idx": 113, "type": "bod", "entity": "T、B细胞" } ] }, { "text": "多数病例早年发病,若基因突变位点影响ADA功能较少,即可在年长儿和成人发病,症状也轻。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "基因突变" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "ADA" } ] }, { "text": "各种T-B-SCID均为常染色体隐性遗传。", "entities": [ { "start_idx": 2, "end_idx": 21, "type": "dis", "entity": "T-B-SCID" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "常染色体" } ] }, { "text": "2.伴高IgM的免疫球蛋白缺乏症高IgM综合征(hyperIgMsyndrome,HM)70%为X连锁遗传,其余为常染色体隐性遗传;特点为B细胞内Ig转换障碍IgM正常或增高IgG、IgA和IgEX连锁高IgM血症因T细胞表面CD40配体基因突变,不能与B细胞表面CD40结合,使B细胞得不到活化刺激,是Ig转换障碍的原因。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "高IgM的免疫球蛋白缺乏症" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "高IgM综合征" }, { "start_idx": 24, "end_idx": 39, "type": "dis", "entity": "hyperIgMsyndrome" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "HM" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "X" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "常染色体" }, { "start_idx": 69, "end_idx": 78, "type": "sym", "entity": "B细胞内Ig转换障碍" }, { "start_idx": 69, "end_idx": 74, "type": "bod", "entity": "B细胞内Ig" }, { "start_idx": 79, "end_idx": 86, "type": "sym", "entity": "IgM正常或增高" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "IgM" }, { "start_idx": 87, "end_idx": 100, "type": "sym", "entity": "IgG、IgA和IgEX连锁" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "IgG" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "IgA" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "IgE" }, { "start_idx": 98, "end_idx": 98, "type": "bod", "entity": "X" }, { "start_idx": 101, "end_idx": 106, "type": "dis", "entity": "高IgM血症" }, { "start_idx": 108, "end_idx": 122, "type": "sym", "entity": "T细胞表面CD40配体基因突变" }, { "start_idx": 108, "end_idx": 120, "type": "bod", "entity": "T细胞表面CD40配体基因" }, { "start_idx": 124, "end_idx": 137, "type": "sym", "entity": "不能与B细胞表面CD40结合" }, { "start_idx": 127, "end_idx": 135, "type": "bod", "entity": "B细胞表面CD40" }, { "start_idx": 140, "end_idx": 149, "type": "sym", "entity": "B细胞得不到活化刺激" }, { "start_idx": 140, "end_idx": 142, "type": "bod", "entity": "B细胞" }, { "start_idx": 152, "end_idx": 157, "type": "sym", "entity": "Ig转换障碍" }, { "start_idx": 152, "end_idx": 153, "type": "bod", "entity": "Ig" } ] }, { "text": "实验室发现T、B细胞计数正常T细胞增殖反应正常依赖T细胞的B细胞增殖反应低下体外淋巴细胞培养中T细胞表达CD40L减少是诊断的要点之一,但要注意常见变异性免疫缺陷病(CVID)等其他一些病也有CD40L表达减少。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "sym", "entity": "T、B细胞计数正常" }, { "start_idx": 5, "end_idx": 11, "type": "ite", "entity": "T、B细胞计数" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "T细胞增殖反应正常" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "T细胞" }, { "start_idx": 23, "end_idx": 37, "type": "sym", "entity": "依赖T细胞的B细胞增殖反应低下" }, { "start_idx": 23, "end_idx": 31, "type": "bod", "entity": "依赖T细胞的B细胞" }, { "start_idx": 38, "end_idx": 45, "type": "pro", "entity": "体外淋巴细胞培养" }, { "start_idx": 47, "end_idx": 58, "type": "sym", "entity": "T细胞表达CD40L减少" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "T细胞" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "CD40L" }, { "start_idx": 74, "end_idx": 81, "type": "dis", "entity": "变异性免疫缺陷病" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "CVID" }, { "start_idx": 96, "end_idx": 104, "type": "sym", "entity": "CD40L表达减少" }, { "start_idx": 96, "end_idx": 100, "type": "bod", "entity": "CD40L" } ] }, { "text": "CD40L基因突变分析可确诊。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "CD40L基因突变分析" } ] }, { "text": "3.嘌呤核苷磷酸化酶(purinenucleosidephosphorylase,PNP)缺陷PNP缺乏致毒性中间代谢产物脱氧三磷酸鸟苷累积,对淋巴细胞,尤其是T细胞损伤尤为严重。", "entities": [ { "start_idx": 2, "end_idx": 46, "type": "dis", "entity": "嘌呤核苷磷酸化酶(purinenucleosidephosphorylase,PNP)缺陷" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "PNP缺乏" }, { "start_idx": 52, "end_idx": 69, "type": "sym", "entity": "致毒性中间代谢产物脱氧三磷酸鸟苷累积" }, { "start_idx": 52, "end_idx": 67, "type": "bod", "entity": "致毒性中间代谢产物脱氧三磷酸鸟苷" }, { "start_idx": 71, "end_idx": 88, "type": "sym", "entity": "对淋巴细胞,尤其是T细胞损伤尤为严重" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "T细胞" } ] }, { "text": "(二)以抗体缺陷为主的免疫缺陷病(表17-4)抗体缺陷可能是B细胞本身发育障碍,也可能是正常B细胞未能接受到缺陷T辅助细胞的协同信号刺激所致,因而将过去分类中的抗体缺陷病改为以抗体缺陷为主的免疫缺陷病。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "以抗体缺陷为主的免疫缺陷病" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "抗体缺陷" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "B细胞本身发育障碍" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "B细胞" }, { "start_idx": 44, "end_idx": 67, "type": "sym", "entity": "正常B细胞未能接受到缺陷T辅助细胞的协同信号刺激" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "正常B细胞" }, { "start_idx": 54, "end_idx": 60, "type": "bod", "entity": "缺陷T辅助细胞" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "抗体缺陷病" }, { "start_idx": 87, "end_idx": 99, "type": "dis", "entity": "以抗体缺陷为主的免疫缺陷病" } ] }, { "text": "其主要临床表现是反复化脓性感染。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "反复化脓性感染" } ] }, { "text": "表17-4以抗体缺陷为主的免疫缺陷病续表注:XLA:X连锁无丙种球蛋白血症;CVID:常见变异型免疫缺陷病;Igk:免疫球蛋白κ链1.X连锁无丙种球蛋白血症(Xlinkedagammaglobulinaemia,XLA)本病又称Bruton病,因定位于Xq12.3-3上的Bruton酪氨酸激酶(Btk)基因缺失或突变,使B细胞发育受阻于原B细胞,极少成熟B细胞(CD20+,CD19+,SIg+B细胞少于2%)。", "entities": [ { "start_idx": 5, "end_idx": 17, "type": "dis", "entity": "以抗体缺陷为主的免疫缺陷病" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "XLA" }, { "start_idx": 26, "end_idx": 36, "type": "dis", "entity": "X连锁无丙种球蛋白血症" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "CVID" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "变异型免疫缺陷病" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "Igk" }, { "start_idx": 58, "end_idx": 64, "type": "bod", "entity": "免疫球蛋白κ链" }, { "start_idx": 67, "end_idx": 77, "type": "dis", "entity": "X连锁无丙种球蛋白血症" }, { "start_idx": 79, "end_idx": 104, "type": "dis", "entity": "Xlinkedagammaglobulinaemia" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "XLA" }, { "start_idx": 114, "end_idx": 120, "type": "dis", "entity": "Bruton病" }, { "start_idx": 123, "end_idx": 158, "type": "sym", "entity": "定位于Xq12.3-3上的Bruton酪氨酸激酶(Btk)基因缺失或突变" }, { "start_idx": 126, "end_idx": 133, "type": "bod", "entity": "Xq12.3-3" }, { "start_idx": 142, "end_idx": 153, "type": "bod", "entity": "酪氨酸激酶(Btk)基因" }, { "start_idx": 161, "end_idx": 163, "type": "bod", "entity": "B细胞" }, { "start_idx": 161, "end_idx": 172, "type": "sym", "entity": "B细胞发育受阻于原B细胞" }, { "start_idx": 169, "end_idx": 172, "type": "bod", "entity": "原B细胞" }, { "start_idx": 174, "end_idx": 180, "type": "sym", "entity": "极少成熟B细胞" }, { "start_idx": 176, "end_idx": 180, "type": "bod", "entity": "成熟B细胞" }, { "start_idx": 182, "end_idx": 222, "type": "sym", "entity": "CD20+,CD19+,SIg+B细胞少于2%" }, { "start_idx": 182, "end_idx": 192, "type": "bod", "entity": "CD20+" }, { "start_idx": 194, "end_idx": 204, "type": "bod", "entity": "CD19+" }, { "start_idx": 206, "end_idx": 218, "type": "bod", "entity": "SIg+B细胞" } ] }, { "text": "表现为外周血B细胞极少或缺如淋巴细胞,B细胞数少于5个),浆细胞亦缺乏淋巴器官生发中心缺如血IgM、IgG和IgA均明显下降或缺如IgG<2g/L,IgA<0.1g/L);T细胞数量和功能正常Btk蛋白表达功能也各异,临床表现轻重不一,因此凡男孩低Ig血症者,均应进行Btk基因筛查。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "外周血B细胞极少或缺如" }, { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "外周血B细胞" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "淋巴细胞,B细胞数少于5个" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "淋巴细胞" }, { "start_idx": 19, "end_idx": 22, "type": "ite", "entity": "B细胞数" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "浆细胞亦缺乏" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "浆细胞" }, { "start_idx": 35, "end_idx": 44, "type": "sym", "entity": "淋巴器官生发中心缺如" }, { "start_idx": 35, "end_idx": 42, "type": "ite", "entity": "淋巴器官生发中心" }, { "start_idx": 45, "end_idx": 64, "type": "sym", "entity": "血IgM、IgG和IgA均明显下降或缺如" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "血IgM" }, { "start_idx": 50, "end_idx": 52, "type": "ite", "entity": "IgG" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "IgA" }, { "start_idx": 65, "end_idx": 72, "type": "sym", "entity": "IgG<2g/L" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "IgG" }, { "start_idx": 74, "end_idx": 83, "type": "sym", "entity": "IgA<0.1g/L" }, { "start_idx": 74, "end_idx": 76, "type": "ite", "entity": "IgA" }, { "start_idx": 86, "end_idx": 95, "type": "sym", "entity": "T细胞数量和功能正常" }, { "start_idx": 86, "end_idx": 88, "type": "ite", "entity": "T细胞" }, { "start_idx": 96, "end_idx": 107, "type": "sym", "entity": "Btk蛋白表达功能也各异" }, { "start_idx": 96, "end_idx": 100, "type": "ite", "entity": "Btk蛋白" }, { "start_idx": 123, "end_idx": 127, "type": "dis", "entity": "低Ig血症" }, { "start_idx": 134, "end_idx": 140, "type": "pro", "entity": "Btk基因筛查" } ] }, { "text": "多数患儿在生后6~12个月时发生反复化脓性感染,以呼吸道为主,也有全身性感染。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "反复化脓性感染" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "以呼吸道为主" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "呼吸道" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "全身性感染" } ] }, { "text": "Btk基因分析可确诊本病,有1/3患儿找不到阳性家族史。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "Btk基因分析" } ] }, { "text": "终身IVIG治疗本病有效,骨髓干细胞移植基因治疗在探索之中。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "终身IVIG" }, { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "骨髓干细胞移植" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "基因治疗" } ] }, { "text": "2.选择性IgG亚类缺陷血清1~2种IgG亚类浓度低于同龄儿童2个标准差时可考虑IgG亚类缺陷。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "选择性IgG亚类缺陷" }, { "start_idx": 12, "end_idx": 35, "type": "sym", "entity": "血清1~2种IgG亚类浓度低于同龄儿童2个标准差" }, { "start_idx": 12, "end_idx": 24, "type": "ite", "entity": "血清1~2种IgG亚类浓度" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "IgG亚类缺陷" } ] }, { "text": "由于IgG1占总IgG的70%,因此IgG1缺陷总是伴有总IgG下降IgG3水平低下常见于成人病例,而儿童则常伴IgG2低下。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "IgG1" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "总IgG" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "IgG1缺陷" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "总IgG下降" }, { "start_idx": 28, "end_idx": 31, "type": "ite", "entity": "总IgG" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "IgG3" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "gG3水平低下" }, { "start_idx": 56, "end_idx": 59, "type": "ite", "entity": "IgG2" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "IgG2低下" } ] }, { "text": "我国儿童IgG亚类缺陷以IgG3为主。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "儿童IgG亚类缺陷" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "IgG3" } ] }, { "text": "IgG4占总IgG的5%以下,正常儿童有时也难以测出,故不宜诊断单纯IgG4缺陷,当IgG2和IgG4联合缺陷时,不能产生对多糖抗原的抗体,如流感杆菌、脑膜炎球菌和肺炎球菌的抗体。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "IgG4" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "总IgG" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "单纯IgG4缺陷" }, { "start_idx": 42, "end_idx": 54, "type": "dis", "entity": "IgG2和IgG4联合缺陷" }, { "start_idx": 62, "end_idx": 68, "type": "bod", "entity": "多糖抗原的抗体" }, { "start_idx": 71, "end_idx": 88, "type": "bod", "entity": "流感杆菌、脑膜炎球菌和肺炎球菌的抗体" } ] }, { "text": "3.常见变异型免疫缺陷病(commonvariableimmunodeficiency,CVID)为一组病因不明,表现为Ig低下的综合征。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "变异型免疫缺陷病" }, { "start_idx": 13, "end_idx": 42, "type": "dis", "entity": "commonvariableimmunodeficiency" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "CVID" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "Ig" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "Ig低下" } ] }, { "text": "多基因遗传理论有待证实,部分IgA缺乏病人可转变为CVID,提示两病可能源于同一基因位点缺陷。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "多基因" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "IgA缺乏" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "CVID" }, { "start_idx": 38, "end_idx": 45, "type": "sym", "entity": "同一基因位点缺陷" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "同一基因位点" } ] }, { "text": "与XLA的外周淋巴器官萎缩相反,CVID患儿外周淋巴结肿大脾肿大自身免疫性疾病、淋巴系统肿瘤和胃肠道恶性瘤的发生率很高。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "XLA" }, { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "外周淋巴器官萎缩" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "CVID" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "外周淋巴结肿大" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "外周淋巴结" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "脾" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "脾肿大" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "淋巴系统肿瘤" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "胃肠道恶性瘤" } ] }, { "text": "CVID常发病于年长儿或成人,男女均可发病,这些不同于XLA。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "CVID" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "XLA" } ] }, { "text": "反复呼吸道感染为其特征,包括鼻窦炎、肺炎和支气管扩张,可发展为慢性阻塞性肺部疾病。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "反复呼吸道感染" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肺炎" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "慢性阻塞性肺部疾病" } ] }, { "text": "也易患幽门螺杆菌、梨形鞭毛虫等胃肠道感染和肠病毒性脑膜炎。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "梨形鞭毛虫" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "胃肠道感染" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "肠病毒性脑膜炎" } ] }, { "text": "多数CVID患儿的外周血B细胞数量大致正常B细胞不能分化为产生Ig的浆细胞,少数CVID患儿外周血B细胞数量减少甚至难以测出,此种情况应与XLA鉴别。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "CVID" }, { "start_idx": 9, "end_idx": 16, "type": "ite", "entity": "外周血B细胞数量" }, { "start_idx": 19, "end_idx": 36, "type": "sym", "entity": "正常B细胞不能分化为产生Ig的浆细胞" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "正常B细胞" }, { "start_idx": 29, "end_idx": 36, "type": "bod", "entity": "产生Ig的浆细胞" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "CVID" }, { "start_idx": 46, "end_idx": 55, "type": "sym", "entity": "外周血B细胞数量减少" }, { "start_idx": 46, "end_idx": 53, "type": "ite", "entity": "外周血B细胞数量" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "XLA" } ] }, { "text": "近年的研究逐渐认识到部分CVID的发病机制与T细胞调节网络失常有关,部分患儿CD8+T细胞增多T细胞减少CD4/CD8T细胞比值下降T细胞分泌的干扰素γ可抑制B细胞合成和分泌Ig,CVID的遗传方式不定,可为常染色体隐性或显性,也可为X连锁,但更常见为无遗传家系的散发病例。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "CVID" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "T细胞" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "T细胞调节网络失常" }, { "start_idx": 38, "end_idx": 52, "type": "sym", "entity": "CD8+T细胞增多" }, { "start_idx": 38, "end_idx": 50, "type": "ite", "entity": "CD8+T细胞" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "T细胞" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "T细胞减少" }, { "start_idx": 58, "end_idx": 71, "type": "sym", "entity": "CD4/CD8T细胞比值下降" }, { "start_idx": 58, "end_idx": 69, "type": "ite", "entity": "CD4/CD8T细胞比值" }, { "start_idx": 72, "end_idx": 81, "type": "bod", "entity": "T细胞分泌的干扰素γ" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "B细胞" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "Ig" }, { "start_idx": 96, "end_idx": 99, "type": "dis", "entity": "CVID" }, { "start_idx": 110, "end_idx": 113, "type": "bod", "entity": "常染色体" }, { "start_idx": 123, "end_idx": 125, "type": "bod", "entity": "X连锁" } ] }, { "text": "病变严重度一般低于XLA。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "XLA" } ] }, { "text": "诊断依赖于排除其他原发性免疫缺陷病。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "原发性免疫缺陷病" } ] }, { "text": "IVIG替代治疗可减轻感染的严重度。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "IVIG" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "替代治疗" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "感染" } ] }, { "text": "4.IgA缺陷IgA缺陷病是较为常见的PID,但人群发病率有差异,白种人发病率约1/500~1/1500,日本人为1/18500,中国人约为1/5000~1/10000。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "IgA缺陷" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "IgA缺陷病" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "PID" } ] }, { "text": "本病发病机制不明,可能与TH2细胞对B细胞合成IgA调控失调有关,目前没有发现有IgA基因缺失或突变,部分病例为常染色体隐性或显性遗传。", "entities": [ { "start_idx": 12, "end_idx": 22, "type": "bod", "entity": "TH2细胞" }, { "start_idx": 13, "end_idx": 35, "type": "sym", "entity": "H2细胞对B细胞合成IgA调控失调" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "B细胞" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "IgA" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "IgA基因" }, { "start_idx": 46, "end_idx": 55, "type": "sym", "entity": "IgA基因缺失或突变" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "常染色体" } ] }, { "text": "轻症患儿可无症状或在婴儿期发生反复呼吸道感染肠道、泌尿道感染血清IgA可逐渐升至正常水平。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "肠道、泌尿道感染" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "血清IgA" } ] }, { "text": "可伴发自身免疫性疾病、哮喘和肠吸收不良血清IgA低于0.05IgM及IgG正常或升高,分泌型IgA也明显减少丙种球蛋白,因其含有微量IgA,可诱导患儿产生抗IgA抗体(属IgG2类),但所幸的是相当一部分IgA缺陷病患儿同时伴发IgG2亚类缺陷,这些人一般不会发生过敏反应。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "哮喘" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肠吸收不良" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "血清IgA" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "血清IgA低于0.05" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "IgM" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "IgG" }, { "start_idx": 43, "end_idx": 53, "type": "sym", "entity": "分泌型IgA也明显减少" }, { "start_idx": 43, "end_idx": 48, "type": "ite", "entity": "分泌型IgA" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "丙种球蛋白" }, { "start_idx": 64, "end_idx": 68, "type": "ite", "entity": "微量IgA" }, { "start_idx": 77, "end_idx": 90, "type": "bod", "entity": "抗IgA抗体(属IgG2类)" }, { "start_idx": 102, "end_idx": 107, "type": "dis", "entity": "IgA缺陷病" }, { "start_idx": 114, "end_idx": 121, "type": "dis", "entity": "IgG2亚类缺陷" }, { "start_idx": 132, "end_idx": 135, "type": "sym", "entity": "过敏反应" } ] }, { "text": "5.婴儿暂时性低丙种球蛋白血症正常婴儿3~4个月时因母体传输来的IgG已消失,血清IgG呈最低水平,随之自身产生的IgG逐渐增多。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "婴儿暂时性低丙种球蛋白血症" }, { "start_idx": 26, "end_idx": 34, "type": "bod", "entity": "母体传输来的IgG" }, { "start_idx": 39, "end_idx": 43, "type": "ite", "entity": "血清IgG" }, { "start_idx": 52, "end_idx": 63, "type": "sym", "entity": "自身产生的IgG逐渐增多" }, { "start_idx": 52, "end_idx": 59, "type": "ite", "entity": "自身产生的IgG" } ] }, { "text": "婴儿暂时性低丙种球蛋白血症(transienthypogammaglobulinemiaofinfant)患儿不能及时产生IgG,故血清IgG水平持续低下。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "婴儿暂时性低丙种球蛋白血症" }, { "start_idx": 14, "end_idx": 51, "type": "dis", "entity": "transienthypogammaglobulinemiaofinfant" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "不能及时产生IgG" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "IgG" }, { "start_idx": 66, "end_idx": 76, "type": "sym", "entity": "血清IgG水平持续低下" }, { "start_idx": 66, "end_idx": 72, "type": "ite", "entity": "血清IgG水平" } ] }, { "text": "(三)以T细胞缺陷为主的免疫缺陷病本组疾病多为新近发现,其分子遗传学和病因学尚不清楚的疾病。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "T细胞缺陷" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "免疫缺陷病" } ] }, { "text": "1.CD4+T细胞缺陷外周血CD4+T细胞计数减少细胞免疫功能低下而血清Ig水平正常或偏高易患隐球菌脑膜炎及念珠菌T细胞缺陷T细胞缺乏IL-2缺陷IL-2mRNA转录表达障碍。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "dis", "entity": "CD4+T细胞缺陷" }, { "start_idx": 17, "end_idx": 36, "type": "sym", "entity": "外周血CD4+T细胞计数减少" }, { "start_idx": 17, "end_idx": 34, "type": "ite", "entity": "外周血CD4+T细胞计数" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "细胞" }, { "start_idx": 37, "end_idx": 44, "type": "sym", "entity": "细胞免疫功能低下" }, { "start_idx": 46, "end_idx": 51, "type": "ite", "entity": "血清Ig水平" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "隐球菌脑膜炎" }, { "start_idx": 66, "end_idx": 73, "type": "dis", "entity": "念珠菌T细胞缺陷" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "T细胞缺乏" }, { "start_idx": 79, "end_idx": 84, "type": "dis", "entity": "IL-2缺陷" }, { "start_idx": 85, "end_idx": 98, "type": "dis", "entity": "IL-2mRNA转录表达障碍" } ] }, { "text": "4.多细胞因子缺陷IL-2、IL-4和IL-5缺陷,缺乏活化T细胞的核因子(NFAT)。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "多细胞因子缺陷" }, { "start_idx": 9, "end_idx": 24, "type": "dis", "entity": "IL-2、IL-4和IL-5缺陷" }, { "start_idx": 26, "end_idx": 42, "type": "sym", "entity": "缺乏活化T细胞的核因子(NFAT)" }, { "start_idx": 28, "end_idx": 36, "type": "bod", "entity": "活化T细胞的核因子" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "NFAT" } ] }, { "text": "5.信息传递障碍当给予抗原刺激后,T细胞钙内流和二酰甘油(DAG)表达障碍,临床表现与SCID或CVID相似。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "信息传递障碍" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "抗原" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "T细胞" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "T细胞钙内流" }, { "start_idx": 24, "end_idx": 36, "type": "sym", "entity": "二酰甘油(DAG)表达障碍" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "二酰甘油" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "DAG" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "SCID" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "CVID" } ] }, { "text": "6.钙内流障碍T细胞钙内流机制失调SCID。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "钙内流障碍" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "T细胞" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "T细胞钙内流机制失调" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "SCID" } ] }, { "text": "(四)免疫缺陷合并其他重要特征这类疾病除免疫缺陷外,尚有非常突出的其他临床表现。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "免疫缺陷" } ] }, { "text": "1.湿疹-血小板减少伴免疫缺陷(WiskottAldrichsyndrome,WAS)WAS蛋白(WASP)编码基因位于Xq11.22,WASP存在于造血干细胞及由其分化而来的细胞的胞浆中,其功能尚不清楚,可能与细胞内信息传递和细胞骨架再组建等功能有关。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "湿疹-血小板减少伴免疫缺陷" }, { "start_idx": 16, "end_idx": 37, "type": "dis", "entity": "WiskottAldrichsyndrome" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "WAS" }, { "start_idx": 43, "end_idx": 57, "type": "bod", "entity": "WAS蛋白(WASP)编码基因" }, { "start_idx": 60, "end_idx": 66, "type": "bod", "entity": "Xq11.22" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "WASP" }, { "start_idx": 75, "end_idx": 93, "type": "bod", "entity": "造血干细胞及由其分化而来的细胞的胞浆中" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "细胞内" }, { "start_idx": 114, "end_idx": 117, "type": "bod", "entity": "细胞骨架" } ] }, { "text": "WASP基因突变或缺失,使淋巴细胞及血小板功能异常。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "WASP基因" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "WASP基因突变或缺失" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 13, "end_idx": 24, "type": "sym", "entity": "淋巴细胞及血小板功能异常" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "血小板" } ] }, { "text": "早期表现为出生后即有出血倾向,皮肤瘀点,血便,颅内出血;湿疹可轻可重,可局限于面部;肝脾肿大反复或慢性感染淋巴瘤和自身免疫性血管炎发生率高。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "出血倾向" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "皮肤瘀点" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "血便" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "便" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "颅内出血" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "湿疹" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "面部" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肝" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "脾" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "慢性感染" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 57, "end_idx": 64, "type": "dis", "entity": "自身免疫性血管炎" } ] }, { "text": "实验室发现IgM下降IgA、IgE上升IgG正常抗体反应差抗同族凝集素效价低下血小板数量减少骨髓移植前,多数患儿在3岁左右因严重出血或感染死亡。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "实验室发现IgM下降" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "IgM" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "IgA" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "IgA、IgE上升" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "IgE" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "IgG" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "抗体" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "抗体反应差" }, { "start_idx": 29, "end_idx": 36, "type": "ite", "entity": "抗同族凝集素效价" }, { "start_idx": 29, "end_idx": 38, "type": "sym", "entity": "抗同族凝集素效价低下" }, { "start_idx": 39, "end_idx": 43, "type": "ite", "entity": "血小板数量" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "血小板数量减少" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "严重出血" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "血" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "感染" } ] }, { "text": "2.共济失调毛细血管扩张综合征(ataxiatelangiectasia,AT)进行性小脑共济失调毛细血管扩张耳垂和球结合膜。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "共济失调毛细血管扩张综合征" }, { "start_idx": 16, "end_idx": 35, "type": "dis", "entity": "ataxiatelangiectasia" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "AT" }, { "start_idx": 40, "end_idx": 48, "type": "dis", "entity": "进行性小脑共济失调" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "毛细血管" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "毛细血管扩张" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "耳垂" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "球结合膜" } ] }, { "text": "血清甲胎蛋白增高见于95%的病例。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "血清甲胎蛋白" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "血清甲胎蛋白增高" } ] }, { "text": "不同程度血清IgG2、IgG4、IgA和IgE下降甚或缺如抗体反应下降T细胞数量和功能多有减弱逐渐出现反复呼吸道感染淋巴细胞对放射线非常敏感,其DNA损伤不易修复。", "entities": [ { "start_idx": 0, "end_idx": 28, "type": "sym", "entity": "不同程度血清IgG2、IgG4、IgA和IgE下降甚或缺如" }, { "start_idx": 4, "end_idx": 22, "type": "ite", "entity": "血清IgG2、IgG4、IgA和IgE" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "抗体反应下降" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "抗体" }, { "start_idx": 35, "end_idx": 46, "type": "sym", "entity": "T细胞数量和功能多有减弱" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "T细胞" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "DNA损伤" } ] }, { "text": "病人易患肿瘤,且常为其死因。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肿瘤" } ] }, { "text": "3.胸腺发育不全综合征(DiGeorgesyndrome)为一系列基因异常综合征中的一部分,约80%~90%病例伴有染色体22q11-ter缺失心脏畸形cardiacabnormalities)、面部异常abnormalfacies)、胸腺发育不良thymichypoplasia)、腭裂cleftpalate)和低钙血症(hypocalcemia),故称为“CATCH22”。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "胸腺发育不全综合征" }, { "start_idx": 12, "end_idx": 27, "type": "dis", "entity": "DiGeorgesyndrome" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "一系列基因异常" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "一系列基因" }, { "start_idx": 58, "end_idx": 71, "type": "dis", "entity": "染色体22q11-ter缺失" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "心脏畸形" }, { "start_idx": 76, "end_idx": 95, "type": "dis", "entity": "cardiacabnormalities" }, { "start_idx": 98, "end_idx": 101, "type": "sym", "entity": "面部异常" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "面部" }, { "start_idx": 102, "end_idx": 115, "type": "sym", "entity": "abnormalfacies" }, { "start_idx": 118, "end_idx": 123, "type": "dis", "entity": "胸腺发育不良" }, { "start_idx": 124, "end_idx": 139, "type": "dis", "entity": "thymichypoplasia" }, { "start_idx": 142, "end_idx": 143, "type": "dis", "entity": "腭裂" }, { "start_idx": 144, "end_idx": 154, "type": "dis", "entity": "cleftpalate" }, { "start_idx": 157, "end_idx": 160, "type": "dis", "entity": "低钙血症" }, { "start_idx": 162, "end_idx": 173, "type": "dis", "entity": "hypocalcemia" }, { "start_idx": 180, "end_idx": 186, "type": "dis", "entity": "CATCH22" } ] }, { "text": "胸腺缺如使T细胞,尤其是CD8+T细胞数量减少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胸腺" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "胸腺缺如" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "T细胞" }, { "start_idx": 5, "end_idx": 28, "type": "sym", "entity": "T细胞,尤其是CD8+T细胞数量减少" }, { "start_idx": 12, "end_idx": 24, "type": "bod", "entity": "CD8+T细胞" } ] }, { "text": "患儿易于病毒感染;因甲状旁腺功能低下,患儿生后将发生低钙惊厥;Ⅰ~Ⅱ咽弓受累时,出现特殊面容眼距宽鼻梁开坦小嘴及耳位低Ⅲ~Ⅳ咽弓发育不良导致先天性心脏病,如大血管转位及法洛四联症。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "病毒感染" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "甲状旁腺功能低下" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "低钙惊厥" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "Ⅰ~Ⅱ咽弓" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "特殊面容" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "面" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "眼" }, { "start_idx": 46, "end_idx": 48, "type": "sym", "entity": "眼距宽" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "鼻" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "鼻梁开坦" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "小嘴" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "小嘴及耳位低" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "耳" }, { "start_idx": 59, "end_idx": 67, "type": "dis", "entity": "Ⅲ~Ⅳ咽弓发育不良" }, { "start_idx": 70, "end_idx": 75, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "大血管转位" }, { "start_idx": 84, "end_idx": 88, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "尽管胸腺体积变小或萎缩而代以外胚叶组织,但本病免疫缺陷表现轻,仅约20%的病例出现T细胞功能异常,多数患儿随年龄增长,T细胞缺陷可自行恢复至正常。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胸腺" }, { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "胸腺体积变小或萎缩" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "外胚叶组织" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "免疫缺陷" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "T细胞" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "T细胞功能异常" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "T细胞缺陷" } ] }, { "text": "先心畸形可行手术治疗,早年反复感染可给予抗感染和对症治疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "先心畸形" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "手术治疗" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "感染" }, { "start_idx": 20, "end_idx": 27, "type": "pro", "entity": "抗感染和对症治疗" } ] }, { "text": "骨髓和胸腺细胞移植已有成功报告。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "骨髓和胸腺细胞移植" } ] }, { "text": "(五)吞噬细胞数量和功能缺陷1.严重先天性中性粒细胞减少症(SCN;Kostmann综合征)外周血中性粒细胞集落刺激因子(G-CSF)受体基因发生转位,而不能表达G-CSF受体蛋白。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "吞噬细胞数量和功能缺陷" }, { "start_idx": 16, "end_idx": 28, "type": "dis", "entity": "严重先天性中性粒细胞减少症" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "SCN" }, { "start_idx": 34, "end_idx": 44, "type": "dis", "entity": "Kostmann综合征" }, { "start_idx": 46, "end_idx": 74, "type": "sym", "entity": "外周血中性粒细胞集落刺激因子(G-CSF)受体基因发生转位" }, { "start_idx": 46, "end_idx": 70, "type": "bod", "entity": "外周血中性粒细胞集落刺激因子(G-CSF)受体基因" }, { "start_idx": 77, "end_idx": 89, "type": "sym", "entity": "不能表达G-CSF受体蛋白" }, { "start_idx": 81, "end_idx": 89, "type": "bod", "entity": "G-CSF受体蛋白" } ] }, { "text": "一些病例发生粒细胞再生障碍或粒细胞性急性白血病。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "粒细胞再生障碍" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "粒细胞性急性白血病" } ] }, { "text": "2.慢性肉芽肿病(chronicgranulomatous,CGD)为一种少见的原发性吞噬细胞功能缺陷病,编码吞噬细胞还原型辅酶Ⅱ(NAPDH)氧化酶基因缺陷,导致吞噬细胞不能杀灭过氧化物酶阳性细菌和真菌,临床表现以反复发生严重感染以及在反复感染部位形成肉芽肿为特征。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "慢性肉芽肿病" }, { "start_idx": 9, "end_idx": 28, "type": "dis", "entity": "chronicgranulomatous" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "CGD" }, { "start_idx": 40, "end_idx": 51, "type": "dis", "entity": "原发性吞噬细胞功能缺陷病" }, { "start_idx": 53, "end_idx": 78, "type": "sym", "entity": "编码吞噬细胞还原型辅酶Ⅱ(NAPDH)氧化酶基因缺陷" }, { "start_idx": 53, "end_idx": 76, "type": "bod", "entity": "编码吞噬细胞还原型辅酶Ⅱ(NAPDH)氧化酶基因" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 90, "end_idx": 101, "type": "mic", "entity": "过氧化物酶阳性细菌和真菌" }, { "start_idx": 114, "end_idx": 115, "type": "dis", "entity": "感染" }, { "start_idx": 121, "end_idx": 122, "type": "dis", "entity": "感染" }, { "start_idx": 125, "end_idx": 129, "type": "sym", "entity": "形成肉芽肿" }, { "start_idx": 127, "end_idx": 129, "type": "dis", "entity": "肉芽肿" } ] }, { "text": "常见病原菌为金黄色葡萄球菌、大肠杆菌、沙门菌属、假单胞菌属和真菌(特别是曲霉菌),近年亦常发现分枝杆菌及结核杆菌感染。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "病原菌" }, { "start_idx": 6, "end_idx": 12, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 14, "end_idx": 17, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 19, "end_idx": 22, "type": "mic", "entity": "沙门菌属" }, { "start_idx": 24, "end_idx": 28, "type": "mic", "entity": "假单胞菌属" }, { "start_idx": 30, "end_idx": 31, "type": "mic", "entity": "真菌" }, { "start_idx": 36, "end_idx": 38, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 47, "end_idx": 57, "type": "dis", "entity": "分枝杆菌及结核杆菌感染" } ] }, { "text": "卡介苗接种后引起播散性卡介苗病者近年亦不少见,严重者危及生命。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "卡介苗" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "接种" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "播散性卡介苗病" } ] }, { "text": "2/3的病例于1岁内发病,肺部、皮肤及消化道感染最为常见,脓肿可发生在机体任何部位。", "entities": [ { "start_idx": 13, "end_idx": 23, "type": "dis", "entity": "肺部、皮肤及消化道感染" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "脓肿" } ] }, { "text": "吞噬细胞杀菌功能低下形成本病的特有表现;大量淋巴细胞,组织细胞聚集的肉芽肿,位于各个部位,出现相应的临床表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "吞噬细胞杀菌功能低下" }, { "start_idx": 5, "end_idx": 5, "type": "mic", "entity": "菌" }, { "start_idx": 20, "end_idx": 36, "type": "bod", "entity": "大量淋巴细胞,组织细胞聚集的肉芽肿" } ] }, { "text": "CGD可分为X-连锁和常染色体隐性遗传两类。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "CGD" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "X-连锁" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "常染色体" } ] }, { "text": "X-连锁的CGD临床表现最重,而常染色体隐性遗传CGD则症状轻微。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "X-连锁的CGD" }, { "start_idx": 16, "end_idx": 26, "type": "dis", "entity": "常染色体隐性遗传CGD" } ] }, { "text": "X-连锁CGD系CYBB基因突变所致,CYBB基因编码NADPH氧化酶的细胞色素b558的gp91phox基因,突变导致吞噬细胞不能产生超氧根、单态氧和H2O2,部分病例为Xp21缺失所致。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "X-连锁CGD" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "CYBB基因突变" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "CYBB基因" }, { "start_idx": 19, "end_idx": 34, "type": "bod", "entity": "CYBB基因编码NADPH氧化酶" }, { "start_idx": 36, "end_idx": 43, "type": "bod", "entity": "细胞色素b558" }, { "start_idx": 45, "end_idx": 54, "type": "bod", "entity": "gp91phox基因" }, { "start_idx": 56, "end_idx": 91, "type": "sym", "entity": "突变导致吞噬细胞不能产生超氧根、单态氧和H2O2" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "超氧根" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "单态氧" }, { "start_idx": 76, "end_idx": 91, "type": "bod", "entity": "H2O2" }, { "start_idx": 98, "end_idx": 103, "type": "sym", "entity": "Xp21缺失" }, { "start_idx": 98, "end_idx": 101, "type": "bod", "entity": "Xp21" } ] }, { "text": "常染色体隐性CGD见于CYBA、NCF2及NCF1基因突变,分别导致细胞色素b558的p22phox亚基失活,或NADPH氧化酶p67phox或p47phox亚基缺陷。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "常染色体隐性CGD" }, { "start_idx": 11, "end_idx": 28, "type": "sym", "entity": "CYBA、NCF2及NCF1基因突变" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "CYBA" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "NCF2" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "NCF1基因" }, { "start_idx": 34, "end_idx": 53, "type": "sym", "entity": "细胞色素b558的p22phox亚基失活" }, { "start_idx": 34, "end_idx": 41, "type": "bod", "entity": "细胞色素b558" }, { "start_idx": 43, "end_idx": 51, "type": "bod", "entity": "p22phox亚基" }, { "start_idx": 56, "end_idx": 82, "type": "sym", "entity": "NADPH氧化酶p67phox或p47phox亚基缺陷" }, { "start_idx": 56, "end_idx": 70, "type": "bod", "entity": "NADPH氧化酶p67phox" }, { "start_idx": 72, "end_idx": 80, "type": "bod", "entity": "p47phox亚基" } ] }, { "text": "四唑氮蓝染料试验(NBT)可作为诊断本病初筛,进一步确诊有赖于NADPH氧化酶活性测定及基因突变分析。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "四唑氮蓝染料试验" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "NBT" }, { "start_idx": 31, "end_idx": 42, "type": "pro", "entity": "NADPH氧化酶活性测定" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "基因突变分析" } ] }, { "text": "约50%CGD患者于30岁前死于感染,治疗原则是预防和治疗感染(如磺胺增效剂和其他敏感的抗生素,保持室内干燥,以免发生真菌性感染)。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "CGD" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "感染" }, { "start_idx": 24, "end_idx": 30, "type": "pro", "entity": "预防和治疗感染" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "磺胺增效剂" }, { "start_idx": 39, "end_idx": 46, "type": "dru", "entity": "其他敏感的抗生素" }, { "start_idx": 48, "end_idx": 53, "type": "pro", "entity": "保持室内干燥" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "真菌性感染" } ] }, { "text": "重组干扰素γ可提高吞噬细胞氧化酶活性(50μg/m2,每周3次,皮下注射),骨髓移植治疗已有成功病例报道。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "重组干扰素γ" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "吞噬细胞氧化酶" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "皮下注射" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "骨髓移植治疗" } ] }, { "text": "(六)补体缺陷(表17-17)表17-5补体缺陷注:AD为常染色体显性遗传,AR为常染色体隐性遗传,XL为X-连锁遗传广义的补体系统涉及一组血浆蛋白质,由两部分组成:①补体各活性成分C1~C9,B因子和甘露糖结合素(mannosebindinglectin,MBL);②补体调节蛋白,C1抑制物,C4结合蛋白,D因子,I因子,备解素,H因子,腐败加速因子(decayacceleratingfactor,DAF,CD55)和保护因子(protectin,CD59)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "补体缺陷" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "补体缺陷" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "AD" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "常染色体" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "AR" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "常染色体" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "XL" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "X-连锁" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "补体系统" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "血浆蛋白质" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "补体" }, { "start_idx": 87, "end_idx": 95, "type": "bod", "entity": "活性成分C1~C9" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "B因子" }, { "start_idx": 101, "end_idx": 106, "type": "bod", "entity": "甘露糖结合素" }, { "start_idx": 108, "end_idx": 127, "type": "bod", "entity": "mannosebindinglectin" }, { "start_idx": 129, "end_idx": 131, "type": "bod", "entity": "MBL" }, { "start_idx": 135, "end_idx": 140, "type": "bod", "entity": "补体调节蛋白" }, { "start_idx": 142, "end_idx": 146, "type": "bod", "entity": "C1抑制物" }, { "start_idx": 148, "end_idx": 153, "type": "bod", "entity": "C4结合蛋白" }, { "start_idx": 155, "end_idx": 157, "type": "bod", "entity": "D因子" }, { "start_idx": 159, "end_idx": 161, "type": "bod", "entity": "I因子" }, { "start_idx": 163, "end_idx": 165, "type": "bod", "entity": "备解素" }, { "start_idx": 167, "end_idx": 169, "type": "bod", "entity": "H因子" }, { "start_idx": 171, "end_idx": 176, "type": "bod", "entity": "腐败加速因子" }, { "start_idx": 178, "end_idx": 200, "type": "bod", "entity": "decayacceleratingfactor" }, { "start_idx": 202, "end_idx": 204, "type": "bod", "entity": "DAF" }, { "start_idx": 206, "end_idx": 209, "type": "bod", "entity": "CD55" }, { "start_idx": 212, "end_idx": 215, "type": "bod", "entity": "保护因子" }, { "start_idx": 217, "end_idx": 225, "type": "bod", "entity": "protectin" }, { "start_idx": 227, "end_idx": 230, "type": "bod", "entity": "CD59" } ] }, { "text": "补体上游成分如C1、C4、C2及C3缺陷,易发生反复化脓性感染,尤其是具荚膜多糖抗原的细菌感染;下游成分C5~9(也含C3)缺陷易发生革兰阴性菌感染,尤其是奈瑟菌感染,约1%~15%患者发生散发性脑膜炎双球菌感染。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "补体上游成分如C1、C4、C2及C3缺陷" }, { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "补体上游成分" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "C1" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "C4" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "C2" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "C3" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "反复化脓性感染" }, { "start_idx": 36, "end_idx": 46, "type": "dis", "entity": "荚膜多糖抗原的细菌感染" }, { "start_idx": 48, "end_idx": 63, "type": "sym", "entity": "下游成分C5~9(也含C3)缺陷" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "C5~9" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "C3" }, { "start_idx": 67, "end_idx": 73, "type": "dis", "entity": "革兰阴性菌感染" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "奈瑟菌感染" }, { "start_idx": 95, "end_idx": 105, "type": "dis", "entity": "散发性脑膜炎双球菌感染" } ] }, { "text": "第十一章系统性红斑狼疮【概述】系统性红斑狼疮(systemiclupuserythematosus,SLE)是一种累及多系统的自身免疫性疾病,特征为广泛的血管炎和结缔组织炎症,存在抗核抗体(ANA),特别是抗dsDNA和抗Sm抗体阳性。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 23, "end_idx": 48, "type": "dis", "entity": "systemiclupuserythematosus" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "SLE" }, { "start_idx": 57, "end_idx": 69, "type": "sym", "entity": "累及多系统的自身免疫性疾病" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "多系统" }, { "start_idx": 63, "end_idx": 69, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 74, "end_idx": 86, "type": "sym", "entity": "广泛的血管炎和结缔组织炎症" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "血管炎" }, { "start_idx": 81, "end_idx": 86, "type": "dis", "entity": "结缔组织炎症" }, { "start_idx": 90, "end_idx": 93, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "ANA" }, { "start_idx": 103, "end_idx": 116, "type": "sym", "entity": "抗dsDNA和抗Sm抗体阳性" }, { "start_idx": 103, "end_idx": 108, "type": "bod", "entity": "抗dsDNA" }, { "start_idx": 110, "end_idx": 114, "type": "bod", "entity": "抗Sm抗体" } ] }, { "text": "临床除发热、皮疹等共同表现外,因受累脏器不同而表现不同,常常先后或同时累及泌尿、神经、心血管及血液等多个系统,常表现为中至重度多脏器损害。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "发热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "皮疹" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "皮" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "受累脏器" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脏器" }, { "start_idx": 35, "end_idx": 53, "type": "sym", "entity": "累及泌尿、神经、心血管及血液等多个系统" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "泌尿" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "神经" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "心血管" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "血液" }, { "start_idx": 59, "end_idx": 67, "type": "dis", "entity": "中至重度多脏器损害" } ] }, { "text": "过程笃重,有潜在致命性,儿童SLE的预后比成人更严重。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "SLE" } ] }, { "text": "【病因和发病机制】本病是在遗传易感体质的基础上,外界环境作用激发机体免疫功能紊乱及免疫调节障碍而引起的自身免疫性疾病。", "entities": [ { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "免疫功能紊乱" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "免疫调节障碍" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "自身免疫性疾病" } ] }, { "text": "遗传因素方面,已有资料表明,SLE发病与HLAⅡ类基因DR、DQ位点的多态性,以及细胞毒T细胞淋巴相关抗原4(CTLA-4)等因素相关。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "SLE" }, { "start_idx": 20, "end_idx": 28, "type": "bod", "entity": "HLAⅡ类基因DR" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "DQ位点" }, { "start_idx": 41, "end_idx": 53, "type": "bod", "entity": "细胞毒T细胞淋巴相关抗原4" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "CTLA-4" } ] }, { "text": "患儿细胞免疫功能低下,T-B淋巴细胞之间,T淋巴细胞亚群之间平衡失调,T细胞绝对值减少及T抑制细胞减少,致使B细胞功能亢进,自发产生大量自身抗体。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "细胞免疫功能低下" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 33, "type": "sym", "entity": "T-B淋巴细胞之间,T淋巴细胞亚群之间平衡失调" }, { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "T-B淋巴细胞之间" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "T淋巴细胞亚群之间" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "T细胞绝对值减少" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "T细胞" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "T抑制细胞减少" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "T抑制细胞" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "B细胞功能亢进" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "B细胞" }, { "start_idx": 62, "end_idx": 71, "type": "sym", "entity": "自发产生大量自身抗体" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "自身抗体" } ] }, { "text": "由于抗淋巴细胞抗体的产生,引起淋巴细胞减少,抗淋巴细胞抗体与神经元组织交叉反应,可引起中枢神经系统病变。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "抗淋巴细胞抗体" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "淋巴细胞减少" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 22, "end_idx": 38, "type": "sym", "entity": "抗淋巴细胞抗体与神经元组织交叉反应" }, { "start_idx": 22, "end_idx": 28, "type": "bod", "entity": "抗淋巴细胞抗体" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "神经元组织" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "中枢神经系统病变" } ] }, { "text": "大量自身抗体与抗原相结合形成抗原抗体复合物沉积在皮肤血管壁、表皮和真皮连接处、肾小球血管壁及其他受累组织,造成多脏器损害。", "entities": [ { "start_idx": 0, "end_idx": 51, "type": "sym", "entity": "大量自身抗体与抗原相结合形成抗原抗体复合物沉积在皮肤血管壁、表皮和真皮连接处、肾小球血管壁及其他受累组织" }, { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "自身抗体与抗原" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "抗原抗体复合物" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "皮肤血管壁" }, { "start_idx": 30, "end_idx": 37, "type": "bod", "entity": "表皮和真皮连接处" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "肾小球血管壁" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "其他受累组织" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "多脏器损害" } ] }, { "text": "【临床表现】(一)一般症状发热,热型不规则,伴全身不适、乏力、食欲缺乏、体重下降及脱发等。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "热型不规则" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "全身不适" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "乏力" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "体重下降" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "脱发" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "发" } ] }, { "text": "(二)皮疹对称性颊部蝶形红斑,跨过鼻梁,边缘清晰,略高出皮面,日晒加重;上胸及肘部等暴露部位可有红斑样斑丘疹;掌跖红斑、指(趾)端掌侧红斑、甲周红斑以及指甲下远端红斑等均为血管炎所致。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "皮" }, { "start_idx": 3, "end_idx": 34, "type": "sym", "entity": "皮疹对称性颊部蝶形红斑,跨过鼻梁,边缘清晰,略高出皮面,日晒加重" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "颊部" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "鼻梁" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "皮面" }, { "start_idx": 36, "end_idx": 53, "type": "sym", "entity": "上胸及肘部等暴露部位可有红斑样斑丘疹" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "上胸" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肘部" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "暴露部位" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "掌跖红斑" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "掌跖" }, { "start_idx": 60, "end_idx": 68, "type": "sym", "entity": "指(趾)端掌侧红斑" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "指(趾)端" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "甲周红斑" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "甲周" }, { "start_idx": 76, "end_idx": 82, "type": "sym", "entity": "指甲下远端红斑" }, { "start_idx": 76, "end_idx": 80, "type": "bod", "entity": "指甲下远端" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "血管炎" } ] }, { "text": "特别要注意鼻腔和口腔黏膜有无溃疡。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "鼻腔" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "溃疡" } ] }, { "text": "(三)关节症状关节、肌肉疼痛,关节肿胀和畸形。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "关节症状关节、肌肉疼痛" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "关节" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "关节" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肌肉" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "关节肿胀和畸形" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "关节" } ] }, { "text": "(四)心脏可累及心内膜、心肌和心包,可表现为心力衰竭。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "sym", "entity": "心脏可累及心内膜、心肌和心包" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "心内膜" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心肌" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心包" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "(五)肾脏从局灶性肾小球肾炎到弥漫性增生性肾小球肾炎,重症可死于尿毒症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "局灶性肾小球肾炎" }, { "start_idx": 15, "end_idx": 25, "type": "dis", "entity": "弥漫性增生性肾小球肾炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "尿毒症" } ] }, { "text": "肾脏受累亦可为首发症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "肾脏受累" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肾脏" } ] }, { "text": "(六)多发性浆膜炎可累及胸膜、心包及腹膜,可单独或同时受累,一般不留后遗症。", "entities": [ { "start_idx": 3, "end_idx": 19, "type": "sym", "entity": "多发性浆膜炎可累及胸膜、心包及腹膜" }, { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "多发性浆膜炎" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胸膜" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心包" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腹膜" } ] }, { "text": "(七)神经系统头痛、性格改变、癫痫、偏瘫及失语等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "神经系统头痛" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "头" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "性格改变" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "癫痫" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "偏瘫" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "失语" } ] }, { "text": "(八)其他肝、脾、淋巴结肿大,可有咳嗽、胸痛及呼吸困难等症状。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肝" }, { "start_idx": 5, "end_idx": 13, "type": "sym", "entity": "肝、脾、淋巴结肿大" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "脾" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "淋巴结" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "咳嗽" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "胸痛" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "胸" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "(九)狼疮危象(lupuscrisis)是由广泛急性血管炎所致急剧发生的全身性疾病,常常危及生命。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "狼疮危象" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "lupuscrisis" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "广泛急性血管炎" } ] }, { "text": "儿童较成人尤易发生危象,表现为:①持续高热,用抗生素无效。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "持续高热" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "抗生素" } ] }, { "text": "⑤严重神经精神狼疮的表现。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "严重神经精神狼疮" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "神经" } ] }, { "text": "【实验室检查】(一)血象白细胞计数减少,常<4×109/L,淋巴细胞减少,常<1.5×109/L,不同程度贫血,Coombs实验可阳性,血小板正常或减少。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "血象白细胞计数减少" }, { "start_idx": 10, "end_idx": 16, "type": "ite", "entity": "血象白细胞计数" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "淋巴细胞减少" }, { "start_idx": 36, "end_idx": 39, "type": "ite", "entity": "淋巴细胞" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "贫血" }, { "start_idx": 68, "end_idx": 78, "type": "sym", "entity": "Coombs实验可阳性" }, { "start_idx": 68, "end_idx": 75, "type": "pro", "entity": "Coombs实验" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "血小板" }, { "start_idx": 86, "end_idx": 87, "type": "sym", "entity": "减少" } ] }, { "text": "(二)抗核抗体多为周边型和斑点型,有抗dsDNA抗体、抗DNP抗体、抗Sm抗体、抗Ro(SSA)抗体以及抗La(SSB)抗体等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 18, "end_idx": 25, "type": "bod", "entity": "抗dsDNA抗体" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "抗DNP抗体" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "抗Sm抗体" }, { "start_idx": 40, "end_idx": 49, "type": "bod", "entity": "抗Ro(SSA)抗体" }, { "start_idx": 52, "end_idx": 61, "type": "bod", "entity": "抗La(SSB)抗体" } ] }, { "text": "(三)免疫学检查C3降低;IgG显著升高,IgA、IgM亦升高,γ球蛋白升高,呈高γ球蛋白血症;循环免疫复合物测定阳性。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "sym", "entity": "免疫学检查C3降低" }, { "start_idx": 3, "end_idx": 15, "type": "pro", "entity": "免疫学检查C3" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "IgG显著升高" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "IgG" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "IgA、IgM亦升高" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "IgA" }, { "start_idx": 31, "end_idx": 33, "type": "ite", "entity": "IgM" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "γ球蛋白升高" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "γ球蛋白" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "高γ球蛋白血症" }, { "start_idx": 54, "end_idx": 64, "type": "sym", "entity": "循环免疫复合物测定阳性" }, { "start_idx": 54, "end_idx": 62, "type": "pro", "entity": "循环免疫复合物测定" } ] }, { "text": "(四)尿常规有尿蛋白、血尿及管型尿,肝肾功能测定可异常。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "sym", "entity": "尿常规有尿蛋白、血尿及管型尿" }, { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "尿常规" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "尿蛋白" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "管型尿" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "肝肾功能测定可异常" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "肝肾功能测定" } ] }, { "text": "(五)狼疮带试验活检取小块皮肤,用直接免疫荧光法观察,可发现表皮与真皮交界线上有颗粒或线状荧光带,为IgG、IgA、IgM及补体沉积所致。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "狼疮带试验活检" }, { "start_idx": 19, "end_idx": 47, "type": "sym", "entity": "免疫荧光法观察,可发现表皮与真皮交界线上有颗粒或线状荧光带" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "表皮" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "真皮" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "IgG" }, { "start_idx": 50, "end_idx": 65, "type": "sym", "entity": "IgG、IgA、IgM及补体沉积" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "IgA" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "IgM" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "补体" } ] }, { "text": "(六)特殊检查肾穿刺活组织检查,对狼疮肾炎的诊断、治疗和预后均有重要价值。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "肾穿刺活组织检查" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "狼疮肾炎" } ] }, { "text": "TCD为狼疮脑病脑血管早期功能性变化的检测提供了一项较敏感和特异的方法。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "TCD" }, { "start_idx": 4, "end_idx": 17, "type": "sym", "entity": "狼疮脑病脑血管早期功能性变化" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "狼疮脑病" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑血管" } ] }, { "text": "【诊断标准】儿童SLE的诊断标准与成人相同,常选用美国风湿病学会1997年修订的SLE诊断标准,其11项诊断条件包括:1.脸颊部蝶形红斑;2.盘状红斑;3.光敏感;4.口腔或鼻黏膜溃疡5.非侵蚀性关节炎;6.肾炎血尿,蛋白尿>0.5g/d,细胞管型;7.脑病癫痫发作或精神症状;8.浆膜炎;9.血细胞减少贫血白细胞减少血小板减少10.ANA阳性抗dsDNA抗体阳性,或抗Sm抗体阳性,或抗磷脂抗体阳性(包含抗心磷脂抗体阳性,或狼疮抗凝物阳性,或持续6个月梅毒血清试验假阳性三者之一);11.荧光抗核抗体阳性。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "SLE" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "风湿病" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "SLE" }, { "start_idx": 61, "end_idx": 67, "type": "sym", "entity": "脸颊部蝶形红斑" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "脸颊部" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "盘状红斑" }, { "start_idx": 78, "end_idx": 80, "type": "sym", "entity": "光敏感" }, { "start_idx": 84, "end_idx": 91, "type": "dis", "entity": "口腔或鼻黏膜溃疡" }, { "start_idx": 94, "end_idx": 100, "type": "dis", "entity": "非侵蚀性关节炎" }, { "start_idx": 104, "end_idx": 107, "type": "sym", "entity": "肾炎血尿" }, { "start_idx": 104, "end_idx": 105, "type": "dis", "entity": "肾炎" }, { "start_idx": 106, "end_idx": 106, "type": "bod", "entity": "血" }, { "start_idx": 107, "end_idx": 107, "type": "bod", "entity": "尿" }, { "start_idx": 109, "end_idx": 111, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 109, "end_idx": 110, "type": "bod", "entity": "蛋白" }, { "start_idx": 111, "end_idx": 111, "type": "bod", "entity": "尿" }, { "start_idx": 120, "end_idx": 123, "type": "sym", "entity": "细胞管型" }, { "start_idx": 120, "end_idx": 121, "type": "bod", "entity": "细胞" }, { "start_idx": 127, "end_idx": 132, "type": "sym", "entity": "脑病癫痫发作" }, { "start_idx": 127, "end_idx": 128, "type": "dis", "entity": "脑病" }, { "start_idx": 129, "end_idx": 130, "type": "dis", "entity": "癫痫" }, { "start_idx": 141, "end_idx": 143, "type": "dis", "entity": "浆膜炎" }, { "start_idx": 147, "end_idx": 151, "type": "sym", "entity": "血细胞减少" }, { "start_idx": 147, "end_idx": 149, "type": "bod", "entity": "血细胞" }, { "start_idx": 152, "end_idx": 153, "type": "dis", "entity": "贫血" }, { "start_idx": 154, "end_idx": 156, "type": "bod", "entity": "白细胞" }, { "start_idx": 154, "end_idx": 158, "type": "sym", "entity": "白细胞减少" }, { "start_idx": 159, "end_idx": 161, "type": "bod", "entity": "血小板" }, { "start_idx": 159, "end_idx": 163, "type": "sym", "entity": "血小板减少" }, { "start_idx": 167, "end_idx": 171, "type": "sym", "entity": "ANA阳性" }, { "start_idx": 167, "end_idx": 169, "type": "bod", "entity": "ANA" }, { "start_idx": 172, "end_idx": 181, "type": "sym", "entity": "抗dsDNA抗体阳性" }, { "start_idx": 172, "end_idx": 179, "type": "bod", "entity": "抗dsDNA抗体" }, { "start_idx": 184, "end_idx": 190, "type": "sym", "entity": "抗Sm抗体阳性" }, { "start_idx": 184, "end_idx": 188, "type": "bod", "entity": "抗Sm抗体" }, { "start_idx": 193, "end_idx": 199, "type": "sym", "entity": "抗磷脂抗体阳性" }, { "start_idx": 193, "end_idx": 197, "type": "bod", "entity": "抗磷脂抗体" }, { "start_idx": 203, "end_idx": 210, "type": "sym", "entity": "抗心磷脂抗体阳性" }, { "start_idx": 203, "end_idx": 208, "type": "bod", "entity": "抗心磷脂抗体" }, { "start_idx": 213, "end_idx": 219, "type": "sym", "entity": "狼疮抗凝物阳性" }, { "start_idx": 213, "end_idx": 217, "type": "bod", "entity": "狼疮抗凝物" }, { "start_idx": 227, "end_idx": 235, "type": "sym", "entity": "梅毒血清试验假阳性" }, { "start_idx": 227, "end_idx": 232, "type": "pro", "entity": "梅毒血清试验" }, { "start_idx": 245, "end_idx": 252, "type": "sym", "entity": "荧光抗核抗体阳性" }, { "start_idx": 245, "end_idx": 250, "type": "bod", "entity": "荧光抗核抗体" } ] }, { "text": "符合上述条件4项或4项以上者即可确诊SLE。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "SLE" } ] }, { "text": "【治疗】(一)一般治疗卧床休息,加强营养,低盐饮食,避免日光曝晒及预防接种,慎用各种药物,以免诱发疾病活动,预防感染。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "卧床休息" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "加强营养" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "低盐饮食" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "避免日光曝晒" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "预防接种" }, { "start_idx": 38, "end_idx": 43, "type": "pro", "entity": "慎用各种药物" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "预防感染" } ] }, { "text": "(二)皮质激素泼尼松2mg/(kg•d),总量≤60mg,分次服用;病情控制,实验室检查基本正常后改为每日或隔日顿服,剂量逐渐减至0.5~1mg/kg,小剂量维持疗法须持续数年。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "皮质激素" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "泼尼松" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "服用" }, { "start_idx": 39, "end_idx": 43, "type": "pro", "entity": "实验室检查" }, { "start_idx": 51, "end_idx": 57, "type": "pro", "entity": "每日或隔日顿服" } ] }, { "text": "重症可用甲泼尼松龙冲击疗法:10~30mg/(kg•d),共3天,3天后用泼尼松1mg/(kg•d),分次服用。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "甲泼尼松龙冲击疗法" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "泼尼松" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "服用" } ] }, { "text": "(三)免疫抑制剂常用药为环磷酰胺(CTX)、硫唑嘌呤和氨甲蝶呤等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "CTX" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "氨甲蝶呤" } ] }, { "text": "由于此类药物对SLE的活动控制不如激素迅速,因此,不提倡作为治疗SLE的单一或首选药。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "SLE" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "激素" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "SLE" } ] }, { "text": "CTX对各类SLE均有效,特别是严重肾脏损害如弥漫性增生性肾炎、中枢神经系统和肺损害,早期与激素联合使用是降低病死率和提高生命质量的关键。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "CTX" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "SLE" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "严重肾脏损害" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "弥漫性增生性肾炎" }, { "start_idx": 32, "end_idx": 41, "type": "dis", "entity": "中枢神经系统和肺损害" }, { "start_idx": 43, "end_idx": 51, "type": "pro", "entity": "早期与激素联合使用" } ] }, { "text": "有人认为CTX静脉冲击治疗是减少肾纤维化、稳定肾衰竭的一种有效方法。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "CTX静脉冲击治疗" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "肾纤维化" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "同时将泼尼松减量至每日0.5mg/kg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "泼尼松" } ] }, { "text": "另一CTX冲击治疗方案为每次8~12mg/kg,每日1次,连用2日1疗程(总量<1g/疗程,至少间隔2周用1疗程,连用6疗程后改为3个月1疗程,维持2年;也有每月1疗程,连用6个月后停药的半年方案以及每月1次连用6个月,再3个月1次维持2年的长疗程治疗方案。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "CTX冲击治疗" } ] }, { "text": "多数学者认为在CTX静脉冲击治疗的同时应强调采用大量输入平衡液体,即水化疗法,以求更加安全(见本书系统性红斑狼疮肾炎章节)。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "pro", "entity": "CTX静脉冲击治疗" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "输入" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "平衡液体" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "水化疗法" }, { "start_idx": 49, "end_idx": 57, "type": "dis", "entity": "系统性红斑狼疮肾炎" } ] }, { "text": "SLE的药物治疗的过程中,要注意以下几点:1.急性肾衰竭当肌酐清除率(Ccr)<20ml/min时,可在甲泼尼龙冲击获得缓解后,再进行CTX冲击。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "SLE" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 29, "end_idx": 38, "type": "ite", "entity": "肌酐清除率(Ccr)" }, { "start_idx": 52, "end_idx": 57, "type": "pro", "entity": "甲泼尼龙冲击" }, { "start_idx": 67, "end_idx": 71, "type": "pro", "entity": "CTX冲击" } ] }, { "text": "2.近2周内有过严重感染,或WBC<4×109/L,或对CTX过敏,或2周内用过其他细胞免疫抑制剂,重症肾病综合征表现,血清白蛋白<2g/L时,应慎用CTX。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "严重感染" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "WBC" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "CTX" }, { "start_idx": 48, "end_idx": 54, "type": "dru", "entity": "细胞免疫抑制剂" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "重症肾病综合征" }, { "start_idx": 66, "end_idx": 70, "type": "ite", "entity": "血清白蛋白" }, { "start_idx": 81, "end_idx": 83, "type": "dru", "entity": "CTX" } ] }, { "text": "由于儿童SLE的发病高峰在11~15岁,因此,治疗时应该考虑青春期发育的问题。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "SLE" } ] }, { "text": "目前,在狼疮肾炎,应用CTX冲击治疗尿蛋白消失后可用硫唑嘌呤维持,剂量为每日1~2.5mg/kg。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "CTX冲击治疗" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "硫唑嘌呤" } ] }, { "text": "3.氨甲蝶呤(MTX)与硫唑嘌呤可分别与激素联合应用,MTX的剂量为0.005~0.01/m2,每周1次顿服,对控制SLE的活动及减少激素应用量有较好的作用,但不适合于重症狼疮肾炎和中枢神经系统狼疮的治疗。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "氨甲蝶呤" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "MTX" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 20, "end_idx": 21, "type": "dru", "entity": "激素" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "联合应用" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "MTX" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "顿服" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "SLE" }, { "start_idx": 73, "end_idx": 74, "type": "dru", "entity": "激素" }, { "start_idx": 90, "end_idx": 95, "type": "dis", "entity": "重症狼疮肾炎" }, { "start_idx": 97, "end_idx": 104, "type": "dis", "entity": "中枢神经系统狼疮" } ] }, { "text": "4.环孢霉素A(CsA)由于该药有肾毒性并使血管收缩可致高血压,故在儿童SLE尚未广泛应用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "环孢霉素A" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "CsA" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肾" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血管" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "高血压" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "儿童SLE" } ] }, { "text": "(四)对症治疗关节症状应用非甾体抗炎药,皮肤症状合并使用羟氯喹。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "对症治疗" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "关节" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "关节症状" }, { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "非甾体抗炎药" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "皮肤症状" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "羟氯喹" } ] }, { "text": "(五)其他重症可用IVIG、血浆置换术以及DNA免疫吸附等。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "IVIG" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "血浆置换术" }, { "start_idx": 21, "end_idx": 27, "type": "pro", "entity": "DNA免疫吸附" } ] }, { "text": "近年来有使用CD20单克隆抗体等生物制剂,以及应用CD34+细胞移植治疗重症SLE的报道。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "dru", "entity": "CD20单克隆抗体" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "生物制剂" }, { "start_idx": 25, "end_idx": 39, "type": "pro", "entity": "CD34+细胞移植" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "SLE" } ] }, { "text": "【预后】儿童SLE的预后与疾病的活动程度(多用SLEDAI计分评估)、肾脏损害的类型和进展情况、临床血管炎的表现以及多系统受累的情况有关。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "童SLE" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "肾脏损害" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "临床血管炎" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "多系统" } ] }, { "text": "弥漫增殖性狼疮肾炎(Ⅳ型)和持续中枢神经系统病变预后最差。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "弥漫增殖性狼疮肾炎(Ⅳ型)" }, { "start_idx": 14, "end_idx": 23, "type": "dis", "entity": "持续中枢神经系统病变" } ] }, { "text": "该病死亡原因常见为感染、肾衰竭、中枢神经系统病变和脑血管意外、肺出血、肺动脉高压及心肌梗死等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "感染" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "中枢神经系统病变" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "脑血管意外" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "肺出血" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "心肌梗死" } ] }, { "text": "参考文献1.SekiM,KobayashiT,KobayashiT,etal.ExternalValidationofaRiskScoretoPredictIntravenousImmunoglobulinResistanceinPatientswithKawasakiDisease.PediatrInfectDis,20102.EleftheriouD,DillonMJ,BroganPA.Advancesinchildhoodvasculitis[J].CurrOpinRheumatol.2009,21(4):411-4183.杨军,李成荣,李永柏,等.肿瘤坏死因子a和白细胞介素10基因启动子区多态性与川崎病相关性研究.中华儿科杂志,2003,41:598-6024.杨军,李成荣,等.甘露糖结合凝集素基因第54号密码子多态性与川崎病的易感性研究.中华儿科杂志,2004,42(3):1765.张乃峥主编.临床风湿病学[M].上海科学技术出版社,1997,246-2466.Seko,Y.GiantcellandTakayasuarteritis.CurrOpinRheumat,2007,19(1):39-43", "entities": [ { "start_idx": 281, "end_idx": 297, "type": "bod", "entity": "肿瘤坏死因子a和白细胞介素10基因" }, { "start_idx": 306, "end_idx": 308, "type": "dis", "entity": "川崎病" }, { "start_idx": 317, "end_idx": 318, "type": "dep", "entity": "儿科" }, { "start_idx": 353, "end_idx": 357, "type": "bod", "entity": "凝集素基因" }, { "start_idx": 369, "end_idx": 371, "type": "dis", "entity": "川崎病" }, { "start_idx": 381, "end_idx": 382, "type": "dep", "entity": "儿科" }, { "start_idx": 410, "end_idx": 412, "type": "dis", "entity": "风湿病" } ] }, { "text": "第十一章心力衰竭心力衰竭(heartfailure)(以下简称心衰)指心脏不能泵出足够的血液以满足机体代谢所需的一种病理生理状态。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 13, "end_idx": 24, "type": "dis", "entity": "heartfailure" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "心衰" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心脏" }, { "start_idx": 35, "end_idx": 63, "type": "sym", "entity": "心脏不能泵出足够的血液以满足机体代谢所需的一种病理生理状态" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "血液" } ] }, { "text": "心肌收缩功能受损所导致的心输出量降低,常见于心肌缺血性心脏病或原发性心肌病患者。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "心肌收缩功能受损" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "心输出量降低" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "心" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "心肌缺血性心脏病" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "原发性心肌病" } ] }, { "text": "因心脏舒张期充盈不足所致心输出量减少者少见,如流入道梗阻(二尖瓣狭窄、三房心)、限制性心肌病、缩窄性心包炎。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "心脏" }, { "start_idx": 1, "end_idx": 9, "type": "sym", "entity": "心脏舒张期充盈不足" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "心输出量减少" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "心" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "流入道梗阻" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "二尖瓣狭窄" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "三房心" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "限制性心肌病" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "缩窄性心包炎" } ] }, { "text": "在小儿,最常见的心力衰竭原因为心脏结构异常所造成的心室负荷异常,尽管此时心肌收缩力可能仍然正常。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心脏" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "心脏结构异常" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心室" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "心室负荷异常" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "心肌" } ] }, { "text": "心脏负荷异常可包括心室压力负荷过重和容量负荷过重。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "心脏负荷异常" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心室" }, { "start_idx": 9, "end_idx": 23, "type": "sym", "entity": "心室压力负荷过重和容量负荷过重" } ] }, { "text": "如存在流出道梗阻(主动脉瓣狭窄、水肿、肺动脉瓣狭窄)时,心室后负荷增加,即压力负荷增加;如有大量左向右分流、瓣膜严重反流或体循环动静脉瘘时则容量负荷增加。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "流出道梗阻" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "水肿" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "心室" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "心室后负荷增加" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "压力负荷增加" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "大量左向右分流" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "瓣膜严重反流" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "体循环动静脉瘘" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "容量负荷增加" } ] }, { "text": "此外,在代谢亢进和(或)后负荷降低时,如甲状腺功能亢进、贫血,心脏需泵出更多血量以提供足够的氧和其他营养物质以满足机体的需要,由此而造成的心力衰竭称高排血量型心力衰竭。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "代谢亢进" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "后负荷降低" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "甲状腺功能亢进" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "贫血" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "心脏" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "血" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 74, "end_idx": 82, "type": "dis", "entity": "高排血量型心力衰竭" } ] }, { "text": "心力衰竭临床综合征在儿科多见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 10, "end_idx": 11, "type": "dep", "entity": "儿科" } ] }, { "text": "本章主要介绍儿科心力衰竭常见原因,包括各年龄组心功能变化、病理生理学和遗传学基础、临床症状和治疗方法。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "心" } ] }, { "text": "【病因学】心力衰竭开始的时间可提供病因学线索(见表9-9)。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "表9-15不同年龄阶段心力衰竭原因(一)胎儿心力衰竭随着胎儿超声检查的广泛应用,临床上越来越多胎儿心力衰竭得到了诊断,其主要表现为腹腔、心包腔、胸腔的积液,严重时可有胎儿水肿。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "胎儿心力衰竭" }, { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "胎儿超声检查" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "胎儿心力衰竭" }, { "start_idx": 65, "end_idx": 76, "type": "sym", "entity": "腹腔、心包腔、胸腔的积液" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "腹腔" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "心包腔" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "胸腔" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "胎儿水肿" } ] }, { "text": "最常见原因为持续性室上性心动过速,可伴或不伴心脏结构异常完全性房室传导阻滞伴缓慢心室率可在母亲患有红斑狼疮时出现。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "持续性室上性心动过速" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "心脏结构异常" }, { "start_idx": 28, "end_idx": 36, "type": "dis", "entity": "完全性房室传导阻滞" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "缓慢心室率" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "红斑狼疮" } ] }, { "text": "心脏结构异常伴严重的瓣膜反流所致者及出生前卵圆孔早闭导致胎儿心力衰竭者较少见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "心脏结构异常" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "严重的瓣膜反流" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "卵圆孔早闭" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "胎儿心力衰竭" } ] }, { "text": "(二)新生儿心力衰竭足月新生儿充血性心力衰竭多因心肌功能障碍所致,常见于围生期窒息所致的一过性心肌缺血,表现为血清心肌酶增高乳头肌功能障碍伴房室瓣严重反流代谢紊乱(低血糖、低血钙)和败血症,病毒性心肌炎为少见原因。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "新生儿心力衰竭" }, { "start_idx": 10, "end_idx": 21, "type": "dis", "entity": "足月新生儿充血性心力衰竭" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "心肌功能障碍" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "围生期窒息" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "一过性心肌缺血" }, { "start_idx": 55, "end_idx": 61, "type": "sym", "entity": "血清心肌酶增高" }, { "start_idx": 55, "end_idx": 59, "type": "ite", "entity": "血清心肌酶" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "乳头肌功能障碍" }, { "start_idx": 70, "end_idx": 76, "type": "dis", "entity": "房室瓣严重反流" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "代谢紊乱" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "低血糖" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "低血钙" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "败血症" }, { "start_idx": 95, "end_idx": 100, "type": "dis", "entity": "病毒性心肌炎" } ] }, { "text": "除前述的各种原因引起的严重贫血外,分娩时婴儿严重出血所致的贫血及其他溶血性贫血可致高排血量型心力衰竭。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "严重贫血" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "严重出血" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "贫血" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 41, "end_idx": 49, "type": "dis", "entity": "高排血量型心力衰竭" } ] }, { "text": "出生后第一天出现心力衰竭的心脏结构异常的心脏病多见于典型的右心室容量负荷过重者,最常见的畸形包括可能因三尖瓣发育不良所致的严重三尖瓣反流、Ebstein畸形、一过性心肌缺血;肺动脉瓣缺如综合征所致严重肺动脉瓣反流少见,此时可闻及高调来回样病理性杂音。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "心脏结构异常" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "心脏病" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "右心室" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "右心室容量负荷过重" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "畸形" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "三尖瓣发育不良" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "严重三尖瓣反流" }, { "start_idx": 69, "end_idx": 77, "type": "dis", "entity": "Ebstein畸形" }, { "start_idx": 79, "end_idx": 85, "type": "dis", "entity": "一过性心肌缺血" }, { "start_idx": 87, "end_idx": 95, "type": "dis", "entity": "肺动脉瓣缺如综合征" }, { "start_idx": 100, "end_idx": 105, "type": "dis", "entity": "肺动脉瓣反流" }, { "start_idx": 111, "end_idx": 123, "type": "sym", "entity": "可闻及高调来回样病理性杂音" } ] }, { "text": "1.新生儿早期心力衰竭(出生后第1周)结构性心脏畸形尤其是左心室流出道梗阻(严重主动脉狭窄、水肿、主动脉弓中断)伴动脉导管闭锁,是导致心力衰竭的最重要原因,典型表现为动脉导管关闭而左心室后负荷急剧增高。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "新生儿早期心力衰竭" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "结构性心脏畸形" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "左心室流出道梗阻" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "主动脉狭窄" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "水肿" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "主动脉弓中断" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "动脉导管闭锁" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "动脉导管关闭" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "动脉导管" }, { "start_idx": 90, "end_idx": 99, "type": "sym", "entity": "左心室后负荷急剧增高" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "左心室" } ] }, { "text": "在左心发育不全综合征时,动脉导管的收缩导致体循环、冠状动脉血流降低,临床上即出现心力衰竭的表现。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "左心发育不全综合征" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "动脉导管" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "体循环、冠状动脉血流降低" }, { "start_idx": 21, "end_idx": 30, "type": "bod", "entity": "体循环、冠状动脉血流" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "严重肺动脉瓣狭窄可表现为右心心力衰竭,但心房水平的右向左分流造成的中央型青紫更多见。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "右心心力衰竭" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "心房水平的右向左分流" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心房" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "中央型青紫" } ] }, { "text": "在早产儿,在肺血管阻力快速降低时,若伴有呼吸窘迫综合征,血液通过未闭的动脉导管形成大量的左向右分流。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "早产儿" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺血管" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "肺血管阻力快速降低" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血液" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "动脉导管" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "大量的左向右分流" } ] }, { "text": "少数非心脏原因如肾脏异常和内分泌异常亦可致心力衰竭。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "肾脏异常" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "内分泌异常" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "非青紫型先天性心脏病包括动脉导管未闭、室间隔缺损、房室间隔缺损可在此时期出现心力衰竭,亦可偶见于房间隔缺损。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "非青紫型先天性心脏病" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "房室间隔缺损" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "青紫型先心病如永存动脉干、不伴肺动脉血流梗阻的单心室和完全性肺静脉异位引流等常因伴氧合和非氧合血的混合和肺血流量增多而出现心力衰竭表现。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "青紫型先心病" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "永存动脉干" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "肺动脉血流梗阻" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "单心室" }, { "start_idx": 27, "end_idx": 36, "type": "dis", "entity": "完全性肺静脉异位引流" }, { "start_idx": 41, "end_idx": 50, "type": "sym", "entity": "氧合和非氧合血的混合" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "血" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "肺血流量增多" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "肺血流" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "同样,左冠状动脉异常起源于肺动脉者,可由于肺动脉压力下降使来自肺动脉的冠脉供血减少而出现心力衰竭。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "左冠状动脉异常" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺动脉" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺动脉" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "肺动脉压力下降" }, { "start_idx": 29, "end_idx": 40, "type": "sym", "entity": "来自肺动脉的冠脉供血减少" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肺动脉" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "冠脉" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "血" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "心肌收缩功能的损害可因扩张型心肌病所致,其病因至今不明,亦可能与代谢性疾病有关。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "心肌收缩功能的损害" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心肌" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "扩张型心肌病" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "代谢性疾病" } ] }, { "text": "婴儿糖原累积症(Ⅱ型)自6周至3个月即可表现为心力衰竭症状及体征,其他症状包括肌张力减低、肌肉无力跟腱反射消失。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "婴儿糖原累积症(Ⅱ型)" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "肌张力减低" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "肌张力" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肌肉" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "肌肉无力" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "跟腱反射消失" }, { "start_idx": 49, "end_idx": 52, "type": "ite", "entity": "跟腱反射" } ] }, { "text": "非心脏原因如肾脏、内分泌疾病亦少见。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "心脏" }, { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "肾脏、内分泌疾病" } ] }, { "text": "因早产儿慢性肺部疾病所致单纯右心力衰竭并不少见,尽管体格检查时仍以胸部体征为主。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "早产儿慢性肺部疾病" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "单纯右心力衰竭" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "体格检查" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "胸部" } ] }, { "text": "3.儿童及青少年心力衰竭在儿童及青春期出现心力衰竭症状者并不常见。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "儿童及青少年心力衰竭" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "在手术前伴有心力衰竭的先天性心脏病患者往往在儿童早期即有心力衰竭的症状。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "手术" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "但本年龄组亦可见许多后天性损害而致心力衰竭者(表9-9)。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "后天性损害" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "【病理生理】心脏异常负荷、心肌收缩或舒张功能异常均可致心力衰竭,心功能变化可用压力-容积关系曲线表示(图9-40)。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "心脏异常负荷" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "心脏" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "心肌收缩或舒张功能异常" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心肌" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "心" }, { "start_idx": 39, "end_idx": 47, "type": "ite", "entity": "压力-容积关系曲线" } ] }, { "text": "随着容量负荷的增加,如大量的左向右分流,心室舒张末期容量增加(图9-40A),充盈压增加,致体肺静脉淤血。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "容量负荷的增加" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "大量的左向右分流" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "心室舒张末期容量增加" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心室" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "充盈压增加" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "体肺静脉淤血" } ] }, { "text": "另一方面,压力负荷增加,如水肿,致每搏量减少(图9-40B)。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "压力负荷增加" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "水肿" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "每搏量减少" } ] }, { "text": "为保持正常每搏量,舒张末期压力及容积增加,临床出现静脉淤血症状。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "每搏量" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "舒张末期压力及容积增加" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "静脉淤血" } ] }, { "text": "心肌收缩功能降低,压力-容积曲线降低,心脏射血功能减少(图9-40C)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "心肌收缩功能降低" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心肌" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "压力-容积曲线降低" }, { "start_idx": 9, "end_idx": 15, "type": "ite", "entity": "压力-容积曲线" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心脏" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "心脏射血功能减少" } ] }, { "text": "为恢复每搏量,舒张末期压力和容积继续增加。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "每搏量" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "舒张末期压力和容积继续增加" } ] }, { "text": "舒张期充盈受损,舒张期压力-容积曲线左移(图9-40D),使一定的舒张末期压力下,每搏输出量减少。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "充盈受损" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "舒张期压力-容积曲线左移" }, { "start_idx": 8, "end_idx": 17, "type": "ite", "entity": "舒张期压力-容积曲线" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "一定的舒张末期压力下" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "每搏输出量减少" } ] }, { "text": "为维持一定的心输出量,必须使血容量增加,以增加心室的充盈。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "心" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "血容量" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心室" } ] }, { "text": "心力衰竭的细胞学表现为肌纤维膜、肌浆网、肌纤维异常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 24, "type": "sym", "entity": "肌纤维膜、肌浆网、肌纤维异常" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肌纤维膜" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肌浆网" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肌纤维" } ] }, { "text": "在心力衰竭患者常存在由钙离子流出所诱发的心奋收缩耦联过程异常。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "钙离子" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "心奋收缩耦联过程异常" } ] }, { "text": "有研究表明,在人类充血性心力衰竭患者及动物实验中肌浆网ATP酶、钙离子摄取功能降低。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "肌浆网ATP酶" }, { "start_idx": 24, "end_idx": 40, "type": "sym", "entity": "肌浆网ATP酶、钙离子摄取功能降低" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "钙离子" } ] }, { "text": "这些异常可降低肌浆网可释放的钙离子浓度而降低心肌收缩力,直接导致舒张期延长。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "bod", "entity": "肌浆网可释放的钙离子" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心肌" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "舒张期延长" } ] }, { "text": "同时对于肾上腺素能兴奋作用反应降低。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 4, "end_idx": 16, "type": "sym", "entity": "肾上腺素能兴奋作用反应降低" } ] }, { "text": "人类心力衰竭患者后期心脏β受体数量减少,同时对于β受体激动剂的正性肌力作用反应降低。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "心脏β受体" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "心脏β受体数量减少" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "β受体激动剂" }, { "start_idx": 24, "end_idx": 40, "type": "sym", "entity": "β受体激动剂的正性肌力作用反应降低" } ] }, { "text": "对于衰竭的心肌该反应利于减少能量消耗,亦是心力衰竭患者使用受体阻滞剂的原因之一。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "衰竭" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心肌" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "受体阻滞剂" } ] }, { "text": "心力衰竭时心脏代偿机制调节心脏及循环系统之间的关系。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心脏" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "循环系统" } ] }, { "text": "神经体液调节导致心力衰竭综合征。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "神经体液调节" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "心力衰竭综合征" } ] }, { "text": "肾素血管紧张素醛固酮系统和交感神经系统的活化直接导致心肌毒性和外周血管收缩心室重构和心室功能恶化心脏扩大心房利钠因子释放尿钠增多、血管扩张的作用,但该因子导致心力衰竭的机制不明。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "bod", "entity": "肾素血管紧张素醛固酮系统" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "交感神经系统" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心肌" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "外周血管" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "心室" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "心室重构" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心室" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "心室功能恶化" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "心脏" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "心房" }, { "start_idx": 52, "end_idx": 59, "type": "sym", "entity": "心房利钠因子释放" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "利钠因子" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "尿钠" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "尿钠增多" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "血管" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "因子" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "根据Frank-Starling机制,心室扩张将导致每搏量增加。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心室" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "心室扩张" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "每搏量增加" } ] }, { "text": "但扩张的心室为维持心室收缩压力需增加室壁张力,这将使耗氧量增加。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "扩张" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "心室" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心室" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "室壁" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "耗氧量增加" } ] }, { "text": "为此心肌逐渐代偿性肥厚以降低室壁张力和降低心肌耗氧量。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心肌" }, { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "心肌逐渐代偿性肥厚" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "室壁" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "心肌耗氧量" } ] }, { "text": "多种机制和体液刺激导致此心肌肥厚。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "体液" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "心肌肥厚" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心肌" } ] }, { "text": "但严重的心肌肥大将导致心内膜下缺血。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "严重的心肌肥大" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "心肌" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "心内膜下缺血" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "心内膜下" } ] }, { "text": "压力负荷过重常导致室壁增厚直至心力衰竭晚期心室才出现扩张心腔扩张在任何时期均为心脏容量负荷过重的表现。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "压力负荷过重" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "室壁增厚" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "室壁" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心室" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "心室才出现扩张" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "心腔扩张" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "心腔" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "心脏容量负荷过重" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "心脏" } ] }, { "text": "为增加心输出量,肾上腺素能活性增加。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "心" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "肾上腺素能活性增加" } ] }, { "text": "β-肾上腺素能活性增强致心率和心肌收缩力增加以改善体循环心输出量。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "β-肾上腺素" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "β-肾上腺素能活性增强" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "心率" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "心率和心肌收缩力增加" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心肌" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "体循环" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "心" } ] }, { "text": "提高α肾上腺素能活性可导致心输出量的重分配,机体可以通过肾脏、胃肠道和皮肤血管床的收缩来减少这些器官的血供,以保证心肌和中枢神经系统的供给。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "α肾上腺素" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "心" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肾脏" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "胃肠道" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "皮肤血管床" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "器官" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "血" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "心肌" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "随着后负荷的增加,心收缩功能将进一步受到损害。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "后负荷的增加" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "心" }, { "start_idx": 9, "end_idx": 21, "type": "sym", "entity": "心收缩功能将进一步受到损害" } ] }, { "text": "此外,心肌的静止张力较任何牵张程度都高,意味着心室顺应性减低,因此不能充分耐受容量负荷的增加,舒张末期压力过高在早期即可发展为肺水肿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心肌" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心室" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "心室顺应性减低" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "容量负荷的增加" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "舒张末期压力过高" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "肺水肿" } ] }, { "text": "心肌收缩使新生儿心肌静息长度下产生的张力低于成人,与其中无收缩成分占优势有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心肌" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心肌" } ] }, { "text": "新生儿尚有心室间的相互依赖。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "心室间" } ] }, { "text": "因此,一侧心室的压力或容量负荷增加将影响充盈特征和另一侧心室的充盈和功能。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "sym", "entity": "一侧心室的压力或容量负荷增加" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心室" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "心室" } ] }, { "text": "图9-40充血性心力衰竭A.左向右分流导致的容量负荷过重;B.水肿导致的压力负荷过重;C.心肌炎所致的心收缩力下降;D.限制型心肌病所致的心脏充盈受限【遗传学】基因表达改变在心力衰竭的病理生理学机制中所起的作用已受到足够地重视。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "左向右分流" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "容量负荷过重" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "水肿" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "压力负荷过重" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "心肌炎" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "心" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "心收缩力下降" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "限制型心肌病" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "心脏" }, { "start_idx": 69, "end_idx": 74, "type": "sym", "entity": "心脏充盈受限" }, { "start_idx": 80, "end_idx": 85, "type": "sym", "entity": "基因表达改变" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "基因" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "大量工作集中于遗传性心肌病的研究。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "遗传性心肌病" } ] }, { "text": "编码肌小节蛋白包括肌球蛋白链、肌钙蛋白和心肌收缩系统的其他成分的基因发生突变已被证明可致家族性肥厚性心肌病。", "entities": [ { "start_idx": 0, "end_idx": 37, "type": "sym", "entity": "编码肌小节蛋白包括肌球蛋白链、肌钙蛋白和心肌收缩系统的其他成分的基因发生突变" }, { "start_idx": 0, "end_idx": 33, "type": "bod", "entity": "编码肌小节蛋白包括肌球蛋白链、肌钙蛋白和心肌收缩系统的其他成分的基因" }, { "start_idx": 44, "end_idx": 52, "type": "dis", "entity": "家族性肥厚性心肌病" } ] }, { "text": "家族性扩张性心肌病被认为与基因突变有关,包括X性连锁扩张性心疾病中的营养障碍基因突变及晚近发现的肌动蛋白基因突变。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "家族性扩张性心肌病" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "基因" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "基因突变" }, { "start_idx": 22, "end_idx": 31, "type": "dis", "entity": "X性连锁扩张性心疾病" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "营养障碍" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "基因突变" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "基因" }, { "start_idx": 48, "end_idx": 55, "type": "sym", "entity": "肌动蛋白基因突变" }, { "start_idx": 48, "end_idx": 53, "type": "bod", "entity": "肌动蛋白基因" } ] }, { "text": "儿童慢性心肌病的其他病因,如先天性心脏病等的分子水平研究较少。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "儿童慢性心肌病" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "许多慢性心肌病都有一共同的基因表达形式,即胎儿基因的表达上调,胎儿肌动蛋白和肌凝蛋白亚型亦出现表达。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "慢性心肌病" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "基因" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "基因的表达上调" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "基因" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "肌动蛋白" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "肌凝蛋白亚型" } ] }, { "text": "此外,已有研究证实,可以改变衰竭心肌获取钙离子能力的钙调蛋白也有显著变化。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "衰竭" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心肌" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "钙离子" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "钙调蛋白" } ] }, { "text": "在形成心功能衰竭的过程中,常伴随有其他蛋白通过转录、翻译、磷酸化激活等方式进行的调节。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "心功能衰竭" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "心" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "蛋白" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "磷酸" } ] }, { "text": "衰竭的心脏可通过增加血管紧张素转换酶活性和心肌张力使心肌细胞局部释放血管紧张素Ⅱ。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "衰竭" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "血管紧张素转换酶" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心肌" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "心肌细胞" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "血管紧张素Ⅱ" } ] }, { "text": "心肌细胞表面血管紧张素Ⅱ受体活性导致磷酸化作用的连锁反应,可以使包括细胞生长和肥大的几个基因出现转录。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "bod", "entity": "心肌细胞表面血管紧张素Ⅱ受体" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "磷酸" }, { "start_idx": 34, "end_idx": 45, "type": "bod", "entity": "细胞生长和肥大的几个基因" } ] }, { "text": "β肾上腺素能系统的重要作用已被转基因鼠模型所证实。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "β肾上腺素能系统" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "基因" } ] }, { "text": "具有心肌特异性β2受体过度表达型的转基因鼠患扩张型心肌病的比例较高。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "sym", "entity": "心肌特异性β2受体过度表达" }, { "start_idx": 2, "end_idx": 16, "type": "bod", "entity": "心肌特异性β2受体" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "扩张型心肌病" } ] }, { "text": "β肾上腺素能信号系统其他方面的过度表达同样可损害心室功能。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "β肾上腺素能信号系统" }, { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "β肾上腺素能信号系统其他方面的过度表达" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "损害心室功能" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心室" } ] }, { "text": "儿科心血管病工作者所面临的挑战是应用这些成果来治疗他们的病人。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dep", "entity": "儿科" }, { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "心血管病" } ] }, { "text": "【临床表现】在充血性心力衰竭的诊断中病史非常重要。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "充血性心力衰竭" } ] }, { "text": "婴儿主要的体力消耗为吃奶,常见症状为吃奶时呼吸急促易疲劳。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "易疲劳" } ] }, { "text": "此外,有反复下呼吸道感染病史。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "反复下呼吸道感染" } ] }, { "text": "肾上腺素能神经紧张性增强致多汗,吃奶时尤甚。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "肾上腺素能神经紧张性增强" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "多汗,吃奶时尤甚" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "汗" } ] }, { "text": "年长儿及青少年可表现为体重减轻、精神不振,另一方面,水潴留可致体重在短期内增加。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "体重减轻" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "体重" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "精神不振" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "水潴留" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "体重在短期内增加" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "体重" } ] }, { "text": "呼吸急促活动能力降低为特征性表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "活动能力降低" } ] }, { "text": "年长儿偶有端坐呼吸或发作性夜间呼吸困难病史,但该主诉在儿科极少见。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "偶有端坐呼吸" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "发作性夜间呼吸困难" }, { "start_idx": 27, "end_idx": 28, "type": "dep", "entity": "儿科" } ] }, { "text": "偶有继发于胃肠道淤血的食欲降低、恶心等症状。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "胃肠道淤血" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "食欲降低" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "恶心" } ] }, { "text": "心力衰竭代偿阶段过度的水盐摄入可加重心力衰竭的症状和体征。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "体格检查可发现体循环心输出量减少,体肺循环静脉淤血,心动过速是机体增加心输出量的一种适应性代偿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "体循环心输出量减少" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "体循环" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "心" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "体肺循环静脉淤血" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "心动过速" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "心" } ] }, { "text": "体循环血量减少肢端发凉、毛细血管再充盈时间延长、周围血管搏动减弱。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "体循环血量减少" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "体循环" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "肢端发凉" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肢端" }, { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "毛细血管再充盈时间延长" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "毛细血管" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "周围血管搏动减弱" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "周围血管" } ] }, { "text": "心脏检查时心力衰竭的征象常被心脏结构异常所遮盖,心脏常扩大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "心脏检查" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "心脏结构异常" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心脏" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "心脏常扩大" } ] }, { "text": "顺应性下降、相对僵硬的心室快速充盈可导致第三心音增强而形成奔马律,此外,还有呼吸急促呼吸困难肋间隙凹陷肺静脉淤血体征水肿可致哮鸣音,湿啰音少见,一旦出现为并发肺炎的表现。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "顺应性下降" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "相对僵硬" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "心室" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "第三心音增强" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "心" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "奔马律" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "呼吸" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "肋间隙" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "肋间隙凹陷" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "肺静脉淤血" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "水肿" }, { "start_idx": 62, "end_idx": 64, "type": "sym", "entity": "哮鸣音" }, { "start_idx": 66, "end_idx": 68, "type": "sym", "entity": "湿啰音" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "肺炎" } ] }, { "text": "严重充血性心力衰竭时,因肺内液体积聚,气体交换出现障碍产生轻度的青紫。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "肺内液体积聚" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺内" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "气体交换出现障碍" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "轻度的青紫" } ] }, { "text": "低心输出量和氧摄取量增加导致周围性发绀,体静脉淤血表现为肝大颈部短,颈静脉扩张不易观察。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "低心输出量" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "氧摄取量增加" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "周围性发绀" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "体静脉淤血" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "体静脉" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肝" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "肝大" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "颈部短" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "颈部" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "颈静脉扩张" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "颈静脉" } ] }, { "text": "外周水肿在婴儿极少见,即使在年长儿亦仅当右侧心力衰竭严重或心室充盈严重受限如限制性心包炎和限制性心肌病时才出现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "外周水肿" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "心室充盈严重受限" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "心室" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "限制性心包炎" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "限制性心肌病" } ] }, { "text": "由纽约心脏协会制定的分类方法对于判定年长儿和青少年心力衰竭严重程度有重要作用。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "该分类方法依据机体因疲劳综合征引起的活动能力受限程度以及是否有因心脏疾病导致的心悸、呼吸困难咽峡炎来进行判定。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "疲劳综合征" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "心脏疾病" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "心悸" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "心" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "呼吸" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "咽峡炎" } ] }, { "text": "对于婴儿和幼儿,Rose等曾提出另一种分类方法,Ⅰ级,无活动受限及症状;Ⅱ级,有中等程度的呼吸急促吃奶时多汗、疲劳及喂奶时间延长、生长发育落后;Ⅲ级,上述症状明显;Ⅳ级,安静时即可有呼吸急促、呻吟或多汗。", "entities": [ { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "吃奶时多汗" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "疲劳" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "喂奶时间延长" }, { "start_idx": 65, "end_idx": 70, "type": "sym", "entity": "生长发育落后" }, { "start_idx": 91, "end_idx": 94, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "呻吟" }, { "start_idx": 99, "end_idx": 100, "type": "sym", "entity": "多汗" } ] }, { "text": "【辅助检查】(一)胸部X线摄片胸部X线片检查均表现为心影扩大,限制性心肌病和缩窄性心包炎例外。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "胸部X线摄片" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "胸部X线片检查" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "胸部X线片检查均表现为心影扩大" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "心" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "限制性心肌病" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "缩窄性心包炎" } ] }, { "text": "肺血管纹理常增多,与肺动静脉淤血胸腔积液。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "肺血管纹理常增多" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺血管" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "肺动静脉淤血" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺动静" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "血" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "胸腔积液" } ] }, { "text": "(二)心电图心电图对诊断心力衰竭无特异性,可表现为非特异性的T波及ST段改变。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "心电图" }, { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "心电图" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 25, "end_idx": 37, "type": "sym", "entity": "非特异性的T波及ST段改变" } ] }, { "text": "(三)实验室检查由于肺静脉严重淤血动脉氧分压降低和呼吸性酸中毒。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺静脉" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "肺静脉严重淤血" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "血" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "动脉氧分压降低" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "动脉氧分压" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "呼吸性酸中毒" } ] }, { "text": "另一方面,代谢性酸中毒意味着严重的体循环障碍。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "体循环障碍" } ] }, { "text": "电解质紊乱包括低钠血症、低氯血症和碳酸氢盐增加。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "电解质紊乱" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低钠血症" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "低氯血症" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "碳酸氢盐增加" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "碳酸氢盐" } ] }, { "text": "低钠血症为水潴留所致,肾脏对呼吸性酸中毒的代偿导致低氯血症和碳酸氢盐增加。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "低钠血症" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "水潴留" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "低氯血症" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "碳酸氢盐增加" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "碳酸氢盐" } ] }, { "text": "(四)超声心动图超声心动图可了解潜在的心脏结构损害及血流动力学异常。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "超声心动图" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "心脏结构损害" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心脏" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "血流动力学异常" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血流" } ] }, { "text": "此外,尚可无创性估计心脏收缩和舒张功能。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" } ] }, { "text": "另外,对心力衰竭患者的随访和对治疗效果的评价的系列研究对临床具有一定的指导意义。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "(五)心导管检查诊断性心导管检查并非必需的检查,但对诊断和治疗有特殊意义时仍需进行。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心导管检查" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "心导管检查" } ] }, { "text": "对疑有心肌病和心内膜弹力纤维增生症者需行心内膜心肌活体组织检查。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "心肌病" }, { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "心内膜弹力纤维增生症" }, { "start_idx": 20, "end_idx": 30, "type": "pro", "entity": "心内膜心肌活体组织检查" } ] }, { "text": "心力衰竭导致心律失常者可考虑心电生理检查。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心律失常" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "心电生理检查" } ] }, { "text": "【治疗】一般治疗包括卧床休息、抬高头部和肩部以改善肺功能,限制液体摄入量,高热能饮食,吸氧,呼吸困难严重时予以机械通气支持。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "卧床休息" }, { "start_idx": 15, "end_idx": 27, "type": "pro", "entity": "抬高头部和肩部以改善肺功能" }, { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "限制液体摄入量" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "高热能饮食" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "吸氧" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "机械通气" } ] }, { "text": "如有大的左向右分流,吸氧宜慎重,因其可降低肺血管阻力而加重左向右的分流。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "大的左向右分流" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "吸氧" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺血管" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "左向右的分流" } ] }, { "text": "但以下原则适用于大多数患者:药物治疗,消除诱发因素(如感染,心律失常,电解质紊乱)及对导致心力衰竭的根本原因进行手术或心导管介入治疗。", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "消除诱发因素" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "感染" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "心律失常" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "电解质紊乱" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "手术" }, { "start_idx": 59, "end_idx": 65, "type": "pro", "entity": "心导管介入治疗" } ] }, { "text": "循环系统机械支持(主动脉内球囊泵或心室辅助系统)可帮助患儿顺利度过危险期。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "循环系统机械支持" }, { "start_idx": 9, "end_idx": 15, "type": "equ", "entity": "主动脉内球囊泵" }, { "start_idx": 17, "end_idx": 22, "type": "equ", "entity": "心室辅助系统" } ] }, { "text": "对于晚期心力衰竭患者心脏移植为唯一的可行措施。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "心脏移植" } ] }, { "text": "(一)利尿剂利尿剂用于减轻心脏过多的容量负荷,降低心室壁压力,从而消除心肌重构的潜在刺激因素。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "利尿剂" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "利尿剂" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "心室壁" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "心肌重构" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心肌" } ] }, { "text": "螺内酯为一种醛固酮拮抗剂,有较轻的利尿效果但因可降低成人心力衰竭患者死亡率和住院率近来正在引起关注。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "螺内酯" }, { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "醛固酮拮抗剂" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "利尿" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "通常和呋塞米联合使用以减少尿中钾离子的丢失。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "呋塞米" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "尿中钾离子的丢失" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "钾离子" } ] }, { "text": "氯噻嗪利尿作用较弱,美托拉宗为一种较强的噻嗪类利尿剂,患儿伴有严重的水潴留且对呋塞米不敏感时使用有确切疗效。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "氯噻嗪" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "利尿" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "美托拉宗" }, { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "噻嗪类利尿剂" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "水潴留" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "呋塞米" } ] }, { "text": "常见并发症有电解质、酸碱平衡紊乱(低钠血症、低钾血症、使用保钾利尿剂所致的高钾血症、低血容量所致的代谢性碱中毒)。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "电解质、酸碱平衡紊乱" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "低钠血症" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "低钾血症" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "保钾利尿剂" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "高钾血症" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "低血容量" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "血" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "代谢性碱中毒" } ] }, { "text": "长期使用袢利尿剂和噻嗪类利尿剂可致高尿酸血症,但患儿常无症状。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "袢利尿剂" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "噻嗪类利尿剂" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "高尿酸血症" } ] }, { "text": "(二)地高辛地高辛为治疗婴儿和儿童心力衰竭的最基本、最常用的洋地黄糖苷类药物,其主要作用为抑制钠钾泵ATP酶活性,减少钠离子由细胞内流出导致钠钙竞争及钠钾交换机制的运行。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "地高辛" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "地高辛" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 30, "end_idx": 37, "type": "dru", "entity": "洋地黄糖苷类药物" }, { "start_idx": 45, "end_idx": 55, "type": "sym", "entity": "抑制钠钾泵ATP酶活性" }, { "start_idx": 47, "end_idx": 53, "type": "bod", "entity": "钠钾泵ATP酶" }, { "start_idx": 59, "end_idx": 67, "type": "sym", "entity": "钠离子由细胞内流出" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "钠离子" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "细胞内" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "钠钙竞争" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "钠" }, { "start_idx": 71, "end_idx": 71, "type": "bod", "entity": "钙" }, { "start_idx": 75, "end_idx": 75, "type": "bod", "entity": "钠" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "钾" } ] }, { "text": "细胞内钙离子浓度逐渐增加,使心肌收缩能力增强。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "细胞内钙离子浓度逐渐增加" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "细胞内" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "钙离子" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "心肌收缩能力增强" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心肌" } ] }, { "text": "心收缩力的增强和临床症状的改善并不一致。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "心收缩力的增强" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "心" } ] }, { "text": "有证据表明,强心甙可以提高副交感神经以及动脉血管压力感受器的活性,从而降低中枢交感神经冲动,产生一种有利的神经体液调节作用。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "强心甙" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "副交感神经" }, { "start_idx": 20, "end_idx": 28, "type": "bod", "entity": "动脉血管压力感受器" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "中枢交感神经" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "神经" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "体液" } ] }, { "text": "地高辛可静脉用于急性的或严重的心力衰竭。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "地高辛" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "静脉" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "但其他可静脉给药的正性肌力药物可能更安全更有效。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "静脉给药" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "正性肌力药物" } ] }, { "text": "许多婴儿和儿童,可不用负荷量只用维持量口服,4至5天内可达洋地黄化量。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "洋地黄" } ] }, { "text": "地高辛治疗量和中毒量非常接近,使用时应慎重,以避免致命的并发症。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "地高辛" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "中毒" } ] }, { "text": "心外表现包括恶心,呕吐,视力障碍和行为异常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心外" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "恶心" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "视力障碍" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "行为异常" } ] }, { "text": "心律失常包括心动过缓、室上性心动过速、室性心动过速、异位节律。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心律失常" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心动过缓" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "室性心动过速" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "异位节律" } ] }, { "text": "地高辛中毒治疗包括停药,测定血药浓度,治疗心律失常,避免低钾血症,如有生命危险可使用特异性抗原结合抗体。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "地高辛中毒" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "停药" }, { "start_idx": 12, "end_idx": 17, "type": "ite", "entity": "测定血药浓度" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "心律失常" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "低钾血症" }, { "start_idx": 40, "end_idx": 50, "type": "pro", "entity": "使用特异性抗原结合抗体" } ] }, { "text": "(三)其他正性肌力药物对于低心排状态的紧急处理可使用某些正性肌力药物静脉滴注,通常此类药物主要具有β1受体兴奋作用。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "正性肌力药物" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "低心排状态" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "心" }, { "start_idx": 28, "end_idx": 33, "type": "dru", "entity": "正性肌力药物" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 49, "end_idx": 58, "type": "bod", "entity": "β1受体" } ] }, { "text": "多巴胺直接刺激β1受体,使心肌释放去甲肾上腺素。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "多巴胺" }, { "start_idx": 7, "end_idx": 16, "type": "bod", "entity": "β1受体" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心肌" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "去甲肾上腺素" } ] }, { "text": "多巴酚丁胺是另一种β1受体兴奋剂,但其影响心肌收缩力的作用与前者相比较弱。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "多巴酚丁胺" }, { "start_idx": 9, "end_idx": 21, "type": "dru", "entity": "β1受体兴奋剂" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心肌" } ] }, { "text": "小剂量的肾上腺素增强心肌收缩力的同时可扩张收缩的血管床,大剂量有强烈的血管收缩作用。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心肌" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "血管" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血管" } ] }, { "text": "异丙肾上腺素只是β1受体和β2受体激动剂,因其可致心律失常临床而少用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "异丙肾上腺素" }, { "start_idx": 8, "end_idx": 31, "type": "dru", "entity": "β1受体和β2受体激动剂" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "心律失常" } ] }, { "text": "(四)血管扩张剂血管扩张剂可降低心脏前后负荷,一定剂量时可降低血压。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "心脏前后" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "血压" } ] }, { "text": "血管扩张剂通过舒张小动脉平滑肌以降低后负荷;另一方面可降低前负荷,以减少肺体循环静脉的淤血。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "小动脉平滑肌" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "肺体循环静脉的淤血" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "肺体循环静脉" } ] }, { "text": "在术后早期,如需控制血压和调节前、后负荷以便达到最大的心输出量,临床上常用硝普钠、硝酸甘油、氨力农、米力农。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "控制血压" }, { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "调节前、后负荷" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "心" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "硝普钠" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "硝酸甘油" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "氨力农" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "米力农" } ] }, { "text": "另一方面,如需长期减轻后负荷,则用硫酸肼屈嗪和血管紧张素转换酶抑制剂。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "长期减轻后负荷" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "硫酸肼屈嗪" }, { "start_idx": 23, "end_idx": 33, "type": "dru", "entity": "血管紧张素转换酶抑制剂" } ] }, { "text": "本类药物中,只有血管紧张素转换酶抑制剂被证实在成人中长期使用可降低死亡率。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "dru", "entity": "血管紧张素转换酶抑制剂" } ] }, { "text": "除血管扩张作用外,ACE抑制剂可防止和逆转心肌纤维化。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血管" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "ACE抑制剂" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "心肌纤维化" } ] }, { "text": "在临床上用血管紧张素转换酶抑制剂治疗患有大量左向右分流的先天性心脏病和扩张型心肌病的婴儿和儿童时,效果良好。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "dru", "entity": "血管紧张素转换酶抑制剂" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "大量左向右分流" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "扩张型心肌病" } ] }, { "text": "临床上多选用卡托普利和依那普利。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "卡托普利" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "依那普利" } ] }, { "text": "应用这些药物可造成高钾血症,因此临床上不应同时补钾,亦不必使用保钾利尿剂(如螺内酯)。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "高钾血症" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "补钾" }, { "start_idx": 31, "end_idx": 35, "type": "dru", "entity": "保钾利尿剂" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "螺内酯" } ] }, { "text": "(五)磷酸二酯酶抑制剂新型的磷酸二酯酶抑制剂可提高心肌收缩力和扩张外周血管。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "磷酸二酯酶抑制剂" }, { "start_idx": 11, "end_idx": 21, "type": "dru", "entity": "新型的磷酸二酯酶抑制剂" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心肌" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "外周血管" } ] }, { "text": "目前,临床上常用的氨力农和米力农主要通过作用于磷酸二酯酶Ⅲ,来抑制cAMP的灭活,心肌细胞内cAMP增加可使细胞内钙离子增加和心肌收缩力增强。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "氨力农" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "米力农" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "磷酸二酯酶Ⅲ" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "cAMP" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "心肌细胞" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "cAMP" }, { "start_idx": 54, "end_idx": 59, "type": "bod", "entity": "细胞内钙离子" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "心肌" } ] }, { "text": "血管平滑肌中cAMP的增加可抑制蛋白激酶活性导致血管扩张和后负荷减低。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "血管平滑肌" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "cAMP" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "蛋白激酶" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "血管" } ] }, { "text": "副反应包括低血压、心律失常和血小板减少,尤在使用氨力农后易发生。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "低血压" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "心律失常" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "血小板减少" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "血小板" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "氨力农" } ] }, { "text": "目前,北美洲多家医疗中心对儿科心脏术后低心排综合征高危人群预防性使用米力农的安全性和有效性的随机双盲安慰剂对照研究正在进行中。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "儿科心脏术" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "低心排综合征" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "米力农" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "安慰剂" } ] }, { "text": "(六)β受体阻滞剂近来的临床应用表明,β受体阻滞剂可通过肌细胞的生物学改变提高心肌收缩力,增加左心室射血分数,降低左心室容量负荷。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "β受体阻滞剂" }, { "start_idx": 19, "end_idx": 24, "type": "dru", "entity": "β受体阻滞剂" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肌细胞" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "心肌" }, { "start_idx": 47, "end_idx": 53, "type": "ite", "entity": "左心室射血分数" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "左心室" } ] }, { "text": "其可能的机制是屏蔽儿茶酚胺的心肌毒性作用,上调β1受体的表达以及逆转过度的神经体液刺激。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "儿茶酚胺" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心肌" }, { "start_idx": 23, "end_idx": 32, "type": "bod", "entity": "β1受体" }, { "start_idx": 38, "end_idx": 48, "type": "sym", "entity": "逆转过度的神经体液刺激" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "神经" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "体液" } ] }, { "text": "有限的研究表明,对儿童此类药物可改善左心室功能,提高运动耐量,减少了特发性心肌病、药物诱发或遗传性心肌病的心脏移植几率。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "左心室" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "特发性心肌病" }, { "start_idx": 41, "end_idx": 51, "type": "dis", "entity": "药物诱发或遗传性心肌病" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "心脏移植" } ] }, { "text": "【展望】随着对心力衰竭发病机制的不断深入了解,新的治疗方法不断涌现。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "新的药物包括血管紧张素受体拮抗剂,内皮素受体拮抗剂,肾素拮抗剂,中枢神经激素调节剂等。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dru", "entity": "血管紧张素受体拮抗剂" }, { "start_idx": 17, "end_idx": 24, "type": "dru", "entity": "内皮素受体拮抗剂" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "肾素拮抗剂" }, { "start_idx": 32, "end_idx": 40, "type": "dru", "entity": "中枢神经激素调节剂" } ] }, { "text": "第三节肺穿刺和肺活检一、肺穿刺肺穿刺是指用针经过胸壁刺入肺内有病变部位,吸取组织、细胞等成分,进行组织学、细胞学检查。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "肺活检" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 22, "end_idx": 22, "type": "equ", "entity": "针" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胸壁" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "肺内有病变部位" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "组织" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "细胞" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "成分" }, { "start_idx": 50, "end_idx": 58, "type": "pro", "entity": "组织学、细胞学检查" } ] }, { "text": "常用于诊断肺内及纵隔内肿块的性质。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "肺内及纵隔内肿块" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肺内" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "纵隔内" } ] }, { "text": "近年来随着X线、超声波、CT及磁共振等影像技术的发展,使肺穿刺吸取活检更为安全可靠。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "X线" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "超声波" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "CT" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "磁共振" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "肺穿刺吸取活检" } ] }, { "text": "因而对肺内及纵隔内局限性肿块以及肺内弥漫性结节状病变,直径在2.0cm以上者均可穿刺活检。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "肺内及纵隔内局限性肿块" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肺内" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "纵隔内" }, { "start_idx": 16, "end_idx": 25, "type": "sym", "entity": "肺内弥漫性结节状病变" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺内" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "穿刺活检" } ] }, { "text": "主要禁忌证包括严重肺气肿、心肺功能不全、病灶周围有肺大疱、活动性肺结核或病灶靠近心脏及大血管、有剧烈咳嗽、出血倾向者及肺内弥漫性病灶直径<2.0cm者。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肺气肿" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "心肺功能不全" }, { "start_idx": 20, "end_idx": 34, "type": "sym", "entity": "病灶周围有肺大疱、活动性肺结核" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "肺大疱" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "活动性肺结核" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "病灶靠近心脏及大血管" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "心脏" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "大血管" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "剧烈咳嗽" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "出血倾向" }, { "start_idx": 59, "end_idx": 73, "type": "sym", "entity": "肺内弥漫性病灶直径<2.0cm" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "肺内" } ] }, { "text": "肺穿刺还可用于细菌性肺炎的病原学检查。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 7, "end_idx": 17, "type": "pro", "entity": "细菌性肺炎的病原学检查" } ] }, { "text": "下呼吸道感染可由不同病原引起,正确的病原学诊断至关重要。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "下呼吸道感染" } ] }, { "text": "但临床常用的呼吸道分泌物取材方法,如咳痰、吸痰,甚至经纤维支气管镜吸引,均不同程度受到鼻咽部正常菌群的污染,而血培养、胸水培养等阳性率较低,因而直接影响了细菌性肺炎的诊断率。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "咳痰" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "吸痰" }, { "start_idx": 27, "end_idx": 34, "type": "pro", "entity": "纤维支气管镜吸引" }, { "start_idx": 43, "end_idx": 52, "type": "sym", "entity": "鼻咽部正常菌群的污染" }, { "start_idx": 43, "end_idx": 49, "type": "mic", "entity": "鼻咽部正常菌群" }, { "start_idx": 55, "end_idx": 65, "type": "sym", "entity": "血培养、胸水培养等阳性" }, { "start_idx": 55, "end_idx": 57, "type": "pro", "entity": "血培养" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "胸水培养" }, { "start_idx": 77, "end_idx": 81, "type": "dis", "entity": "细菌性肺炎" } ] }, { "text": "肺穿刺目前仍作为细菌性肺炎诊断的金标准。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "细菌性肺炎" } ] }, { "text": "但由于其潜在的并发症,不易被医生和患者及其家长接受,因而不宜作为肺炎的常规检查法。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "肺炎" } ] }, { "text": "对肺部感染严重,需立即明确病原体以指导治疗者,或经抗生素治疗无进步者可考虑进行肺穿刺培养。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "肺部感染严重" }, { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "肺部感染" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 39, "end_idx": 43, "type": "pro", "entity": "肺穿刺培养" } ] }, { "text": "具体方法:先通过体格检查及影像学检查定位。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "体格检查" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "影像学检查" } ] }, { "text": "患儿取坐位,如在上叶,亦可采用俯伏位或仰卧位,常规碘酒和70%乙醇消毒,局部麻醉。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "上叶" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "碘酒" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "70%乙醇" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "局部麻醉" } ] }, { "text": "常用7~12号腰穿刺针或其改良针。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "equ", "entity": "7~12号腰穿刺针" }, { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "改良针" } ] }, { "text": "进针时注意避开心脏、大血管、肝脏等重要结构。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "equ", "entity": "针" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "大血管" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肝脏" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "重要结构" } ] }, { "text": "刺针进入肺组织后用负压抽吸,吸出物送检。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "刺针" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肺组织" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "负压抽吸" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "吸出物送检" } ] }, { "text": "肺穿刺最常见的并发症是气胸和咯血,前者可持续数日,而咯血大多较轻,可自行停止。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "气胸" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "咯血" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "血" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "咯血" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" } ] }, { "text": "穿刺过程中应避免患儿深呼吸和剧烈咳嗽,如有头晕、面色苍白、多汗、心悸、剧烈咳嗽者应停止操作。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "穿刺" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "剧烈咳嗽" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "头晕" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "头" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "面色苍白" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "面" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "多汗" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "心悸" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "心" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "剧烈咳嗽" } ] }, { "text": "穿刺后密切观察患儿病情变化,如有烦躁不安、剧咳不止、呼吸急促、痰中带血者,立即进行X线检查。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "穿刺" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "剧咳不止" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "痰中带血" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "痰" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "血" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "X线检查" } ] }, { "text": "病情稳定者,24小时后摄胸片。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "胸片" } ] }, { "text": "最近Vuori-Holopainen对59个肺穿刺研究进行了再评价,认为肺穿刺比一般想象的要安全,与其他常规方法相比,具有许多优点,术后发生的气胸常无症状,多可自然缓解。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 36, "end_idx": 38, "type": "pro", "entity": "肺穿刺" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "气胸" } ] }, { "text": "二、肾脏超声检查小儿肾脏随着年龄增长而生长发育,其本身均有一定的解剖结构,对超声同样形成很好的声学界面,构成肾脏固定的超声形态。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "肾脏超声检查小儿肾脏" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "超声" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肾脏" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "超声" } ] }, { "text": "成为超声检查显示较好的脏器之一,超声不仅显示肾脏的位置、大小、形态和内部结构,还能观察肾脏及周围的各种病变。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "超声检查" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脏器" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "超声" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肾脏" }, { "start_idx": 43, "end_idx": 52, "type": "sym", "entity": "肾脏及周围的各种病变" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肾脏" } ] }, { "text": "尤其是超声检查无痛苦、无创伤、不受肾功能影响、迅速以及可复性强等优点,更适合小儿,成为比较理想的检查方法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "超声检查" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "痛苦" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "创伤" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肾" } ] }, { "text": "1.先天性肾脏发育异常可了解肾缺如、孤立肾、重复肾、肾发育不全、蹄铁形肾以及异位肾。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "先天性肾脏发育异常" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肾缺如" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "孤立肾" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "重复肾" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "肾发育不全" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "蹄铁形肾" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "异位肾" } ] }, { "text": "2.肾内囊性病变如肾囊肿、多囊肾、海绵肾和肾积水等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肾内囊性病变" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肾囊肿" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "多囊肾" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "海绵肾" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肾积水" } ] }, { "text": "3.肾肿瘤小儿肾肿瘤很少为良性,其恶性肿瘤为小儿肿瘤死亡率之前列。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肾肿瘤" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "小儿肾肿瘤" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "小儿肿瘤" } ] }, { "text": "最多见的是肾胚瘤,又称肾母细胞瘤或Wilms瘤,肾癌多见于较大儿童。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肾胚瘤" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "Wilms瘤" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "肾癌" } ] }, { "text": "肾盂肿瘤发病率较肾实质肿瘤为低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肾盂肿瘤" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肾实质肿瘤" } ] }, { "text": "4.结石小儿泌尿系结石发病率低于成人,肾结石更低于膀胱及尿道结石。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "结石" }, { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "小儿泌尿系结石" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "肾结石" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "膀胱及尿道结石" } ] }, { "text": "小儿肾结石有些合并各种各样尿路梗阻、肾畸形以及肾异位等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小儿肾结石" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肾畸形" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肾异位" } ] }, { "text": "无论X线片显示为阳性结石或不显影的阴性结石,超声均可检出。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "X线片显示为阳性结石" }, { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "X线片" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "结石" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "结石" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "超声" } ] }, { "text": "5.肾外伤肾轻度裂伤常不显示,或在肾实质见到片状或不规则之低回声区,肾盏回声光点不规则并增多。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肾外伤" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肾轻度裂伤" }, { "start_idx": 17, "end_idx": 32, "type": "sym", "entity": "肾实质见到片状或不规则之低回声区" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肾实质" }, { "start_idx": 34, "end_idx": 45, "type": "sym", "entity": "肾盏回声光点不规则并增多" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肾盏" } ] }, { "text": "肾盂有积血和肾周有血肿时,可了解出血的多少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肾盂有积血" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肾盂" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "肾周有血肿" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肾周" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "血" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "出血" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "血" } ] }, { "text": "6.感染性肾脏疾病如肾脓肿、脓肾、肾周脓肿以及肾盂肾炎等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "感染性肾脏疾病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾脓肿" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "脓肾" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "肾周脓肿" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "重型肾结核肾实质破坏严重,肾体积增大,形态不规则,受累的肾实质肿胀增厚或变薄,肾盏回声模糊且不规则,肾盂扩张。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "重型肾结核" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "肾实质破坏严重" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾实质" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肾" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "肾体积增大,形态不规则" }, { "start_idx": 25, "end_idx": 37, "type": "sym", "entity": "受累的肾实质肿胀增厚或变薄" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "受累的肾实质" }, { "start_idx": 39, "end_idx": 48, "type": "sym", "entity": "肾盏回声模糊且不规则" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肾盏" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "肾盂扩张" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "肾盂" } ] }, { "text": "肾结核钙化时,可见到强光团或光带回声伴声影。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾结核" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "钙化" }, { "start_idx": 8, "end_idx": 20, "type": "sym", "entity": "见到强光团或光带回声伴声影" } ] }, { "text": "7.弥漫性肾脏疾病弥漫性肾病是多种原因引起的肾实质损害且有不同的病理变化。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "弥漫性肾脏疾病" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "弥漫性肾病" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "肾实质损害" } ] }, { "text": "但通常可归为三大类:第一类是肾实质以充血、水肿为主,包括急性肾小球肾炎、肾病综合征以及狼疮肾;第二类是以结缔组织增生为主,包括慢性肾小球肾炎、肾盂肾炎、肾淀粉样变、肾中毒以及狼疮肾;第三类是肾实质萎缩、纤维化,包括慢性肾小球肾炎、肾盂肾炎、肾硬化、高血压病晚期以及肾动脉狭窄。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肾实质" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "充血" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "水肿" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "急性肾小球肾炎" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "狼疮肾" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "结缔组织增生" }, { "start_idx": 63, "end_idx": 69, "type": "dis", "entity": "慢性肾小球肾炎" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 76, "end_idx": 80, "type": "sym", "entity": "肾淀粉样变" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "肾" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "肾中毒" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "狼疮肾" }, { "start_idx": 95, "end_idx": 103, "type": "sym", "entity": "肾实质萎缩、纤维化" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "肾实质萎缩" }, { "start_idx": 107, "end_idx": 113, "type": "dis", "entity": "慢性肾小球肾炎" }, { "start_idx": 115, "end_idx": 118, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 120, "end_idx": 122, "type": "dis", "entity": "肾硬化" }, { "start_idx": 124, "end_idx": 129, "type": "dis", "entity": "高血压病晚期" }, { "start_idx": 132, "end_idx": 136, "type": "dis", "entity": "肾动脉狭窄" } ] }, { "text": "肾明显缩小,表面不光滑,可为双侧或单侧。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "肾明显缩小,表面不光滑,可为双侧或单侧" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肾" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "表面" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "双侧" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "单侧" } ] }, { "text": "肾实质明显变薄、回声增强、肾锥体与皮质分辨不清,与肾窦分界不清,肾窦回声缩小。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "肾实质明显变薄、回声增强" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肾实质" }, { "start_idx": 13, "end_idx": 30, "type": "sym", "entity": "肾锥体与皮质分辨不清,与肾窦分界不清" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肾锥体" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "皮质" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肾窦" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "肾窦回声缩小" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肾窦" } ] }, { "text": "第八节肾小管间质性肾炎肾小管间质性肾炎(tubulointerstitialnephritis,TIN)是指主要累及肾小管和肾间质的炎症,而肾小球及血管受累相对不明显的一种疾患。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肾小管间质性肾炎" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "肾小管间质性肾炎" }, { "start_idx": 20, "end_idx": 46, "type": "dis", "entity": "tubulointerstitialnephritis" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "TIN" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "肾小管" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "肾间质" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "炎症" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "肾小球" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "血管" } ] }, { "text": "但多年来它的意义特别是在急性或慢性肾衰竭中的意义很少受到重视。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "急性或慢性肾衰竭" } ] }, { "text": "故一旦小儿出现无明确原因的肾功能不全时应想到本症:因急性TIN是可逆的,及时治疗可防治肾功能的恶化。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肾功能不全" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "急性TIN" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肾" } ] }, { "text": "【病因和发病机制】(一)急性TIN在小儿由全身性感染和药物引起者为主。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "急性TIN" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "全身性感染" } ] }, { "text": "1.感染可由病原体直接侵袭间质(肾盂肾炎)或间接(亦称反应性)机制引起。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "感染" }, { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "病原体" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "间质" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "后者如布氏杆菌、白喉棒状杆菌、A族溶血链球菌、支原体及沙门菌;病毒如EB病毒、乙肝病毒、人免疫缺陷病毒(HIV)、川崎病、风疹以及麻疹病毒,也见于寄生虫(蛔虫、利什曼原虫及弓形虫属)感染。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "mic", "entity": "布氏杆菌" }, { "start_idx": 8, "end_idx": 13, "type": "mic", "entity": "白喉棒状杆菌" }, { "start_idx": 15, "end_idx": 21, "type": "mic", "entity": "A族溶血链球菌" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "支原体" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "沙门菌" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 37, "type": "mic", "entity": "EB病毒" }, { "start_idx": 39, "end_idx": 42, "type": "mic", "entity": "乙肝病毒" }, { "start_idx": 44, "end_idx": 50, "type": "mic", "entity": "人免疫缺陷病毒" }, { "start_idx": 52, "end_idx": 54, "type": "mic", "entity": "HIV" }, { "start_idx": 57, "end_idx": 68, "type": "mic", "entity": "川崎病、风疹以及麻疹病毒" }, { "start_idx": 73, "end_idx": 92, "type": "dis", "entity": "寄生虫(蛔虫、利什曼原虫及弓形虫属)感染" } ] }, { "text": "2.药物多种药物可通过过敏机制引起TIN,如抗癫痫药(卡马西平、苯巴比妥及苯妥英钠)、抗炎药(磺胺药)、止痛药(NSAID)、抗生素(尤其是p-内酰胺类,如头孢菌素和青霉素及其衍生物)以及利尿剂等。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "过敏" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "TIN" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "抗癫痫药" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "卡马西平" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "抗炎药" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "磺胺药" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "止痛药" }, { "start_idx": 56, "end_idx": 60, "type": "dru", "entity": "NSAID" }, { "start_idx": 63, "end_idx": 65, "type": "dru", "entity": "抗生素" }, { "start_idx": 70, "end_idx": 75, "type": "dru", "entity": "p-内酰胺类" }, { "start_idx": 78, "end_idx": 81, "type": "dru", "entity": "头孢菌素" }, { "start_idx": 83, "end_idx": 90, "type": "dru", "entity": "青霉素及其衍生物" }, { "start_idx": 94, "end_idx": 96, "type": "dru", "entity": "利尿剂" } ] }, { "text": "某些药物还可在引起微小病变肾病综合征同时发生TIN(如氨苄西林、二苯基乙内酰脲、干扰素、锂、NSAID及利福平)。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "微小病变肾病综合征" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "TIN" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 32, "end_idx": 38, "type": "dru", "entity": "二苯基乙内酰脲" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "干扰素" }, { "start_idx": 44, "end_idx": 44, "type": "dru", "entity": "锂" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "NSAID" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "利福平" } ] }, { "text": "3.免疫性疾病时的TIN全身性免疫性疾患时可同时有肾小球和肾小管间质受累。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "免疫性疾病时的TIN" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "全身性免疫性疾患" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肾小球" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "肾小管间质" } ] }, { "text": "另有作者报告IgA肾病中37%肾小管有免疫复合物沉积,且此类病人肾功恶化之几率亦高。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾小管" }, { "start_idx": 15, "end_idx": 25, "type": "sym", "entity": "肾小管有免疫复合物沉积" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "肾功恶化" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "肾功" } ] }, { "text": "实验室检查有血沉快,血IgG增高,血浆总蛋白增高(>8g/dl),氮质血症,贫血,尿中有白细胞,蛋白尿,糖尿,间质性肾炎改变可自发缓解或于应用皮质激素后完全缓解,但眼色素膜炎常易复发。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "实验室检查" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "血沉快" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "血沉" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "血IgG" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "血IgG增高" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "血浆总蛋白" }, { "start_idx": 17, "end_idx": 31, "type": "sym", "entity": "血浆总蛋白增高(>8g/dl)" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "氮质血症" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "贫血" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "尿" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "尿中有白细胞" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "白细胞" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "蛋白" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "尿" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "糖尿" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "糖" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "尿" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "间质性肾炎" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "皮质激素" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "眼色素膜炎" } ] }, { "text": "(二)慢性TIN可有多种原因,且任何未经控制的急性者也可进入慢性。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "慢性TIN" } ] }, { "text": "尤其<5岁且伴有反复尿路感染者。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "反复尿路感染" } ] }, { "text": "其次为结石、外来肿物压迫及外科手术所致梗阻。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "结石" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "外来肿物压迫" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "外科手术" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "梗阻" } ] }, { "text": "遗传性疾患也可造成慢性TIN,如Alportsyndrome、髓质囊性病、多囊肾(AD,AR)、家族性幼年肾单位肾结核(juvenilenephrophthiasis)以及髓质海绵肾等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传性疾患" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "慢性TIN" }, { "start_idx": 16, "end_idx": 29, "type": "dis", "entity": "Alportsyndrome" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "髓质囊性病" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "多囊肾" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "AD" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "AR" }, { "start_idx": 48, "end_idx": 58, "type": "dis", "entity": "家族性幼年肾单位肾结核" }, { "start_idx": 60, "end_idx": 82, "type": "dis", "entity": "juvenilenephrophthiasis" }, { "start_idx": 86, "end_idx": 90, "type": "dis", "entity": "髓质海绵肾" } ] }, { "text": "病损先见于近曲小管(该处分泌草酸盐),但严重处常见于髓质(该处管内浓度高),且此类病人之草酸钙结石则由于梗阻更加重TIN。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "病损" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "近曲小管" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "草酸盐" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "髓质" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "草酸钙结石" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "梗阻" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "TIN" } ] }, { "text": "任何原因致高血钙则首先可见髓质小管上皮细胞局灶褪变和坏死,后因受累小管萎缩和梗阻致近端小管扩张。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "高血钙" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "髓质小管上皮细胞" }, { "start_idx": 13, "end_idx": 27, "type": "sym", "entity": "髓质小管上皮细胞局灶褪变和坏死" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "坏死" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "受累小管萎缩和梗阻" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "小管萎缩" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "梗阻" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "近端小管" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "近端小管扩张" } ] }, { "text": "受损处的钙沉着可致肾钙化。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "受损处的钙沉着" }, { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "受损处" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "钙" }, { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "肾钙化" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肾" } ] }, { "text": "动物实验证实持久的低钾可致肾间质纤维化和疤痕。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "低钾" }, { "start_idx": 10, "end_idx": 10, "type": "ite", "entity": "钾" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肾间质" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "肾间质纤维化和疤痕" } ] }, { "text": "【病理】急性者主要是肾间质细胞浸润(以淋巴细胞为主,但也可有单核巨噬细胞、嗜酸细胞以及浆细胞和成纤维细胞),水肿和肾小管细胞变平、萎缩、退行性病变及刷状缘消失。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "肾间质细胞" }, { "start_idx": 10, "end_idx": 52, "type": "sym", "entity": "肾间质细胞浸润(以淋巴细胞为主,但也可有单核巨噬细胞、嗜酸细胞以及浆细胞和成纤维细胞)" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "单核巨噬细胞" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "嗜酸细胞" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "浆细胞" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "水肿" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "肾小管细胞" }, { "start_idx": 57, "end_idx": 78, "type": "sym", "entity": "肾小管细胞变平、萎缩、退行性病变及刷状缘消失" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "刷状缘" } ] }, { "text": "电镜下有线粒体损伤、胞浆空泡变性及粗面内质网扩张。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "线粒体损伤" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "胞浆空泡变性" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "胞浆空泡" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "粗面内质网扩张" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "粗面内质网" } ] }, { "text": "免疫荧光检查,一般Ig和补体阴性,但由红斑狼疮、梅毒和乙肝病毒感染引起者可见免疫复合物沉积。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "免疫荧光检查" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "Ig" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "补体" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "红斑狼疮" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "梅毒" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "乙肝病毒感染" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "免疫复合物沉积" } ] }, { "text": "慢性者特点是间质纤维化和小管萎缩,并也常见肾小球硬化、萎缩及肾小球周围纤维化。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "间质" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "间质纤维化" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "小管萎缩" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肾小球" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "肾小球硬化、萎缩" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "肾小球周围纤维化" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "肾小球周围" } ] }, { "text": "【临床表现】急性者病情轻重悬殊,此与病因及肾间质受损和部位有关。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "肾间质受损" } ] }, { "text": "由感染引起者有发热,发生于感染初几天,而很少在10~12天后(此与感染致肾小球损害者不一);由药物过敏引起者有发热(30%~100%)、皮疹(30%~50%)及嗜酸性细胞增多三大症状,此外,还有关节疼(15%~20%)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "感染" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "感染" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肾小球损害" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "药物过敏" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "发热" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "皮疹" }, { "start_idx": 68, "end_idx": 68, "type": "bod", "entity": "皮" }, { "start_idx": 80, "end_idx": 84, "type": "ite", "entity": "嗜酸性细胞" }, { "start_idx": 80, "end_idx": 86, "type": "sym", "entity": "嗜酸性细胞增多" }, { "start_idx": 97, "end_idx": 99, "type": "sym", "entity": "关节疼" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "关节" } ] }, { "text": "由本症导致的急性肾衰中30%~40%为非少尿型。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "急性肾衰" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "尿" } ] }, { "text": "病人可有多饮多尿,夜尿,体重下降,乏力。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "多饮" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "多尿" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "尿" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "尿" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "体重下降" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "体重" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "乏力" } ] }, { "text": "高血压常为后期表见,一般无水肿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血压" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "水肿" } ] }, { "text": "【实验室检查】(一)尿液检查急性者最常见为蛋白尿和镜下血尿。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "尿液检查" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "蛋白" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "镜下血尿" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" } ] }, { "text": "由肾小管损伤所致蛋白尿一般为轻至中度(<1g/24h),其中β2-微球蛋白和其他小分子量蛋白约占50%。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "肾小管损伤" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "蛋白" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "尿" }, { "start_idx": 30, "end_idx": 47, "type": "bod", "entity": "β2-微球蛋白" }, { "start_idx": 49, "end_idx": 56, "type": "bod", "entity": "其他小分子量蛋白" } ] }, { "text": "由药物引起者多有镜下血尿,偶见红细胞管型。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "镜下血尿" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "血" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "尿" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "偶见红细胞管型" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "红细胞管型" } ] }, { "text": "当近端小管功能障碍时有糖尿、磷尿、氨基酸尿和重碳酸盐尿。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "近端小管功能障碍" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "糖尿" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "糖" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "磷尿" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "磷" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "氨基酸尿" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "氨基酸" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "重碳酸盐尿" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "重碳酸盐" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "尿" } ] }, { "text": "药物引起者可仅为糖尿。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "糖尿" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "糖" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" } ] }, { "text": "此外检测磷酸盐重吸收(<80%为异常)和尿钠排泄分数(>3%为异常)可证实近端小管受损。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "ite", "entity": "磷酸盐重吸收" }, { "start_idx": 20, "end_idx": 25, "type": "ite", "entity": "尿钠排泄分数" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "近端小管受损" } ] }, { "text": "远端小管受累可致重碳酸盐尿及肾小管酸中毒,但最常见的是尿浓缩功能减退。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "远端小管" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "重碳酸盐尿" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "重碳酸盐" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "肾小管酸中毒" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "尿浓缩功能减退" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "尿" } ] }, { "text": "慢性TIN也可有上述尿异常,但以失盐和尿浓缩功能减退为最常见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "慢性TIN" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "尿异常" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "尿" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "失盐" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "盐" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "尿浓缩功能减退" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "尿" } ] }, { "text": "病程后期尿呈等张,比重固定在1.015,尿渗透压<300mOsm/L。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "比重" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "尿渗透压" } ] }, { "text": "(二)患者常见贫血,血白细胞增多由药物引起者60%~100%有嗜酸细胞增多;还常伴血中IgE增高(50%病例)。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "贫血" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "血白细胞" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "血白细胞增多" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "嗜酸细胞" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "嗜酸细胞增多" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "血中IgE增高" }, { "start_idx": 41, "end_idx": 45, "type": "ite", "entity": "血中IgE" } ] }, { "text": "急性TIN常见高钾高氯性代谢性酸中毒,此由远端小管功能障碍所致;近端小管障碍则高氯性酸中毒、低磷血症和低尿酸血症,高氯性代谢性酸中毒为诊断急性TIN的重要线索,并有助于区别由急性肾小管坏死或急进性肾炎所致的急性肾衰竭。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "急性TIN" }, { "start_idx": 7, "end_idx": 17, "type": "dis", "entity": "高钾高氯性代谢性酸中毒" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "远端小管功能障碍" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "近端小管障碍" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "高氯性酸中毒" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "低磷血症" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "低尿酸血症" }, { "start_idx": 57, "end_idx": 65, "type": "dis", "entity": "高氯性代谢性酸中毒" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "急性TIN" }, { "start_idx": 87, "end_idx": 93, "type": "dis", "entity": "急性肾小管坏死" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "急进性肾炎" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "【鉴别诊断】急性TIN应与急性肾小球肾炎、急性肾小管坏死(ATN)和血管炎区别。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "急性TIN" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "急性肾小球肾炎" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "急性肾小管坏死" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "ATN" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "血管炎" } ] }, { "text": "AGN多同时有水肿及血压高等表现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "AGN" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "水肿" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "血压" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "血压高" } ] }, { "text": "当病人有用药史,发生急性肾衰竭时应区别ATN和TIN。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "ATN" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "TIN" } ] }, { "text": "注意TIN可能有发热、皮疹及关节痛等过敏反应的表现,血中IgE增高,嗜酸细胞增多,高氯性(阴离子间隙正常)代谢性酸中毒,此外尿/血浆渗透压比例高,尿钠水平低,也助于区别ATN。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "TIN" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "皮疹" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "皮" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "关节" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "关节痛" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "过敏反应" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "血中IgE增高" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "血中IgE" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "嗜酸细胞" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "嗜酸细胞增多" }, { "start_idx": 41, "end_idx": 58, "type": "dis", "entity": "高氯性(阴离子间隙正常)代谢性酸中毒" }, { "start_idx": 62, "end_idx": 68, "type": "ite", "entity": "尿/血浆渗透压" }, { "start_idx": 62, "end_idx": 71, "type": "sym", "entity": "尿/血浆渗透压比例高" }, { "start_idx": 73, "end_idx": 76, "type": "ite", "entity": "尿钠水平" }, { "start_idx": 73, "end_idx": 77, "type": "sym", "entity": "尿钠水平低" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "ATN" } ] }, { "text": "镓扫描发现肾摄取增加提示非特异间质炎症反应。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "镓扫描" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "镓扫描发现肾摄取增加" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肾" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "非特异间质炎症反应" } ] }, { "text": "此外本症停药后90%以上肾功能可改善,确诊尚依赖于肾活体组织检查。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肾" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "【治疗】(一)恰当的治疗涉及各种病因考虑与药物有关应停用并且注意勿用与原药有交叉反应者,如有报告发现由甲氧苯青霉素引起者,当换用萘夫西林或头孢噻吩而再次发生ATN者。", "entities": [ { "start_idx": 51, "end_idx": 56, "type": "dru", "entity": "甲氧苯青霉素" }, { "start_idx": 64, "end_idx": 67, "type": "dru", "entity": "萘夫西林" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "头孢噻吩" }, { "start_idx": 78, "end_idx": 80, "type": "dis", "entity": "ATN" } ] }, { "text": "由感染导致者应治疗感染,小儿由UTO或VUR引起者易反复感染和进行性肾损害,故应考虑给予外科手术矫正。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "感染" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "感染" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "UTO" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "VUR" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "反复感染" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "进行性肾损害" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "外科手术" } ] }, { "text": "(二)支持治疗包括纠正水、电解质紊乱,必要时需行透析。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "支持治疗" }, { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "纠正水、电解质紊乱" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "透析" } ] }, { "text": "(三)有关激素和(或)细胞毒药物之应用因缺乏前瞻对照研究,目前未获结论。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "激素" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "细胞毒药物" } ] }, { "text": "目前一般看法是开始一般治疗后肾功能不见好转或继续恶化者以及少尿型急性肾衰竭时给予泼尼松,小儿患者的效应较快,并常可于2~4周内迅速减量。", "entities": [ { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肾" }, { "start_idx": 14, "end_idx": 25, "type": "sym", "entity": "肾功能不见好转或继续恶化" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "少尿型急性肾衰竭" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "泼尼松" } ] }, { "text": "第三章染色体疾病第一节21-三体综合征【概述】21-三体综合征又称Down综合征(DS),亦称先天愚型,是人类最早被确定的染色体病,是人类最早发现、最为常见的染色体畸变,占小儿染色体病的70%~80%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "染色体疾病" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "Down综合征" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "DS" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "染色体病" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "染色体畸变" }, { "start_idx": 86, "end_idx": 91, "type": "dis", "entity": "小儿染色体病" } ] }, { "text": "60%患儿在胎内早期即夭折流产,存活者有明显的智能落后、特殊面容、生长发育障碍和多发畸形。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "流产" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "明显的智能落后" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "特殊面容" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "面" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "生长发育障碍" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "多发畸形" } ] }, { "text": "【流行病学】本病为小儿染色体病中最常见的一种,活产婴儿中发生率约1∶600~1∶1000,发病率随孕妇年龄增高而增加,母亲怀孕年龄愈大,发病率愈高。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "小儿染色体病" } ] }, { "text": "【病理生理和发病机制】21-三体综合征发生率与母亲怀孕年龄有相关,其发生机制系因亲代(多数为母方)的生殖细胞在减数分裂时染色体不分离所致。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "染色体不分离" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "染色体" } ] }, { "text": "孕妇年龄越大,21-三体综合征发生的可能性越大(表14-6)。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "除有染色体易位外,双亲外周血淋巴细胞核型大都正常21-三体综合征发病率关系【临床表现】本病主要特征为智能落后、特殊面容和生长发育迟缓,并可伴有多种畸形。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "染色体易位" }, { "start_idx": 11, "end_idx": 19, "type": "ite", "entity": "外周血淋巴细胞核型" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "智能落后" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "特殊面容" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "面" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "生长发育迟缓" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "多种畸形" } ] }, { "text": "21-三体综合征的主要特征(表14-14)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "1.特殊面容出生时即有明显的特殊面容,表情呆滞。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "特殊面容" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "面" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "明显的特殊面容" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "面" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "表情呆滞" } ] }, { "text": "眼裂小,眼距宽双眼外眦上斜内眦赘皮鼻梁低平外耳小硬腭窄小常张口伸舌,流涎多,头小而圆,前囟大且关闭延迟,颈短而宽,常呈现嗜睡和喂养困难。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "眼裂小" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "眼裂" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "眼距" }, { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "眼距宽" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "双眼外眦" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "双眼外眦上斜" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "内眦" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "内眦赘皮" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "鼻梁" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "鼻梁低平" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "外耳" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "外耳小" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "硬腭" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "硬腭窄小" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "常张口伸舌" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "口" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "舌" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "流涎多" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "头" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "头小而圆" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "前囟" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "前囟大且关闭延迟" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "颈" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "颈短而宽" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "嗜睡" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "喂养困难" } ] }, { "text": "2.智能落后这是本病最突出、最严重的临床表现。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "智能落后" } ] }, { "text": "绝大部分患儿都有不同程度的智能发育障碍,随年龄的增长日益明显。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "智能发育障碍" } ] }, { "text": "嵌合体型患儿若正常细胞比例较大则智能障碍较轻。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "正常细胞比例较大" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "正常细胞" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "智能障碍" } ] }, { "text": "3.生长发育迟缓患儿出生的身长和体重均较正常儿低,生后体格发育、动作发育均迟缓,身材矮小骨龄落后于实际年龄,出牙迟且顺序异常;四肢短韧带松弛关节可过度弯曲肌张力低下腹膨隆脐疝;手指粗短小指尤短男孩可有隐睾,成年后大多无生育能力。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "生长发育迟缓" }, { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "出生的身长和体重均较正常儿低" }, { "start_idx": 10, "end_idx": 17, "type": "ite", "entity": "出生的身长和体重" }, { "start_idx": 27, "end_idx": 38, "type": "sym", "entity": "体格发育、动作发育均迟缓" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "身材矮小" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "骨龄落后于实际年龄" }, { "start_idx": 54, "end_idx": 61, "type": "sym", "entity": "出牙迟且顺序异常" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "牙" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "四肢短韧带松弛" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "四肢短韧带" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "关节" }, { "start_idx": 70, "end_idx": 76, "type": "sym", "entity": "关节可过度弯曲" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "肌张力" }, { "start_idx": 77, "end_idx": 81, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 82, "end_idx": 82, "type": "bod", "entity": "腹" }, { "start_idx": 82, "end_idx": 84, "type": "sym", "entity": "腹膨隆" }, { "start_idx": 85, "end_idx": 86, "type": "dis", "entity": "脐疝" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "手指" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "手指粗短" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "小指" }, { "start_idx": 92, "end_idx": 95, "type": "sym", "entity": "小指尤短" }, { "start_idx": 100, "end_idx": 101, "type": "dis", "entity": "隐睾" }, { "start_idx": 108, "end_idx": 112, "type": "sym", "entity": "无生育能力" } ] }, { "text": "女孩无月经,仅少数可有生育能力。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "女孩无月经" } ] }, { "text": "约50%患儿伴有先天性心脏病,其次是消化道畸形。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "消化道畸形" } ] }, { "text": "5.皮纹特点手掌出现猿线通关手)、轴三角的atd角度一般大于45°,第4、5指桡箕增多。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "皮纹" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "皮" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "手掌" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "手掌出现猿线" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "通关手" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "手" }, { "start_idx": 17, "end_idx": 25, "type": "ite", "entity": "轴三角的atd角度" }, { "start_idx": 17, "end_idx": 32, "type": "sym", "entity": "轴三角的atd角度一般大于45°" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "第4、5指桡箕增多" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "第4、5指" } ] }, { "text": "【实验室检查】1.外周血细胞染色体核型分析按染色体核型分析可将21-三体综合征患儿分为三型:(1)标准型:患儿体细胞染色体为47条有一个额外的21号染色体核型为47,XX(或XY),+21,此型占全部病例的95%。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 9, "end_idx": 20, "type": "pro", "entity": "外周血细胞染色体核型分析" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "染色体核型分析" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "体细胞染色体" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "体细胞染色体为47条" }, { "start_idx": 65, "end_idx": 76, "type": "sym", "entity": "有一个额外的21号染色体" }, { "start_idx": 68, "end_idx": 76, "type": "bod", "entity": "额外的21号染色体" } ] }, { "text": "(2)易位型:约占2.5%~5%,患儿的染色体总数为46条发生在近端着丝粒染色体的一种相互易位多为D/G易位,D组中以14号染色体为主型为46,XX(或XY),-14,+t(14q21q);少数为15号染色体易位,这种易位型患儿约半数为遗传性,即亲代中有平衡易位染色体携带者。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "染色体" }, { "start_idx": 32, "end_idx": 46, "type": "sym", "entity": "近端着丝粒染色体的一种相互易位" }, { "start_idx": 32, "end_idx": 39, "type": "bod", "entity": "近端着丝粒染色体" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "D/G易位" }, { "start_idx": 59, "end_idx": 64, "type": "bod", "entity": "14号染色体" }, { "start_idx": 98, "end_idx": 105, "type": "sym", "entity": "15号染色体易位" }, { "start_idx": 98, "end_idx": 103, "type": "bod", "entity": "15号染色体" }, { "start_idx": 127, "end_idx": 133, "type": "bod", "entity": "平衡易位染色体" } ] }, { "text": "另一种为G/G易位,较少见,是由于G组中两个21号染色体发生着丝粒融合,形成等臂染色体t(21q21q),或一个21号易位到一个22号染色体上。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "G/G易位" }, { "start_idx": 17, "end_idx": 34, "type": "sym", "entity": "G组中两个21号染色体发生着丝粒融合" }, { "start_idx": 17, "end_idx": 27, "type": "bod", "entity": "G组中两个21号染色体" }, { "start_idx": 36, "end_idx": 51, "type": "sym", "entity": "形成等臂染色体t(21q21q)" }, { "start_idx": 38, "end_idx": 51, "type": "bod", "entity": "等臂染色体t(21q21q)" }, { "start_idx": 54, "end_idx": 70, "type": "sym", "entity": "一个21号易位到一个22号染色体上" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "一个21号" }, { "start_idx": 62, "end_idx": 69, "type": "bod", "entity": "一个22号染色体" } ] }, { "text": "(3)嵌合体型(mosaic)此型约占2%~4%,患者体内具有两种以上细胞系,由于受精卵在早期分裂过程中发生了21号染色体不分离21-三体细胞,形成嵌合体,90%其核型为46,XY(或XX)/47,XY(或XX),+21。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "体内" }, { "start_idx": 31, "end_idx": 37, "type": "bod", "entity": "两种以上细胞系" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "受精卵" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "21号染色体" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "21号染色体不分离" }, { "start_idx": 64, "end_idx": 70, "type": "bod", "entity": "21-三体细胞" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "嵌合体" } ] }, { "text": "细胞遗传学研究发现,在21号染色体长臂21q22区带为三体时,该个体具有完全类似21-三体综合征的临床表现,相反,该区带为非三体的个体则无此典型症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 25, "type": "bod", "entity": "21号染色体长臂21q22区带" }, { "start_idx": 11, "end_idx": 28, "type": "sym", "entity": "21号染色体长臂21q22区带为三体" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 61, "end_idx": 63, "type": "sym", "entity": "非三体" } ] }, { "text": "由此推论,21q22区可能是21-三体综合征的基因关键区带,又称为Down综合征区。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "21q22区" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "基因关键区带" }, { "start_idx": 33, "end_idx": 40, "type": "bod", "entity": "Down综合征区" } ] }, { "text": "2.羊水细胞染色体检查羊水细胞染色体检查是21-三体综合征产前诊断的一种有效方法,常见核型与外周血细胞染色体核型相同。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "羊水细胞染色体检查" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "羊水细胞染色体检查" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "产前诊断" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "核型" }, { "start_idx": 46, "end_idx": 55, "type": "ite", "entity": "外周血细胞染色体核型" } ] }, { "text": "3.荧光原位杂交以21号染色体的相应部位序列作探针,与外周血中的淋巴细胞或羊水细胞进行杂交,可快速、准确进行诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "荧光原位杂交" }, { "start_idx": 9, "end_idx": 21, "type": "bod", "entity": "21号染色体的相应部位序列" }, { "start_idx": 27, "end_idx": 35, "type": "bod", "entity": "外周血中的淋巴细胞" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "羊水细胞" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "进行杂交" } ] }, { "text": "在本病患者的细胞中呈现3个21号染色体的荧光信号。", "entities": [ { "start_idx": 6, "end_idx": 23, "type": "sym", "entity": "细胞中呈现3个21号染色体的荧光信号" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "细胞中" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "21号染色体" } ] }, { "text": "若选择DS关键决定区域的特异序列作探针进行FISH杂交分析,可以对第21号常染色体的异常部位进行精确定位,从而提高检测第21号染色体数目和结构异常的精确性。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "DS" }, { "start_idx": 21, "end_idx": 28, "type": "pro", "entity": "FISH杂交分析" }, { "start_idx": 33, "end_idx": 40, "type": "bod", "entity": "第21号常染色体" }, { "start_idx": 57, "end_idx": 70, "type": "pro", "entity": "检测第21号染色体数目和结构" } ] }, { "text": "4.产前筛查血清标志物目前可在所有孕妇中进行孕早期或者孕中期21-三体综合征产前筛查,采用测定孕妇血清绒毛膜促性腺激素(βHCG)、甲胎蛋白(AFP)及游离雌三醇(FE3),根据孕妇检测此三项值的结果,并结合孕妇年龄,计算出本病的危险度,以决定是否进行产前诊断。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "产前筛查血清标志物" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "产前筛查" }, { "start_idx": 45, "end_idx": 91, "type": "ite", "entity": "测定孕妇血清绒毛膜促性腺激素(βHCG)、甲胎蛋白(AFP)及游离雌三醇(FE3)" } ] }, { "text": "采用这一方法可以检出大约50%~75%的21-三体综合征胎儿。", "entities": [ { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "近年发现的妊娠相关血浆蛋白A(PAPP-A)的诊断价值日益受到重视。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "bod", "entity": "妊娠相关血浆蛋白A" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "PAPP-A" } ] }, { "text": "PAPP-A为胎盘滋养层细胞产生,早期怀21-三体综合征胎儿的孕妇血清水平明显降低,推测可能与滋养层细胞功能降低有关。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "PAPP-A" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "胎盘滋养层细胞" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 33, "end_idx": 36, "type": "ite", "entity": "血清水平" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "血清水平明显降低" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "滋养层细胞" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "滋养层细胞功能降低" } ] }, { "text": "采用PAPP-A、hCG及AFP等指标筛查不仅可筛查出21-三体综合征,还可检出18-三体综合征、先天性神经管缺陷以及先天性腹壁缺损等其他先天异常。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "pro", "entity": "PAPP-A、hCG及AFP等指标筛查" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "18-三体综合征" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "先天性神经管缺陷" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "先天性腹壁缺损" } ] }, { "text": "此外,通过B超测量胎儿颈项皮肤厚度也是诊断21-三体综合征的重要指标。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "equ", "entity": "B超" }, { "start_idx": 7, "end_idx": 16, "type": "pro", "entity": "测量胎儿颈项皮肤厚度" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "【诊断】该病的先天愚型面容、手的特点和智能低下虽然能为临床诊断提供重要线索,但是诊断的建立必须有赖于染色体核型分析,因此染色体核型分析和FISH技术是21-三体综合征的主要实验室检查技术。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "先天愚型面容" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "面" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "手" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "智能低下" }, { "start_idx": 50, "end_idx": 56, "type": "pro", "entity": "染色体核型分析" }, { "start_idx": 60, "end_idx": 66, "type": "pro", "entity": "染色体核型分析" }, { "start_idx": 68, "end_idx": 73, "type": "pro", "entity": "FISH技术" }, { "start_idx": 75, "end_idx": 82, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 86, "end_idx": 92, "type": "pro", "entity": "实验室检查技术" } ] }, { "text": "这两项检查还对21-三体综合征嵌合型的预后估计有积极意义,由于嵌合畸形患儿的表型差异悬殊,可从正常或接近正常到典型的临床表现,他们的预后主要取决于患儿体细胞中正常细胞株所占的百分比率。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "dis", "entity": "21-三体综合征嵌合型" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "嵌合畸形" }, { "start_idx": 77, "end_idx": 77, "type": "bod", "entity": "胞" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "正常细胞株" } ] }, { "text": "因此了解嵌合型患儿体细胞中正常核型细胞与21-三体核型细胞的比例,可以根据其具体情况指导患儿的家庭及社会对其进行教育。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "体细胞中" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "正常核型细胞" }, { "start_idx": 20, "end_idx": 28, "type": "bod", "entity": "21-三体核型细胞" } ] }, { "text": "本病应与先天性甲状腺功能减低症鉴别,后者在出生后即可有嗜睡、哭声嘶哑、喂养困难、腹胀及便秘等症状,舌大而厚,但无本症的特殊面容。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "dis", "entity": "先天性甲状腺功能减低症" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "嗜睡" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "哭声嘶哑" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "喂养困难" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "腹胀" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "腹" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "便秘" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "舌大而厚" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "舌" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "特殊面容" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "面" } ] }, { "text": "可检测血清TSH、T4和染色体核型分析进行鉴别。", "entities": [ { "start_idx": 1, "end_idx": 16, "type": "ite", "entity": "检测血清TSH、T4" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "染色体核型分析" } ] }, { "text": "对患儿宜注意预防感染,如伴有先天性心脏病、胃肠道或其他畸形,可考虑手术矫治。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "感染" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "胃肠道" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "畸形" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "手术矫治" } ] }, { "text": "【预防】1.遗传咨询母亲年龄愈大,风险率愈高。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "遗传咨询" } ] }, { "text": "标准型21-三体综合征的再发风险率为l%。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "标准型21-三体综合征" } ] }, { "text": "易位型患儿的双亲应进行核型分析,以便发现平衡易位携带者:如母方为D/G易位,则每一胎都有10%的风险率;如为父方D/G易位,则风险率为4%。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "核型分析" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "D/G易位" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "D/G易位" } ] }, { "text": "2.产前诊断是防止21-三体综合征患儿出生的有效措施。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "产前诊断" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "已有该病生育史的夫妇再次生育时应作产前诊断,即染色体核型分析,所采用的材料包括孕中期羊膜腔穿刺作羊水细胞、孕中期胚胎绒毛细胞和孕中期脐带血淋巴细胞等分析。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "产前诊断" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "染色体核型分析" }, { "start_idx": 39, "end_idx": 46, "type": "pro", "entity": "孕中期羊膜腔穿刺" }, { "start_idx": 47, "end_idx": 75, "type": "pro", "entity": "作羊水细胞、孕中期胚胎绒毛细胞和孕中期脐带血淋巴细胞等分析" } ] }, { "text": "第七节单心室单心室(singleventricle)是指两个房室瓣仅与一个心室腔连接或与一个伴一个对应的小心室腔主心室连接。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "单心室" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "单心室" }, { "start_idx": 10, "end_idx": 24, "type": "dis", "entity": "singleventricle" }, { "start_idx": 28, "end_idx": 60, "type": "sym", "entity": "两个房室瓣仅与一个心室腔连接或与一个伴一个对应的小心室腔主心室连接" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "两个房室瓣" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "一个心室腔" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "小心室腔主心室" } ] }, { "text": "占婴儿期先天性心脏病发病的第13位,发生率占活产新生儿的0.05‰~0.1‰。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "在胚胎发育早期,以后发育为二尖瓣和三尖瓣房室管与以后发育为左心室的原始心管相连接。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "房室管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "左心室" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心管" } ] }, { "text": "血流从原始心管的心室部分流向原始心球,后者将随后发育为右心室。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心管" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心室" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心球" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "右心室" } ] }, { "text": "当胚胎发育过程中存在任何原因导致心室分隔异常即可形成左心室双入口合并残余右心室流出道腔,此时心室多为反位(左襻)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "胚胎" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "心室分隔异常" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心室" }, { "start_idx": 26, "end_idx": 42, "type": "sym", "entity": "左心室双入口合并残余右心室流出道腔" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "左心室" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "右心室" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "道腔" }, { "start_idx": 46, "end_idx": 55, "type": "sym", "entity": "心室多为反位(左襻)" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "心室" } ] }, { "text": "【病理解剖】单心室下的主心室可为左心室型或右心室型,可通过心室壁的肌小梁形态及房室瓣的位置和形态解剖加以区别。", "entities": [ { "start_idx": 6, "end_idx": 24, "type": "sym", "entity": "单心室下的主心室可为左心室型或右心室型" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "单心室" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "主心室" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "左心室" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "右心室" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "心室壁" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肌小梁" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "房室瓣" } ] }, { "text": "通常肺动脉位于后方,二尖瓣位于右侧,三尖瓣位于左侧。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺动脉" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "三尖瓣" } ] }, { "text": "此种类型的单心室占单心室尸解病例的74%。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "单心室" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "单心室" } ] }, { "text": "有时左主心室与残余右心室腔之间的室间隔缺损随时间推移可逐渐缩小,导致功能性的主动脉下狭窄。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "sym", "entity": "左主心室与残余右心室腔之间的室间隔缺损" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "左主心室" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "右心室腔" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 34, "end_idx": 43, "type": "sym", "entity": "功能性的主动脉下狭窄" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "主动脉下" } ] }, { "text": "近50%的病例伴有肺动脉瓣狭窄或闭锁。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "肺动脉瓣狭窄或闭锁" } ] }, { "text": "伴发水肿或主动脉弓中断多见。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "水肿" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "主动脉弓中断" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "主动脉弓" } ] }, { "text": "无脾综合征多合并右心室单心室中。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "无脾综合征" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "右心室单心室" } ] }, { "text": "【病理生理】由于肺动脉狭窄或肺血管阻力增高使肺循环血流量减少。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "肺血管阻力增高" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺血管" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "肺循环血流量减少" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" } ] }, { "text": "肺循环血流量的大小决定了单心室患儿在婴儿期病情的严重程度。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "肺循环血流量" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "单心室" } ] }, { "text": "青紫的程度取决于来自动脉动脉导管未闭、主肺动脉间侧支血管或支气管循环的肺血流量大小。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "青紫" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "动脉" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "动脉导管" }, { "start_idx": 19, "end_idx": 27, "type": "bod", "entity": "主肺动脉间侧支血管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "支气管" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "肺血流量" } ] }, { "text": "在80%的患儿中,单一的主心室起到一个共同的混合腔的作用而使主动脉和肺动脉内仍能保持理想的氧饱和度。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "单一的主心室" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "混合腔" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "主动脉" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "肺动脉内" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "氧饱和度" } ] }, { "text": "但尽管同样为单心室,有部分患儿虽然有足够的体肺静脉回流,却仍然不能达到充分的混合。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "单心室" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "体肺静脉" } ] }, { "text": "可能的原因是尽管主、肺动脉均起自同一主心室但仍然可存在主动脉接受绝大多数体循环回流血流而肺动脉接受绝大多数肺循环血流的状况。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "主、肺动脉" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "主心室" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "主动脉" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "血" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肺动脉" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "肺" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "血" } ] }, { "text": "甚至有少数患儿在作心房内转换术后体循环氧饱和度改善的报道。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "心房内转换术" }, { "start_idx": 16, "end_idx": 22, "type": "ite", "entity": "体循环氧饱和度" } ] }, { "text": "对于绝大多数的患儿,是否合并大血管异位并不影响血流动力学状况。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "大血管异位" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "大血管" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "血" } ] }, { "text": "在伴有流出道腔入口狭窄的患儿,通常伴有肺动脉环缩或肺动脉狭窄,可导致单心室内压力超过体循环,此时二尖瓣或三尖瓣异常多见。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "流出道腔入口狭窄" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "肺动脉环缩" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "单心室内压力超过体循环" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "单心室内" }, { "start_idx": 48, "end_idx": 56, "type": "sym", "entity": "二尖瓣或三尖瓣异常" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "三尖瓣" } ] }, { "text": "【临床表现】绝大多数的单心室患儿在出生后第一天或出生后数周内被发现。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "单心室" } ] }, { "text": "尽管所有的单心室均伴有青紫,但在无肺动脉狭窄伴充血性心力衰竭的患儿,青紫可较轻而被忽略。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "单心室" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "青紫" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "青紫" } ] }, { "text": "在伴有肺动脉狭窄的患儿,心前区可及明显的收缩期杂音。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 12, "end_idx": 24, "type": "sym", "entity": "心前区可及明显的收缩期杂音" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "心前区" } ] }, { "text": "二尖瓣或三尖瓣反流亦可导致收缩期杂音。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "二尖瓣或三尖瓣反流" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "收缩期杂音" } ] }, { "text": "连续性杂音提示有动脉导管未闭或侧支血管形成。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "连续性杂音" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "动脉导管" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "侧支血管形成" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "侧支血管" } ] }, { "text": "上下肢血压或搏动的差异提示有主动脉弓缩窄的存在。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "上下肢血压或搏动的差异提示有主动脉弓缩窄" }, { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "上下肢血压" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "搏动" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "主动脉弓" } ] }, { "text": "【辅助检查】(一)心电图检查由于心电图通常表现为心室增大,以左心室肥厚常见,因此无特异性。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "心电图检查" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "心电图" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "心室增大" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心室" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "左心室肥厚" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "左心室" } ] }, { "text": "随年龄的增长,心脏节律异常逐渐增多,包括自发的完全性房室传导阻滞和交界性节律。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "心脏节律异常逐渐增多" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "完全性房室传导阻滞" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "交界性节律" } ] }, { "text": "(二)胸部X线检查如无肺动脉狭窄胸片表现为心脏增大肺血管影增粗肺血越多越容易出现充血性心力衰竭,肺血越少,青紫越明显。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 16, "end_idx": 57, "type": "sym", "entity": "胸片表现为心脏增大肺血管影增粗肺血越多越容易出现充血性心力衰竭,肺血越少,青紫越明显" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "胸片" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心脏" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血管" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肺血" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "肺血" } ] }, { "text": "(三)超声心动图检查超声心动图可对单心室进行诊断。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "超声心动图" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "单心室" } ] }, { "text": "尤其在婴儿期超声心动图可进行精确的诊断。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "超声心动图" } ] }, { "text": "因其诊断无特异的切面,检查时需进行系统扫查以寻找残余的心室,明确大动脉的起源,了解房室瓣的位置、形态和活动度,测量残余心室入口的大小及判断可能存在的肺动脉狭窄畸形。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "残余的心室" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心室" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "大动脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "房室瓣" }, { "start_idx": 55, "end_idx": 65, "type": "pro", "entity": "测量残余心室入口的大小" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "畸形" } ] }, { "text": "两个房室瓣入口及室间隔的缺如可通过心室的长轴和短轴切面的扫查得到证实。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "两个房室瓣入口及室间隔的缺如" }, { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "两个房室瓣" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "室间隔" }, { "start_idx": 17, "end_idx": 29, "type": "pro", "entity": "心室的长轴和短轴切面的扫查" } ] }, { "text": "单心室的类型可通过心室腔的解剖形态特征进行判断。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "单心室" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "心室腔" } ] }, { "text": "超声心动图检查时,尤其在长轴切面,右心室型单心室的心室腔中存在的粗大的肌束有时易与室间隔混淆。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "长轴切面" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "右心室型单心室" }, { "start_idx": 25, "end_idx": 36, "type": "sym", "entity": "心室腔中存在的粗大的肌束" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "心室腔" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肌束" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "室间隔" } ] }, { "text": "但在短轴切面时可显示长段的肌束游离在心室腔中,而真正的室间隔往往有很大一部分的组织与前或(和)后游离壁相连。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "短轴切面" }, { "start_idx": 10, "end_idx": 21, "type": "sym", "entity": "长段的肌束游离在心室腔中" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "长段的肌束" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "心室腔" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "室间隔" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "组织" } ] }, { "text": "心尖及剑突下四腔切面可用于显示房室瓣的大小和功能。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "心尖及剑突下四腔切面" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "房室瓣" } ] }, { "text": "在左襻的左心室型单心室,左侧房室瓣(三尖瓣)多为发育不良右侧房室瓣(三尖瓣)往往有明显的反流心尖或胸骨旁彩色多普勒显像可理想显示反流血流,而心尖的脉冲和连续波多普勒检测对测定房室瓣狭窄有用。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "左心室型单心室" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "左侧房室瓣" }, { "start_idx": 12, "end_idx": 27, "type": "sym", "entity": "左侧房室瓣(三尖瓣)多为发育不良" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "右侧房室瓣" }, { "start_idx": 28, "end_idx": 45, "type": "sym", "entity": "右侧房室瓣(三尖瓣)往往有明显的反流" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 46, "end_idx": 67, "type": "sym", "entity": "心尖或胸骨旁彩色多普勒显像可理想显示反流血流" }, { "start_idx": 46, "end_idx": 58, "type": "pro", "entity": "心尖或胸骨旁彩色多普勒显像" }, { "start_idx": 70, "end_idx": 83, "type": "pro", "entity": "心尖的脉冲和连续波多普勒检测" }, { "start_idx": 87, "end_idx": 91, "type": "dis", "entity": "房室瓣狭窄" } ] }, { "text": "当合并有大型的房间隔缺损时,即使有严重的房室瓣狭窄,多普勒超声亦不能检测到任何房室阶差。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "严重的房室瓣狭窄" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "房室瓣狭窄" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "多普勒超声" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "房室阶差" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "房室" } ] }, { "text": "在主、肺动脉位置正常时,肺动脉通常发自流出道腔而主动脉发自左心室型单心室左心室;当存在大动脉异位时,主动脉则发自流出道腔而肺动脉发自左心室型左心室。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "主、肺动脉" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肺动脉" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "道腔" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "主动脉" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "左心室型单心室" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "左心室" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "大动脉异位" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "大动脉" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "主动脉" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "道腔" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "肺动脉" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "左心室型左心室" } ] }, { "text": "肺动脉狭窄可用脉冲或连续波多普勒在心尖或胸骨上窝探测到;在大动脉相对位置正常时,狭窄通常为肌性、肺动脉下或(和)肺动脉瓣狭窄。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 7, "end_idx": 25, "type": "pro", "entity": "脉冲或连续波多普勒在心尖或胸骨上窝探测" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "大动脉" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "狭窄通常为肌性" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肌" }, { "start_idx": 48, "end_idx": 61, "type": "dis", "entity": "肺动脉下或(和)肺动脉瓣狭窄" } ] }, { "text": "在大血管异位时,限制型的室间隔缺损可以导致主动脉下的功能性狭窄。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "大血管异位" }, { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "大血管" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "限制型的室间隔缺损" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "主动脉下的功能性狭窄" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "主动脉下" } ] }, { "text": "在新生儿,多普勒超声可能检测不到限制型室间隔缺损两侧的压差,此时应注重室间隔缺损的大小。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "多普勒超声" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "限制型室间隔缺损" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "超声心动图可显示两种室间隔缺损:①主动脉下室间隔缺损:多伴有圆锥隔的发育不良和对位不良,缺损直接位于瓣下且由于圆锥隔向后方偏移而出现肺动脉下狭窄;②肌部室间隔缺损半月瓣而接近心尖部,通常为限制型。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "超声心动图" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "主动脉下室间隔缺损" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "圆锥隔的发育不良和对位不良" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "圆锥隔" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "缺损直接位于瓣下" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "瓣下" }, { "start_idx": 55, "end_idx": 71, "type": "sym", "entity": "圆锥隔向后方偏移而出现肺动脉下狭窄" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "圆锥隔" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "肺动脉下狭窄" }, { "start_idx": 74, "end_idx": 89, "type": "sym", "entity": "肌部室间隔缺损半月瓣而接近心尖部" }, { "start_idx": 74, "end_idx": 78, "type": "bod", "entity": "肌部室间隔" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "半月瓣" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "心尖部" }, { "start_idx": 94, "end_idx": 96, "type": "dis", "entity": "限制型" } ] }, { "text": "主动脉瓣下狭窄常伴有水肿。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "主动脉瓣下狭窄" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "水肿" } ] }, { "text": "主肺动脉及其分支可在胸骨旁或胸骨上切面进行显示和测量。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "主肺动脉及其分支" }, { "start_idx": 10, "end_idx": 18, "type": "bod", "entity": "胸骨旁或胸骨上切面" } ] }, { "text": "(四)心导管造影检查任何有症状的单心室均需进行心导管造影检查,无症状患儿包括轻度青紫或无充血性心力衰竭的患儿可在生后1月后进行。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "心导管造影检查" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "单心室" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "心导管造影检查" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "轻度青紫" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "充血性心力衰竭" } ] }, { "text": "检查时,需详细检测动脉血氧饱和度、跨瓣压力阶差,尤其需检查残余心室腔入口的压力阶差。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "ite", "entity": "检测动脉血氧饱和度" }, { "start_idx": 17, "end_idx": 22, "type": "ite", "entity": "跨瓣压力阶差" }, { "start_idx": 27, "end_idx": 40, "type": "pro", "entity": "检查残余心室腔入口的压力阶差" } ] }, { "text": "尽管超声心动图可进行流出道梗阻压差的测定,但当在重症患儿心排量较低时超声阶差的测量不敏感,此时需用心导管方法进行测量。", "entities": [ { "start_idx": 2, "end_idx": 19, "type": "pro", "entity": "超声心动图可进行流出道梗阻压差的测定" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "心排量较低" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "心" }, { "start_idx": 34, "end_idx": 40, "type": "pro", "entity": "超声阶差的测量" }, { "start_idx": 49, "end_idx": 57, "type": "pro", "entity": "心导管方法进行测量" } ] }, { "text": "心血管造影进行准确的解剖诊断,应用球囊导管和猪尾巴导管在心室尖部造影效果更佳。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "心血管造影" }, { "start_idx": 15, "end_idx": 33, "type": "pro", "entity": "应用球囊导管和猪尾巴导管在心室尖部造影" } ] }, { "text": "一般多采用2.0~2.5ml/kg造影剂尽快注射。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "造影剂" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "注射" } ] }, { "text": "通常需心房、心室、主动脉和肺动脉造影。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "pro", "entity": "心房、心室、主动脉和肺动脉造影" } ] }, { "text": "【治疗】伴有肺动脉闭锁患儿的存活是由于动脉导管未闭的存在,当出生后动脉导管未闭逐渐关闭时,患儿即处于危重状态。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肺动脉闭锁" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "动脉导管" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "动脉导管" } ] }, { "text": "此时应用前列腺素E可维持动脉导管开放且常需进行体-肺动脉分流术(blalockshunt)。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "前列腺素E" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "动脉导管" }, { "start_idx": 21, "end_idx": 30, "type": "pro", "entity": "进行体-肺动脉分流术" }, { "start_idx": 32, "end_idx": 43, "type": "pro", "entity": "blalockshunt" } ] }, { "text": "对于充血性心力衰竭的患儿尚可进行肺动脉环扎术以帮助其渡过新生儿期。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "肺动脉环扎术" } ] }, { "text": "患儿如病情较稳定,可先处理心外或其他心血管畸形气管食管瘘限制型室间隔缺损导致主动脉下狭窄的程度,而且具有逐渐加重的趋势,因此,任何疑似的残余流出道管腔入口狭窄均必须及时手术处理。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "心外或其他心血管畸形" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "气管食管瘘" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "限制型室间隔缺损" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "主动脉下狭窄" }, { "start_idx": 68, "end_idx": 78, "type": "sym", "entity": "残余流出道管腔入口狭窄" }, { "start_idx": 68, "end_idx": 76, "type": "bod", "entity": "残余流出道管腔入口" }, { "start_idx": 84, "end_idx": 87, "type": "pro", "entity": "手术处理" } ] }, { "text": "对于婴儿患者处理的目的是使其能够尽可能逐渐发育至能够成功进行Fontan手术的年龄,而且能够达到Fontan手术的要求,包括肺动脉平均压和肺阻力正常、心室功能良好、房室瓣完好及肺血管分支形态正常。", "entities": [ { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 48, "end_idx": 55, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 62, "end_idx": 67, "type": "ite", "entity": "肺动脉平均压" }, { "start_idx": 69, "end_idx": 71, "type": "ite", "entity": "肺阻力" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "心室" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "房室瓣" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "肺血管" } ] }, { "text": "理想的肺血流体循环血流并使动脉血氧饱和度维持在75%~85%之间。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "肺血流" }, { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "体循环血流" }, { "start_idx": 13, "end_idx": 19, "type": "ite", "entity": "动脉血氧饱和度" } ] }, { "text": "符合条件的患儿可进行Fontan手术或改良Fontan手术。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 19, "end_idx": 28, "type": "pro", "entity": "改良Fontan手术" } ] }, { "text": "手术应考虑是否将肺静脉和体静脉循环在腔-房水平分开、是否保留两侧房室瓣或将肺静脉隔入完好的一侧房室瓣而关闭另一侧病变的房室瓣。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺静脉" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "体静脉循环" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "腔-房" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "两侧房室瓣" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肺静脉" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "一侧房室瓣" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "房室瓣" } ] }, { "text": "体静脉将直接改道越过心室而直接进入肺动脉。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "体静脉" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心室" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肺动脉" } ] }, { "text": "如果手术指征掌握恰当,Fontan手术的存活率仍较高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "手术" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "Fontan手术" } ] }, { "text": "目前为止,尚无有关多少百分率的单心室婴儿可存活并可适合进行Fontan手术的资料。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "单心室" }, { "start_idx": 29, "end_idx": 36, "type": "pro", "entity": "Fontan手术" } ] }, { "text": "1970年的一份资料提示,近50%的单心室患儿在生后1岁以内死亡。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "单心室" } ] }, { "text": "二、血制品输注不良反应1.即时输血反应异体血制品输入时可出现过敏反应(输血反应),出现发热、皮疹、瘙痒、哮喘等反应,严重时出现过敏性休克。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血制品输注" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "即时输血反应" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "异体血制品" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "输入" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "过敏反应" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "输血" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "输血反应" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "发热" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "皮疹" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "皮" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "瘙痒" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "哮喘" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "过敏性休克" } ] }, { "text": "出现输血反应时应立即停止输入,给予地塞米松5~10mg静脉推注,异丙嗪1mg/kg肌内注射。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "输血" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "输血反应" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "立即停止输入" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "地塞米松" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "静脉推注" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "异丙嗪" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "肌内注射" } ] }, { "text": "2.经输血感染原因有以下几点:①对已知血行传播的传染病检测手段的敏感度不可能达到100%精确;②新出现的未被认识的血源性传染病感染;③输入受污染血制品,造成菌血症、败血症等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "输血" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "感染" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "血行传播的传染病" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "血源性传染病" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "感染" }, { "start_idx": 67, "end_idx": 68, "type": "pro", "entity": "输入" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "血制品" }, { "start_idx": 78, "end_idx": 80, "type": "dis", "entity": "菌血症" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "败血症" } ] }, { "text": "3.多次输注血制品机体反复经受异体弱抗原的刺激,最终可产生足量抗体,增加输血反应,并使血制品输注疗效减低。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "输注血制品" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "异体弱抗原" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "抗体" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "输血" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "输血反应" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "血制品输注" } ] }, { "text": "4.配血或输血错误可出现严重的临床情况表现为寒战、高热、腰背酸痛、黄疸、贫血、少尿、无尿、休克等,严重时可致死。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "配血" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "输血" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "寒战" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "高热" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "腰背酸痛" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "腰" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "背" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "黄疸" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "贫血" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "少尿" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "尿" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "无尿" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "尿" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "休克" } ] }, { "text": "因此实验室及临床工作人员必须仔细核对、严格认真对待输血治疗。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "输血治疗" } ] }, { "text": "临床治疗以糖皮质激素、利尿、抗休克、保持水电解质平衡及对症处理。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "利尿" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "抗休克" }, { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "保持水电解质平衡" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "对症处理" } ] }, { "text": "参考文献1.陈清棠,余宗颐.临床神经病学.北京:北京科学技术出版社,20002.张迅,朱军,须昌隆,等.围产儿先天性脑积水的流行病学调查.现代预防医学,1997,24(3):276-2763.王艳萍,梁娟,朱军,等.1988~1992年我国先天性脑积水发生率的动态变化.实用儿科临床杂志,2000,15(6):324-3244.詹升全,林志俊,李昭杰,等.神经内窥镜手术治疗小儿脑积水.中华神经外科杂志,2000,16(1):14-145.Menkes,JH,SarnatHB.ChildNeurology.6thed.Philadelphia:LippncottWilliams&Wilkins,20006.JohnstonI,TeoC.DisordersofCSFhydrodynamics.ChildsNervSyst,2000,16(10-10):776-7767.MitraDK,SrinivasM.Congenitalhydrocephalus.IndianJPediatr,1997,64(6Suppl):15-158.UpadhyayaP.Hydrocephalus:past,presentandfuture.IndianJPediatr,1997,64(6Suppl):4-49.DrakeJM,KestleJR,TuliS.CSFshunts50yearsonpast,presentandfuture.ChildsNervSyst,2000,16(10-10):800-80410.SatoO,YamguchiT,KittakaM,etal.Hydrocephalusandepilepsy.ChildsNervSyst,2001,17(1-1):76-8611.崔凤成,孔昭江.外部性脑积水.临床儿科杂志,1998,16(3):204-20412.毛萌,朱军.出生缺陷检测研究现状.实用儿科临床杂志,2009,24(11):801-80313.RekateHL.Acontemporarydefinitionandclassificationofhydrocephalus.SeminPediatrNeurol,2009,16(1):9-9", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "神经病" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 120, "end_idx": 125, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 137, "end_idx": 138, "type": "dep", "entity": "儿科" }, { "start_idx": 178, "end_idx": 184, "type": "pro", "entity": "神经内窥镜手术" }, { "start_idx": 187, "end_idx": 191, "type": "dis", "entity": "小儿脑积水" }, { "start_idx": 195, "end_idx": 198, "type": "dep", "entity": "神经外科" }, { "start_idx": 758, "end_idx": 763, "type": "dis", "entity": "外部性脑积水" }, { "start_idx": 767, "end_idx": 768, "type": "dep", "entity": "儿科" }, { "start_idx": 799, "end_idx": 804, "type": "pro", "entity": "出生缺陷检测" }, { "start_idx": 812, "end_idx": 813, "type": "dep", "entity": "儿科" } ] }, { "text": "第八节心室颤动心室颤动(简称室颤)为QRS-T波群消失,呈现不规则的、形状和振幅各异的颤动波,频率在150~500次/分。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心室颤动" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "心室颤动" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "室颤" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "QRS-T波群消失" }, { "start_idx": 28, "end_idx": 59, "type": "sym", "entity": "呈现不规则的、形状和振幅各异的颤动波,频率在150~500次/分" } ] }, { "text": "患儿如不迅速恢复有效心搏则死亡。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "心" } ] }, { "text": "胸前区重击有时可恢复窦性心律。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "胸前区重击" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "窦性心律" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "心" } ] }, { "text": "抢救应人工通气下胸外按摩,直流除颤。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "人工通气下胸外按摩" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "直流除颤" } ] }, { "text": "如除颤无效或室颤复发,可静脉应用溴苄铵托西酸盐,然后再予除颤。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "除颤" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "室颤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "静脉" }, { "start_idx": 16, "end_idx": 22, "type": "dru", "entity": "溴苄铵托西酸盐" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "除颤" } ] }, { "text": "室颤恢复后应寻找基础病因。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "室颤" } ] }, { "text": "应常规测量QT间期排除QT间期延长综合征。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "pro", "entity": "常规测量QT间期" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "QT间期延长综合征" } ] }, { "text": "第九章风湿热【概述】风湿热(rheumaticfever)是A组β溶血性链球菌感染后发生的一种免疫性炎性疾病,特征是累及心脏、关节、中枢神经系统、皮肤及皮下组织等各器官,其中以心脏的非化脓性炎症最为严重且常见。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "风湿热" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "风湿热" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "rheumaticfever" }, { "start_idx": 30, "end_idx": 40, "type": "dis", "entity": "A组β溶血性链球菌感染" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "免疫性炎性疾病" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "心脏" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "关节" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "皮肤" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "皮下组织" }, { "start_idx": 88, "end_idx": 96, "type": "dis", "entity": "心脏的非化脓性炎症" } ] }, { "text": "急性重症风湿热可导致患儿死亡,慢性反复发作可形成风湿性心瓣膜病。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "急性重症风湿热" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "风湿性心瓣膜病" } ] }, { "text": "目前风湿热仍然是全世界儿童和青少年后天性心脏病中最常见的病因之一,也是40岁内人群最常见的心血管病死因之一。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "风湿热" }, { "start_idx": 11, "end_idx": 22, "type": "dis", "entity": "儿童和青少年后天性心脏病" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "心血管病" } ] }, { "text": "从20世纪50年代至80年代,工业发达国家中风湿热的发病率明显下降,美国下降至0.64/10万,下降的原因多认为是风湿热的筛查和预防,青霉素或其他抗菌药物的应用之故,风湿源性(rheumatogenic)菌株减少也可能有关系。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "风湿热" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "风湿热" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "青霉素" }, { "start_idx": 73, "end_idx": 76, "type": "dru", "entity": "抗菌药物" }, { "start_idx": 83, "end_idx": 103, "type": "mic", "entity": "风湿源性(rheumatogenic)菌株" } ] }, { "text": "但是20世纪80年代中期以来,西方发达国家出现风湿热新的局部地区性流行。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "风湿热" } ] }, { "text": "在发展中国家,风湿热和风湿性心脏病仍是常见和严重的。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "风湿热" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "风湿性心脏病" } ] }, { "text": "我国各地发病情况不一样,1986—1990年广东省心血管研究所调查风湿热发病率从33.79/10万下降至22.30/10万,风湿性心脏病患病率从0.79‰下降至0.61‰,虽低于其他发展中国家,但仍明显高于西方发达国家;特别是农村和边远地区发病率仍高,且近年来风湿热发病率有回升趋势,值得重视。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "心血管" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "风湿热" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "风湿性心脏病" }, { "start_idx": 130, "end_idx": 132, "type": "dis", "entity": "风湿热" } ] }, { "text": "【病因和发病机制】风湿热是A组β溶血性链球菌咽峡炎后的自身免疫性疾病,其他组链球菌和其他细菌均证明与风湿热无关。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "风湿热" }, { "start_idx": 13, "end_idx": 24, "type": "dis", "entity": "A组β溶血性链球菌咽峡炎" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 37, "end_idx": 40, "type": "mic", "entity": "组链球菌" }, { "start_idx": 44, "end_idx": 45, "type": "mic", "entity": "细菌" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "风湿热" } ] }, { "text": "风湿热的发病机制与A组β溶血性链球菌的特殊结构成分和细胞外产物有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "风湿热" }, { "start_idx": 9, "end_idx": 17, "type": "mic", "entity": "A组β溶血性链球菌" }, { "start_idx": 26, "end_idx": 30, "type": "mic", "entity": "细胞外产物" } ] }, { "text": "A组β溶血性链球菌的抗原性很复杂,其荚膜由透明质酸组成,与人体关节、滑膜有共同抗原;其细胞壁外层蛋白质中M蛋白和M相关蛋白、中层多糖中N-乙酰葡萄胺和鼠李糖均与人体心肌和瓣膜有共同抗原;其细胞膜的蛋白与人体心肌肌膜和丘脑下核、尾状核之间有共同抗原。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "mic", "entity": "A组β溶血性链球菌" }, { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "抗原" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "荚膜" }, { "start_idx": 21, "end_idx": 24, "type": "mic", "entity": "透明质酸" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "关节" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "滑膜" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "抗原" }, { "start_idx": 43, "end_idx": 50, "type": "bod", "entity": "细胞壁外层蛋白质" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "M蛋白" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "M相关蛋白" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "中层多糖" }, { "start_idx": 67, "end_idx": 73, "type": "bod", "entity": "N-乙酰葡萄胺" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "鼠李糖" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "心肌" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "瓣膜" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "抗原" }, { "start_idx": 94, "end_idx": 99, "type": "bod", "entity": "细胞膜的蛋白" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "心肌肌膜" }, { "start_idx": 108, "end_idx": 111, "type": "bod", "entity": "丘脑下核" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "尾状核" }, { "start_idx": 121, "end_idx": 122, "type": "bod", "entity": "抗原" } ] }, { "text": "这样链球菌感染后,肌体产生抗链球菌抗体,一方面可清除链球菌起保护作用,另一方面可与人体组织产生免疫交叉反应导致器官损害,链球菌抗原的分子模拟是风湿热发病的主要机制。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肌体" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "抗链球菌抗体" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "链球菌" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "组织" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "器官损害" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "链球菌抗原" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "风湿热" } ] }, { "text": "链球菌抗原与抗链球菌抗体还可以形成循环免疫复合物在人体关节滑膜、心肌及心瓣膜等沉积后,激活补体成分产生炎性病变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "链球菌抗原" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "抗链球菌抗体" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "循环免疫复合物" }, { "start_idx": 17, "end_idx": 40, "type": "sym", "entity": "循环免疫复合物在人体关节滑膜、心肌及心瓣膜等沉积" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "关节滑膜" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "心肌" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "心瓣膜" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "补体" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "炎性病变" } ] }, { "text": "此外A组链球菌还可以产生多种外毒素和胞外酶,部分对人体组织如心肌和关节有毒性作用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "mic", "entity": "A组链球菌" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "外毒素" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "胞外酶" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "组织" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心肌" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "关节" } ] }, { "text": "宿主的遗传易感染性或免疫应答性改变在风湿热发病机制中起一定作用。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "sym", "entity": "遗传易感染性或免疫应答性改变" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "风湿热" } ] }, { "text": "【病理】可分为以下几个期:(一)变性渗出期受累部位如心脏、关节及皮肤等结缔组织变性和水肿,淋巴细胞和浆细胞浸润;心包膜纤维素性渗出,关节腔内浆液性渗出。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心脏" }, { "start_idx": 26, "end_idx": 43, "type": "sym", "entity": "心脏、关节及皮肤等结缔组织变性和水肿" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "关节" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "皮肤" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "结缔组织" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 45, "end_idx": 54, "type": "sym", "entity": "淋巴细胞和浆细胞浸润" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "浆细胞" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "心包膜" }, { "start_idx": 56, "end_idx": 64, "type": "sym", "entity": "心包膜纤维素性渗出" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "关节腔内" }, { "start_idx": 66, "end_idx": 74, "type": "sym", "entity": "关节腔内浆液性渗出" } ] }, { "text": "(二)增殖期本期特点为Aschoff小体(风湿小体)的形成。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "Aschoff小体" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "风湿小体" } ] }, { "text": "Aschoff小体为一位于血管周围的局灶性胶原纤维素样坏死,外周有淋巴细胞、浆细胞和巨大的多核细胞(风湿细胞)的浸润结缔组织,好发部位为心肌、心瓣膜、心外膜、关节处皮下组织和腱鞘,是诊断风湿热的病理依据,表示风湿活动。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "Aschoff小体" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "血管周围" }, { "start_idx": 13, "end_idx": 28, "type": "sym", "entity": "血管周围的局灶性胶原纤维素样坏死" }, { "start_idx": 18, "end_idx": 28, "type": "dis", "entity": "局灶性胶原纤维素样坏死" }, { "start_idx": 30, "end_idx": 61, "type": "sym", "entity": "外周有淋巴细胞、浆细胞和巨大的多核细胞(风湿细胞)的浸润结缔组织" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "浆细胞" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "巨大的多核细胞" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "风湿细胞" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "结缔组织" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "心肌" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "心瓣膜" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "心外膜" }, { "start_idx": 79, "end_idx": 85, "type": "bod", "entity": "关节处皮下组织" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "腱鞘" }, { "start_idx": 93, "end_idx": 95, "type": "dis", "entity": "风湿热" }, { "start_idx": 104, "end_idx": 105, "type": "dis", "entity": "风湿" } ] }, { "text": "(三)硬化期Aschoff小体中央变性和坏死物质被吸收,炎症细胞减少,纤维组织增生和疤痕形成;心瓣膜增厚形成疤痕,本期约持续2~3个月。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "bod", "entity": "Aschoff小体" }, { "start_idx": 6, "end_idx": 18, "type": "sym", "entity": "Aschoff小体中央变性" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "坏死物质" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "炎症细胞减少" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "炎症细胞" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "纤维组织增生" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "疤痕形成" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "心瓣膜" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "心瓣膜增厚" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "疤痕" } ] }, { "text": "此外,大脑皮层、小脑及基底核可见散在非特异性细胞变性。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 3, "end_idx": 25, "type": "sym", "entity": "大脑皮层、小脑及基底核可见散在非特异性细胞变性" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "小脑" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "基底核" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "细胞" } ] }, { "text": "【临床表现】风湿热临床表现轻重不一,取决于疾病侵犯部位和程度。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "风湿热" } ] }, { "text": "风湿热仅发生于上呼吸道链球菌感染后,潜伏期1周至数周;发作活动期如不经治疗,一般不超过6个月;如不进行预防,可以反复周期性发作。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "风湿热" }, { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "上呼吸道链球菌感染" } ] }, { "text": "风湿热多呈急性起病,亦可为隐匿性进程,风湿热临床表现主要为心脏炎、关节炎、舞蹈症、皮下小结和环形红斑;发热和关节炎是最常见的主诉,证明原有链球菌感染是必需的诊断条件;咽拭培养阳性或抗链球菌抗体阳性可证明有过链球菌感染。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "风湿热" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "风湿热" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "心脏炎" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "关节炎" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "舞蹈症" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "皮下小结" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "环形红斑" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "发热" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "关节炎" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 83, "end_idx": 86, "type": "pro", "entity": "咽拭培养" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "咽拭培养阳性" }, { "start_idx": 90, "end_idx": 97, "type": "sym", "entity": "抗链球菌抗体阳性" }, { "start_idx": 90, "end_idx": 95, "type": "ite", "entity": "抗链球菌抗体" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "链球菌感染" } ] }, { "text": "(一)一般表现发热、不适、疲倦、胃纳不佳、面色苍白、多汗和腹痛等,个别有胸膜炎和肺炎。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "疲倦" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "胃纳不佳" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "胃" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "面色苍白" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "面" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "多汗" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "汗" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "腹痛" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "腹" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "肺炎" } ] }, { "text": "(二)心脏炎急性风湿热最特征的表现是心脏炎,是唯一的持续性器官损害,初次发作时心肌、心内膜和心包膜均可累及,以心肌炎和心内膜炎最多见,亦可发生全心炎,发生率为40%~50%,一般起病1~2周内出现症状。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "心脏炎" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "急性风湿热" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "心脏炎" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "持续性器官损害" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "心肌" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "心内膜" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "心包膜" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "心肌炎" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "全心炎" } ] }, { "text": "1.心肌炎轻者可无症状,重者可伴不同程度的心力衰竭;安静时心动过速,与体温升高不成比例;心脏扩大,心尖搏动弥散;心音低钝,可见奔马律;心尖部可闻及轻度收缩期杂音,主动脉瓣区可闻及舒张中期杂音。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "心肌炎" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "心动过速" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "体温升高" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "体温" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "心脏" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "心尖搏动弥散" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "心尖" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "心音低钝" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "心" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "见奔马律" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "心尖部" }, { "start_idx": 67, "end_idx": 79, "type": "sym", "entity": "心尖部可闻及轻度收缩期杂音" }, { "start_idx": 81, "end_idx": 85, "type": "bod", "entity": "主动脉瓣区" }, { "start_idx": 81, "end_idx": 94, "type": "sym", "entity": "主动脉瓣区可闻及舒张中期杂音" } ] }, { "text": "X线检查有心脏扩大,心脏搏动减弱;ECG示P-R间期延长,伴有T波低平和ST段异常,或有心律失常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "X线检查" }, { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "X线检查有心脏扩大,心脏搏动减弱" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心脏" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "ECG" }, { "start_idx": 17, "end_idx": 40, "type": "sym", "entity": "ECG示P-R间期延长,伴有T波低平和ST段异常" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "心律失常" } ] }, { "text": "2.心内膜炎主要侵犯二尖瓣和/或主动脉瓣,造成关闭不全;二尖瓣关闭不全表现为心尖部2~3/6级吹风样全收缩期杂音,向腋下传导,有时可闻及二尖瓣相对狭窄所致舒张中期杂音;主动脉瓣关闭不全时胸骨左缘第三肋间可闻及舒张期叹气样杂音;急性期瓣膜损害多为充血性水肿,恢复期可渐消失;多次复发可造成心瓣膜永久性瘢痕形成,导致风湿性心瓣膜病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "关闭不全" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "二尖瓣关闭不全" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "心尖部" }, { "start_idx": 38, "end_idx": 61, "type": "sym", "entity": "心尖部2~3/6级吹风样全收缩期杂音,向腋下传导" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "腋下" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "二尖瓣相对狭窄" }, { "start_idx": 77, "end_idx": 82, "type": "sym", "entity": "舒张中期杂音" }, { "start_idx": 84, "end_idx": 91, "type": "dis", "entity": "主动脉瓣关闭不全" }, { "start_idx": 93, "end_idx": 111, "type": "sym", "entity": "胸骨左缘第三肋间可闻及舒张期叹气样杂音" }, { "start_idx": 93, "end_idx": 100, "type": "bod", "entity": "胸骨左缘第三肋间" }, { "start_idx": 113, "end_idx": 119, "type": "dis", "entity": "急性期瓣膜损害" }, { "start_idx": 122, "end_idx": 126, "type": "dis", "entity": "充血性水肿" }, { "start_idx": 143, "end_idx": 152, "type": "sym", "entity": "心瓣膜永久性瘢痕形成" }, { "start_idx": 143, "end_idx": 145, "type": "bod", "entity": "心瓣膜" }, { "start_idx": 156, "end_idx": 162, "type": "dis", "entity": "风湿性心瓣膜病" } ] }, { "text": "超声心动图检查能更敏感地发现临床听诊无异常的隐匿性心瓣膜炎。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "听诊" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "隐匿性心瓣膜炎" } ] }, { "text": "3.心包炎积液量很少时,临床上难以发现;典型症状为心前区疼痛,心底部听到心包摩擦音;积液量多时心前区搏动消失,心音遥远,有颈静脉怒张、肝肿大等心包填塞表现;X线检查心影向两侧扩大呈烧瓶形,ECG示低电压,早期ST段抬高,随后ST段回到等电位,并出现T波改变超声心动图可确诊少量心包积液。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "心包炎" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "积液" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "积液量很少" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "心前区疼痛" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "心前区" }, { "start_idx": 31, "end_idx": 40, "type": "sym", "entity": "心底部听到心包摩擦音" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "心底部" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "心包" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "积液量多" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "积液" }, { "start_idx": 47, "end_idx": 53, "type": "sym", "entity": "心前区搏动消失" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "心前区" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "心音遥远" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "心" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "颈静脉怒张" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "颈静脉" }, { "start_idx": 67, "end_idx": 69, "type": "sym", "entity": "肝肿大" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "肝" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "心包填塞" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "心包" }, { "start_idx": 78, "end_idx": 81, "type": "pro", "entity": "X线检查" }, { "start_idx": 78, "end_idx": 92, "type": "sym", "entity": "X线检查心影向两侧扩大呈烧瓶形" }, { "start_idx": 82, "end_idx": 82, "type": "bod", "entity": "心" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "两侧" }, { "start_idx": 94, "end_idx": 96, "type": "pro", "entity": "ECG" }, { "start_idx": 94, "end_idx": 127, "type": "sym", "entity": "ECG示低电压,早期ST段抬高,随后ST段回到等电位,并出现T波改变" }, { "start_idx": 128, "end_idx": 132, "type": "pro", "entity": "超声心动图" }, { "start_idx": 136, "end_idx": 141, "type": "dis", "entity": "少量心包积液" } ] }, { "text": "临床上有心包炎表现者,提示心脏炎严重。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "心包炎" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "心脏炎" } ] }, { "text": "风湿性心脏炎初次发作约有5%~10%患儿发生充血性心力衰竭,再发时发生率更高。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "风湿性心脏炎" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "充血性心力衰竭" } ] }, { "text": "近期发生风湿热的病例如果伴有心力衰竭,提示有活动性心脏炎存在。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "风湿热" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "心脏炎" } ] }, { "text": "(三)关节炎见于75%初次发作患儿,侵犯大关节,以膝、踝、肘和腕多见,表现为关节红肿热痛,活动受限,可同时侵犯数个关节,或从1个关节到另1个关节游走;关节炎最终消退不留畸形。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "关节炎" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "大关节" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "膝" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "踝" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肘" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "腕" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "关节" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "关节红肿热痛" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "活动受限" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "数个关节" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "1个关节" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "另1个关节" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "关节炎" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "畸形" } ] }, { "text": "(四)舞蹈病也称Sydenham舞蹈病,表现为全身或部分肌肉的无目的的不自主快速运动,如伸舌歪嘴,挤眉弄眼,耸肩缩颈,言语障碍,书写困难,细微动作不协调,在兴奋或注意力集中时加剧,入睡后即可消失,伴肌无力,情绪不稳定;占风湿热患儿10%,常在其他症状后约数月出现,如风湿热发作较轻,舞蹈病可能为首发症状。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "舞蹈病" }, { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "Sydenham舞蹈病" }, { "start_idx": 23, "end_idx": 41, "type": "sym", "entity": "全身或部分肌肉的无目的的不自主快速运动" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "全身或部分肌肉" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "伸舌歪嘴" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "舌" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "嘴" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "挤眉弄眼" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "眉" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "眼" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "耸肩缩颈" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "肩" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "颈" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "言语障碍" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "书写困难" }, { "start_idx": 69, "end_idx": 75, "type": "sym", "entity": "细微动作不协调" }, { "start_idx": 99, "end_idx": 101, "type": "sym", "entity": "肌无力" }, { "start_idx": 99, "end_idx": 99, "type": "bod", "entity": "肌" }, { "start_idx": 103, "end_idx": 107, "type": "sym", "entity": "情绪不稳定" }, { "start_idx": 110, "end_idx": 112, "type": "dis", "entity": "风湿热" }, { "start_idx": 133, "end_idx": 135, "type": "dis", "entity": "风湿热" }, { "start_idx": 141, "end_idx": 143, "type": "dis", "entity": "舞蹈病" } ] }, { "text": "(五)皮肤症状见于5%患儿:1.环形红斑较少见,环形或半环形边界明显的粉红色红斑,大小变化很大,中心苍白,出现在躯干和四肢近端,呈一过性,或时隐时现呈迁延性,可持续数周。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "皮肤症状" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "环形红斑" }, { "start_idx": 24, "end_idx": 77, "type": "sym", "entity": "环形或半环形边界明显的粉红色红斑,大小变化很大,中心苍白,出现在躯干和四肢近端,呈一过性,或时隐时现呈迁延性" } ] }, { "text": "2.皮下结节少见,常伴有严重心脏炎,呈坚硬无痛结节,与皮肤不粘连,直径0.1~1cm,出现于肘、腕及踝等关节伸面,或枕部、前额头皮以及胸、腰椎脊突链球菌感染证据咽拭培养可发现A组β溶血性链球菌;近年来开展的咽分泌物A组链球菌抗原的快速鉴定,敏感性与特异性均很高,阳性率达90%左右;测定血清抗链球菌抗体,链球菌感染1周后血清ASO滴度开始上升,2个月后逐渐下降,80%患儿ASO升高;同时测定抗脱氧核糖核酸酶B(Anti-DNaseB)、抗链球菌激酶(ASK)及抗透明质酸酶(AH)则阳性率可提高到95%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "皮下结节" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "皮下" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "心脏炎" }, { "start_idx": 19, "end_idx": 41, "type": "sym", "entity": "坚硬无痛结节,与皮肤不粘连,直径0.1~1cm" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "皮肤" }, { "start_idx": 46, "end_idx": 55, "type": "bod", "entity": "肘、腕及踝等关节伸面" }, { "start_idx": 58, "end_idx": 64, "type": "bod", "entity": "枕部、前额头皮" }, { "start_idx": 67, "end_idx": 72, "type": "bod", "entity": "胸、腰椎脊突" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "咽拭培养" }, { "start_idx": 87, "end_idx": 95, "type": "mic", "entity": "A组β溶血性链球菌" }, { "start_idx": 103, "end_idx": 118, "type": "pro", "entity": "咽分泌物A组链球菌抗原的快速鉴定" }, { "start_idx": 141, "end_idx": 150, "type": "pro", "entity": "测定血清抗链球菌抗体" }, { "start_idx": 152, "end_idx": 156, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 160, "end_idx": 166, "type": "ite", "entity": "血清ASO滴度" }, { "start_idx": 186, "end_idx": 190, "type": "sym", "entity": "ASO升高" }, { "start_idx": 186, "end_idx": 188, "type": "ite", "entity": "ASO" }, { "start_idx": 194, "end_idx": 240, "type": "pro", "entity": "测定抗脱氧核糖核酸酶B(Anti-DNaseB)、抗链球菌激酶(ASK)及抗透明质酸酶(AH)" } ] }, { "text": "(二)风湿热活动性指标包括白细胞计数和中性粒细胞增高、血沉增快、C-反应蛋白阳性、α2球蛋白增高以及黏蛋白增高等,但均为非特异性。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "风湿热" }, { "start_idx": 13, "end_idx": 25, "type": "sym", "entity": "白细胞计数和中性粒细胞增高" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "白细胞计数" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "中性粒细胞" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "血沉增快" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "血沉" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "C-反应蛋白阳性" }, { "start_idx": 32, "end_idx": 37, "type": "ite", "entity": "C-反应蛋白" }, { "start_idx": 41, "end_idx": 53, "type": "sym", "entity": "α2球蛋白增高" }, { "start_idx": 41, "end_idx": 51, "type": "ite", "entity": "α2球蛋白" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "黏蛋白增高" }, { "start_idx": 56, "end_idx": 58, "type": "ite", "entity": "黏蛋白" } ] }, { "text": "【诊断和鉴别诊断】(一)诊断标准风湿热的诊断有赖于临床表现和实验室检查的综合分析;1992年修改的Jones诊断标准包括3个部分,在确定链球菌感染证据的前提下,有2项主要表现或1项主要表现伴2项次要表现即可作出诊断(表17-17)。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "风湿热" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "实验室检查" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "Jones" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "链球菌感染" } ] }, { "text": "表17-14风湿热的诊断标准注:主要表现为关节炎者,关节痛不再作为次要表现;主要表现为心脏炎者,P-R间期延长不再作为次要表现(二)鉴别诊断风湿热需与下列疾病进行鉴别:1.幼年型类风湿性关节炎常侵犯指趾小关节,关节炎无游走性特点。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "风湿热" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "关节炎" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "关节痛" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "关节" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "心脏炎" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "P-R间期延长" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "风湿热" }, { "start_idx": 86, "end_idx": 95, "type": "dis", "entity": "幼年型类风湿性关节炎" }, { "start_idx": 99, "end_idx": 103, "type": "bod", "entity": "指趾小关节" }, { "start_idx": 105, "end_idx": 107, "type": "dis", "entity": "关节炎" } ] }, { "text": "反复发作后遗留关节畸形,病程长者X线骨关节摄片可见关节面破坏、关节间隙变狭窄和邻近骨骼骨质疏松,很少侵犯心脏,心瓣膜病更少见。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "关节畸形" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "X线" }, { "start_idx": 16, "end_idx": 46, "type": "sym", "entity": "X线骨关节摄片可见关节面破坏、关节间隙变狭窄和邻近骨骼骨质疏松" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "骨关节摄片" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "关节面" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "关节间隙" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "邻近骨骼" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "心脏" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "心瓣膜病" } ] }, { "text": "2.急性白血病除发热、骨关节疼痛、贫血、出血倾向、肝、脾及淋巴结肿大。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "急性白血病" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "骨关节" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "骨关节疼痛" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "贫血" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "出血倾向" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "肝、脾及淋巴结肿大" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肝" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "脾" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "淋巴结" } ] }, { "text": "周围血片可见幼稚白细胞,骨髓检查可予鉴别。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "周围血片可见幼稚白细胞" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "幼稚白细胞" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "骨髓检查" } ] }, { "text": "3.感染性心内膜炎先天性心脏病或风湿心脏病合并感染性心内膜炎时,易与风湿性心脏病伴风湿活动相混淆,贫血、肝脾肿大、皮肤瘀斑或其他栓塞症状有助诊断,超声心动图可看到心瓣膜或心内膜有赘生物,血培养阳性可确诊。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "风湿心脏病" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "风湿性心脏病" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "风湿" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "贫血" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "肝" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "脾" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "皮肤瘀斑" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "皮肤" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "栓塞" }, { "start_idx": 73, "end_idx": 77, "type": "pro", "entity": "超声心动图" }, { "start_idx": 73, "end_idx": 91, "type": "sym", "entity": "超声心动图可看到心瓣膜或心内膜有赘生物" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "心瓣膜" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "心内膜" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "赘生物" }, { "start_idx": 93, "end_idx": 97, "type": "sym", "entity": "血培养阳性" }, { "start_idx": 93, "end_idx": 95, "type": "pro", "entity": "血培养" } ] }, { "text": "【治疗】(一)休息卧床休息的期限取决于心脏受累程度和心功能状态;急性期无心脏炎患儿卧床休息2周,随后逐渐恢复活动,4周后达正常活动水平;心脏炎无心力衰竭患儿则卧床休息4周,8周内逐渐恢复活动;心脏炎伴充血性心力衰竭患儿则卧床休息至少8周,在4~6个月内逐渐恢复正常活动量。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "休息" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "卧床休息" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心脏" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "心" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "心脏炎" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "卧床休息" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "心脏炎" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 79, "end_idx": 82, "type": "pro", "entity": "卧床休息" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "心脏炎" }, { "start_idx": 100, "end_idx": 106, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 110, "end_idx": 113, "type": "pro", "entity": "卧床休息" } ] }, { "text": "(二)控制链球菌感染应用大剂量青霉素静脉滴注,或肌肉注射10~14天;以彻底清除链球菌感染。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "青霉素" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "肌肉注射" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "链球菌感染" } ] }, { "text": "青霉素过敏可改用其他有效抗生素如红霉素等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "青霉素过敏" }, { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "青霉素" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "抗生素" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "红霉素" } ] }, { "text": "(三)抗风湿治疗心脏炎时宜早期使用肾上腺皮质激素,泼尼松2mg/(kg•d),最大量≤60mg/d,分次口服,2~4周后减量,总疗程8~12周;无心脏炎患儿可用阿司匹林,80~100mg/(kg•d)最大量≤3g/d,分次服用,2周后逐渐减量,疗程4~8周。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗风湿治疗" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "心脏炎" }, { "start_idx": 17, "end_idx": 23, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "泼尼松" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "口服" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "心脏炎" }, { "start_idx": 80, "end_idx": 83, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "(四)对症治疗有充血性心力衰竭时除低盐饮食及氧气吸入外可给予利尿剂、洋地黄制剂和血管扩张剂,并注意限制液体入量,纠正电解质紊乱;舞蹈病时可用苯巴比妥及安定等镇静剂。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "对症治疗" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "低盐饮食" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "氧气吸入" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "利尿剂" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "洋地黄制剂" }, { "start_idx": 40, "end_idx": 44, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 47, "end_idx": 54, "type": "pro", "entity": "注意限制液体入量" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "电解质紊乱" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "舞蹈病" }, { "start_idx": 70, "end_idx": 73, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 75, "end_idx": 76, "type": "dru", "entity": "安定" }, { "start_idx": 78, "end_idx": 80, "type": "dru", "entity": "镇静剂" } ] }, { "text": "关节肿痛时应予制动。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "关节" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "关节肿痛" } ] }, { "text": "【预防和预后】风湿热预后主要取决于心脏炎的严重程度、首次发作是否得到正确治疗以及是否按期进行预防风湿热复发措施。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "风湿热" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "心脏炎" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "风湿热" } ] }, { "text": "严重心脏炎伴充血性心力衰竭患儿预后较差。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "心脏炎" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "充血性心力衰竭" } ] }, { "text": "每3~4周肌肉注射苄星青霉素G(长效青霉素)120万单位,预防注射期限至少5年,最好持续至25岁。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "肌肉注射" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "苄星青霉素G" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "长效青霉素" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "预防注射" } ] }, { "text": "有风湿性心脏病者,宜作终生药物预防。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "风湿性心脏病" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "终生药物预防" } ] }, { "text": "对青霉素过敏者可改用红霉素类药物口服,每月6~7天,持续时间同前。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "青霉素" }, { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "青霉素过敏" }, { "start_idx": 10, "end_idx": 15, "type": "dru", "entity": "红霉素类药物" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "口服" } ] }, { "text": "有作者认为目前A组溶血性链球菌对红霉素耐药菌株增多,而对复方新诺明耐药率仅为3.4%,因此主张对青霉素过敏的风热湿热患者,二级预防应首选磺胺药。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "mic", "entity": "A组溶血性链球菌" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "红霉素" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "菌株" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "复方新诺明" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "青霉素" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "青霉素过敏" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "风热湿热" }, { "start_idx": 68, "end_idx": 70, "type": "dru", "entity": "磺胺药" } ] }, { "text": "风湿热或风湿性心脏病患儿,当拔牙或行其他手术时,术前及术后应用抗生素静脉注射,以预防感染性心内膜炎。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "风湿热" }, { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "风湿性心脏病" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "拔牙" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "手术" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "抗生素" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "静脉注射" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "感染性心内膜炎" } ] }, { "text": "参考文献1.韩玉昆,陈树宝,杨锡强,等.实用儿科诊断治疗学.安徽科技出版社,2000,1:396-4002.唐晋清,盛芳芸.小儿肾血管性高血压的诊断探讨.中国实用儿科杂志,1997,12(5):277-2773.楊丙昂,李军,王执民,等.肾功能狭窄双功能多普勒与数学减数造影对照研究.中国超声医学杂志,1997,13(6):48-484.WellsTG,BelshaCW.Pediatricrenovascularhypertension.CurrOpinPediatr,1996,8(2):128-1285.DongQ,SchocenbergSB,CarlosRC,etal.DiagnosisofrenalvasculardiseasewithMRangiography.Radiographics,1999,19(6):1535-15356.NovickAC.Currentconceptsinthemanagementofrenovascularhypertensionandischemicrenalfailure.AmJKidDis,1989,8(6)suppl1:33-337.TonkiIL,StapletonFB,RoyS,etal.Digitalsubstractionangiographyintheevaluationofrenalvascularhypertensioninchildren.Pediatrics,1988,81(1):150-150", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "dep", "entity": "儿科" }, { "start_idx": 62, "end_idx": 70, "type": "dis", "entity": "小儿肾血管性高血压" }, { "start_idx": 81, "end_idx": 82, "type": "dep", "entity": "儿科" }, { "start_idx": 119, "end_idx": 123, "type": "dis", "entity": "肾功能狭窄" }, { "start_idx": 124, "end_idx": 129, "type": "pro", "entity": "双功能多普勒" }, { "start_idx": 131, "end_idx": 136, "type": "pro", "entity": "数学减数造影" }, { "start_idx": 144, "end_idx": 145, "type": "pro", "entity": "超声" } ] }, { "text": "六、恶性血管内皮瘤恶性血管内皮瘤(malignantmesothelioma)又称血管肉瘤,小儿少见。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "恶性血管内皮瘤" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "恶性血管内皮瘤" }, { "start_idx": 17, "end_idx": 37, "type": "dis", "entity": "malignantmesothelioma" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "血管肉瘤" } ] }, { "text": "该肿瘤的发生有人认为与慢性淋巴水肿有关,也有认为放射性射线辐射可导致本瘤。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "慢性淋巴水肿" }, { "start_idx": 35, "end_idx": 35, "type": "dis", "entity": "瘤" } ] }, { "text": "肿瘤直径多在几厘米范围,切面灰白或灰红色,形态不规则,质软,肿瘤内可见大小不一的腔隙显微镜下可见广泛增生的内皮细胞其结构呈形状不规则的血管腔相互吻合成网。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "切面灰白或灰红色" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "切面" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "形态不规则" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "质软" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "肿瘤内可见大小不一的腔隙" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肿瘤" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "腔隙" }, { "start_idx": 42, "end_idx": 44, "type": "equ", "entity": "显微镜" }, { "start_idx": 42, "end_idx": 75, "type": "sym", "entity": "显微镜下可见广泛增生的内皮细胞其结构呈形状不规则的血管腔相互吻合成网" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "血管腔" } ] }, { "text": "细胞核分裂多见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "细胞核" } ] }, { "text": "八、松果体区肿瘤松果体区肿瘤占儿童中枢神经系统肿瘤的0.4%~2%,包括生殖细胞肿瘤(40%~65%)、松果体实质细胞瘤(17%)和星形细胞瘤(15%);其中生殖细胞肿瘤又可分为生殖细胞瘤、畸胎瘤和混合型生殖细胞肿瘤。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "松果体区肿瘤" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "松果体区肿瘤" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "儿童中枢神经系统肿瘤" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "生殖细胞肿瘤" }, { "start_idx": 52, "end_idx": 59, "type": "dis", "entity": "松果体实质细胞瘤" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "星形细胞瘤" }, { "start_idx": 79, "end_idx": 84, "type": "dis", "entity": "生殖细胞肿瘤" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "生殖细胞瘤" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 99, "end_idx": 107, "type": "dis", "entity": "混合型生殖细胞肿瘤" } ] }, { "text": "主要有以下三大类:①肿瘤阻塞第三脑室出口引起梗阻性脑积水及颅高压;②邻近结构受压症,如压迫中脑四叠体上丘导致双眼不能上视内分泌紊乱:松果体区肿瘤可分泌褪黑激素,影响垂体分泌促性腺激素,可表现为发育迟缓,性功能减退、肥胖、嗜睡等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "肿瘤阻塞第三脑室出口" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "第三脑室出口" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "梗阻性脑积水" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "颅高压" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "中脑四叠体上丘" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "双眼" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "双眼不能上视" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "内分泌紊乱" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "松果体区肿瘤" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "褪黑激素" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "垂体" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "促性腺激素" }, { "start_idx": 96, "end_idx": 99, "type": "sym", "entity": "发育迟缓" }, { "start_idx": 101, "end_idx": 105, "type": "sym", "entity": "性功能减退" }, { "start_idx": 107, "end_idx": 108, "type": "sym", "entity": "肥胖" }, { "start_idx": 110, "end_idx": 111, "type": "sym", "entity": "嗜睡" } ] }, { "text": "化疗对延长生存期有一定帮助。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" } ] }, { "text": "视肿瘤性质、大小、侵犯部位、有否转移及治疗是否彻底而定。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" } ] }, { "text": "三、放射学检查钡剂胃肠造影不仅排除胃肠道内器质性病变,而且通过钡剂在胃肠道中的运动来检测胃肠动力。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "放射学检查" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "钡剂胃肠造影" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "胃肠道内器质性病变" }, { "start_idx": 29, "end_idx": 47, "type": "pro", "entity": "通过钡剂在胃肠道中的运动来检测胃肠动力" } ] }, { "text": "观察了解食管和胃的形态、收缩、蠕动、排空以及有无反流,也可用于小肠及大肠的动力学检测,为胃肠动力检测提供定性评估。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "食管" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "胃" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "反流" }, { "start_idx": 31, "end_idx": 41, "type": "pro", "entity": "小肠及大肠的动力学检测" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "胃肠动力检测" } ] }, { "text": "给受试者口服一定量X线不能穿透的小标记物,如小钡条胶囊,每隔一定时间透视或摄片,以标志物计数来计算胃排空和结肠通过时间。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "X线" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "小钡条胶囊" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "透视" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "摄片" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "胃" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "结肠" } ] }, { "text": "在X线下观察胃肠道运动,符合生理要求,设备简单,但费时,多次曝光,对患儿健康不利。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "X线" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胃肠道" } ] }, { "text": "三、配型问题HLA配型仍为最重要。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "HLA配型" } ] }, { "text": "但在多囊肾病人的肾移植时发现,如HLA不全配仍有较高的生存率(尸肾移植60%,活肾移植78%)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "多囊肾" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "肾移植" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "HLA" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "尸肾移植" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "活肾移植" } ] }, { "text": "而传统意义上的ABO配型在活体肾移植中已通过移植前的血浆置换、免疫吸附处理及移植后免疫抑制剂的有效应用成功克服ABO障碍,这更加拓宽了儿童肾移植的供肾来源。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "ABO配型" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "活体肾移植" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "移植" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "血浆置换" }, { "start_idx": 31, "end_idx": 36, "type": "pro", "entity": "免疫吸附处理" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "移植" }, { "start_idx": 41, "end_idx": 45, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 69, "end_idx": 71, "type": "pro", "entity": "肾移植" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "肾" } ] }, { "text": "第七节儿童肾移植儿童肾移植自20世纪60年代自美国首例开展以来,经历了近50年的发展历程,已与透析一起成为儿童终末期肾衰竭的最重要的替代性治疗手段。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "透析" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "儿童终末期肾衰竭" }, { "start_idx": 66, "end_idx": 70, "type": "pro", "entity": "替代性治疗" } ] }, { "text": "据统计,每百万人口中就有约2~3名终末期肾病患儿需要血液透析或肾移植治疗;而据美国医疗保险机构统计,每名肾移植患儿的一年医疗费用14000美元,每名透析患儿的一年医疗费用43000美元。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "终末期肾病" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "血液透析" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "肾移植治疗" }, { "start_idx": 52, "end_idx": 54, "type": "pro", "entity": "肾移植" }, { "start_idx": 74, "end_idx": 75, "type": "pro", "entity": "透析" } ] }, { "text": "越来越多的医疗人员提出,良好的移植肾功能对儿童生活质量的改善远较透析优越。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "移植肾" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "透析" } ] }, { "text": "20世纪80年代以来,随着组织相容性检测的提高、手术技术的完善以及更新的免疫抑制剂的应用,儿童肾移植已作为一项成熟的医疗手段在世界各国包括美国、英国、德国、荷兰、意大利、加拿大、澳大利亚、日本、巴西以及泰国等得到广泛开展。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "组织相容性检测" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "手术技术" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 45, "end_idx": 49, "type": "pro", "entity": "儿童肾移植" } ] }, { "text": "北美移植中心USRUS及欧洲透析与移植协会EDTA报道,儿童肾移植的10年生存率81%~92%,来自德国的儿童肾移植后的心理社会康复水平评价:致残率30%,就业率86%;87%的儿童能够正常上学,50%能够结婚,24%能够生育。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "移植" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "透析" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "移植" }, { "start_idx": 28, "end_idx": 32, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 53, "end_idx": 57, "type": "pro", "entity": "儿童肾移植" } ] }, { "text": "一、受体(一)受体的选择Topel统计,欧洲37个肾移植中心总结286例15岁以下儿童肾移植病例其终末期肾病的分布:慢性肾小球肾炎52.3%,慢性肾盂肾炎20.8%,遗传性肾病8.0%,血管性肾病4.5%,多囊肾3.0%,药物引起性肾病2.4%,先天性肾发育不全1.6%,其他(包括胱氨酸沉积症、草酸盐沉积症、Alport综合征和溶血尿毒综合征)7.4%。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "pro", "entity": "肾移植" }, { "start_idx": 41, "end_idx": 45, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "终末期肾病" }, { "start_idx": 58, "end_idx": 64, "type": "dis", "entity": "慢性肾小球肾炎" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "遗传性肾病" }, { "start_idx": 93, "end_idx": 97, "type": "dis", "entity": "血管性肾病" }, { "start_idx": 103, "end_idx": 105, "type": "dis", "entity": "多囊肾" }, { "start_idx": 111, "end_idx": 117, "type": "dis", "entity": "药物引起性肾病" }, { "start_idx": 123, "end_idx": 130, "type": "dis", "entity": "先天性肾发育不全" }, { "start_idx": 141, "end_idx": 146, "type": "dis", "entity": "胱氨酸沉积症" }, { "start_idx": 148, "end_idx": 153, "type": "dis", "entity": "草酸盐沉积症" }, { "start_idx": 155, "end_idx": 163, "type": "dis", "entity": "Alport综合征" }, { "start_idx": 165, "end_idx": 171, "type": "dis", "entity": "溶血尿毒综合征" } ] }, { "text": "(二)肾移植的时机当肾衰竭已进入不可逆阶段(Ccr<5~10ml/min)时可考虑肾移植。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "肾移植" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 41, "end_idx": 43, "type": "pro", "entity": "肾移植" } ] }, { "text": "但如出现尿毒症引起的并发症严重,应积极内科治疗(包括透析)为肾移植手术创造条件。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "尿毒症" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "内科治疗" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "透析" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "肾移植手术" } ] }, { "text": "传统认为5岁为接受肾移植的年龄,但近5年来许多医疗人员不再严格限制儿童肾移植的年龄,而更加强调的是缩短待肾时间及未经透析的干肾移植。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "肾移植" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "肾" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "透析" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "干肾移植" } ] }, { "text": "美国明尼苏达大学研究发现<1岁年龄组与5~13岁年龄组儿童肾移植的生存率无显著差别,而1岁以内的肾移植患儿移植后排斥反应的发生率有所降低,小儿体重明显增加,但在迟发性肾功能不全与血栓发生率有所提高;表明只要合适的选择移植肾、手术成功完成及良好的护理,完全可以保证低龄幼儿的肾移植的成功,没有必要限定进行肾移植的最小年龄。", "entities": [ { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "肾移植" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "移植" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "排斥反应" }, { "start_idx": 71, "end_idx": 76, "type": "sym", "entity": "体重明显增加" }, { "start_idx": 71, "end_idx": 72, "type": "ite", "entity": "体重" }, { "start_idx": 80, "end_idx": 87, "type": "dis", "entity": "迟发性肾功能不全" }, { "start_idx": 89, "end_idx": 90, "type": "sym", "entity": "血栓" }, { "start_idx": 89, "end_idx": 89, "type": "bod", "entity": "血" }, { "start_idx": 108, "end_idx": 110, "type": "pro", "entity": "移植肾" }, { "start_idx": 112, "end_idx": 113, "type": "pro", "entity": "手术" }, { "start_idx": 136, "end_idx": 138, "type": "pro", "entity": "肾移植" }, { "start_idx": 151, "end_idx": 153, "type": "pro", "entity": "肾移植" } ] }, { "text": "待肾时间愈短,预后愈佳。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肾" } ] }, { "text": "在待肾期间未进行透析者的预后以往多认为优于移植前已进行透析者,但近年报道二者之间无明显差异。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肾" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "透析" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "移植" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "透析" } ] }, { "text": "(三)儿童肾移植的反指征存在不适于应用免疫抑制剂的疾病:如严重的全身感染、肝功能严重损害、活动性结核以及糖尿病等,术后应慎重选用免疫抑制剂。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "全身感染" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "肝功能严重损害" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "活动性结核" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "糖尿病" }, { "start_idx": 64, "end_idx": 68, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "恶性肿瘤、不能修复的严重下尿路畸形(如脑脊膜膨出合并神经源性膀胱等)都是肾移植的禁忌证。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "尿路畸形" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "脑脊膜膨出" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 36, "end_idx": 38, "type": "pro", "entity": "肾移植" } ] }, { "text": "第七节系统性红斑狼疮性肾炎系统性红斑狼疮(systemiclupuserythematosus,SLE)是一种公认的自身免疫性疾病,其病变大多累及数个系统或器官。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "系统性红斑狼疮性肾炎" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 21, "end_idx": 46, "type": "dis", "entity": "systemiclupuserythematosus" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "SLE" }, { "start_idx": 58, "end_idx": 64, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 67, "end_idx": 79, "type": "sym", "entity": "病变大多累及数个系统或器官" }, { "start_idx": 73, "end_idx": 79, "type": "bod", "entity": "数个系统或器官" } ] }, { "text": "肾脏病变在SLE病人中很常见,约40%~70%SLE患儿有狼疮肾炎(lupusnephritis,LN)的临床表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肾脏病变" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "SLE" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "SLE" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 34, "end_idx": 47, "type": "dis", "entity": "lupusnephritis" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "LN" } ] }, { "text": "肾活体组织检查一般病理检查发现肾病变者可达90%,进一步作免疫荧光及电镜检查发现有不同程度肾病变者近100%。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "病理检查" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肾病变" }, { "start_idx": 29, "end_idx": 37, "type": "pro", "entity": "免疫荧光及电镜检查" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "肾病变" } ] }, { "text": "LN患儿约占在全部LN病人的4%~10%,但儿童LN病变往往严重,难治病例更多。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "LN" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "LN" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "儿童LN病变" } ] }, { "text": "SLE部分病人以肾外症状为主,肾损害轻;另一部病人则以肾损害为主要表现,肾外症状不明显,后者易误诊为原发性肾小球疾病。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "SLE" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "肾外症状" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肾外" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肾" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "肾损害轻" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肾损害" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "肾外症状" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肾外" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "原发性肾小球疾病" } ] }, { "text": "【病因】SLE病因尚未阐明,多数学者认为是有一定遗传特征的个体,在多种触发因素(如感染及理化环境因素)作用下,发生免疫紊乱所致的自身免疫性损伤,LN具有明显的免疫复合物性肾炎特征。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "SLE" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "感染" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "免疫紊乱" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "自身免疫性损伤" }, { "start_idx": 72, "end_idx": 73, "type": "dis", "entity": "LN" }, { "start_idx": 79, "end_idx": 86, "type": "dis", "entity": "免疫复合物性肾炎" } ] }, { "text": "(一)遗传因素遗传流行病学资料发现SLE具有家族聚集倾向,同卵双生子SLE发病一致率达25%~70%,明显高于异卵双生子(2%~9%)。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "SLE" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "SLE" } ] }, { "text": "但大量的遗传病学研究分析证实SLE是多基因遗传,位于第6对染色体中的多个基因位点与发病有关,尤其是遗传性补体基因缺陷(C1r、C1s、C2及C4等早期补体成分缺陷)。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "SLE" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "基因" }, { "start_idx": 26, "end_idx": 39, "type": "bod", "entity": "第6对染色体中的多个基因位点" }, { "start_idx": 49, "end_idx": 57, "type": "dis", "entity": "遗传性补体基因缺陷" }, { "start_idx": 59, "end_idx": 72, "type": "bod", "entity": "C1r" }, { "start_idx": 59, "end_idx": 124, "type": "sym", "entity": "C1r、C1s、C2及C4等早期补体成分缺陷" }, { "start_idx": 74, "end_idx": 87, "type": "bod", "entity": "C1s" }, { "start_idx": 89, "end_idx": 101, "type": "bod", "entity": "C2" }, { "start_idx": 103, "end_idx": 115, "type": "bod", "entity": "C4" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "早期补体" } ] }, { "text": "人类白细胞抗原(HLA)基因(HLA-B8、BW15、DR2及DR3)、T细胞表面抗原受体(TCR)基因以及免疫球蛋白基因等经典免疫应答基因的多态性也与罹患SLE有关。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "bod", "entity": "人类白细胞抗原(HLA)基因" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "HLA-B8" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "BW15" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "DR2" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "DR3" }, { "start_idx": 36, "end_idx": 51, "type": "bod", "entity": "T细胞表面抗原受体(TCR)基因" }, { "start_idx": 54, "end_idx": 60, "type": "bod", "entity": "免疫球蛋白基因" }, { "start_idx": 62, "end_idx": 69, "type": "bod", "entity": "经典免疫应答基因" }, { "start_idx": 78, "end_idx": 80, "type": "dis", "entity": "SLE" } ] }, { "text": "其中日本人和中国人HLA-DR2位点频率增高,西欧血统白人HLA-DR2和/或DR3位点频率增高,我国南方汉人SLE发病与DRB1*0301及DQB1*0608有关,美国黑人与DRB1*1503、DQA1*0102和DQB1*0602有关。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "HLA-DR2" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "HLA-DR2" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "DR3" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "SLE" }, { "start_idx": 61, "end_idx": 69, "type": "bod", "entity": "DRB1*0301" }, { "start_idx": 71, "end_idx": 79, "type": "bod", "entity": "DQB1*0608" }, { "start_idx": 88, "end_idx": 96, "type": "bod", "entity": "DRB1*1503" }, { "start_idx": 98, "end_idx": 106, "type": "bod", "entity": "DQA1*0102" }, { "start_idx": 108, "end_idx": 116, "type": "bod", "entity": "DQB1*0602" } ] }, { "text": "但其他人群研究未发现HLA-Ⅱ类基因与SLE发病有如此相关性。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "HLA-Ⅱ类基因" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "SLE" } ] }, { "text": "进一步研究发现某些HLA-Ⅱ类基因位点多态性与SLE患者产生自身抗体有关,尤其是不同HLA-DQ等位基因所共有的多态性序列可能导致某种自身抗体的产生。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "bod", "entity": "HLA-Ⅱ类基因" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "SLE" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "自身抗体" }, { "start_idx": 42, "end_idx": 51, "type": "bod", "entity": "HLA-DQ等位基因" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "自身抗体" } ] }, { "text": "如含高水平dsDNA抗体患者中,96%具有HLA-DQB1*0201(与HLADR3和DR7连锁)、DQB1*0602(与DR2和DRW6连锁)或DQB1*0302(与HLA-DR4单倍型连锁)等位基因。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "高水平dsDNA抗体" }, { "start_idx": 5, "end_idx": 11, "type": "ite", "entity": "dsDNA抗体" }, { "start_idx": 21, "end_idx": 100, "type": "bod", "entity": "HLA-DQB1*0201(与HLADR3和DR7连锁)、DQB1*0602(与DR2和DRW6连锁)或DQB1*0302(与HLA-DR4单倍型连锁)等位基因" } ] }, { "text": "另一些人发现抗心磷脂抗体阳性的SLE患者与HLA-DQB1*0301(DQw7)、*0302(DQw8)、*0303(DQw9)及*0602(DQw6)等位基因密切相关。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "ite", "entity": "抗心磷脂抗体" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "抗心磷脂抗体阳性" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "SLE" }, { "start_idx": 21, "end_idx": 79, "type": "bod", "entity": "HLA-DQB1*0301(DQw7)、*0302(DQw8)、*0303(DQw9)及*0602(DQw6)等位基因" } ] }, { "text": "因此,推测SLE患病基因位于MHC区域,与HLA-Ⅰ类及Ⅱ类基因呈连锁不平衡性。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "SLE" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "MHC区域" }, { "start_idx": 21, "end_idx": 31, "type": "bod", "entity": "HLA-Ⅰ类及Ⅱ类基因" } ] }, { "text": "正常情况下补体成分在免疫复合物的固定和有效清除中起着关键作用,这些成分因遗传基因缺陷而缺乏时,将导致免疫复合物在肾脏沉积而得病。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "补体" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "遗传基因缺陷" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "缺乏" }, { "start_idx": 50, "end_idx": 59, "type": "sym", "entity": "免疫复合物在肾脏沉积" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "肾脏" } ] }, { "text": "但资料表明补体缺陷在SLE中并不多见,且补体缺陷者肾病变也常不严重,临床表现不典型,累及男孩多,因此它不代表多数SLE的发病特征,同时表明致SLE的遗传基因肯定具有多种复杂特征。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "补体缺陷" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "SLE" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "补体缺陷" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "肾病变" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "SLE" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "SLE" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "基因" } ] }, { "text": "(二)环境与感染因素紫外线被认为是触发SLE的病因之一;实验发现紫外线(主要是紫外线290~320nm)可诱使皮肤角质细胞产生白细胞介素-1(IL-1)、IL-3、IL-6及肿瘤坏死因子(TNF);紫外线还可以减弱巨噬细胞对抗原的清除以及抑制T细胞活化;约有1/3的SLE患者对光过敏或紫外线照射后发病。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "感染" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "SLE" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "皮肤角质细胞" }, { "start_idx": 63, "end_idx": 69, "type": "bod", "entity": "白细胞介素-1" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "IL-1" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "IL-3" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "IL-6" }, { "start_idx": 87, "end_idx": 92, "type": "bod", "entity": "肿瘤坏死因子" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "TNF" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "抗原" }, { "start_idx": 121, "end_idx": 123, "type": "bod", "entity": "T细胞" }, { "start_idx": 133, "end_idx": 135, "type": "dis", "entity": "SLE" }, { "start_idx": 139, "end_idx": 141, "type": "sym", "entity": "光过敏" } ] }, { "text": "资料表明紫外线可使细胞内DNA转化为胸腺嘧啶二聚体,使其抗原性增强,诱生抗DNA抗体。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "细胞内DNA" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "胸腺嘧啶二聚体" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "抗原" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "抗DNA抗体" } ] }, { "text": "某些药物可促使SLE患者光过敏,如磺胺药及四环素;有些药物可诱发产生自身抗体如普鲁卡因胺和肼苯达嗪等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "SLE" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "光过敏" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "磺胺药" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "四环素" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "自身抗体" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "肼苯达嗪" } ] }, { "text": "有些香料、染料、染发水、烟火熏烤食品及菌类也可诱发SLE。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "mic", "entity": "菌类" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "SLE" } ] }, { "text": "有人认为这药物或化学物质与细胞核蛋白结合后,发生抗原性变性,也是引发机体自身免疫损伤的重要原因。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "细胞核蛋白" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "抗原性变性" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "机体自身免疫损伤" } ] }, { "text": "近年资料发现人类免疫缺陷病毒(HIV)感染者可发生SLE;感染单纯性疱疹病毒可引起患者血清Sm抗原浓度升高;SLE患者血清中常见多种病毒抗体滴度增加(如风疹、EB病毒、流感及麻疹等),尤其是C型RNA病毒。", "entities": [ { "start_idx": 8, "end_idx": 20, "type": "dis", "entity": "免疫缺陷病毒(HIV)感染" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "SLE" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "感染单纯性疱疹病毒" }, { "start_idx": 43, "end_idx": 52, "type": "sym", "entity": "血清Sm抗原浓度升高" }, { "start_idx": 43, "end_idx": 50, "type": "ite", "entity": "血清Sm抗原浓度" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "SLE" }, { "start_idx": 59, "end_idx": 73, "type": "sym", "entity": "血清中常见多种病毒抗体滴度增加" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "血清" }, { "start_idx": 64, "end_idx": 71, "type": "ite", "entity": "多种病毒抗体滴度" }, { "start_idx": 76, "end_idx": 82, "type": "mic", "entity": "风疹、EB病毒" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "流感" }, { "start_idx": 87, "end_idx": 88, "type": "dis", "entity": "麻疹" }, { "start_idx": 95, "end_idx": 101, "type": "mic", "entity": "C型RNA病毒" } ] }, { "text": "(三)内分泌因素SLE患者多数为女性,且不论男女,患者雌激素水平均增高,雄激素水平降低。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "SLE" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "雌激素水平均增高" }, { "start_idx": 27, "end_idx": 31, "type": "ite", "entity": "雌激素水平" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "雄激素水平降低" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "雄激素水平" } ] }, { "text": "推测高水平雌激素可直接作用B细胞,使其活化,导致分泌自身抗体的活化B细胞大量扩增。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "高水平雌激素" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "雌激素" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "B细胞" }, { "start_idx": 24, "end_idx": 39, "type": "sym", "entity": "分泌自身抗体的活化B细胞大量扩增" }, { "start_idx": 24, "end_idx": 35, "type": "bod", "entity": "分泌自身抗体的活化B细胞" } ] }, { "text": "在实验动物中发现雌激素可使其病情加重,而雄激素可使病情减轻。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "雌激素" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "雄激素" } ] }, { "text": "(四)自身组织抗原变异紫外线照射、药物、化学物质以及病原感染等多种因素均可能破坏自身组织,暴露组织隐蔽抗原或使正常组织抗原结构改变,激发机体自身免疫损伤。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "自身组织抗原变异" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "病原感染" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "破坏自身组织" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "自身组织" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "组织" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "抗原" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "正常组织抗原结构改变" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "正常组织" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "抗原" }, { "start_idx": 68, "end_idx": 75, "type": "dis", "entity": "机体自身免疫损伤" } ] }, { "text": "【发病机制】目前有关SLE发病机制尚无一致结论,多数学者认为发病环节可能是多元性的。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "SLE" } ] }, { "text": "较为一致的结论是具有一定遗传趋向的个体,在某些触发因素作用下,发生以自身组织为靶目标的异常免疫反应。", "entities": [ { "start_idx": 33, "end_idx": 48, "type": "sym", "entity": "以自身组织为靶目标的异常免疫反应" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "自身组织" } ] }, { "text": "其最终免疫损伤的机制是T细胞功能紊乱,B细胞多克隆活化,自身抗体与自身组织抗原结合后发生免疫复合物性疾病,LN更具有免疫复合物性炎症的明显特征。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "免疫损伤" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "T细胞功能紊乱" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "B细胞多克隆活化" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "B细胞" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "自身抗体" }, { "start_idx": 28, "end_idx": 40, "type": "sym", "entity": "自身抗体与自身组织抗原结合" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "自身组织抗原" }, { "start_idx": 44, "end_idx": 51, "type": "dis", "entity": "免疫复合物性疾病" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "LN" }, { "start_idx": 58, "end_idx": 65, "type": "dis", "entity": "免疫复合物性炎症" } ] }, { "text": "(一)T细胞功能紊乱SLE患儿细胞免疫功能低下,T细胞亚群间失衡,T细胞绝对数减少,且其程度与疾病活动性有关。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "T细胞功能紊乱" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "SLE" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "细胞" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "细胞免疫功能低下" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "T细胞亚群间失衡" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "T细胞亚群" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "T细胞绝对数减少" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "T细胞" } ] }, { "text": "T细胞对B细胞的调控功能异常,致病性B细胞克隆活性增强,自身抗体水平上升。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "T细胞" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "T细胞对B细胞的调控功能异常" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "B细胞" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "致病性B细胞" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "致病性B细胞克隆活性增强" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "自身抗体" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "自身抗体水平上升" } ] }, { "text": "(二)B细胞多克隆活化B细胞多克隆活化,诱发产生过多的致病性抗DNA抗体,大量资料证明SLE患者在活动期有类似B细胞多克隆活化证据,且预示病情严重与疾病进展。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "B细胞" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "B细胞多克隆活化" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "B细胞" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "B细胞多克隆活化" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "产生过多的致病性抗DNA抗体" }, { "start_idx": 27, "end_idx": 35, "type": "bod", "entity": "致病性抗DNA抗体" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "SLE" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "B细胞" }, { "start_idx": 55, "end_idx": 62, "type": "sym", "entity": "B细胞多克隆活化" } ] }, { "text": "(三)免疫复合物致病研究表明DNA-抗DNA抗体是引起肾脏损害的一对主要抗原抗体复合物(免疫复合物),除此之外Sm抗原、SSA抗原、肾小球基底膜(GBM)抗原、肾小管基底膜(TBM)抗原与相应的抗体结合形成的免疫复合物均可能与肾组织损伤有关。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 14, "end_idx": 23, "type": "bod", "entity": "DNA-抗DNA抗体" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "肾脏损害" }, { "start_idx": 36, "end_idx": 42, "type": "bod", "entity": "抗原抗体复合物" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 55, "end_idx": 119, "type": "bod", "entity": "Sm抗原、SSA抗原、肾小球基底膜(GBM)抗原、肾小管基底膜(TBM)抗原与相应的抗体结合形成的免疫复合物" }, { "start_idx": 124, "end_idx": 128, "type": "dis", "entity": "肾组织损伤" } ] }, { "text": "且不同抗体的免疫复合物与不同类型肾损害有关;如抗RNP(核糖核蛋白)及Sm抗体阳性时,肾损害者少。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "抗体的免疫复合物" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肾损害" }, { "start_idx": 23, "end_idx": 40, "type": "sym", "entity": "抗RNP(核糖核蛋白)及Sm抗体阳性" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "抗RNP" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "核糖核蛋白" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "Sm抗体" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "肾损害" } ] }, { "text": "但另有研究发现抗SSA、RNP及Sm抗体阳性时,多为膜性肾病;弥漫增殖性狼疮肾炎上述抗体阳性率均低或滴度低;高亲和力DNA抗体阳性及低补体血症者多为弥漫增殖性肾炎。", "entities": [ { "start_idx": 7, "end_idx": 30, "type": "ite", "entity": "抗SSA、RNP及Sm抗体" }, { "start_idx": 7, "end_idx": 32, "type": "sym", "entity": "抗SSA、RNP及Sm抗体阳性" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 42, "end_idx": 50, "type": "dis", "entity": "弥漫增殖性狼疮肾炎" }, { "start_idx": 53, "end_idx": 54, "type": "ite", "entity": "抗体" }, { "start_idx": 61, "end_idx": 62, "type": "ite", "entity": "滴度" }, { "start_idx": 65, "end_idx": 73, "type": "ite", "entity": "高亲和力DNA抗体" }, { "start_idx": 65, "end_idx": 75, "type": "sym", "entity": "高亲和力DNA抗体阳性" }, { "start_idx": 77, "end_idx": 81, "type": "dis", "entity": "低补体血症" }, { "start_idx": 85, "end_idx": 91, "type": "dis", "entity": "弥漫增殖性肾炎" } ] }, { "text": "除T、B细胞功能紊乱产生大量致病性自身抗体的直接损伤外,免疫复合物是一个重要的致病原因,其主要机制是:1.循环免疫复合物抗体与各种抗原在循环中形成免疫复合物后,经循环沉积于肾脏,由经典途径激活补体,吸引中性粒细胞,释放炎症介质,引起肾脏损害。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "T、B细胞功能紊乱" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "致病性自身抗体" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "损伤" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 53, "end_idx": 59, "type": "bod", "entity": "循环免疫复合物" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "抗体" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "抗原" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "循环" }, { "start_idx": 73, "end_idx": 77, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "循环" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "肾脏" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "补体" }, { "start_idx": 101, "end_idx": 105, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 109, "end_idx": 112, "type": "bod", "entity": "炎症介质" }, { "start_idx": 116, "end_idx": 119, "type": "dis", "entity": "肾脏损害" } ] }, { "text": "2.原位免疫复合物实验发现ssDNA对肾小球基底膜有亲和力,经循环ssDNA先植入肾小球,再吸引循环中的抗ssDNA抗体与之结合,在原位形成免疫复合物,激活补体,诱生炎症,这种肾炎常为膜性狼疮性肾炎。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "原位免疫复合物" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "ssDNA" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "肾小球基底膜" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "循环" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "ssDNA" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "肾小球" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "循环" }, { "start_idx": 52, "end_idx": 59, "type": "bod", "entity": "抗ssDNA抗体" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "补体" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "炎症" }, { "start_idx": 88, "end_idx": 89, "type": "dis", "entity": "肾炎" }, { "start_idx": 92, "end_idx": 98, "type": "dis", "entity": "膜性狼疮性肾炎" } ] }, { "text": "3.抗GBM及抗TBM抗体这些抗体直接与肾组织(GBM和TBM)反应,引起肾损伤,若发现免疫荧光在GBM呈线样IgG沉积,提示狼疮肾炎因抗肾组织抗体介导而致病。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "bod", "entity": "抗GBM及抗TBM抗体" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "抗体" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肾组织" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "GBM" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "TBM" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "肾损伤" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 44, "end_idx": 59, "type": "sym", "entity": "免疫荧光在GBM呈线样IgG沉积" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "GBM" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "IgG" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 68, "end_idx": 73, "type": "bod", "entity": "抗肾组织抗体" } ] }, { "text": "4.免疫复合物清除障碍正常人可以通过多种途径清除不断产生的免疫复合物,其中补体途径最为重要,SLE患者因C3缺乏或红细胞膜上C3b受体减少,导致巨噬细胞清除机制减弱,是免疫复合物沉积及致病的重要原因。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "免疫复合物清除障碍" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "补体" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "SLE" }, { "start_idx": 52, "end_idx": 59, "type": "bod", "entity": "C3" }, { "start_idx": 52, "end_idx": 61, "type": "sym", "entity": "C3缺乏" }, { "start_idx": 63, "end_idx": 80, "type": "sym", "entity": "红细胞膜上C3b受体减少" }, { "start_idx": 63, "end_idx": 78, "type": "bod", "entity": "红细胞膜上C3b受体" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 84, "end_idx": 93, "type": "sym", "entity": "巨噬细胞清除机制减弱" }, { "start_idx": 96, "end_idx": 102, "type": "sym", "entity": "免疫复合物沉积" }, { "start_idx": 96, "end_idx": 100, "type": "bod", "entity": "免疫复合物" } ] }, { "text": "【病理改变】狼疮肾炎病变既可累及肾小球,也可累及肾小管以及肾血管及间质。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "狼疮肾炎病变" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肾小球" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肾小管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肾血管" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "间质" } ] }, { "text": "儿童狼疮肾炎多使用WHO分类法及国际小儿肾脏病科研协作组(ISKDC)分类法,并用Pirani积分法作为补充(表12-12),且Pirani积分法较病理分型更能反映肾病变的严重性和活动性,也能反映狼疮肾炎的治疗效果。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "儿童狼疮肾炎" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "小儿肾脏病" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "肾病变" }, { "start_idx": 98, "end_idx": 101, "type": "dis", "entity": "狼疮肾炎" } ] }, { "text": "表12-2Pirani积分法(一)WHO病理分型括弧中为国际小儿肾脏病科研协作组(ISKDC)分类法。", "entities": [ { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "小儿肾脏病" } ] }, { "text": "1.WHOⅠ型(ISKDC1a,1b)本型罕见,为正常肾小球或轻微病变,极少部分患儿免疫荧光或电镜下可见肾小球有少许沉积物。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "WHOⅠ型" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "ISKDC1a" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "1b" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肾小球" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "轻微病变" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 42, "end_idx": 60, "type": "sym", "entity": "免疫荧光或电镜下可见肾小球有少许沉积物" }, { "start_idx": 47, "end_idx": 48, "type": "equ", "entity": "电镜" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "肾小球" } ] }, { "text": "2.WHOⅡ型(ISKDC2a,2b)系膜增殖型肾小球肾炎,病变局限于系膜区,表现为程度不等的系膜细胞和基质增多,系膜区免疫沉积物阳性,仅有轻度节段性系膜增生者为2a型,系膜和系膜细胞增生为2b型。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "WHOⅡ型" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "ISKDC2a" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "2b" }, { "start_idx": 19, "end_idx": 28, "type": "dis", "entity": "系膜增殖型肾小球肾炎" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "病变局限于系膜区" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "系膜区" }, { "start_idx": 42, "end_idx": 55, "type": "sym", "entity": "程度不等的系膜细胞和基质增多" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "系膜细胞" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "基质" }, { "start_idx": 57, "end_idx": 66, "type": "sym", "entity": "系膜区免疫沉积物阳性" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "系膜区" }, { "start_idx": 60, "end_idx": 64, "type": "ite", "entity": "免疫沉积物" }, { "start_idx": 70, "end_idx": 78, "type": "dis", "entity": "轻度节段性系膜增生" }, { "start_idx": 85, "end_idx": 93, "type": "dis", "entity": "系膜和系膜细胞增生" } ] }, { "text": "本型多表现为轻度血尿或蛋白尿,很少发生肾功能不全。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "轻度血尿或蛋白尿" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "血" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "蛋白" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "肾功能不全" } ] }, { "text": "3.WHOⅢ型(ISKDC3a、3b和4a)局灶节段增殖型肾小球肾炎,部分肾小球存在急性或慢性病变,如节段性细胞增生,细胞坏死,内皮细胞增生,纤维素样坏死,白细胞浸润,透明血栓,系膜区和毛细血管壁见IgG、IgA、C1q、C3、C4及白细胞介素等沉积。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "WHOⅢ型" }, { "start_idx": 8, "end_idx": 20, "type": "dis", "entity": "ISKDC3a、3b和4a" }, { "start_idx": 22, "end_idx": 33, "type": "dis", "entity": "局灶节段增殖型肾小球肾炎" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肾小球" }, { "start_idx": 37, "end_idx": 48, "type": "sym", "entity": "肾小球存在急性或慢性病变" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "节段性细胞增生" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "细胞坏死" }, { "start_idx": 64, "end_idx": 69, "type": "dis", "entity": "内皮细胞增生" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "纤维素样坏死" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "白细胞" }, { "start_idx": 78, "end_idx": 82, "type": "sym", "entity": "白细胞浸润" }, { "start_idx": 84, "end_idx": 87, "type": "sym", "entity": "透明血栓" }, { "start_idx": 86, "end_idx": 86, "type": "bod", "entity": "血" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "系膜区" }, { "start_idx": 89, "end_idx": 179, "type": "sym", "entity": "系膜区和毛细血管壁见IgG、IgA、C1q、C3、C4及白细胞介素等沉积" }, { "start_idx": 93, "end_idx": 97, "type": "bod", "entity": "毛细血管壁" }, { "start_idx": 99, "end_idx": 112, "type": "bod", "entity": "IgG" }, { "start_idx": 114, "end_idx": 127, "type": "bod", "entity": "IgA" }, { "start_idx": 129, "end_idx": 142, "type": "bod", "entity": "C1q" }, { "start_idx": 144, "end_idx": 156, "type": "bod", "entity": "C3" }, { "start_idx": 158, "end_idx": 170, "type": "bod", "entity": "C4" }, { "start_idx": 172, "end_idx": 176, "type": "bod", "entity": "白细胞介素" } ] }, { "text": "约半数以上肾小球正常。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾小球" } ] }, { "text": "临床上可表现为蛋白尿、血尿,高血压和轻度肾功能不全,亦可为肾病综合征。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "蛋白" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "高血压" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "轻度肾功能不全" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "ISKDC4a指50%以上肾小球受累。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "ISKDC4a" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肾小球" } ] }, { "text": "4.WHOⅣ型(ISKDC5a,5b)弥漫增生性肾炎,狼疮肾炎中半数以上是本型,病变广泛且严重,几乎全部肾小球受累,呈活动性毛细血管内增殖性改变,中性粒细胞渗出,纤维素样坏死;毛细血管壁显著增厚,管壁内透明血栓;坏死节段常见细胞性新月体;严重病例呈弥漫性坏死和新月体性肾炎,部分病例呈不同程度肾小球硬化。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "WHOⅣ型" }, { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "ISKDC5a,5b" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "弥漫增生性肾炎" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "病变广泛且严重" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "肾小球" }, { "start_idx": 59, "end_idx": 65, "type": "bod", "entity": "活动性毛细血管" }, { "start_idx": 59, "end_idx": 71, "type": "sym", "entity": "活动性毛细血管内增殖性改变" }, { "start_idx": 73, "end_idx": 77, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 73, "end_idx": 79, "type": "sym", "entity": "中性粒细胞渗出" }, { "start_idx": 81, "end_idx": 86, "type": "dis", "entity": "纤维素样坏死" }, { "start_idx": 88, "end_idx": 92, "type": "bod", "entity": "毛细血管壁" }, { "start_idx": 88, "end_idx": 96, "type": "sym", "entity": "毛细血管壁显著增厚" }, { "start_idx": 98, "end_idx": 104, "type": "sym", "entity": "管壁内透明血栓" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "管壁内" }, { "start_idx": 103, "end_idx": 103, "type": "bod", "entity": "血" }, { "start_idx": 106, "end_idx": 117, "type": "sym", "entity": "坏死节段常见细胞性新月体" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "细胞" }, { "start_idx": 124, "end_idx": 128, "type": "dis", "entity": "弥漫性坏死" }, { "start_idx": 130, "end_idx": 135, "type": "dis", "entity": "新月体性肾炎" }, { "start_idx": 146, "end_idx": 150, "type": "dis", "entity": "肾小球硬化" } ] }, { "text": "免疫荧光见所有肾小球、肾小管、包氏囊及球外毛细血管基底膜有各种免疫球蛋白及补体沉积,尤其是内皮下沉积明显,呈“满堂亮”现象。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 0, "end_idx": 60, "type": "sym", "entity": "免疫荧光见所有肾小球、肾小管、包氏囊及球外毛细血管基底膜有各种免疫球蛋白及补体沉积,尤其是内皮下沉积明显,呈“满堂亮”现象" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾小球" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肾小管" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "包氏囊" }, { "start_idx": 19, "end_idx": 27, "type": "bod", "entity": "球外毛细血管基底膜" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "补体" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "内皮下" } ] }, { "text": "不规则大块内皮下沉积物使光镜下见毛细血管袢僵硬,毛细血管基底膜增厚呈“白金耳”现象(wireloops)。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "不规则大块内皮下沉积物" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "皮下" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "光镜" }, { "start_idx": 12, "end_idx": 40, "type": "sym", "entity": "光镜下见毛细血管袢僵硬,毛细血管基底膜增厚呈“白金耳”现象" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "毛细血管袢" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "毛细血管基底膜" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "wireloops" } ] }, { "text": "本型还存在严重的小管间质病变、显著的单核细胞浸润以及坏死性血管炎。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "小管间质病变" }, { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "显著的单核细胞浸润" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "单核细胞" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "坏死性血管炎" } ] }, { "text": "临床上本型患儿多为重症;血尿、蛋白尿、高血压、肾病综合征及肾功能不全,如不给予积极治疗,易进展为终末期肾衰竭。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "血尿" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "血" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "蛋白" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "尿" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "高血压" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "肾功能不全" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "终末期肾衰竭" } ] }, { "text": "5.WHOⅤ型(ISKDC6)膜性肾病,病变似特发性膜性肾病,表现为毛细血管袢的弥漫性增厚,后期基底膜增厚呈钉突样表现,但不同的是同时也见一定程度系膜与内皮细胞增生及系膜基质扩张。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "WHOⅤ型" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "ISKDC6" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "特发性膜性肾病" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "毛细血管袢" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "毛细血管袢的弥漫性增厚" }, { "start_idx": 48, "end_idx": 56, "type": "sym", "entity": "基底膜增厚呈钉突样" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "基底膜" }, { "start_idx": 69, "end_idx": 81, "type": "sym", "entity": "一定程度系膜与内皮细胞增生" }, { "start_idx": 73, "end_idx": 81, "type": "dis", "entity": "系膜与内皮细胞增生" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "系膜基质" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "系膜基质扩张" } ] }, { "text": "本型可进一步分为Va型:与原发性膜性肾病极似,细胞增生及浸润不明显;Vb型:伴弥漫性系膜病变;Vc型:伴局灶节段性细胞增生,浸润与硬化;Vd型:伴弥漫增生性病变或新月体形成;a、b亚型较c、d亚型预后好,表明附加病变影响预后。", "entities": [ { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "原发性膜性肾病" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "细胞增生及浸润" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "细胞" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "弥漫性系膜病变" }, { "start_idx": 85, "end_idx": 99, "type": "sym", "entity": "局灶节段性细胞增生,浸润与硬化" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "细胞" }, { "start_idx": 117, "end_idx": 123, "type": "dis", "entity": "弥漫增生性病变" }, { "start_idx": 125, "end_idx": 129, "type": "sym", "entity": "新月体形成" } ] }, { "text": "6.WHOⅥ型肾小球硬化型,此型与其他肾小球疾病晚期硬化相似,常伴随以上各型肾小球病变,如局灶节段或弥漫增殖性病变。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "WHOⅥ型" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾小球" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "肾小球疾病" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "硬化" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "肾小球病变" }, { "start_idx": 45, "end_idx": 56, "type": "dis", "entity": "局灶节段或弥漫增殖性病变" } ] }, { "text": "部分人表现为单纯肾小球硬化。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "单纯肾小球硬化" } ] }, { "text": "狼疮肾炎可以发生病理类型转化,如局灶增殖转化为弥漫性增殖,膜性肾炎转化为局灶节段增殖或弥漫增殖,系膜增殖可转变为局灶节段增殖等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "局灶增殖" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "弥漫性增殖" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "膜性肾炎" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "局灶节段增殖" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "弥漫增殖" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "系膜增殖" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "系膜" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "局灶节段增殖" } ] }, { "text": "(二)肾小管及间质病变狼疮肾炎中约50%~70%有肾小管间质病变,常见于弥漫增殖型,也见于局灶型,少见于膜型肾炎,罕见于系膜增生型。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肾小管及间质病变" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "肾小管间质病变" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "弥漫增殖型" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "膜型肾炎" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "系膜" } ] }, { "text": "病变以小管萎缩,小管基底膜增厚,电子致密物沉积于小管基底膜及间质,严重者出现小管坏死。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "小管萎缩" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "小管基底膜" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "小管基底膜增厚" }, { "start_idx": 16, "end_idx": 31, "type": "sym", "entity": "电子致密物沉积于小管基底膜及间质" }, { "start_idx": 24, "end_idx": 31, "type": "bod", "entity": "小管基底膜及间质" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "小管坏死" } ] }, { "text": "(三)肾小血管病变常见以下几种类型;①高血压引起的血管病变常见;②小叶间动脉及出入球小动脉呈内皮细胞肿胀、破坏,血管内血栓,IgG及C3沉积于血管壁,无炎症反应;③坏死性小血管炎,抗中性粒细胞胞浆抗体(ANCA)阳性;④肾脏血栓微血管病(renalthromboticmicroangiopathy),在无坏死的基础上出现肾小动脉及间质毛细血管血栓,继而发展为肾小球硬化。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肾小血管病变" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "高血压" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "血管病变" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "小叶间动脉" }, { "start_idx": 33, "end_idx": 54, "type": "sym", "entity": "小叶间动脉及出入球小动脉呈内皮细胞肿胀、破坏" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "出入球小动脉" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "血管内血栓" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "血管内" }, { "start_idx": 62, "end_idx": 75, "type": "bod", "entity": "IgG" }, { "start_idx": 62, "end_idx": 90, "type": "sym", "entity": "IgG及C3沉积于血管壁" }, { "start_idx": 77, "end_idx": 84, "type": "bod", "entity": "C3" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "血管壁" }, { "start_idx": 93, "end_idx": 96, "type": "sym", "entity": "炎症反应" }, { "start_idx": 99, "end_idx": 105, "type": "dis", "entity": "坏死性小血管炎" }, { "start_idx": 107, "end_idx": 116, "type": "ite", "entity": "抗中性粒细胞胞浆抗体" }, { "start_idx": 107, "end_idx": 124, "type": "sym", "entity": "抗中性粒细胞胞浆抗体(ANCA)阳性" }, { "start_idx": 118, "end_idx": 121, "type": "ite", "entity": "ANCA" }, { "start_idx": 127, "end_idx": 134, "type": "dis", "entity": "肾脏血栓微血管病" }, { "start_idx": 136, "end_idx": 165, "type": "dis", "entity": "renalthromboticmicroangiopathy" }, { "start_idx": 170, "end_idx": 171, "type": "dis", "entity": "坏死" }, { "start_idx": 178, "end_idx": 181, "type": "bod", "entity": "肾小动脉" }, { "start_idx": 178, "end_idx": 190, "type": "sym", "entity": "肾小动脉及间质毛细血管血栓" }, { "start_idx": 183, "end_idx": 188, "type": "bod", "entity": "间质毛细血管" }, { "start_idx": 197, "end_idx": 201, "type": "dis", "entity": "肾小球硬化" } ] }, { "text": "(四)活动性与慢性病变的判断肾活检后可用半定量积分子方法评定病变情况,指导治疗:公认的活动性指标,如①肾小球节段性坏死;②肾小球细胞明显增生;③基底膜铁丝圈样改变;④内皮下及系膜区较多电子致密物沉积,核碎片及苏木素小体;⑤细胞新月体;⑥肾小血管病变;⑦间质广泛水肿及单核细胞浸润。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "慢性病变" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "肾活检" }, { "start_idx": 20, "end_idx": 27, "type": "pro", "entity": "半定量积分子方法" }, { "start_idx": 51, "end_idx": 58, "type": "dis", "entity": "肾小球节段性坏死" }, { "start_idx": 61, "end_idx": 65, "type": "bod", "entity": "肾小球细胞" }, { "start_idx": 61, "end_idx": 69, "type": "sym", "entity": "肾小球细胞明显增生" }, { "start_idx": 72, "end_idx": 80, "type": "sym", "entity": "基底膜铁丝圈样改变" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "基底膜" }, { "start_idx": 83, "end_idx": 89, "type": "bod", "entity": "内皮下及系膜区" }, { "start_idx": 83, "end_idx": 108, "type": "sym", "entity": "内皮下及系膜区较多电子致密物沉积,核碎片及苏木素小体" }, { "start_idx": 111, "end_idx": 115, "type": "bod", "entity": "细胞新月体" }, { "start_idx": 118, "end_idx": 123, "type": "dis", "entity": "肾小血管病变" }, { "start_idx": 126, "end_idx": 127, "type": "bod", "entity": "间质" }, { "start_idx": 126, "end_idx": 131, "type": "sym", "entity": "间质广泛水肿" }, { "start_idx": 133, "end_idx": 136, "type": "bod", "entity": "单核细胞" }, { "start_idx": 133, "end_idx": 138, "type": "sym", "entity": "单核细胞浸润" } ] }, { "text": "有活动性病变者主张积极给予皮质激素及免疫抑制剂治疗。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "皮质激素" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "慢性病变的证据,如①肾小球硬化;②纤维新月体;③肾小管萎缩;④肾间质纤维化;⑤肾小囊粘连;⑥肾小血管硬化。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性病变" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肾小球硬化" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "纤维新月体" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "肾小管萎缩" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肾间质" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "肾间质纤维化" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肾小囊" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "肾小囊粘连" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "肾小血管" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "肾小血管硬化" } ] }, { "text": "成年病人的资料认为这些慢性化指标,对预后的价值,就Ⅳ型病变而言有用,其五年存活率明显降低,重复肾活体组织检查动态观察意义更大。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "Ⅳ型病变" }, { "start_idx": 47, "end_idx": 53, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "【临床表现】(一)全身性表现多种多样,80%以上有发热,热型多样,高热、低热、间歇或持续发热。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "发热" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "热型多样" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "高热" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "低热" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "间歇或持续发热" } ] }, { "text": "均有不同程度的食欲缺乏、乏力和体重下降。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "乏力" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "体重下降" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "体重" } ] }, { "text": "(二)皮肤黏膜症状70%~80%狼疮患儿有皮肤黏膜损害,典型的蝶形红斑仅见于50%病例,皮疹位于两颊和鼻梁,为鲜红色,边缘清晰,呈轻度水肿性红斑,可见毛细血管扩张和鳞屑。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "皮肤黏膜症状" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "狼疮" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "皮肤黏膜损害" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "蝶形红斑" }, { "start_idx": 44, "end_idx": 62, "type": "sym", "entity": "皮疹位于两颊和鼻梁,为鲜红色,边缘清晰" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "皮" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "轻度水肿性红斑" }, { "start_idx": 75, "end_idx": 80, "type": "sym", "entity": "毛细血管扩张" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "毛细血管" }, { "start_idx": 82, "end_idx": 83, "type": "sym", "entity": "鳞屑" } ] }, { "text": "炎症重时可见水疱及痂皮。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "炎症" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "水疱" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "痂皮" } ] }, { "text": "红斑消退后一般无瘢痕,无色素沉着。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "红斑" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "瘢痕" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "色素沉着" } ] }, { "text": "(三)其他皮肤黏膜症状小儿盘状红斑较成人少,可见出血疹、斑疹、网状青斑、荨麻疹、紫癜、口腔溃疡及鼻黏膜溃疡。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "皮肤黏膜症状" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "盘状红斑" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "出血疹" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "斑疹" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "网状青斑" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "荨麻疹" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "紫癜" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "口腔溃疡" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "鼻黏膜溃疡" } ] }, { "text": "患儿日光照身后皮损加重或出现新的皮疹。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "皮损加重" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "皮" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "出现新的皮疹" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "皮" } ] }, { "text": "约10%~20%患儿始终无皮疹表现。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "皮疹" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "皮" } ] }, { "text": "(四)肌肉骨骼症状约70%~90%患儿有关节和肌肉症状,如关节炎和关节痛,约1/3患儿伴有肌痛。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "肌肉骨骼症状" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肌肉" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "骨骼" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "关节和肌肉症状" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "关节" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肌肉" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "关节炎" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "关节痛" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "关节" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "肌痛" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肌" } ] }, { "text": "关节炎既可呈游走性,也可呈持续性,很少见关节破坏和畸形。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "关节炎" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "关节破坏和畸形" } ] }, { "text": "(五)心血管症状可见心包炎、心脏炎、全心炎及各种小血管炎,雷诺现象在儿科少见。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "心血管症状" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "心血管" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "心包炎" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "心脏炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "全心炎" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "小血管炎" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "雷诺现象" }, { "start_idx": 34, "end_idx": 35, "type": "dep", "entity": "儿科" } ] }, { "text": "(六)浆膜炎30%患儿出现多浆膜炎,如无菌性胸膜炎、腹膜炎、急性狼疮性肺炎及肺出血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "浆膜炎" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "多浆膜炎" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "无菌性胸膜炎" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "急性狼疮性肺炎" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "肺出血" } ] }, { "text": "上述病变可表现为急性发热、呼吸困难、咳嗽、胸痛及胸水症;腹痛、腹泻、恶心、呕吐及腹水症,若发生肠道坏死、穿孔,需外科治疗;严重肺出血可迅速死亡。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "急性发热" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "咳嗽" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "胸痛" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "胸" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "胸水症" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "腹痛" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "腹" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "腹泻" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "腹" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "恶心" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "呕吐" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "腹水症" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "肠道坏死、穿孔" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "外科治疗" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "严重肺出血" } ] }, { "text": "(七)血液系统症状多有不同程度贫血,50%患儿外周血白细胞数减少,15%~30%患儿血小板减少,少数患儿以血小板减少为首发症状。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "血液系统" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "血液系统症状" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "贫血" }, { "start_idx": 23, "end_idx": 29, "type": "ite", "entity": "外周血白细胞数" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "外周血白细胞数减少" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "血小板" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "血小板减少" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "血小板" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "血小板减少" } ] }, { "text": "(八)神经系统症状狼疮脑炎是SLE严重的并发症,相对发生率约30%(20%~50%),有5%患儿以神经系统症状为首发症状,表现为弥漫性脑功能障碍(意识和定向障碍,智能和记忆力下降,精神异常等)或局限性脑功能障碍,如癫痫和脑血管意外,偏瘫及失语。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "狼疮脑炎" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "SLE" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "神经系统" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 64, "end_idx": 71, "type": "dis", "entity": "弥漫性脑功能障碍" }, { "start_idx": 73, "end_idx": 79, "type": "dis", "entity": "意识和定向障碍" }, { "start_idx": 81, "end_idx": 88, "type": "sym", "entity": "智能和记忆力下降" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "精神异常" }, { "start_idx": 97, "end_idx": 104, "type": "dis", "entity": "局限性脑功能障碍" }, { "start_idx": 107, "end_idx": 108, "type": "dis", "entity": "癫痫" }, { "start_idx": 110, "end_idx": 114, "type": "dis", "entity": "脑血管意外" }, { "start_idx": 116, "end_idx": 117, "type": "dis", "entity": "偏瘫" }, { "start_idx": 119, "end_idx": 120, "type": "dis", "entity": "失语" } ] }, { "text": "周围神经病变少见,表现为多发性周围神经炎。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "周围神经病变" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "多发性周围神经炎" } ] }, { "text": "(九)其他症状有肝脏肿大(75%)、肝功异常以及脾肿大(25%)。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肝脏" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "肝脏肿大" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "肝功异常" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "肝功" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "脾" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "脾肿大" } ] }, { "text": "浅表淋巴结肿大(约50%)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "浅表淋巴结" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "浅表淋巴结肿大" } ] }, { "text": "(十)肾脏症状狼疮肾炎在SLE中很常见,且是危及远期生命质量的关键因素。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肾脏症状" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "SLE" } ] }, { "text": "狼疮肾炎临床表现主要有以下6种形式。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "狼疮肾炎" } ] }, { "text": "1.轻型无症状蛋白尿或(及)血尿(及)血尿,约30%~50%LN患儿表现此型,无水肿,无高血压,仅表现为轻~中度蛋白尿(常<2.5g/d)和/或血尿。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "蛋白" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "血尿" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "血尿" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "LN" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "水肿" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "高血压" }, { "start_idx": 52, "end_idx": 68, "type": "sym", "entity": "轻~中度蛋白尿(常<2.5g/d)" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "蛋白" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "尿" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "血尿" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "血" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "尿" } ] }, { "text": "2.慢性肾炎型起病隐匿,缓慢进展的肾炎综合征。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "肾炎综合征" } ] }, { "text": "有不同程度肾功能不全,高血压。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肾功能不全" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "高血压" } ] }, { "text": "3.急性肾炎或急进性肾炎综合征其中35%~50%患者有高血慢性肾炎型同程度蛋白尿,尿沉渣中有较多红细胞管型,肾功能不全或衰竭。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "急进性肾炎综合征" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "蛋白" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "尿" }, { "start_idx": 41, "end_idx": 52, "type": "sym", "entity": "尿沉渣中有较多红细胞管型" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "尿" }, { "start_idx": 46, "end_idx": 52, "type": "bod", "entity": "较多红细胞管型" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "肾功能不全或衰竭" } ] }, { "text": "急性肾炎起病急性肾炎菌感染后急性肾炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "急性肾炎菌感染" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "急性肾炎" } ] }, { "text": "急进性肾炎起病类似其他急进性肾炎,表现为急性进展的少尿性急性肾衰竭。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "急进性肾炎" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "急进性肾炎" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "少尿性急性肾衰竭" } ] }, { "text": "但这两种起病方式在LN中均少见。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "LN" } ] }, { "text": "4.肾病综合征此型约占LN总数的40%,临床上可表现为单纯性肾病综合征或肾病综合征伴明显肾炎综合征。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "LN" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "单纯性肾病综合征" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "肾炎综合征" } ] }, { "text": "5.肾小管损害型肾小管酸中毒伴肾钙化、肾结石及尿镁丢失,LN病人中约44%有不同程度肾小管功能损害。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肾小管损害" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "肾小管酸中毒" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "肾钙化" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肾" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "肾结石" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "尿镁丢失" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "镁" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "LN" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "肾小管功能损害" } ] }, { "text": "临床类型间也可转变,当血尿、蛋白尿、肾功能减退及高血压加重时均提示临床类型或病理类型发生转变,预后不良。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "蛋白" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "肾功能减退" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "高血压" } ] }, { "text": "【实验室检查】(一)尿液检查蛋白尿、血尿、细胞及蛋白管型常见。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "尿液检查" }, { "start_idx": 10, "end_idx": 27, "type": "sym", "entity": "尿液检查蛋白尿、血尿、细胞及蛋白管型" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "蛋白" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "血" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "尿" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "细胞及蛋白管型" } ] }, { "text": "(二)血液检查大多有不同程度贫血,部分人白细胞减少血小板减少,90%以上患者血沉明显增快,血白蛋白降低,球蛋白升高,以球蛋白升高为主,但若有重度蛋白尿,球蛋白绝对值也降低。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血液检查" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "贫血" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "白细胞" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "白细胞减少" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "血小板" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "血小板减少" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "血沉明显增快" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "血沉" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "血白蛋白" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "血白蛋白降低" }, { "start_idx": 52, "end_idx": 54, "type": "ite", "entity": "球蛋白" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "球蛋白升高" }, { "start_idx": 59, "end_idx": 61, "type": "ite", "entity": "球蛋白" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "球蛋白升高" }, { "start_idx": 70, "end_idx": 74, "type": "sym", "entity": "重度蛋白尿" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "蛋白" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "尿" }, { "start_idx": 76, "end_idx": 81, "type": "ite", "entity": "球蛋白绝对值" }, { "start_idx": 76, "end_idx": 84, "type": "sym", "entity": "球蛋白绝对值也降低" } ] }, { "text": "(三)免疫学检查1.抗核抗体(antinuclearantibodies,ANAs)若免疫荧光分析ANA呈周边型对SLE诊断最有意义,提示dsDNA抗体阳性,该抗体对SLE有高度特异性,且与疾病活动性相关。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "免疫学检查" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 15, "end_idx": 35, "type": "bod", "entity": "antinuclearantibodies" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "ANAs" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "ANA" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "SLE" }, { "start_idx": 69, "end_idx": 75, "type": "ite", "entity": "dsDNA抗体" }, { "start_idx": 69, "end_idx": 77, "type": "sym", "entity": "dsDNA抗体阳性" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "抗体" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "SLE" } ] }, { "text": "2.抗双链DNA(dsDNA)抗体直接检测dsDNA抗体阳性率为50%~80%,但特异性大于90%,且往往提示有肾脏损害,偶见于干燥综合征、类风湿性关节炎及活动性肝炎。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "pro", "entity": "抗双链DNA(dsDNA)抗体直接检测" }, { "start_idx": 21, "end_idx": 27, "type": "ite", "entity": "dsDNA抗体" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "肾脏损害" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "干燥综合征" }, { "start_idx": 70, "end_idx": 76, "type": "dis", "entity": "类风湿性关节炎" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "活动性肝炎" } ] }, { "text": "3.抗Sm抗体约25%~40%病人抗Sm抗体阳性,但其特异性可达99%。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "抗Sm抗体" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "抗Sm抗体" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "抗Sm抗体阳性" } ] }, { "text": "4.其他自身抗体抗单链DNA(ssDNA)抗体,阳性率高,特异性不强,26%~45%病人抗核糖核蛋白(RNP身体)抗体阳性,但特异性不高。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "自身抗体" }, { "start_idx": 8, "end_idx": 22, "type": "bod", "entity": "抗单链DNA(ssDNA)抗体" }, { "start_idx": 44, "end_idx": 60, "type": "sym", "entity": "抗核糖核蛋白(RNP身体)抗体阳性" }, { "start_idx": 44, "end_idx": 57, "type": "ite", "entity": "抗核糖核蛋白(RNP身体)抗" } ] }, { "text": "抗干燥综合征(SS)A、B抗体敏感性及特异性均差。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "bod", "entity": "抗干燥综合征(SS)A、B抗体" } ] }, { "text": "有坏死性血管炎时抗中性粒细胞胞浆抗体(ANCA)阳性,抗心磷脂抗体阳性病例常见病情呈复发性,多发性动、静脉栓塞,血小板减少及流产。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "坏死性血管炎" }, { "start_idx": 8, "end_idx": 17, "type": "ite", "entity": "抗中性粒细胞胞浆抗体" }, { "start_idx": 8, "end_idx": 25, "type": "sym", "entity": "抗中性粒细胞胞浆抗体(ANCA)阳性" }, { "start_idx": 19, "end_idx": 22, "type": "ite", "entity": "ANCA" }, { "start_idx": 27, "end_idx": 32, "type": "ite", "entity": "抗心磷脂抗体" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "抗心磷脂抗体阳性" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "多发性动、静脉栓塞" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "动、静脉" }, { "start_idx": 56, "end_idx": 58, "type": "ite", "entity": "血小板" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "血小板减少" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "流产" } ] }, { "text": "5.补体C1q、C3、C4及CH50在SLE活动期常降低。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "补体" }, { "start_idx": 4, "end_idx": 12, "type": "ite", "entity": "C1q" }, { "start_idx": 14, "end_idx": 21, "type": "ite", "entity": "C3" }, { "start_idx": 23, "end_idx": 30, "type": "ite", "entity": "C4" }, { "start_idx": 32, "end_idx": 41, "type": "ite", "entity": "CH50" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "SLE" } ] }, { "text": "6.循环免疫复合物阳性。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "循环免疫复合物阳性" }, { "start_idx": 2, "end_idx": 8, "type": "ite", "entity": "循环免疫复合物" } ] }, { "text": "(四)狼疮细胞狼疮细胞(lupuserythematosuscell,LEC)在SLE病人中阳性率可达60%~85%,但也可见于其他结缔组织病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "狼疮细胞" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "狼疮细胞" }, { "start_idx": 12, "end_idx": 33, "type": "ite", "entity": "lupuserythematosuscell" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "LEC" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "SLE" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "结缔组织病" } ] }, { "text": "(五)狼疮带试验取材于暴露在阳光下的正常皮肤,用直接免疫荧光检测表皮与真皮连接处,可见一条IgG和C3沉积的荧光带,80%活动期SLE病人阳性,其他自身免疫性疾病也可呈阳性。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "狼疮带试验" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "直接免疫荧光检测" }, { "start_idx": 32, "end_idx": 39, "type": "bod", "entity": "表皮与真皮连接处" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "IgG" }, { "start_idx": 49, "end_idx": 56, "type": "bod", "entity": "C3" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "SLE" }, { "start_idx": 80, "end_idx": 86, "type": "dis", "entity": "自身免疫性疾病" } ] }, { "text": "【诊断与鉴别诊断】(一)诊断标准本病诊断标准大多参考美国风湿病学会1982年提出的诊断条件,在11项标准中符合4项或以上即可诊断本病(表12-12)。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "风湿病" } ] }, { "text": "中华风湿病协会1987年提出的标准增加了低补体C3及皮肤狼疮带试验及肾活检特征后,其诊断特异性为93.6%,敏感性提高到97.5%,并可早期发现以原发性肾病综合征起病的患者。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "风湿病" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "低补体C3" }, { "start_idx": 21, "end_idx": 30, "type": "ite", "entity": "补体C3" }, { "start_idx": 32, "end_idx": 38, "type": "pro", "entity": "皮肤狼疮带试验" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "肾活检" }, { "start_idx": 79, "end_idx": 86, "type": "dis", "entity": "原发性肾病综合征" } ] }, { "text": "表12-3系统性红斑狼疮诊断标准(美国风湿病学会修订,1982年)(二)鉴别诊断注意与其他风湿性疾病,如幼年类风湿性关节炎全身型和多关节型、皮肌炎、硬皮症、混合性结缔组织病以及多发性血管炎等鉴别。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "风湿病" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 52, "end_idx": 68, "type": "dis", "entity": "幼年类风湿性关节炎全身型和多关节型" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "硬皮症" }, { "start_idx": 78, "end_idx": 85, "type": "dis", "entity": "混合性结缔组织病" }, { "start_idx": 88, "end_idx": 93, "type": "dis", "entity": "多发性血管炎" } ] }, { "text": "本病也易与各类肾病、心脏病、溶血性贫血、血小板减少性紫癜、组织细胞增多症、慢性活动性肝炎及神经系统疾病混淆。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肾病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "心脏病" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "血小板减少性紫癜" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "组织细胞增多症" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "慢性活动性肝炎" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "神经系统疾病" } ] }, { "text": "特别是要注意心、肾及神经系统并发症的及时干预治疗,充分考虑药物治疗的利弊及得失后确定近期和远期的治疗方案,并认真评价治疗风险与效益,让患儿监护人充分知晓。", "entities": [ { "start_idx": 3, "end_idx": 23, "type": "pro", "entity": "要注意心、肾及神经系统并发症的及时干预治疗" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "药物治疗" }, { "start_idx": 40, "end_idx": 51, "type": "pro", "entity": "确定近期和远期的治疗方案" } ] }, { "text": "(二)免疫抑制剂1.糖皮质激素是治疗SLE基本药物,主要作用于G0期淋巴细胞,有强烈抗炎作用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "SLE" }, { "start_idx": 31, "end_idx": 48, "type": "bod", "entity": "G0期淋巴细胞" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "抗炎" } ] }, { "text": "常用量为泼尼松1~2mg/(kg•d)(总量<60mg/d),分3次口服,病情缓解、实验室检查基本正常后改为隔日顿服,病情稳定后可以减至小剂量(0.5~1mg/kg,隔日)长期用药,维持疗效。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "泼尼松" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "口服" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "实验室检查" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "隔日顿服" } ] }, { "text": "临床发现多数狼疮肾炎患儿单用泼尼松治疗无效,尤其是Ⅳ型狼疮肾炎,急进性狼疮肾炎肾上腺皮质激素治疗更不敏感。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "泼尼松" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "Ⅳ型狼疮肾炎" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "急进性狼疮肾炎" }, { "start_idx": 39, "end_idx": 47, "type": "pro", "entity": "肾上腺皮质激素治疗" } ] }, { "text": "甲泼尼龙冲击治疗(每次15~30mg/kg,总量<1g/次,每日1次,3次一疗程,间隔1~2周可重复一疗程,共2~3疗程后用中、小剂量泼尼松维持治疗,可使部分狼疮肾炎患儿迅速缓解,肾功能较快好转。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "甲泼尼龙冲击治疗" }, { "start_idx": 64, "end_idx": 73, "type": "pro", "entity": "小剂量泼尼松维持治疗" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 90, "end_idx": 92, "type": "ite", "entity": "肾功能" } ] }, { "text": "糖皮质激素长期使用,易发生条件致病菌感染、骨质疏松、高血压、水电解质紊乱、精神病以及消化道出血等多种毒副作用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "致病菌感染" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "骨质疏松" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "高血压" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "水电解质紊乱" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "精神病" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "消化道出血" } ] }, { "text": "2.细胞毒类药物很多观察均认为皮质激素联合细胞毒性药物治疗狼疮肾炎,疗效远较单用皮质激素或单用细胞毒类药物好。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "细胞毒类药物" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "皮质激素" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "细胞毒性药物" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "皮质激素" }, { "start_idx": 47, "end_idx": 52, "type": "dru", "entity": "细胞毒类药物" } ] }, { "text": "联合用药还可大大减少皮质激素的用药量,提高疗效。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "联合用药" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "皮质激素" } ] }, { "text": "常用的细胞毒类药物有环磷酰胺(CTX)、硫唑嘌呤以及氮芥。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "细胞毒类药物" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "CTX" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 26, "end_idx": 27, "type": "dru", "entity": "氮芥" } ] }, { "text": "其中以CTX使用最广泛,疗效最好。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "CTX" } ] }, { "text": "CTX主要作用于S期,对整个细胞周期均有作用,能有效抑制抗体产生,抗细胞毒及抗炎症介质作用也很明显,其免疫抑制效应强烈而持久。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "CTX" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "抗体" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "细胞" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "炎症介质" } ] }, { "text": "皮质激素联合CTX(2~2.5mg/(kg•d))对保存肾功能有明显作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "皮质激素" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "CTX" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "肾功能" } ] }, { "text": "近年资料表明CTX大剂量冲击用药,较口服CTX不良反应更少,肾脏保护效果更好。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "CTX" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "CTX" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肾脏" } ] }, { "text": "CTX冲击方案尚未成熟,最积极的方案是每次8~12mg/kg,每日1次,连用2日一疗程(总量<1g/疗程),至少间隔2周用一疗程,连用六疗程后改为3个月一疗程,维持2年;也有每月一疗程,连用6个月后停药的半年方案以及每月1次连用6个月,再3个月一次维持2年的长疗程治疗方案。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "CTX冲击方案" } ] }, { "text": "1992年NIH研究小组报告的前瞻性研究结果认为,长疗程较半年疗程在保护肾功能方面疗效更好,只有10%病人进入终末期肾衰。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "肾功能" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "终末期肾衰" } ] }, { "text": "CTX大剂量冲击治疗应注意消化道副反应和采取水化措施(60~80ml/(kg•d)或2000ml/m2电解质平衡液持续静滴),防止出血性膀胱炎。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "CTX大剂量冲击治疗" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "消化道" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "消化道副反应" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "水化措施" }, { "start_idx": 57, "end_idx": 62, "type": "dru", "entity": "电解质平衡液" }, { "start_idx": 63, "end_idx": 66, "type": "pro", "entity": "持续静滴" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "出血性膀胱炎" } ] }, { "text": "目前尚无资料确切证明口服方案与冲击方案对性腺影响的大小。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "口服方案" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "冲击方案" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "性腺" } ] }, { "text": "3.硫唑嘌呤每天2.5mg/kg治疗严重弥漫增殖型LN,可减少皮质激素用量,与皮质激素联合口服CTX效果相同。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "严重弥漫增殖型LN" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "皮质激素" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "皮质激素" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "联合口服" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "CTX" } ] }, { "text": "甲泼尼龙冲击治疗后可用小剂量泼尼松及硫唑嘌呤维持治疗。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "甲泼尼龙冲击治疗" }, { "start_idx": 11, "end_idx": 25, "type": "pro", "entity": "小剂量泼尼松及硫唑嘌呤维持治疗" } ] }, { "text": "4.苯丁酸氮芥0.2mg/(kg•d)分3次口服,疗程2~3个月,其对性腺的CTX反应与致癌作用并不比CTX小。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "苯丁酸氮芥" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "口服" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "性腺" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "CTX" }, { "start_idx": 45, "end_idx": 45, "type": "dis", "entity": "癌" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "CTX" } ] }, { "text": "5.环孢霉素A选择性作用于辅助性T细胞,间接抑制B细胞产生抗体,但毒副作用大,尤其是肾脏的毒副作用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "环孢霉素A" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "辅助性T细胞" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "B细胞" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "抗体" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肾脏" } ] }, { "text": "一般仅在CTX不能使病情缓解者选用环孢毒素A;急性期用药5~7mg/(kg•d),维持用药4mg/(kg•d),可作为激素、细胞毒类及抗凝剂三联用药抗凝剂三联用药的候选药物之一。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "CTX" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "环孢毒素A" }, { "start_idx": 59, "end_idx": 76, "type": "dru", "entity": "激素、细胞毒类及抗凝剂三联用药抗凝剂" }, { "start_idx": 77, "end_idx": 80, "type": "pro", "entity": "三联用药" } ] }, { "text": "(三)抗凝剂狼疮肾炎病人多呈高凝状态,尤其是使用肾上腺皮质激素之后,血小板聚集力增强,血纤维蛋白原升高,不但可发生肾小球毛细血管血栓,还易并发肾静脉等大血管血栓,应予抗凝治疗。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "抗凝剂" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "高凝状态" }, { "start_idx": 24, "end_idx": 30, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "血小板聚集力增强" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "血小板" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "血纤维蛋白原升高" }, { "start_idx": 43, "end_idx": 48, "type": "ite", "entity": "血纤维蛋白原" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "肾小球毛细血管血栓" }, { "start_idx": 57, "end_idx": 63, "type": "bod", "entity": "肾小球毛细血管" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "血" }, { "start_idx": 71, "end_idx": 79, "type": "sym", "entity": "肾静脉等大血管血栓" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "肾静脉" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "大血管" }, { "start_idx": 78, "end_idx": 78, "type": "bod", "entity": "血" }, { "start_idx": 83, "end_idx": 86, "type": "pro", "entity": "抗凝治疗" } ] }, { "text": "严重弥漫增殖型LN可用肝素100U/kg或蝮蛇抗栓酶0.01U/kg(<0.25U/次,每日1~2次)静滴或口服双嘧达莫3~8mg/(kg•d)。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "严重弥漫增殖型LN" }, { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "肝素" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "蝮蛇抗栓酶" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "静滴或口服" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "双嘧达莫" } ] }, { "text": "有肯定血栓形成者可用尿激酶每次200~600U/kg,溶于葡萄糖水200ml中静滴,每日一次,14天一疗程。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "血栓形成" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "尿激酶" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "葡萄糖水" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "静滴" } ] }, { "text": "(四)血浆置换可清除部分致病性抗体、抗原及免疫复合物,但价格昂贵,多用于对其他治疗无反应的严重LN患儿,对狼疮脑患儿效果较好。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "血浆置换" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "致病性抗体" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "抗原" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "严重LN" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "狼疮脑" } ] }, { "text": "也有人主张在急进性LN患儿给甲泼尼龙冲击治疗同时给予血浆置换疗法,每日置换2~4L,连续3天。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "急进性LN" }, { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "甲泼尼龙冲击治疗" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "血浆置换疗法" }, { "start_idx": 33, "end_idx": 40, "type": "pro", "entity": "每日置换2~4L" } ] }, { "text": "(五)静脉注射用丙种球蛋白静脉注射用丙种球蛋白(IVIG)对部分狼疮患儿有一定疗效,可抑制B细胞产生抗体,可改变抗体及抗原比例,使免疫复合物易于清除。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dru", "entity": "静脉注射用丙种球蛋白" }, { "start_idx": 13, "end_idx": 22, "type": "dru", "entity": "静脉注射用丙种球蛋白" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "IVIG" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "狼疮" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "B细胞" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "抗体" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "抗体" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "抗原" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "免疫复合物" } ] }, { "text": "可使部分CTX耐药的患儿病情缓解。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "CTX" } ] }, { "text": "(六)全身淋巴结X线照射有报告用X线照射全身淋巴结(20Gy/4~6周),可使部分病人取得一定疗效,肌酐清除率好转,dsDNA抗体减少,甚或停用泼尼松。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "全身淋巴结X线照射" }, { "start_idx": 16, "end_idx": 24, "type": "pro", "entity": "X线照射全身淋巴结" }, { "start_idx": 50, "end_idx": 54, "type": "ite", "entity": "肌酐清除率" }, { "start_idx": 58, "end_idx": 64, "type": "ite", "entity": "dsDNA抗体" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "泼尼松" } ] }, { "text": "(七)抗CD4单克隆抗体可使T细胞数下降,B细胞抑制,蛋白尿减少,血浆蛋白升高。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dru", "entity": "抗CD4单克隆抗体" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "T细胞数" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "B细胞" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" }, { "start_idx": 33, "end_idx": 36, "type": "ite", "entity": "血浆蛋白" } ] }, { "text": "【预后】早年LN患儿多死于尿毒症,死亡率达60%~80%,近年因正确诊断,分型及诊疗手段改变,其死亡率已下降至18.9%~25.4%。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "LN" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "尿毒症" } ] }, { "text": "下列因素可能影响预后:(一)临床表现持续大量蛋白尿、血尿、高血压、贫血及血肌酐水平已升高者预后不良,反复感染也影响预后。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "持续大量蛋白尿" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "尿" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "血尿" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "尿" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "高血压" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "贫血" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "血肌酐水平已升高" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "血肌酐水平" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "反复感染" } ] }, { "text": "(二)病理类型Ⅰ、Ⅱ型一般不发展为终末期肾,预后不良者多死于并发症;Ⅲ型可能发展成慢性肾衰竭,但5年存活率仍达75.8%。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "终末期肾" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "Ⅴ型若有附加增生性病变(c、d亚型)则预后不良,与Ⅳ型相似。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "附加增生性病变" } ] }, { "text": "公认有大量内皮下电子致密物沉着、合并血管病变及肾功能恶化需替代治疗者预后恶劣。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "大量内皮下电子致密物沉着" }, { "start_idx": 3, "end_idx": 12, "type": "bod", "entity": "大量内皮下电子致密物" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "血管病变" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "肾功能" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "肾功能恶化" } ] }, { "text": "二、过敏性口炎过敏性口炎亦称变态反应性口炎(allergicstomatitis),是由于个体差异,一些普通无害的东西如各种口腔药物漱口水、牙膏碘合剂或药物作为抗原刺激黏膜,使局部产生抗原抗体反应而引起的黏膜损害。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "过敏性口炎" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "过敏性口炎" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "变态反应性口炎" }, { "start_idx": 22, "end_idx": 39, "type": "dis", "entity": "allergicstomatitis" }, { "start_idx": 80, "end_idx": 85, "type": "sym", "entity": "抗原刺激黏膜" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "黏膜" }, { "start_idx": 88, "end_idx": 97, "type": "sym", "entity": "局部产生抗原抗体反应" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "抗体" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "黏膜损害" }, { "start_idx": 102, "end_idx": 103, "type": "bod", "entity": "黏膜" } ] }, { "text": "致敏物质去除后,口腔炎症还要持续一段时间。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "口腔炎症" } ] }, { "text": "五、遗传性疾病的治疗目前遗传病的治疗主要针对部分遗传代谢病,可以根据疾病作以下选择:1.饮食治疗减少或除去有害物质,主要适用于一些遗传代谢病,如对苯丙酮尿症限制苯丙氨酸的摄入,半乳糖血症停奶及乳制品,但可以进食鱼和肉。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "遗传病" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "饮食治疗" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 78, "end_idx": 86, "type": "pro", "entity": "限制苯丙氨酸的摄入" }, { "start_idx": 88, "end_idx": 92, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 93, "end_idx": 98, "type": "pro", "entity": "停奶及乳制品" } ] }, { "text": "2.药物治疗主要补充体内代谢必须的维生素、激素、电解质以及对症治疗,如BH4缺乏引起的非经典型苯丙酮尿症给予L-多巴、5-羟色胺和BH4治疗,先天性肾上腺皮质增生症采用肾上腺皮质激素治疗,同型胱氨酸尿给予大剂量维生素B6,枫糖尿病用维生素B1,对原因不明的代谢病,可给予大剂量多种维生素,有时可收到疗效。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "药物治疗" }, { "start_idx": 8, "end_idx": 26, "type": "pro", "entity": "补充体内代谢必须的维生素、激素、电解质" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "对症治疗" }, { "start_idx": 35, "end_idx": 45, "type": "sym", "entity": "BH4缺乏" }, { "start_idx": 35, "end_idx": 43, "type": "bod", "entity": "BH4" }, { "start_idx": 49, "end_idx": 57, "type": "dis", "entity": "非经典型苯丙酮尿症" }, { "start_idx": 60, "end_idx": 63, "type": "dru", "entity": "L-多巴" }, { "start_idx": 65, "end_idx": 69, "type": "dru", "entity": "5-羟色胺" }, { "start_idx": 71, "end_idx": 79, "type": "dru", "entity": "BH4" }, { "start_idx": 83, "end_idx": 93, "type": "dis", "entity": "先天性肾上腺皮质增生症" }, { "start_idx": 96, "end_idx": 102, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 106, "end_idx": 111, "type": "dis", "entity": "同型胱氨酸尿" }, { "start_idx": 117, "end_idx": 127, "type": "dru", "entity": "维生素B6" }, { "start_idx": 129, "end_idx": 132, "type": "dis", "entity": "枫糖尿病" }, { "start_idx": 134, "end_idx": 144, "type": "dru", "entity": "维生素B1" }, { "start_idx": 152, "end_idx": 154, "type": "dis", "entity": "代谢病" }, { "start_idx": 164, "end_idx": 166, "type": "dru", "entity": "维生素" } ] }, { "text": "3.酶蛋白的替代治疗通过分离出特定的基因,在体外进行DNA重组,然后转移至细胞或者细菌内表达,提纯酶蛋白,用来治疗遗传代谢缺陷病。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "酶蛋白" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "替代治疗" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "分离出特定的基因" }, { "start_idx": 21, "end_idx": 30, "type": "pro", "entity": "在体外进行DNA重组" }, { "start_idx": 34, "end_idx": 45, "type": "pro", "entity": "转移至细胞或者细菌内表达" }, { "start_idx": 47, "end_idx": 51, "type": "pro", "entity": "提纯酶蛋白" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "遗传代谢缺陷病" } ] }, { "text": "通过此类方法制造出的胰岛素及生长激素等重组蛋白,为治疗遗传代谢病提示了新的方向。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "胰岛素" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "生长激素" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "重组蛋白" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "4.外科手术如女性先天性肾上腺皮质增生症可做外科整形,有些疾病可做脏器移植。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "外科手术" }, { "start_idx": 7, "end_idx": 19, "type": "dis", "entity": "女性先天性肾上腺皮质增生症" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "外科整形" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "脏器移植" } ] }, { "text": "5.干细胞移植及骨髓移植是将有功能的细胞或者组织转移到病人的体内,发挥功能,纠正遗传缺陷。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "干细胞移植" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 13, "end_idx": 31, "type": "pro", "entity": "将有功能的细胞或者组织转移到病人的体内" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "遗传缺陷" } ] }, { "text": "干细胞移植技术飞速进展,更为安全有效,已成为治疗遗传性疾病的重要手段,治愈的病种还在不断的扩大。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "干细胞移植" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "遗传性疾病" } ] }, { "text": "6.基因治疗与传统治疗的明显不同之处是以特定的DNA构件导入病人体内。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "基因治疗" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "传统治疗" }, { "start_idx": 19, "end_idx": 33, "type": "pro", "entity": "以特定的DNA构件导入病人体内" } ] }, { "text": "第二节儿童急性非淋巴细胞白血病儿童急性非淋巴细胞白血病(acutenonlymphocyticleukemiaAML)约占儿童白血病的15%~20%,随着生物分子学的进展,对AML的发病机制及生物学特性有了更进一步的了解,目前在合理治疗后约有30%~45%的患儿可获得长期无病生存,较急性淋巴细胞白血病(ALL)的预后仍然明显差。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "儿童急性非淋巴细胞白血病" }, { "start_idx": 15, "end_idx": 26, "type": "dis", "entity": "儿童急性非淋巴细胞白血病" }, { "start_idx": 28, "end_idx": 57, "type": "dis", "entity": "acutenonlymphocyticleukemiaAML" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "儿童白血病" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "AML" }, { "start_idx": 142, "end_idx": 150, "type": "dis", "entity": "急性淋巴细胞白血病" }, { "start_idx": 152, "end_idx": 154, "type": "dis", "entity": "ALL" } ] }, { "text": "【流行病学】在儿童中AML与ALL发病比例约为1∶4~5,但在出生后4周内诊断的先天性白血病几乎均为AML。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "AML" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "ALL" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "先天性白血病" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "AML" } ] }, { "text": "根据上海市1990~1992年的肿瘤登记资料,上海地区0~15岁的AML年发病率为4.92/100万。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "AML" } ] }, { "text": "【病因学】(一)理化因素如苯和电离辐射的暴露与AML发病有关,但能明确的个体致病因素仅占发病数中的极少部分。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "AML" } ] }, { "text": "日本在原子弹爆炸后AML的发病率增高20倍,其高峰在接受辐射后6~8年。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "AML" } ] }, { "text": "孕期吸烟吸烟也增高AML的发病率。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "AML" } ] }, { "text": "(二)病毒尚未证实与AML发病有关的病毒。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "病毒" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "AML" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "病毒" } ] }, { "text": "(三)先天性遗传至今在大多数儿童AML中未能证实遗传因素的参与,偶有同胞发病或家族性发病的报告。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "儿童AML" } ] }, { "text": "唐氏综合征患儿发生AML的机会比正常人群高14倍。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "唐氏综合征" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "AML" } ] }, { "text": "一些先天性骨髓性疾病中发生AML的机会也增加。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "先天性骨髓性疾病" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "AML" } ] }, { "text": "【生物学特征】AML存在逃脱顺序化死亡调控的机制,部分细胞永生化。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "AML" }, { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "存在逃脱顺序化死亡调控的机制" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "部分细胞永生化" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "部分细胞" } ] }, { "text": "在M3中已明确由于t(15;17)造成PML/RARa融合,阻断了对细胞的正常分化调控。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "M3" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "t(15;17)" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "PML/RARa融合" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "PML" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "RARa" }, { "start_idx": 36, "end_idx": 48, "type": "sym", "entity": "阻断了对细胞的正常分化调控" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "细胞" } ] }, { "text": "在AML治疗中易产生多药耐药,有多种耐药机制参与,包括P糖蛋白,它由多药耐药基因家族中的MDR编码,主要功能为主动泵出多种进入细胞的药物而使肿瘤细胞获得耐药性。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "AML" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "易产生多药耐药" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "P糖蛋白" }, { "start_idx": 34, "end_idx": 46, "type": "bod", "entity": "多药耐药基因家族中的MDR" }, { "start_idx": 55, "end_idx": 67, "type": "sym", "entity": "主动泵出多种进入细胞的药物" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "细胞" }, { "start_idx": 69, "end_idx": 78, "type": "sym", "entity": "使肿瘤细胞获得耐药性" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "钙通道阻滞剂和环胞霉素A可阻断P糖蛋白的功能。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "钙通道阻滞剂" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "环胞霉素A" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "P糖蛋白" } ] }, { "text": "AML常见的特征性染色体异常有M2的t(8;21)和t(3;21),M3的t(15;17)和t(11;17),M4Eo的inv(16)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "AML" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "特征性染色体异常" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "染色体" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "M2" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "t(8;21)" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "t(3;21)" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "M3" }, { "start_idx": 49, "end_idx": 56, "type": "sym", "entity": "t(15;17)" }, { "start_idx": 58, "end_idx": 65, "type": "sym", "entity": "t(11;17)" }, { "start_idx": 67, "end_idx": 76, "type": "dis", "entity": "M4Eo" }, { "start_idx": 78, "end_idx": 84, "type": "sym", "entity": "inv(16)" } ] }, { "text": "【形态学分类】根据较公认的法、美、英(FAB)形态学分类,将AML分为M1至M7七个类型,各型特征(表11-8)。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "AML" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "M1" }, { "start_idx": 44, "end_idx": 51, "type": "dis", "entity": "M7" } ] }, { "text": "表11-8AML各亚型特征MP:髓过氧化酶;PAS:糖原;NSE:非特异性酯酶;perox:过氧化酶【临床表现】儿童急性白血病的临床表现有共性,主要表现为贫血、皮肤黏膜或内脏出血倾向发热及各种类型感染。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "AML" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "MP" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "髓过氧化酶" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "PAS" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "糖原" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "NSE" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "非特异性酯酶" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "perox" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "过氧化酶" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "儿童急性白血病" }, { "start_idx": 77, "end_idx": 78, "type": "dis", "entity": "贫血" }, { "start_idx": 80, "end_idx": 90, "type": "sym", "entity": "皮肤黏膜或内脏出血倾向" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "内脏" }, { "start_idx": 88, "end_idx": 88, "type": "bod", "entity": "血" }, { "start_idx": 91, "end_idx": 92, "type": "sym", "entity": "发热" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "各种类型感染" } ] }, { "text": "除M4、M5以外,其他AML浸润中枢神经系统的机会比ALL少。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "M4" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "M5" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "AML" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "浸润中枢神经系统" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "ALL" } ] }, { "text": "【诊断】白血病诊断应包括以下三个内容:细胞形态学、免疫表型和细胞遗传学,随着发展还应包括基因型的分析。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "白血病" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "细胞形态学" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "免疫表型" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "细胞遗传学" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "基因型的分析" } ] }, { "text": "(一)骨髓细胞形态学当临床怀疑白血病时必须进行骨髓涂片作形态学、组织化学染色检查才能明确诊断。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "骨髓细胞形态学" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "白血病" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 28, "end_idx": 39, "type": "pro", "entity": "形态学、组织化学染色检查" } ] }, { "text": "骨髓涂片显示常为高度增生,幼稚细胞≥30%,在30%~100%之间,约4/5病人在形态学和组织化学染色检查后能作出明确的形态分型诊断,另1/5病人需进行免疫表型和细胞遗传学分析来进一步鉴别AML和ALL。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "骨髓涂片显示常为高度增生" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "高度增生" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "幼稚细胞" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "幼稚细胞≥30%" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "在30%~100%之间" }, { "start_idx": 41, "end_idx": 52, "type": "pro", "entity": "形态学和组织化学染色检查" }, { "start_idx": 60, "end_idx": 65, "type": "pro", "entity": "形态分型诊断" }, { "start_idx": 76, "end_idx": 87, "type": "pro", "entity": "免疫表型和细胞遗传学分析" }, { "start_idx": 94, "end_idx": 96, "type": "dis", "entity": "AML" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "ALL" } ] }, { "text": "AML各型中组织化学染色特征(表11-8)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "AML" }, { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "组织化学染色" } ] }, { "text": "(二)免疫表型当骨髓造血干细胞逐步分化成熟时可表达与特定细胞系和分化阶段相关的免疫表型,在细胞形态学和组织化学染色结果不一致时,免疫表型对诊断起重要作用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "免疫表型" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "骨髓造血干细胞" }, { "start_idx": 25, "end_idx": 42, "type": "bod", "entity": "与特定细胞系和分化阶段相关的免疫表型" }, { "start_idx": 45, "end_idx": 49, "type": "pro", "entity": "细胞形态学" }, { "start_idx": 51, "end_idx": 56, "type": "pro", "entity": "组织化学染色" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "免疫表型" } ] }, { "text": "90%的AML病人中至少表达MPO、CD33、CD13、CD15、CD11b和CD36中的一个。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "AML" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "MPO" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "CD33" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "CD13" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "CD15" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "CD11" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "CD36" } ] }, { "text": "有些髓系抗原也表达于幼稚淋巴细胞上,因此也不能仅根据免疫表型来作诊断,4%~25%的ALL可表达至少1个髓系抗原,11%~28%的AML同时表达淋巴系抗原。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "髓系抗原" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "幼稚淋巴细胞" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "免疫表型" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "ALL" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "1个髓系抗原" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "AML" }, { "start_idx": 72, "end_idx": 76, "type": "bod", "entity": "淋巴系抗原" } ] }, { "text": "(三)细胞遗传学AML中常见的并有特征性的染色体异常有t(8;21)、t(3;21),常见于M2;t(15;17)、t(11;17),常见于M3;inv(16)常见于M4Eo。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "细胞遗传学" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "AML" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "染色体异常" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "染色体" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "t(8;21)" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "t(3;21)" }, { "start_idx": 46, "end_idx": 53, "type": "dis", "entity": "M2" }, { "start_idx": 55, "end_idx": 62, "type": "sym", "entity": "t(15;17)" }, { "start_idx": 64, "end_idx": 71, "type": "sym", "entity": "t(11;17)" }, { "start_idx": 76, "end_idx": 83, "type": "dis", "entity": "M3" }, { "start_idx": 85, "end_idx": 91, "type": "sym", "entity": "inv(16)" }, { "start_idx": 95, "end_idx": 104, "type": "dis", "entity": "M4Eo" } ] }, { "text": "【预后】较为公认的影响AML预后不良因素有以下几点:1.起病时白细胞总数>100×109/L。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "AML" }, { "start_idx": 31, "end_idx": 51, "type": "sym", "entity": "白细胞总数>100×109/L" }, { "start_idx": 31, "end_idx": 35, "type": "ite", "entity": "白细胞总数" } ] }, { "text": "2.AML为第二肿瘤。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "AML" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肿瘤" } ] }, { "text": "3.细胞遗传学示单倍体7,及由MDS转化而来的AML。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "单倍体7" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "MDS" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "AML" } ] }, { "text": "除此以外,巨脾、M4或M5、一个疗程未获缓解也被认为是影响预后的不良因素。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "巨脾" } ] }, { "text": "【治疗】AML治疗原则是有效药物联合强化疗、注意诱导缓解及继续治疗中的药物剂量强度和给药时间强度,小剂量长期维持治疗对AML的无病生存率无影响。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "AML" }, { "start_idx": 12, "end_idx": 20, "type": "pro", "entity": "有效药物联合强化疗" }, { "start_idx": 49, "end_idx": 57, "type": "pro", "entity": "小剂量长期维持治疗" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "AML" } ] }, { "text": "(一)诱导治疗国际常用的诱导治疗方案为DA方案,即柔红霉素40mg/m2,qd×3天;阿糖胞苷(Ara-c)每次75~100mg,q12h×7天。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "诱导治疗" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "诱导治疗" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "DA方案" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "柔红霉素" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "阿糖胞苷" }, { "start_idx": 48, "end_idx": 52, "type": "dru", "entity": "Ara-c" } ] }, { "text": "我国特有的三尖杉酯碱对儿童AML也有效,与Ara-C联合为HA方案,即(H)三尖杉酯碱4mg/m2,qd×9天;Ara-C每次75~100mg,q12h×7天,其诱导缓解率与DA方案相似。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "三尖杉酯碱" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "儿童AML" }, { "start_idx": 20, "end_idx": 27, "type": "pro", "entity": "与Ara-C联合" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "HA方案" }, { "start_idx": 36, "end_idx": 36, "type": "dru", "entity": "H" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "三尖杉酯碱" }, { "start_idx": 56, "end_idx": 60, "type": "dru", "entity": "Ara-C" }, { "start_idx": 87, "end_idx": 90, "type": "pro", "entity": "DA方案" } ] }, { "text": "(二)继续化疗AML在缓解后可再继续用原方案1~2疗程作为巩固治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "继续化疗" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "AML" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "巩固治疗" } ] }, { "text": "从目前进展来说,包含大剂量Ara-C的方案可改善AML的预后。", "entities": [ { "start_idx": 8, "end_idx": 20, "type": "pro", "entity": "包含大剂量Ara-C的方案" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "AML" } ] }, { "text": "常用方法为Ara-C每次2~3g/m2,q12h×6次;联合柔红霉素40mg/m2×2天或VP-16160mg/m2×2天,每2~3周1疗程,在巩固治疗之后连续3个疗程作为强化治疗。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "Ara-C" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "柔红霉素" }, { "start_idx": 45, "end_idx": 49, "type": "dru", "entity": "VP-16" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "巩固治疗" }, { "start_idx": 86, "end_idx": 89, "type": "pro", "entity": "强化治疗" } ] }, { "text": "与AML不同,ALL需较长期的低剂量维持化疗,但在AML中需连续的骨髓抑制性化疗,持续化疗至12个月左右即可停药,延长化疗时间并不改善预后,持续化疗方案可用HA、DA、大剂量Ara-C交替,注意整个治疗期蒽环类抗肿瘤类药物累积剂量,一般控制在350mg/m2以下。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "AML" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "ALL" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "化疗" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "AML" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "骨髓抑制性化疗" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "化疗" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "化疗" }, { "start_idx": 70, "end_idx": 75, "type": "pro", "entity": "持续化疗方案" }, { "start_idx": 78, "end_idx": 93, "type": "pro", "entity": "HA、DA、大剂量Ara-C交替" }, { "start_idx": 102, "end_idx": 109, "type": "dru", "entity": "蒽环类抗肿瘤类药" } ] }, { "text": "图11-1AML治疗计划○HA或DA;◎大剂量Ara-c+柔红霉素;●大剂量Ara-c+VP-16;◇HA|鞘注化疗Ara-c1mg/kg,MTX12.5mg/m2(最大12.5mg),DX5mg;A组包括M1、M2、M3、M6、M7,B组为M4、M5全反式维A酸对M3有效,可诱导其分化成熟,约60%~70%病人在单用维A酸后可获得缓解,但如不加用化疗,仍复发。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "AML" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "○HA" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "DA" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "Ara-c" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "柔红霉素" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "Ara-c" }, { "start_idx": 44, "end_idx": 48, "type": "dru", "entity": "VP-16" }, { "start_idx": 51, "end_idx": 53, "type": "pro", "entity": "HA|" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "鞘注化疗" }, { "start_idx": 58, "end_idx": 62, "type": "dru", "entity": "Ara-c" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "MTX" }, { "start_idx": 99, "end_idx": 100, "type": "dru", "entity": "DX" }, { "start_idx": 132, "end_idx": 137, "type": "dru", "entity": "全反式维A酸" }, { "start_idx": 172, "end_idx": 174, "type": "dru", "entity": "维A酸" }, { "start_idx": 187, "end_idx": 188, "type": "pro", "entity": "化疗" } ] }, { "text": "目前主张对M3病人用3~10天维A酸后起用AML诱导化疗,并按AML继续化疗。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "维A酸" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "AML" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "化疗" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "AML" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "化疗" } ] }, { "text": "维A酸的应用可明显减少化疗诱导的DIC发生率,化疗联合维A酸的治疗方式对无病生存率的影响优于单用化疗,维A酸在缓解后间断给药,如每2~3个月给7~14天,化疗仍按AML进行。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "维A酸" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "化疗" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "DIC" }, { "start_idx": 23, "end_idx": 32, "type": "pro", "entity": "化疗联合维A酸的治疗" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "化疗" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "维A酸" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "间断给药" }, { "start_idx": 77, "end_idx": 78, "type": "pro", "entity": "化疗" }, { "start_idx": 81, "end_idx": 83, "type": "dis", "entity": "AML" } ] }, { "text": "(三)中枢神经系统浸润预防对M4、M5需与ALL相仿的鞘内化疗作中枢神经系统浸润预防,用法见图11-11中所示。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "中枢神经系统浸润预防" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "ALL" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "鞘内化疗" }, { "start_idx": 44, "end_idx": 53, "type": "pro", "entity": "中枢神经系统浸润预防" } ] }, { "text": "对其他AML是否需预防尚有争论。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "AML" } ] }, { "text": "(四)骨髓移植AML预后较ALL明显差,特别是有高危因素者更差,因此对这部分病人如有HLA相合的相关家属供体应考虑作异体基因基因造血干细胞移植。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "AML" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "ALL" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "HLA" }, { "start_idx": 58, "end_idx": 70, "type": "pro", "entity": "异体基因基因造血干细胞移植" } ] }, { "text": "自身造血干细胞移植是否有效尚有争论,多数报告为与常规化疗者无差异。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "自身造血干细胞移植" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "化疗" } ] }, { "text": "(五)复发与再治AML复发后再治困难,尤其是治疗中复发者,如能获得再次缓解,应争取在短期内作异体基因造血干细胞移植,因再次缓解时间常短暂。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "AML" }, { "start_idx": 46, "end_idx": 56, "type": "pro", "entity": "异体基因造血干细胞移植" } ] }, { "text": "第五章呼吸系统疾病的常用治疗方法第一节抗生素治疗针对细菌感染的抗生素治疗是20世纪医学最重要的发展,它极大地降低了感染性疾病的死亡率;然而随着滥用抗生素出现的大量耐药菌株,以及缺少新型有效的抗生素,使得临床医生在处理一些严重细菌感染疾病时,感到力不从心。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "呼吸系统疾病" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "细菌感染" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 74, "end_idx": 76, "type": "dru", "entity": "抗生素" }, { "start_idx": 82, "end_idx": 85, "type": "mic", "entity": "耐药菌株" }, { "start_idx": 96, "end_idx": 98, "type": "dru", "entity": "抗生素" }, { "start_idx": 113, "end_idx": 118, "type": "dis", "entity": "细菌感染疾病" } ] }, { "text": "合理有效地使用抗生素是阻止耐药菌株产生的最有效手段。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "抗生素" }, { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "耐药菌株" } ] }, { "text": "正确选择合适抗生素治疗呼吸道感染包括以下几个方面:一、根据临床表现,初步判断与疾病有关最常见的病原种类(一)临床和影像学特点1.急性发病、高热、寒战、胸膜疼痛、肺叶实变是肺炎链球菌的特点。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "急性发病" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "高热" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "寒战" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "胸膜疼痛" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "胸膜" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "肺叶实变" }, { "start_idx": 85, "end_idx": 89, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "2.金黄色葡萄球菌、肠道革兰阴性杆菌(EGNB)、厌氧菌、真菌和分枝杆菌常引起肺组织空洞,而肺炎链球菌、肺炎支原体、肺炎衣原体很少有空洞形成。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 10, "end_idx": 17, "type": "mic", "entity": "肠道革兰阴性杆菌" }, { "start_idx": 19, "end_idx": 22, "type": "mic", "entity": "EGNB" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 29, "end_idx": 30, "type": "mic", "entity": "真菌" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "分枝杆菌" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "肺组织空洞" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肺组织" }, { "start_idx": 46, "end_idx": 50, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 52, "end_idx": 56, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 58, "end_idx": 62, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "少有空洞" } ] }, { "text": "3.吸入性肺炎或肺脓疡,常常是厌氧菌和需氧菌的混合感染。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 15, "end_idx": 26, "type": "dis", "entity": "厌氧菌和需氧菌的混合感染" } ] }, { "text": "4.脓胸、脓气胸及肺大疱是金黄色葡萄球菌感染比较常见的表现之一。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "脓胸" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "脓气胸" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肺大疱" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "金黄色葡萄球菌感染" } ] }, { "text": "(二)当地或医院内的病原学监测资料由于取材、使用抗生素及培养各个环节的原因,个例肺炎的病原检出率较低,仅为30%~60%,加上检查结果的滞后性,在不能准确或及时获知病原菌的情况下,可以参照当地或医院内的病原学监测资料,判断感染的可能病原菌和耐药性,在临床经验性抗感染治疗中非常重要。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "病原学监测" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "抗生素" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "肺炎" }, { "start_idx": 82, "end_idx": 84, "type": "mic", "entity": "病原菌" }, { "start_idx": 101, "end_idx": 105, "type": "pro", "entity": "病原学监测" }, { "start_idx": 111, "end_idx": 112, "type": "dis", "entity": "感染" }, { "start_idx": 116, "end_idx": 118, "type": "mic", "entity": "病原菌" }, { "start_idx": 127, "end_idx": 134, "type": "pro", "entity": "经验性抗感染治疗" } ] }, { "text": "(三)患儿的年龄新生儿感染性肺炎:产前和产时感染性肺炎病原菌常见有肠科杆菌(包括大肠杆菌和克雷伯杆菌)、B族链球菌(GBS)和李斯特菌;巨细胞病毒、风疹病毒、梅毒螺旋体、沙眼衣原体、弓形虫等也可以引起产前和产时感染性肺炎,但临床表现较晚。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "新生儿感染性肺炎" }, { "start_idx": 17, "end_idx": 26, "type": "dis", "entity": "产前和产时感染性肺炎" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "病原菌" }, { "start_idx": 33, "end_idx": 36, "type": "mic", "entity": "肠科杆菌" }, { "start_idx": 40, "end_idx": 43, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 45, "end_idx": 49, "type": "mic", "entity": "克雷伯杆菌" }, { "start_idx": 52, "end_idx": 56, "type": "mic", "entity": "B族链球菌" }, { "start_idx": 58, "end_idx": 60, "type": "mic", "entity": "GBS" }, { "start_idx": 63, "end_idx": 66, "type": "mic", "entity": "李斯特菌" }, { "start_idx": 68, "end_idx": 72, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 74, "end_idx": 77, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 79, "end_idx": 83, "type": "mic", "entity": "梅毒螺旋体" }, { "start_idx": 85, "end_idx": 89, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 91, "end_idx": 93, "type": "mic", "entity": "弓形虫" }, { "start_idx": 100, "end_idx": 109, "type": "dis", "entity": "产前和产时感染性肺炎" } ] }, { "text": "产后感染性肺炎病原菌常见有金黄色葡萄球菌、大肠杆菌、变形杆菌、肺炎克雷伯杆菌、假单胞杆菌属、肺炎链球菌、流感嗜血杆菌等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "产后感染性肺炎" }, { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "病原菌" }, { "start_idx": 13, "end_idx": 19, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 21, "end_idx": 24, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 26, "end_idx": 29, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 31, "end_idx": 37, "type": "mic", "entity": "肺炎克雷伯杆菌" }, { "start_idx": 39, "end_idx": 44, "type": "mic", "entity": "假单胞杆菌属" }, { "start_idx": 46, "end_idx": 50, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 52, "end_idx": 57, "type": "mic", "entity": "流感嗜血杆菌" } ] }, { "text": "婴幼儿肺炎:多见肺炎链球菌感染,较重的肺炎(如有肺大疱或胸腔积液)多为金黄色葡萄球菌、A组链球菌、流感嗜血杆菌或其他革兰阴性杆菌感染所致。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "婴幼儿肺炎" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "肺炎链球菌感染" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "肺炎" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "肺大疱" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "胸腔积液" }, { "start_idx": 35, "end_idx": 41, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 43, "end_idx": 47, "type": "mic", "entity": "A组链球菌" }, { "start_idx": 49, "end_idx": 54, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 58, "end_idx": 63, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "感染" } ] }, { "text": "儿童肺炎:多见肺炎支原体、链球菌及肺炎衣原体感染。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "儿童肺炎" }, { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "链球菌" }, { "start_idx": 17, "end_idx": 21, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "感染" } ] }, { "text": "(四)根据患儿感染来源1.社区获得性肺炎(communityacquiredpneumonia,CAP)是指无明显免疫抑制的患儿在医院外或住院48小时内发生的肺炎,病毒、细菌、支原体、衣原体是CAP的主要病原;病毒感染在肺炎初始阶段居重要地位;仅10%~15%由细菌引起,而且以肺炎链球菌、流感嗜血杆菌、卡他莫拉菌和甲氧西林敏感的金黄色葡萄球菌为主;在5岁以上的儿童肺炎支原体和肺炎衣原体感染明显增加。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "感染" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "社区获得性肺炎" }, { "start_idx": 21, "end_idx": 46, "type": "dis", "entity": "communityacquiredpneumonia" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "CAP" }, { "start_idx": 69, "end_idx": 70, "type": "pro", "entity": "住院" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "肺炎" }, { "start_idx": 82, "end_idx": 83, "type": "mic", "entity": "病毒" }, { "start_idx": 85, "end_idx": 86, "type": "mic", "entity": "细菌" }, { "start_idx": 88, "end_idx": 90, "type": "mic", "entity": "支原体" }, { "start_idx": 92, "end_idx": 94, "type": "mic", "entity": "衣原体" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "CAP" }, { "start_idx": 105, "end_idx": 108, "type": "dis", "entity": "病毒感染" }, { "start_idx": 110, "end_idx": 111, "type": "dis", "entity": "肺炎" }, { "start_idx": 131, "end_idx": 132, "type": "mic", "entity": "细菌" }, { "start_idx": 139, "end_idx": 143, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 145, "end_idx": 150, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 152, "end_idx": 156, "type": "mic", "entity": "卡他莫拉菌" }, { "start_idx": 158, "end_idx": 161, "type": "dru", "entity": "甲氧西林" }, { "start_idx": 165, "end_idx": 171, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 183, "end_idx": 187, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 189, "end_idx": 193, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 194, "end_idx": 195, "type": "dis", "entity": "感染" } ] }, { "text": "2.院内获得性肺炎(hospitalacquiredpneumonia,HAP)指住院48小时后发生的肺炎,HAP病原除了CAP的常见病原外,还应考虑:①原寄生在患儿口腔或肠道的细菌,多为革兰阴性杆菌,常见肠科杆菌,如肺炎克雷伯杆菌、大肠埃希菌、肠杆菌、沙雷菌、变形杆菌和枸橼酸杆菌等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "院内获得性肺炎" }, { "start_idx": 10, "end_idx": 34, "type": "dis", "entity": "hospitalacquiredpneumonia" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "HAP" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "住院" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肺炎" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "HAP" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "CAP" }, { "start_idx": 77, "end_idx": 90, "type": "mic", "entity": "原寄生在患儿口腔或肠道的细菌" }, { "start_idx": 94, "end_idx": 99, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 103, "end_idx": 106, "type": "mic", "entity": "肠科杆菌" }, { "start_idx": 109, "end_idx": 115, "type": "mic", "entity": "肺炎克雷伯杆菌" }, { "start_idx": 117, "end_idx": 121, "type": "mic", "entity": "大肠埃希菌" }, { "start_idx": 123, "end_idx": 125, "type": "mic", "entity": "肠杆菌" }, { "start_idx": 127, "end_idx": 129, "type": "mic", "entity": "沙雷菌" }, { "start_idx": 131, "end_idx": 134, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 136, "end_idx": 140, "type": "mic", "entity": "枸橼酸杆菌" } ] }, { "text": "这些细菌可以通过产生染色体或质粒介导的AmpC酶或质粒介导的ESBLs,对大多数β-内酰胺类抗生素耐药(表8-8)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "细菌" }, { "start_idx": 10, "end_idx": 23, "type": "mic", "entity": "染色体或质粒介导的AmpC酶" }, { "start_idx": 25, "end_idx": 34, "type": "mic", "entity": "质粒介导的ESBLs" }, { "start_idx": 40, "end_idx": 48, "type": "dru", "entity": "β-内酰胺类抗生素" } ] }, { "text": "此外,铜绿假单胞杆菌、不动杆菌属、嗜麦芽窄食单胞菌等细菌也是常见的HAP病原菌,这些病原菌除了产ESBLs、AmpC酶和碳青霉烯酶外,还通过改变细胞壁通透性、外膜蛋白D2丢失、抗生素作用靶位改变及主动外泵机制等途径对多种抗生素产生耐药。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "mic", "entity": "铜绿假单胞杆菌" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "不动杆菌属" }, { "start_idx": 17, "end_idx": 24, "type": "mic", "entity": "嗜麦芽窄食单胞菌" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "细菌" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "HAP" }, { "start_idx": 36, "end_idx": 38, "type": "mic", "entity": "病原菌" }, { "start_idx": 42, "end_idx": 44, "type": "mic", "entity": "病原菌" }, { "start_idx": 48, "end_idx": 52, "type": "mic", "entity": "ESBLs" }, { "start_idx": 54, "end_idx": 58, "type": "mic", "entity": "AmpC酶" }, { "start_idx": 60, "end_idx": 64, "type": "mic", "entity": "碳青霉烯酶" }, { "start_idx": 72, "end_idx": 74, "type": "mic", "entity": "细胞壁" }, { "start_idx": 79, "end_idx": 84, "type": "mic", "entity": "外膜蛋白D2" }, { "start_idx": 88, "end_idx": 90, "type": "dru", "entity": "抗生素" }, { "start_idx": 110, "end_idx": 112, "type": "dru", "entity": "抗生素" } ] }, { "text": "②甲氧西林耐药的金黄色葡萄球菌(MRSA)、甲氧西林耐药的表皮葡萄球菌(MRSE),除了产β-内酰胺酶外,还通过PBP的变异,不仅对甲氧西林耐药,而且对所有β-内酰胺类及红霉素、氨基糖苷类、四环素、克林霉素耐药;常常与铜绿假单胞杆菌或肠道革兰阴性杆菌(EGNB)混合感染。", "entities": [ { "start_idx": 1, "end_idx": 14, "type": "mic", "entity": "甲氧西林耐药的金黄色葡萄球菌" }, { "start_idx": 16, "end_idx": 19, "type": "mic", "entity": "MRSA" }, { "start_idx": 22, "end_idx": 34, "type": "mic", "entity": "甲氧西林耐药的表皮葡萄球菌" }, { "start_idx": 36, "end_idx": 39, "type": "mic", "entity": "MRSE" }, { "start_idx": 44, "end_idx": 50, "type": "mic", "entity": "产β-内酰胺酶" }, { "start_idx": 56, "end_idx": 58, "type": "mic", "entity": "PBP" }, { "start_idx": 66, "end_idx": 69, "type": "dru", "entity": "甲氧西林" }, { "start_idx": 78, "end_idx": 83, "type": "dru", "entity": "β-内酰胺类" }, { "start_idx": 85, "end_idx": 87, "type": "dru", "entity": "红霉素" }, { "start_idx": 89, "end_idx": 93, "type": "dru", "entity": "氨基糖苷类" }, { "start_idx": 95, "end_idx": 97, "type": "dru", "entity": "四环素" }, { "start_idx": 99, "end_idx": 102, "type": "dru", "entity": "克林霉素" }, { "start_idx": 109, "end_idx": 115, "type": "mic", "entity": "铜绿假单胞杆菌" }, { "start_idx": 117, "end_idx": 124, "type": "mic", "entity": "肠道革兰阴性杆菌" }, { "start_idx": 126, "end_idx": 129, "type": "mic", "entity": "EGNB" }, { "start_idx": 131, "end_idx": 134, "type": "dis", "entity": "混合感染" } ] }, { "text": "③厌氧菌,包括脆弱拟杆菌属,是吸入性肺炎和肺脓疡的主要病原菌;多数与需氧的革兰阳性菌(见于CAP)或需氧的EGNB(见于HAP)混合感染。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 7, "end_idx": 12, "type": "mic", "entity": "脆弱拟杆菌属" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "病原菌" }, { "start_idx": 34, "end_idx": 41, "type": "mic", "entity": "需氧的革兰阳性菌" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "CAP" }, { "start_idx": 50, "end_idx": 56, "type": "mic", "entity": "需氧的EGNB" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "HAP" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "混合感染" } ] }, { "text": "90%的脆弱拟杆菌属对青霉素G和氨苄西林耐药。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "mic", "entity": "脆弱拟杆菌属" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "青霉素G" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "④真菌,以白色念株菌为主;为大量使用抗生素或免疫功能异常造成的两重感染。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "真菌" }, { "start_idx": 5, "end_idx": 9, "type": "mic", "entity": "白色念株菌" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "抗生素" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "免疫功能异常" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "两重感染" } ] }, { "text": "⑤卡氏肺孢子虫病,常见于免疫功能异常或服用免疫抑制剂的患儿。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "卡氏肺孢子虫病" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "免疫功能异常" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "表8-7产AmpC酶ESBLs的革兰阴性杆菌对各种β内酰胺类的敏感性*包括克拉维酸、舒巴坦和他唑巴坦(五)根据患儿免疫状态以及基础疾病免疫抑制状态患儿包括原发性和继发性免疫功能低下(如长期使用糖皮质激素和免疫抑制药物、营养不良)或获得性免疫缺陷(AIDS)。", "entities": [ { "start_idx": 4, "end_idx": 21, "type": "mic", "entity": "产AmpC酶ESBLs的革兰阴性杆菌" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "β内酰胺类" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "克拉维酸" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "舒巴坦" }, { "start_idx": 57, "end_idx": 60, "type": "dru", "entity": "他唑巴坦" }, { "start_idx": 88, "end_idx": 100, "type": "dis", "entity": "原发性和继发性免疫功能低下" }, { "start_idx": 107, "end_idx": 111, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 113, "end_idx": 118, "type": "dru", "entity": "免疫抑制药物" }, { "start_idx": 120, "end_idx": 123, "type": "dis", "entity": "营养不良" }, { "start_idx": 126, "end_idx": 132, "type": "dis", "entity": "获得性免疫缺陷" }, { "start_idx": 134, "end_idx": 137, "type": "dis", "entity": "AIDS" } ] }, { "text": "所患肺炎的病原除了常见病原外,还可有一些少见病原感染,如弓形虫、卡氏肺孢子虫、真菌、G-肠道细菌、厌氧菌、努卡菌、军团菌及病毒(单纯疱疹病毒、巨细胞病毒和水痘带状疱疹病毒)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肺炎" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "病原感染" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "弓形虫" }, { "start_idx": 32, "end_idx": 37, "type": "mic", "entity": "卡氏肺孢子虫" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "真菌" }, { "start_idx": 42, "end_idx": 58, "type": "mic", "entity": "G-肠道细菌" }, { "start_idx": 60, "end_idx": 62, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 64, "end_idx": 66, "type": "mic", "entity": "努卡菌" }, { "start_idx": 68, "end_idx": 70, "type": "mic", "entity": "军团菌" }, { "start_idx": 72, "end_idx": 73, "type": "mic", "entity": "病毒" }, { "start_idx": 75, "end_idx": 80, "type": "mic", "entity": "单纯疱疹病毒" }, { "start_idx": 82, "end_idx": 86, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 88, "end_idx": 95, "type": "mic", "entity": "水痘带状疱疹病毒" } ] }, { "text": "HAP的一个重要发病原因是内源性口咽部吸入气道和肺泡组织,一些基础疾病以及造成胃pH上升的药物,可以造成患儿口咽部和气管内定植的EGNB显著增加,使得局部防御能力低下的病重患儿(气管插管、机械通气和昏迷患儿)容易吸入而造成下呼吸道感染。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "HAP" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "口咽部" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "气道" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "肺泡组织" }, { "start_idx": 37, "end_idx": 46, "type": "dru", "entity": "造成胃pH上升的药物" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "口咽部" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "气管内" }, { "start_idx": 64, "end_idx": 67, "type": "mic", "entity": "EGNB" }, { "start_idx": 75, "end_idx": 82, "type": "sym", "entity": "局部防御能力低下" }, { "start_idx": 89, "end_idx": 92, "type": "pro", "entity": "气管插管" }, { "start_idx": 94, "end_idx": 97, "type": "pro", "entity": "机械通气" }, { "start_idx": 99, "end_idx": 100, "type": "sym", "entity": "昏迷" }, { "start_idx": 111, "end_idx": 116, "type": "dis", "entity": "下呼吸道感染" } ] }, { "text": "二、临床表现(一)脑出血系指脑实质内血管破裂所致的出血。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "脑出血" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "脑实质内血管破裂" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "脑实质内血管" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "出血" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "血" } ] }, { "text": "常见于大脑半球,幕下脑出血(小脑或脑干)较少见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "大脑半球" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "幕下脑出血" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "小脑" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脑干" } ] }, { "text": "发病前可有外伤以及过度兴奋等诱因。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "外伤" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "过度兴奋" } ] }, { "text": "重症患儿一般均有明显的生命体征的改变,并易伴发消化道出血、心肺功能异常以及水电解质紊乱,特别严重者可伴发脑疝死亡。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "明显的生命体征的改变" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "消化道出血" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "心肺功能异常" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "水电解质紊乱" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "脑疝" } ] }, { "text": "(二)原发性蛛网膜下腔出血原发性蛛网膜下腔出血是指非外伤性原因所致的颅底或脑表面血管破裂,大量血液直接流入蛛网膜下腔;而继发性者是由于脑出血后,血流穿破脑组织而蔓延至脑室及蛛网膜下腔所致。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "原发性蛛网膜下腔出血" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "原发性蛛网膜下腔出血" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "外伤" }, { "start_idx": 34, "end_idx": 43, "type": "sym", "entity": "颅底或脑表面血管破裂" }, { "start_idx": 34, "end_idx": 41, "type": "bod", "entity": "颅底或脑表面血管" }, { "start_idx": 45, "end_idx": 57, "type": "sym", "entity": "大量血液直接流入蛛网膜下腔" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "大量血液" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "脑出血" }, { "start_idx": 72, "end_idx": 90, "type": "sym", "entity": "血流穿破脑组织而蔓延至脑室及蛛网膜下腔" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "血流" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "脑组织" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "脑室" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "蛛网膜下腔" } ] }, { "text": "小儿蛛网膜下腔出血比成人少见。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "小儿蛛网膜下腔出血" } ] }, { "text": "大脑凸面血管破裂所致的蛛网膜下腔出血,若病变部位靠近额叶及颞叶时,常可出现明显的精神症状,可表现为胡言乱语、自言自语、模仿语言和摸空动作等。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "大脑凸面血管破裂" }, { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "大脑凸面血管" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "额叶" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "颞叶" }, { "start_idx": 37, "end_idx": 43, "type": "sym", "entity": "明显的精神症状" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "胡言乱语" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "自言自语" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "模仿语言" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "摸空动作" } ] }, { "text": "可伴发血肿或脑梗死而出现局灶性神经体征,如肢体瘫痪及颅神经异常等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "血肿" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑梗死" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "出现局灶性神经体征" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "肢体瘫痪" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "颅神经异常" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "颅神经" } ] }, { "text": "眼底检查可见玻璃体下出血。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "眼底检查可见玻璃体下出血" }, { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "眼底检查" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "玻璃体下" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" } ] }, { "text": "(三)硬膜下出血婴幼儿多见。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "硬膜下出血" } ] }, { "text": "通常分为小脑幕上和小脑幕下两种类型,前者最常见,多因大脑表面的细小桥静脉撕裂出血所致;后者多由于小脑幕撕裂所致。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "小脑幕上" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "小脑幕下" }, { "start_idx": 26, "end_idx": 39, "type": "sym", "entity": "大脑表面的细小桥静脉撕裂出血" }, { "start_idx": 26, "end_idx": 35, "type": "bod", "entity": "大脑表面的细小桥静脉" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "血" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "小脑幕撕裂" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "小脑幕" } ] }, { "text": "位于大脑半球凸面的硬膜下出血,若出血量很小,可无明显症状;若出血量较大,则可出现颅内压增高、意识障碍、惊厥发作或偏瘫、斜视等局灶体征,甚至继发脑疝导致死亡。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "sym", "entity": "大脑半球凸面的硬膜下出血" }, { "start_idx": 2, "end_idx": 11, "type": "bod", "entity": "大脑半球凸面的硬膜下" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "血" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "出血量很小" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "血" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "出血量较大" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "血" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "意识障碍" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "惊厥发作" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "偏瘫" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "斜视" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "脑疝" } ] }, { "text": "幕下硬膜下血肿通常出血较多,往往迅速出现昏迷、眼球活动障碍、瞳孔不等大且对光反射消失、呼吸不整等脑干受压症状,病情进展极为迅速,多在数小时内呼吸停止而死亡。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "幕下硬膜下血肿通常出血较多" }, { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "幕下硬膜下" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "血" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "昏迷" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "眼球活动障碍" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "眼球" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "瞳孔不等大" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "瞳孔" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "光反射消失" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "呼吸不整" }, { "start_idx": 48, "end_idx": 53, "type": "sym", "entity": "脑干受压症状" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "脑干" }, { "start_idx": 64, "end_idx": 73, "type": "sym", "entity": "多在数小时内呼吸停止" } ] }, { "text": "(四)NICH主要包括脑室周围-脑室内出血、小脑出血、原发性蛛网膜下腔出血和硬膜下出血四种类型。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "NICH" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "脑室周围-脑室内出血" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "小脑出血" }, { "start_idx": 27, "end_idx": 36, "type": "dis", "entity": "原发性蛛网膜下腔出血" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "硬膜下出血" } ] }, { "text": "小脑出血可因压迫脑干而出现四肢瘫痪、呼吸浅表以及反复窒息发作等,均于病后36小时内死亡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "小脑出血" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "压迫脑干" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "脑干" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "四肢瘫痪" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "呼吸浅表" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "反复窒息发作" } ] }, { "text": "新生儿蛛网膜下腔出血临床表现与出血量有关,轻微出血时可无任何症状与体征,仅有血性脑脊液,常见于早产儿;出血较多时,常于生后2~3天出现嗜睡和惊厥,可致出血后脑积水,多见于足月儿;大量出血较罕见,病情严重,生后不久即死亡。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "新生儿蛛网膜下腔出血" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "出血" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "轻微出血" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "血" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "血性脑脊液" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "血" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "脑脊液" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "早产儿" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "出血较多" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "血" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "嗜睡" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "惊厥" }, { "start_idx": 75, "end_idx": 80, "type": "dis", "entity": "出血后脑积水" }, { "start_idx": 89, "end_idx": 92, "type": "sym", "entity": "大量出血" }, { "start_idx": 92, "end_idx": 92, "type": "bod", "entity": "血" } ] }, { "text": "新生儿硬膜下出血临床表现与前面所谈到的硬膜下出血相类似。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "新生儿硬膜下出血" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "硬膜下出血" } ] }, { "text": "第三节肺结核病儿童时期肺结核(pulmonarytuberculosis)主要是结核杆菌第一次侵入人体而致病,因此主要是原发性感染及其演变。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肺结核病" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肺结核" }, { "start_idx": 15, "end_idx": 35, "type": "dis", "entity": "pulmonarytuberculosis" }, { "start_idx": 40, "end_idx": 43, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "原发性感染" } ] }, { "text": "在原发感染经过一段静止时期甚至临床痊愈后,再发生活动性肺结核病即属继发性结核。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "原发感染" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "活动性肺结核病" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "继发性结核" } ] }, { "text": "儿童各型肺结核病的临床特点如下:(一)原发型肺结核原发型肺结核是结核杆菌初次侵入肺部后发生的原发感染,包括原发综合征(肺原发病灶、局部淋巴结病变和两者相连的淋巴管炎)及支气管淋巴结结核(图7-7)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "肺结核病" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "原发型肺结核" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "原发型肺结核" }, { "start_idx": 32, "end_idx": 41, "type": "sym", "entity": "结核杆菌初次侵入肺部" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肺部" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "原发感染" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "原发综合征" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "肺原发病灶" }, { "start_idx": 65, "end_idx": 71, "type": "dis", "entity": "局部淋巴结病变" }, { "start_idx": 73, "end_idx": 81, "type": "dis", "entity": "两者相连的淋巴管炎" }, { "start_idx": 84, "end_idx": 91, "type": "dis", "entity": "支气管淋巴结结核" } ] }, { "text": "起病急缓轻重不一,可无症状,于体检时发现;或有低热、盗汗等结核中毒症状;也可有突然高热,2~3周后转为低热。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "体检" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "低热" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "盗汗" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "汗" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "结核中毒" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "突然高热" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "低热" } ] }, { "text": "有结核中毒症状的表现。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "结核中毒症状" }, { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "结核中毒" } ] }, { "text": "有结核过敏症状,如皮肤结节性红斑、疱疹性角膜结膜炎(皮疹表现为细小灰白色或灰黄色的小结节)及过敏性关节炎等。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "结核过敏症状" }, { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "结核" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "皮肤结节性红斑" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "皮肤" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "疱疹性角膜结膜炎" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "皮疹" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "皮" }, { "start_idx": 31, "end_idx": 43, "type": "sym", "entity": "细小灰白色或灰黄色的小结节" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "过敏性关节炎" } ] }, { "text": "胸腔内淋巴结明显肿大压迫症状和体征,压迫迷走神经可出现类似百日咳样咳嗽支气管哮喘压迫喉返神经声音嘶哑;压迫大支气管可出现双音性咳嗽或金属样痉挛性咳嗽,或有喘鸣等气道梗阻的表现;支气管受压严重局限性肺不张儿童原发肺结核的研究表明,各个肺叶受累的危险性是相同的。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "胸腔内淋巴结明显肿大" }, { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "胸腔内淋巴结" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "压迫症状和体征" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "压迫迷走神经" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "迷走神经" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "类似百日咳样咳嗽" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "百日咳" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "压迫喉返神经" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "喉返神经" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "压迫大支气管" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "大支气管" }, { "start_idx": 60, "end_idx": 64, "type": "sym", "entity": "双音性咳嗽" }, { "start_idx": 66, "end_idx": 73, "type": "sym", "entity": "金属样痉挛性咳嗽" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "喘鸣" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "气道梗阻" }, { "start_idx": 88, "end_idx": 94, "type": "sym", "entity": "支气管受压严重" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "支气管" }, { "start_idx": 95, "end_idx": 100, "type": "dis", "entity": "局限性肺不张" }, { "start_idx": 101, "end_idx": 107, "type": "dis", "entity": "儿童原发肺结核" }, { "start_idx": 116, "end_idx": 117, "type": "bod", "entity": "肺叶" } ] }, { "text": "成人复燃的肺结核易侵及肺尖形成空洞,但在儿童原发肺结核没有这个特点。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "sym", "entity": "复燃的肺结核易侵及肺尖形成空洞" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肺结核" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肺尖" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "儿童原发肺结核" } ] }, { "text": "原发综合征典型X线表现是由肺原发病灶、淋巴管炎和肿大淋巴结所组成哑铃状的“双极影”,在急性进展阶段可常见到病变融合成大片状阴影或仅见肺野阴影及同侧肿大淋巴结。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "原发综合征" }, { "start_idx": 5, "end_idx": 40, "type": "sym", "entity": "典型X线表现是由肺原发病灶、淋巴管炎和肿大淋巴结所组成哑铃状的“双极影”" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "X线" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肺原发病灶" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "淋巴管炎" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "肿大淋巴结" }, { "start_idx": 42, "end_idx": 62, "type": "sym", "entity": "在急性进展阶段可常见到病变融合成大片状阴影" }, { "start_idx": 64, "end_idx": 77, "type": "sym", "entity": "仅见肺野阴影及同侧肿大淋巴结" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "肺野" }, { "start_idx": 71, "end_idx": 77, "type": "bod", "entity": "同侧肿大淋巴结" } ] }, { "text": "支气管淋巴结结核X线表现主要有肺门区可见肿大淋巴结的团块状阴影肿大淋巴结及其周围炎融合成片状阴影肺门淋巴结肿大,没有肺部浸润病灶,在儿科患者也很常见,这在侧位胸片上最容易看到。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "支气管淋巴结结核" }, { "start_idx": 8, "end_idx": 30, "type": "sym", "entity": "X线表现主要有肺门区可见肿大淋巴结的团块状阴影" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "X线" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺门区" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "肿大淋巴结" }, { "start_idx": 31, "end_idx": 47, "type": "sym", "entity": "肿大淋巴结及其周围炎融合成片状阴影" }, { "start_idx": 31, "end_idx": 40, "type": "dis", "entity": "肿大淋巴结及其周围炎" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "肺门淋巴结肿大" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "肺门淋巴结" }, { "start_idx": 56, "end_idx": 63, "type": "sym", "entity": "没有肺部浸润病灶" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "肺部" }, { "start_idx": 66, "end_idx": 67, "type": "dep", "entity": "儿科" }, { "start_idx": 77, "end_idx": 80, "type": "pro", "entity": "侧位胸片" } ] }, { "text": "如肿大淋巴结压迫支气管肺气肿或肺不张等改变。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "肿大淋巴结压迫支气管" }, { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "肿大淋巴结" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "支气管" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肺气肿" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肺不张" } ] }, { "text": "图7-1胸X线片:左侧肺门淋巴结肿大,左肺原发灶(二)急性粟粒型肺结核当机体免疫状态发生改变,如急性传染病、营养不良等造成机体抵抗力降低时,有结核菌血症存在则容易诱发急性粟粒型肺结核,且多同时引起脑、脑膜、肺、肝、胃、肠等脏器的病变,发生全身性粟粒结核病。", "entities": [ { "start_idx": 4, "end_idx": 23, "type": "sym", "entity": "胸X线片:左侧肺门淋巴结肿大,左肺原发灶" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "胸X线片" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "左侧肺门淋巴结" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "左肺原发灶" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "急性粟粒型肺结核" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "急性传染病" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "营养不良" }, { "start_idx": 61, "end_idx": 67, "type": "sym", "entity": "机体抵抗力降低" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "结核菌血症" }, { "start_idx": 83, "end_idx": 90, "type": "dis", "entity": "急性粟粒型肺结核" }, { "start_idx": 98, "end_idx": 98, "type": "bod", "entity": "脑" }, { "start_idx": 98, "end_idx": 115, "type": "sym", "entity": "脑、脑膜、肺、肝、胃、肠等脏器的病变" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "脑膜" }, { "start_idx": 103, "end_idx": 103, "type": "bod", "entity": "肺" }, { "start_idx": 105, "end_idx": 105, "type": "bod", "entity": "肝" }, { "start_idx": 107, "end_idx": 107, "type": "bod", "entity": "胃" }, { "start_idx": 109, "end_idx": 109, "type": "bod", "entity": "肠" }, { "start_idx": 111, "end_idx": 112, "type": "bod", "entity": "脏器" }, { "start_idx": 119, "end_idx": 126, "type": "dis", "entity": "全身性粟粒结核病" } ] }, { "text": "患儿大部分急性起病,持续高热,中毒症状重,持续时间长。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "急性起病" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "持续高热" }, { "start_idx": 15, "end_idx": 25, "type": "sym", "entity": "中毒症状重,持续时间长" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "中毒" } ] }, { "text": "肺部体征不多,但咳、喘、发热等呼吸道症状较严重。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 8, "end_idx": 8, "type": "sym", "entity": "咳" }, { "start_idx": 10, "end_idx": 10, "type": "sym", "entity": "喘" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "发热" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "呼吸道症状" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "呼吸道" } ] }, { "text": "多数有肺外结核病变,如体表淋巴结肿大、肝脾肿大、眼底有结核结节等,大多数伴有结核性脑膜炎。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肺外结核病变" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "体表淋巴结肿大" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "体表淋巴结" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肝" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "脾" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "眼底有结核结节" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "眼底" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "结核" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "结核性脑膜炎" } ] }, { "text": "可有血行播散性皮肤结核的表现,如粟粒性结核疹、丘疹坏死性结核疹、硬红斑等。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "血行播散性皮肤结核" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "粟粒性结核疹" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "丘疹坏死性结核疹" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "硬红斑" } ] }, { "text": "胸部X线检查一般在出现症状数周后才见到粟粒阴影,呈两肺对称性均匀一致粟粒状阴影或小点状阴影弥漫、分布均匀的小结节状肺部浸润影大小如小米粒或草籽,直径约1~2mm。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "粟粒阴影" }, { "start_idx": 25, "end_idx": 44, "type": "sym", "entity": "两肺对称性均匀一致粟粒状阴影或小点状阴影" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "两肺" }, { "start_idx": 45, "end_idx": 61, "type": "sym", "entity": "弥漫、分布均匀的小结节状肺部浸润影" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肺部" }, { "start_idx": 62, "end_idx": 79, "type": "sym", "entity": "大小如小米粒或草籽,直径约1~2mm" } ] }, { "text": "但结节的大小可不等,可能小到刚刚能够看见,直径只有1mm,或大到非常明显的6mm的结节(图7-7)。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "结节的大小可不等" }, { "start_idx": 10, "end_idx": 27, "type": "sym", "entity": "可能小到刚刚能够看见,直径只有1mm" }, { "start_idx": 29, "end_idx": 42, "type": "sym", "entity": "或大到非常明显的6mm的结节" } ] }, { "text": "肺部的粟粒结节提示结核杆菌可能已在体内播散,其中包括进入脑膜而形成结核性脑膜炎。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "肺部的粟粒结节" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "结核杆菌可能已在体内播散" }, { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "脑膜" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "结核性脑膜炎" } ] }, { "text": "急性粟粒型肺结核患儿由于免疫力低下,约有1/3的患儿对结核菌素试验不起反应。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "急性粟粒型肺结核" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "免疫力低下" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "对结核菌素试验不起反应" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "结核菌素试验" } ] }, { "text": "(三)继发型肺结核多见于10岁以上的年长儿及青少年,一般是在原发病变痊愈一个时期后,已形成硬结或钙化的原发灶或其播散灶发生内源性复燃,或者外源性再次感染,又发生活动性肺结核。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "继发型肺结核" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "原发病变" }, { "start_idx": 42, "end_idx": 65, "type": "sym", "entity": "已形成硬结或钙化的原发灶或其播散灶发生内源性复燃" }, { "start_idx": 69, "end_idx": 75, "type": "dis", "entity": "外源性再次感染" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "活动性肺结核" } ] }, { "text": "起病可急、可缓,起病急者出现高热,咳嗽、咯血、胸痛等呼吸道症状较明显。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "高热" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "咳嗽" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "咯血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "胸痛" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "胸" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "呼吸道症状" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "呼吸道" } ] }, { "text": "体格检查一般无阳性体征,若病变较大时,可有叩诊浊音,听诊呼吸音低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "叩诊浊音" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "叩诊" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "听诊呼吸音低" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "听诊" } ] }, { "text": "干酪性肺炎者结核中毒症状较严重,肺部可听到支气管呼吸音大量响亮的中小水泡音。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "干酪性肺炎" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "结核中毒症状" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "结核" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "中毒" }, { "start_idx": 16, "end_idx": 26, "type": "sym", "entity": "肺部可听到支气管呼吸音" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺部" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "支气管" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "大量响亮的中小水泡音" } ] }, { "text": "图7-2胸X线片:肺内弥漫分布粟粒性结节X线胸片一般呈圆形或絮状或团块状阴影,多见于肺上部,成人肺结核病常有空洞,在儿童则很罕见,但可能发生在小婴幼儿严重病例和青春期患者。", "entities": [ { "start_idx": 4, "end_idx": 19, "type": "sym", "entity": "胸X线片:肺内弥漫分布粟粒性结节" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "胸X线片" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肺内" }, { "start_idx": 20, "end_idx": 37, "type": "sym", "entity": "X线胸片一般呈圆形或絮状或团块状阴影" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "X线胸片" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "多见于肺上部" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肺上部" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "成人肺结核病" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "常有空洞" } ] }, { "text": "也可见已愈的原发性肺结核的钙化灶。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "原发性肺结核" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "钙化灶" } ] }, { "text": "(四)结核性胸膜炎一般发生在原发结核感染后半年内,多见于3岁以上儿童。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "结核性胸膜炎" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "原发结核感染" } ] }, { "text": "起病多较急,高热,1~3周后转为低热。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "起病多较急" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "高热" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "低热" } ] }, { "text": "除结核中毒症状外,可有胸痛、咳嗽、气促等,胸水多时可有呼吸困难。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "结核中毒症状" }, { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "结核" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "胸痛" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "胸" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "咳嗽" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "气促" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "胸水多时可有呼吸困难" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "胸" } ] }, { "text": "病侧胸廓活动受限,气管和心脏向对侧移位叩诊浊音,呼吸音减弱或消失。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "病侧胸廓活动受限" }, { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "病侧胸廓" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "气管和心脏向对侧移位" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "气管" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心脏" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "叩诊浊音" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "叩诊" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "呼吸音减弱或消失" } ] }, { "text": "X线胸片示胸腔积液。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "X线胸片示胸腔积液" }, { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "X线胸片" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胸腔" } ] }, { "text": "胸水比重一般为1.012~1.025,蛋白2~4g/dl,糖减低,但通常在正常低限(20~40mg/dl),细胞数每立方毫米数百到数千,早期中性多形核粒细胞为主,以后逐渐变为淋巴细胞为主。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "胸水比重一般为1.012~1.025" }, { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "胸水比重" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "蛋白2~4g/dl" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "蛋白" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "糖减低" }, { "start_idx": 29, "end_idx": 29, "type": "ite", "entity": "糖" }, { "start_idx": 34, "end_idx": 52, "type": "sym", "entity": "通常在正常低限(20~40mg/dl)" }, { "start_idx": 54, "end_idx": 66, "type": "sym", "entity": "细胞数每立方毫米数百到数千" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "细胞数" }, { "start_idx": 68, "end_idx": 79, "type": "sym", "entity": "早期中性多形核粒细胞为主" }, { "start_idx": 70, "end_idx": 77, "type": "ite", "entity": "中性多形核粒细胞" }, { "start_idx": 83, "end_idx": 92, "type": "sym", "entity": "逐渐变为淋巴细胞为主" }, { "start_idx": 87, "end_idx": 90, "type": "ite", "entity": "淋巴细胞" } ] }, { "text": "对激素治疗敏感,治愈后很少发生广泛的胸膜增厚。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "激素治疗" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "广泛的胸膜增厚" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胸膜" } ] }, { "text": "参考文献1.林庚金主编.消化病新概念.第11版.上海:上海医科大学出版社,1996:46-462.沈小明,许春娣.幽门螺杆菌感染的临床与病理.实用儿科临床杂志,1999,14(4):203-2033.中华儿科杂志编辑委员会.小儿慢性胃炎、消化性溃疡、胃镜诊断标准(试行).中华儿科杂志,1996,34(5):296-2964.MarionR,BrendanD.ClinicalsignificanclofHelicobacterinfectioninchildren.BritishMediealBulletin,1998,54(1):95-955.PiotrowskiJ,PiotrowskiE,SkrodzkaDetal.InductionofacutegastritisandepithelialapoposisbyHelicobacterpyloriLipopolysaecharideScandJgastroenterol,1997,2(3):203-2036.RibeiroVL,BarbosaAJ.Lymphocyticgastritis:astudyofitsfrequencyandreviewoftheliterature.ArqGastroenterol,1998,35(1):26-267.SipponenP,NaturalcourseofHelicobacterpylorigastricinfection.ItalJGastroenterolHepatol,1998,30(3):S270-2708.SlomianyBL,PiotrowskiJ.SlomonanyA.Inductionofcaspase-3andnitricoxidesynthase-2duringgastricmucosalinflammatoryreactiontoHelicobacterpyloriLipopolysaecharide.BiochenMolBiolInt,1998,46(5):1063-10639.YamasakiK,ArakawaT,TakaishiOetal.Influenceofrebamipideonindometacin-inducedgastrichemorrhageinratsunderrestraintstress.Arzneimforsch/DrugRes,1999,49(4):359-359", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "消化病" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 73, "end_idx": 74, "type": "dep", "entity": "儿科" }, { "start_idx": 102, "end_idx": 103, "type": "dep", "entity": "儿科" }, { "start_idx": 112, "end_idx": 117, "type": "dis", "entity": "小儿慢性胃炎" }, { "start_idx": 119, "end_idx": 123, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 125, "end_idx": 126, "type": "pro", "entity": "胃镜" }, { "start_idx": 138, "end_idx": 139, "type": "dep", "entity": "儿科" } ] }, { "text": "参考文献1.许春娣,奚容平,陈舜年,等.幽门螺杆菌感染的患儿人类白细胞抗原DQA1的免疫遗传学分析.中华儿科杂志,2000,38:746-7462.许春娣,陈舜年,徐家裕,等.儿童幽门螺杆菌感染的流行病学研究.中华儿科杂志,1999,37:318-3183.许春娣,周建元,薛英,等.无症状儿童人群中幽门螺杆菌感染的血清流行病学.中华儿科杂志,1999,37:412-4144.许春娣,苏林,陈舜年,等.幽门螺杆菌感染与小儿胃十二指肠疾病的关系.上海医学,1999,22:466-4685.许春娣,季月华,陈舜年,等.胃幽门螺杆菌感染患儿细胞毒素相关基因A和细胞空泡毒素A与十二指肠疾病.中华儿科杂志,1999,37:552-5526.中华儿科杂志编辑委员会.中华医学会儿科学会感染消化组.小儿慢性胃炎.消化性溃疡诊断治疗推荐方案.中华儿科杂志,2000,38:201-2017.BehrensR,LangT,KellerKM,etal.DualversustripletherapyofHelicobacterpyloriinfection:resultsofamulticentretrial.ArchDisChild,1999,81:688.Ellc,SchoernerC,SolbachW,etal.TheAMORstudy:arandomizeed,doubleblindedtrialofomeprazoleversusranitidinetogetherwithamoxycillinandmetronidazoleforeradicationofHelicobacterpylori.EurJGastroentrolHepatol,2001,13:685-6919.LamSK,TalleyNJ.Reportofthe1997AsiaPacIFicConsensusConferenceonthemanagementofHelicobacterpyloriinfection.JGastroenterolHepatol.1998,13:110.MoshkowitzM,ReIFS,BrillS,etal.Oneweektripletherapywithomeprazole,clarithromycin,andnitroimidazoleforHelicobacterpyloriinfectioninchildrenandadolescents.Pediatrics,1998,102:e1411.PanJJ,VanderHulstRWM,FellerM,etal.EquallyhighprevalenceofinfectionwithCagApositiveHelicobacterpyloriinChinesepatientwithpepticulcerdiseaseandthosewithchronicgastritisassociateddyspepsia.JClinMicrobiol,1997,35:1344-134412.ShashidharH,PetersJ,LinCH,etal.AprospectivetrialofLansoprazoletripletherapyforpediatricHelicobacterpyloriinfection.JPediatricGastroenterolNutr,2000,30:27613.ShamalyH,BerkowitzD,RosenthalE,etal.EfficacyofbismuthbasedtripletherapyinchildrenwithabdominalpainandHelicobacterpylorigastritis.JPediatrGastroenterolNutr,2000,30:19814.TheEuropeanHelicobacterpyloriStudyGroup.CurrentEuropeanconceptsinthemanagementofHelicobacterpyloriinfection.TheMaastrichtConsensusReportGut,1997,41:815.VandenplasY,bleckerU.Helicobacterpyloriinfectioninchildren,ActaPediatr,1998,87:1105-110516.WarburtonVJ,EverettS,MapastoneNP,etal.ClinicalandhistologicalassociationsofcagAandvacAgenotypesinHelicobacterpylorigastritis,JClinPathol,1998,51:55-6117.中华医学会消化病学会幽门螺杆菌科研协作组.第三次全国幽门螺杆菌感染若干问题共识报告(2007.庐山).现代消化及介入诊所,2008,13(1):73-73", "entities": [ { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 30, "end_idx": 40, "type": "bod", "entity": "人类白细胞抗原DQA1" }, { "start_idx": 42, "end_idx": 48, "type": "pro", "entity": "免疫遗传学分析" }, { "start_idx": 52, "end_idx": 53, "type": "dep", "entity": "儿科" }, { "start_idx": 88, "end_idx": 96, "type": "dis", "entity": "儿童幽门螺杆菌感染" }, { "start_idx": 98, "end_idx": 103, "type": "pro", "entity": "流行病学研究" }, { "start_idx": 107, "end_idx": 108, "type": "dep", "entity": "儿科" }, { "start_idx": 150, "end_idx": 156, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 158, "end_idx": 162, "type": "dis", "entity": "血清流行病" }, { "start_idx": 167, "end_idx": 168, "type": "dep", "entity": "儿科" }, { "start_idx": 202, "end_idx": 208, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 210, "end_idx": 218, "type": "dis", "entity": "小儿胃十二指肠疾病" }, { "start_idx": 259, "end_idx": 266, "type": "dis", "entity": "胃幽门螺杆菌感染" }, { "start_idx": 269, "end_idx": 277, "type": "mic", "entity": "细胞毒素相关基因A" }, { "start_idx": 279, "end_idx": 285, "type": "mic", "entity": "细胞空泡毒素A" }, { "start_idx": 287, "end_idx": 292, "type": "dis", "entity": "十二指肠疾病" }, { "start_idx": 296, "end_idx": 297, "type": "dep", "entity": "儿科" }, { "start_idx": 320, "end_idx": 321, "type": "dep", "entity": "儿科" }, { "start_idx": 335, "end_idx": 336, "type": "dep", "entity": "儿科" }, { "start_idx": 339, "end_idx": 340, "type": "dis", "entity": "感染" }, { "start_idx": 345, "end_idx": 350, "type": "dis", "entity": "小儿慢性胃炎" }, { "start_idx": 352, "end_idx": 356, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 368, "end_idx": 369, "type": "dep", "entity": "儿科" }, { "start_idx": 2006, "end_idx": 2008, "type": "dis", "entity": "消化病" }, { "start_idx": 2011, "end_idx": 2015, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 2027, "end_idx": 2033, "type": "dis", "entity": "幽门螺杆菌感染" } ] }, { "text": "药物的吸收和分布受一系列生物膜的阻挡,因此生物膜的转运机制与药物的体内转运密切相关,亦与周围环境有关。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "生物膜" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "生物膜" } ] }, { "text": "生物利用度常用来描述血管外用药后吸收进入体内循环的药量与用药量的比例。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "生物利用度" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "血管外用药" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "体内循环" } ] }, { "text": "可通过计算血管外用药后血药浓度-时间曲线下面积(AUC)与静脉用药后AUC之比,即口服AUC/静脉AUC而得出。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "血管外用药" }, { "start_idx": 11, "end_idx": 22, "type": "ite", "entity": "血药浓度-时间曲线下面积" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "AUC" }, { "start_idx": 29, "end_idx": 36, "type": "ite", "entity": "静脉用药后AUC" }, { "start_idx": 41, "end_idx": 51, "type": "ite", "entity": "口服AUC/静脉AUC" } ] }, { "text": "生物利用度受多种生理、病理因素的影响,例如胃、十二指肠中存在食物可降低口服药物进入体循环的速率,从而推迟药物达到高峰血清浓度的时间,但大多数口服药物的吸收总量一般不影响。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "生物利用度" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "胃" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "十二指肠" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "口服" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "体循环" }, { "start_idx": 56, "end_idx": 61, "type": "ite", "entity": "高峰血清浓度" }, { "start_idx": 70, "end_idx": 71, "type": "pro", "entity": "口服" } ] }, { "text": "六、预防(一)发现感染儿童在所有结核病的控制方案中首先是发现和治疗患者,以切断密切接触传播的感染途径。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "感染" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "结核病" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "发现和治疗患者" }, { "start_idx": 37, "end_idx": 49, "type": "pro", "entity": "切断密切接触传播的感染途径" } ] }, { "text": "对接触过开放性肺结核患者的小儿和成人应立即给予结核菌素试验检查。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "开放性肺结核" }, { "start_idx": 19, "end_idx": 30, "type": "pro", "entity": "立即给予结核菌素试验检查" } ] }, { "text": "家庭中接触开放性结核患者的成员中平均30%~50%结核菌素皮试阳性结核病。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "开放性结核" }, { "start_idx": 25, "end_idx": 35, "type": "dis", "entity": "结核菌素皮试阳性结核病" } ] }, { "text": "(二)接种卡介苗唯一的预防结核病疫苗为卡介苗(BCG),以两名法国研制者Calmette和Guerin命名。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "接种" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "卡介苗" }, { "start_idx": 11, "end_idx": 17, "type": "dru", "entity": "预防结核病疫苗" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "卡介苗" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "BCG" } ] }, { "text": "接种卡介苗的途径和剂量不同,产生的免疫力亦不同。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "接种" }, { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "卡介苗" } ] }, { "text": "以皮内注射为较好的途径。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "皮内注射" } ] }, { "text": "BCG对免疫健全的宿主绝对安全。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "BCG" } ] }, { "text": "约有0.1%~1%的人在接种后发生局部溃疡和局部淋巴结化脓免疫缺陷亦不影响疫苗提供的免疫力。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "接种" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "局部溃疡" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "局部淋巴结化脓" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "局部淋巴结" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 37, "end_idx": 38, "type": "dru", "entity": "疫苗" } ] }, { "text": "局部病变可自行缓解,偶尔需要化学药物治疗,很少需要外科手术切除流脓的淋巴结。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "局部病变" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "化学药物" }, { "start_idx": 25, "end_idx": 36, "type": "pro", "entity": "外科手术切除流脓的淋巴结" } ] }, { "text": "发热、惊厥、食欲缺乏和烦躁是接种BCG后非常罕见的全身表现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "发热" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "惊厥" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "烦躁" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "接种" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "BCG" } ] }, { "text": "明显的免疫损害者接种BCG后可发生播散性卡介苗病。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "明显的免疫损害" }, { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "免疫损害" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "接种" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "BCG" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "播散性卡介苗病" } ] }, { "text": "HIV感染的小儿较免疫力正常儿的局部反应发生率高,然而这些患儿在接种BCG数月至数年后播散感染的发生率目前尚不清楚。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HIV感染" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "局部反应发生率高" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "接种" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "BCG" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "播散感染" } ] }, { "text": "当前,在我国卡介苗接种作为预防儿童结核的一项有效措施,仍应予以高度重视,应继续做好对新生儿(婴幼儿)卡介苗接种的计划免疫工作。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "卡介苗" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "接种" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "结核" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "卡介苗" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "接种" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "计划免疫" } ] }, { "text": "第十二章幼年型皮肌炎【概述】幼年型皮肌炎(juveniledermatomyositis,JDM)是一种多系统疾病,特点是横纹肌和皮肤的急性或慢性的非化脓性炎症,早期存在不同程度的闭塞性血管病,晚期发生钙化。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "幼年型皮肌炎" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "幼年型皮肌炎" }, { "start_idx": 21, "end_idx": 43, "type": "dis", "entity": "juveniledermatomyositis" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "JDM" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "多系统疾病" }, { "start_idx": 61, "end_idx": 79, "type": "sym", "entity": "横纹肌和皮肤的急性或慢性的非化脓性炎症" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "横纹肌" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "皮肤" }, { "start_idx": 74, "end_idx": 79, "type": "dis", "entity": "非化脓性炎症" }, { "start_idx": 90, "end_idx": 95, "type": "dis", "entity": "闭塞性血管病" }, { "start_idx": 101, "end_idx": 102, "type": "sym", "entity": "钙化" } ] }, { "text": "成人皮肌炎中20%由儿童期起病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "成人皮肌炎" } ] }, { "text": "死因主要为呼吸衰竭和胃肠道溃疡、出血。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "胃肠道溃疡、出血" } ] }, { "text": "其发病与感染和免疫功能紊乱有关。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "感染" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "免疫功能紊乱" } ] }, { "text": "感染引起淋巴细胞释放细胞因子等机制损害肌纤维;同时肌肉蛋白变性而具有抗原性,产生的自身抗原抗体反应也可能起一定作用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "感染" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "细胞因子" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "损害肌纤维" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肌纤维" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "肌肉蛋白变性" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "肌肉蛋白" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "自身抗原抗体反应" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "抗原" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "抗体" } ] }, { "text": "一般认为本病为细胞介导的免疫失调所引起的骨骼肌疾病。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "细胞介导的免疫失调" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "细胞" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "骨骼肌疾病" } ] }, { "text": "皮肌炎患儿HLA-B8和DR3明显增加,且与家庭遗传有相关性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 5, "end_idx": 18, "type": "sym", "entity": "HLA-B8和DR3明显增加" }, { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "HLA-B8" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "DR3" } ] }, { "text": "【临床表现】一般为隐匿性起病,1/3急性起病,发热不规则,38~40℃,常诉乏力、不适、关节痛、厌食和体重减轻,易激惹,大运动量活动减低。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "隐匿性起病" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "急性起病" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "发热不规则" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "38~40℃" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "乏力" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "不适" }, { "start_idx": 44, "end_idx": 46, "type": "sym", "entity": "关节痛" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "关节" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "厌食" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "体重减轻" }, { "start_idx": 51, "end_idx": 52, "type": "ite", "entity": "体重" }, { "start_idx": 56, "end_idx": 58, "type": "sym", "entity": "易激惹" }, { "start_idx": 60, "end_idx": 67, "type": "sym", "entity": "大运动量活动减低" } ] }, { "text": "(一)肌肉症状患儿诉轻度肌痛或肌肉僵硬、肌无力,起病时多见于下肢肢带肌,导致不能行走,不能上楼梯,颈前屈肌和背肌无力导致不能抬头和维持坐位。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肌肉症状" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肌肉" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "轻度肌痛" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肌" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "肌肉僵硬" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肌肉" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "肌无力" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "肌" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "多见于下肢肢带肌" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "下肢肢带肌" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "不能行走" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "不能上楼梯" }, { "start_idx": 49, "end_idx": 57, "type": "sym", "entity": "颈前屈肌和背肌无力" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "颈前屈肌" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "背肌" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "不能抬头" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "头" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "维持坐位" } ] }, { "text": "病变肌肉呈对称分布,近端肌明显,如髋、肩、颈屈肌和腹肌;受累肌肉偶呈水肿样,稍硬,轻压痛;肌力减退,患儿不能从卧位站起,不能下蹲或下蹲后不能起立,上下楼梯困难;重症累及肢体远端肌肉,患儿可完全不能动弹。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "病变肌肉" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "病变肌肉呈对称分布" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "近端肌" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "近端肌明显" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "髋" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肩" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "颈屈肌" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "腹肌" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "受累肌肉偶呈水肿样" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肌肉" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "稍硬" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "轻压痛" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "肌力减退" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "肌力" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "从卧位站起" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "不能下蹲" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "下蹲后不能起立" }, { "start_idx": 73, "end_idx": 78, "type": "sym", "entity": "上下楼梯困难" }, { "start_idx": 84, "end_idx": 89, "type": "bod", "entity": "肢体远端肌肉" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "完全不能动弹" } ] }, { "text": "(二)皮肤症状3/4患儿有典型皮肤改变,可为首发症状,亦可在肌肉症状出现数周后才有皮肤病变:1.上眼睑皮肤变为紫红色,伴有水肿;面部弥漫呈紫色或紫红色。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "皮肤症状" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "典型皮肤改变" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肌肉" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "肌肉症状" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "皮肤病变" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "上眼睑皮肤" }, { "start_idx": 48, "end_idx": 57, "type": "sym", "entity": "上眼睑皮肤变为紫红色" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "水肿" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "面部" }, { "start_idx": 64, "end_idx": 74, "type": "sym", "entity": "面部弥漫呈紫色或紫红色" } ] }, { "text": "颈部和上胸部“V”字区、躯干部及四肢伸侧等处可出现弥漫性或局限性暗红色斑。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "颈部" }, { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "上胸部“V”字区" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "躯干部" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "四肢伸侧" }, { "start_idx": 25, "end_idx": 35, "type": "sym", "entity": "弥漫性或局限性暗红色斑" } ] }, { "text": "2.Gottron征,关节伸侧对称性变化,为有光泽的红斑样萎缩性鳞片状斑。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "Gottron征" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "关节伸侧对称性变化" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "关节伸侧" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "有光泽的红斑样萎缩性鳞片状斑" } ] }, { "text": "皮肤萎缩区呈淡粉红色,早期皮肤增厚呈白色,Gottron征常见于近端指间关节,其次掌指关节及远端指间关节;脚趾罕见;肘、膝、踝关节伸侧亦可累及。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "皮肤萎缩区呈淡粉红色" }, { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "皮肤萎缩区" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "皮肤增厚呈白色" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮肤" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "Gottron征" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "近端指间关节" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "掌指关节" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "远端指间关节" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "脚趾" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "肘" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "膝" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "踝关节伸侧" } ] }, { "text": "3.甲皱皮肤明显发红,甲皱毛细血管扩张,毛细血管增厚、弯曲和中断。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "甲皱皮肤明显发红" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "甲皱皮肤" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "甲皱毛细血管扩张" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "甲皱毛细血管" }, { "start_idx": 20, "end_idx": 31, "type": "sym", "entity": "毛细血管增厚、弯曲和中断" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "毛细血管" } ] }, { "text": "4.晚期可产生皮下钙化和皮肤溃疡,从破溃处排出白色钙盐。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "皮下" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "皮下钙化" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "皮肤溃疡" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "破溃处排出白色钙盐" } ] }, { "text": "(三)消化道症状有口咽部溃疡、全腹痛、黑粪,偶有呕血,示消化道黏膜弥漫性出血性炎症或急性肠系膜动脉栓塞;胃肠道穿孔时膈下有游离气体;腹胀时应疑及麻痹性肠梗阻。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "消化道症状" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "消化道" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "口咽部溃疡" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "口咽部" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "全腹痛" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "全腹" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "黑粪" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "粪" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "呕血" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" }, { "start_idx": 28, "end_idx": 40, "type": "dis", "entity": "消化道黏膜弥漫性出血性炎症" }, { "start_idx": 42, "end_idx": 50, "type": "dis", "entity": "急性肠系膜动脉栓塞" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "胃肠道穿孔" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "膈下有游离气体" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "膈下" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "腹胀" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "腹" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "麻痹性肠梗阻" } ] }, { "text": "(四)其他可有黄疸、肝大和肝功能异常、淋巴结肿大、雷诺现象及脾肿大,常可累及心、肺和中枢神经系统,肾脏病变较少见。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "黄疸" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "肝大" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肝" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "肝功能异常" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肝" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "淋巴结肿大" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "淋巴结" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "雷诺现象" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "脾肿大" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "脾" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "心" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "肺" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "肾脏病变" } ] }, { "text": "【实验室检查】(一)血象急性期白细胞增多,晚期有贫血。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "血象" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "白细胞增多" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "白细胞" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "贫血" } ] }, { "text": "(二)急性期反应物血沉增快,α2和γ球蛋白增高,CRP阳性,但变化较轻微。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "sym", "entity": "急性期反应物血沉增快" }, { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "急性期反应物" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "血沉" }, { "start_idx": 14, "end_idx": 21, "type": "ite", "entity": "α2" }, { "start_idx": 15, "end_idx": 28, "type": "sym", "entity": "2和γ球蛋白增高" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "γ球蛋白" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "CRP阳性" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "CRP" } ] }, { "text": "(三)血清酶学检查肌酸激酶(CK)、乳酸脱氢酶(LDH)及谷草转氨酶(AST)等明显升高,CK同工酶CK-MB增高。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "血清酶学检查" }, { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "肌酸激酶" }, { "start_idx": 9, "end_idx": 43, "type": "sym", "entity": "肌酸激酶(CK)、乳酸脱氢酶(LDH)及谷草转氨酶(AST)等明显升高" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "CK" }, { "start_idx": 18, "end_idx": 22, "type": "ite", "entity": "乳酸脱氢酶" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "LDH" }, { "start_idx": 29, "end_idx": 33, "type": "ite", "entity": "谷草转氨酶" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "AST" }, { "start_idx": 45, "end_idx": 56, "type": "sym", "entity": "CK同工酶CK-MB增高" }, { "start_idx": 45, "end_idx": 54, "type": "ite", "entity": "CK同工酶CK-MB" } ] }, { "text": "(四)抗核抗体ANA50%阳性,但无dsDNA和抗Sm抗体,可有特异性抗JO-1抗体。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "抗核抗体ANA50%阳性" }, { "start_idx": 3, "end_idx": 9, "type": "ite", "entity": "抗核抗体ANA" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "无dsDNA和抗Sm抗体" }, { "start_idx": 18, "end_idx": 22, "type": "ite", "entity": "dsDNA" }, { "start_idx": 24, "end_idx": 28, "type": "ite", "entity": "抗Sm抗体" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "可有特异性抗JO-1抗体" }, { "start_idx": 32, "end_idx": 41, "type": "ite", "entity": "特异性抗JO-1抗体" } ] }, { "text": "(五)特殊检查1.X线检查骨关节周围有钙化,或弥漫性软组织及皮肤钙化。", "entities": [ { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "X线检查骨关节周围有钙化" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "X线检查" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "骨关节周围" }, { "start_idx": 23, "end_idx": 33, "type": "sym", "entity": "弥漫性软组织及皮肤钙化" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "软组织" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "皮肤" } ] }, { "text": "2.肌电图呈肌源性变化,表现为:(1)静息时自发性纤颤电位、正锐波及插入激惹;(2)收缩时呈短时限、低振幅及多相性电位;(3)刺激时出现反复高频放电。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "肌电图呈肌源性变化" }, { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肌电图" }, { "start_idx": 19, "end_idx": 28, "type": "sym", "entity": "静息时自发性纤颤电位" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "正锐波及插入激惹" }, { "start_idx": 42, "end_idx": 58, "type": "sym", "entity": "收缩时呈短时限、低振幅及多相性电位" }, { "start_idx": 63, "end_idx": 73, "type": "sym", "entity": "刺激时出现反复高频放电" } ] }, { "text": "3.MRI可显示肌肉异常部位及范围,有利于监测病情和指导肌活检部位。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "MRI" }, { "start_idx": 2, "end_idx": 16, "type": "sym", "entity": "MRI可显示肌肉异常部位及范围" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "肌肉异常部位" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "监测病情" }, { "start_idx": 28, "end_idx": 30, "type": "pro", "entity": "肌活检" } ] }, { "text": "4.肌肉活体组织检查一般为三角肌或四头肌以及经肌电图或MRI证实的病变部位,应无肌肉萎缩,标本宜较大(2~3cm);不合适的部位和不合适的标本大小可使肌活检结果阴性,疾病晚期不宜做肌活体组织检查,因此时病变已不再有特异性。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "三角肌" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "四头肌" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "肌电图" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "MRI" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "肌肉萎缩" }, { "start_idx": 75, "end_idx": 77, "type": "pro", "entity": "肌活检" }, { "start_idx": 90, "end_idx": 96, "type": "pro", "entity": "肌活体组织检查" } ] }, { "text": "活体组织检查标本可见到:(1)血管周围炎性细胞浸润;(2)肌束周围有肌纤维萎缩和坏死;(3)肌纤维再生现象。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "血管周围炎性细胞浸润" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "血管周围" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "肌束周围有肌纤维萎缩和坏死" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "肌束周围" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "肌纤维" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "坏死" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "肌纤维再生现象" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "肌纤维" } ] }, { "text": "【治疗】(一)一般治疗注意避免阳光照射,出门宜带帽子和手套,皮肤护理,避免外伤引起溃疡和溃破处继发感染;注意心脏功能和呼吸情况;低盐饮食;肢体注意功能位,及时进行按摩和理疗。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "避免阳光照射" }, { "start_idx": 20, "end_idx": 28, "type": "pro", "entity": "出门宜带帽子和手套" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "皮肤护理" }, { "start_idx": 35, "end_idx": 50, "type": "pro", "entity": "避免外伤引起溃疡和溃破处继发感染" }, { "start_idx": 52, "end_idx": 62, "type": "pro", "entity": "注意心脏功能和呼吸情况" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "低盐饮食" }, { "start_idx": 69, "end_idx": 75, "type": "pro", "entity": "肢体注意功能位" }, { "start_idx": 77, "end_idx": 85, "type": "pro", "entity": "及时进行按摩和理疗" } ] }, { "text": "(二)皮质激素泼尼松2mg/(kg•d),最大量≤60mg/d,分次服用,共服用1个月,后改为1mg/(kg•d),随后逐渐减量,连用2年以上;急性期可用甲泼尼龙大剂量冲击疗法,10~30mg/(kg•d)(≤1000mg/d)静脉滴注1~3天。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "皮质激素" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "泼尼松" }, { "start_idx": 77, "end_idx": 80, "type": "dru", "entity": "甲泼尼龙" }, { "start_idx": 81, "end_idx": 87, "type": "pro", "entity": "大剂量冲击疗法" }, { "start_idx": 114, "end_idx": 117, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "注意本病如伴消化道吸收障碍,口服泼尼松不能吸收,则宜改用静注。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "消化道吸收障碍" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "口服" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "泼尼松" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "静注" } ] }, { "text": "(三)羟氯喹剂量6mg/(kg•d),可控制皮肤病变发展。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "羟氯喹" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "皮肤病变" } ] }, { "text": "(四)免疫抑制剂可选用:1.氨甲蝶呤(MTX)每次0.35~0.65mg/kg,每周一次,PO或IV/IM。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "氨甲蝶呤" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "MTX" } ] }, { "text": "2.环磷酰胺(CTX)每次0.5~0.75g/m2,每月一次静滴。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "CTX" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "静滴" } ] }, { "text": "3.硫唑嘌呤(azathioprine)1~3mg/(kg•d),口服。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 7, "end_idx": 18, "type": "dru", "entity": "azathioprine" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "口服" } ] }, { "text": "4.环孢素(cyclosporineA)2.5~7.5mg/(kg•d),口服。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "环孢素" }, { "start_idx": 6, "end_idx": 18, "type": "dru", "entity": "cyclosporineA" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "口服" } ] }, { "text": "重症可选用2种免疫抑制剂。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "(五)IVIG每次1~2g/kg,每月1次,对皮质激素耐药或皮质激素依赖患儿可应用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "IVIG" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "皮质激素" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "皮质激素" } ] }, { "text": "(六)其他生物制剂如TNF-α抑制剂以及CD20单抗的应用,为顽固难治的JDM提供了新的治疗手段。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "生物制剂" }, { "start_idx": 10, "end_idx": 17, "type": "dru", "entity": "TNF-α抑制剂" }, { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "CD20单抗" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "JDM" } ] }, { "text": "血浆置换及造血干细胞移植对重症、危及生命的JDM可能有效。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "血浆置换" }, { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "造血干细胞移植" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "JDM" } ] }, { "text": "(一)铅中毒铅是一种具有神经毒性的重金属元素,目前大量的临床研究证明,血铅浓度超过0.483μmol/L(10μg/dl)就会损害健康。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "铅中毒" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "神经" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "血铅浓度" }, { "start_idx": 35, "end_idx": 60, "type": "sym", "entity": "血铅浓度超过0.483μmol/L(10μg/dl)" } ] }, { "text": "国内流行病学的调查资料表明,在工业区内,儿童青少年铅中毒(leadpoisoning)的流行率较高,约85%;即使在没有明显污染的普通市区,儿童的血铅水平也在临界值,这一现象值得引起人们的关注。", "entities": [ { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "儿童青少年铅中毒" }, { "start_idx": 29, "end_idx": 41, "type": "dis", "entity": "leadpoisoning" }, { "start_idx": 73, "end_idx": 74, "type": "ite", "entity": "血铅" }, { "start_idx": 73, "end_idx": 81, "type": "sym", "entity": "血铅水平也在临界值" } ] }, { "text": "研究表明,铅中毒会影响智力。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "铅中毒" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "影响智力" } ] }, { "text": "高血铅的群体在韦氏智力量表测试的结果,即总智商和操作智商均比低铅群体分别低14分和13分,有非常显著的统计学意义,而且这些高血铅者在脱离铅污染区3年后,虽然血铅浓度明显下降,但并不能使受损的心理发育恢复到原先水平。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血铅" }, { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "韦氏智力量表测试" }, { "start_idx": 20, "end_idx": 43, "type": "sym", "entity": "总智商和操作智商均比低铅群体分别低14分和13分" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "总智商" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "操作智商" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "高血铅" }, { "start_idx": 78, "end_idx": 81, "type": "ite", "entity": "血铅浓度" }, { "start_idx": 78, "end_idx": 85, "type": "sym", "entity": "血铅浓度明显下降" } ] }, { "text": "不仅如此,铅中毒还可致体格生长落后,研究证实血铅每升高0.483μmol/L,身高降低1.3cm。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "铅中毒" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "体格生长落后" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "血铅" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "身高" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "身高降低1.3cm" } ] }, { "text": "铅中毒预防重于治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "铅中毒" } ] }, { "text": "对已经铅中毒的青少年,也可根据血铅浓度进行排铅,轻、中度铅中毒用钙制剂排铅,重度铅中毒则需用二巯基丙醇和依地酸二钠钙进行驱铅治疗。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "铅中毒" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "血铅浓度" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "排铅" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "轻、中度铅中毒" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "钙制剂" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "排铅" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "重度铅中毒" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "二巯基丙醇" }, { "start_idx": 52, "end_idx": 57, "type": "dru", "entity": "依地酸二钠钙" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "驱铅治疗" } ] }, { "text": "吸烟所造成的危害远非尼古丁的慢性中毒,而是烟草中几十种毒性物质的综合影响,例如吸烟会降低学习能力,使记忆能力下降。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "慢性中毒" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "降低学习能力" }, { "start_idx": 50, "end_idx": 55, "type": "sym", "entity": "记忆能力下降" } ] }, { "text": "吸烟时还吸入大量的一氧化碳,一氧化碳与血红蛋白的亲和力比氧气大240倍,经常吸烟的青少年就可能有脑部缺氧引起的头痛、头晕、四肢无力。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "脑部缺氧" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "脑部" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "头痛" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "头" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "头晕" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "头" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "四肢无力" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "四肢" } ] }, { "text": "吸烟还会造成性功能障碍,有研究表明,吸烟的青少年血浆睾酮浓度比不吸烟者低20%~30%。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "性功能障碍" }, { "start_idx": 24, "end_idx": 29, "type": "ite", "entity": "血浆睾酮浓度" }, { "start_idx": 24, "end_idx": 42, "type": "sym", "entity": "血浆睾酮浓度比不吸烟者低20%~30%" } ] }, { "text": "吸烟对心血管系统也有影响,使血管痉挛、心压增高、心跳加快等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "心血管系统" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "血管痉挛" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "心压增高" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "心压" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "心跳加快" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "心跳" } ] }, { "text": "十一、肺脓疡肺脓疡(lungabscess)又称肺化脓症,由多种病原菌引起。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 10, "end_idx": 20, "type": "dis", "entity": "lungabscess" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "肺化脓症" }, { "start_idx": 32, "end_idx": 34, "type": "mic", "entity": "病原菌" } ] }, { "text": "由于抗生素的广泛应用,目前已较少见。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "抗生素" } ] }, { "text": "起病急剧,有畏寒、高热,伴阵咳、咳出大量脓痰,病程长者可反复咯血、贫血、消瘦等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "畏寒" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "高热" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "阵咳" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "咳出大量脓痰" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "痰" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "反复咯血" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "血" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "贫血" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "消瘦" } ] }, { "text": "最好根据细菌培养和药物敏感试验结果选用。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "细菌培养" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "药物敏感试验" } ] }, { "text": "第六节心房颤动心房颤动(简称房颤)在儿童少见,在婴儿罕见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心房颤动" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "心房颤动" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "房颤" } ] }, { "text": "房颤通常是心房长期牵张扩大的结果,多见于患风湿性二尖瓣病变的年长儿。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "房颤" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心房" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "心房长期牵张扩大" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "风湿性二尖瓣病变" } ] }, { "text": "房颤也可是家族性的。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "房颤" } ] }, { "text": "慢性房颤患儿易发生血栓栓塞疾病及中风,应予以华法林抗凝。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性房颤" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "血栓栓塞疾病" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "中风" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "华法林" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "抗凝" } ] }, { "text": "十一、幕上原始神经外胚叶肿瘤占儿童中枢神经系统肿瘤2%~3%,发病年龄多在5岁以内,90%位于大脑半球,其余在脑中线位,如脑室、胼胝体、丘脑、基底节等。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "幕上原始神经外胚叶肿瘤" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "儿童中枢神经系统肿瘤" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "大脑半球" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "脑中线位" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "脑室" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "胼胝体" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "丘脑" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "基底节" } ] }, { "text": "可有脑脊液播散或骨、肺等远处转移。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "脑脊液" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "骨" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肺" } ] }, { "text": "临床表现多有头痛、呕吐等颅内高压症状,其次可有癫痫、偏瘫等局部神经损害症检查CT及MRI检查有助于明确诊断。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "头痛" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "头" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "颅内高压症状" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "颅内" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "癫痫" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "偏瘫" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "局部神经损害症" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "检查CT" }, { "start_idx": 41, "end_idx": 45, "type": "pro", "entity": "MRI检查" } ] }, { "text": "治疗需靠手术、放疗及化疗综合处理。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "手术" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "放疗" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "化疗" } ] }, { "text": "手术肿瘤切除的程度与预后无明显关系,术后放疗需包括全脑及脊髓照射。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "肿瘤切除" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "术后放疗" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "全脑及脊髓照射" } ] }, { "text": "预后差,尤其是小于3岁患儿;另外实质性肿瘤比囊性肿瘤预后差。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "实质性肿瘤" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "囊性肿瘤" } ] }, { "text": "第十二章淹溺【概述】淹溺是指包括水在内的各种液体淹没面部及上呼吸道,导致人体因呼吸功能丧失所窒息而引起的生命危险状态或死亡。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "淹溺" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "淹溺" }, { "start_idx": 16, "end_idx": 32, "type": "sym", "entity": "水在内的各种液体淹没面部及上呼吸道" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "面部" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "呼吸功能丧失" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "窒息" } ] }, { "text": "淹溺的严重后果在于,即使生命得以复苏,淹溺引起的神经系统损害常足以使患儿丧失正常生活能力。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "淹溺" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "神经系统损害" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "神经系统" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "丧失正常生活能力" } ] }, { "text": "国外资料显示,在急症室接受心肺复苏并存活的淹溺患儿中有60%~100%存在严重脑损害。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "淹溺" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "严重脑损害" } ] }, { "text": "继发性溺死(secondarydrowning)是指淹溺后短期内死于并发症的患者;在溺水后初期病情不重,但以后病情恶化,最终导致死亡。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "继发性溺死" }, { "start_idx": 6, "end_idx": 22, "type": "dis", "entity": "secondarydrowning" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "淹溺" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "溺水" } ] }, { "text": "浸渍综合征(immersionsyndrome)是指在跌入冰水时突然死亡。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "浸渍综合征" }, { "start_idx": 6, "end_idx": 22, "type": "dis", "entity": "immersionsyndrome" } ] }, { "text": "目前认为其发生与迷走神经过度兴奋导致心跳停止或心室颤动有关。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "迷走神经过度兴奋" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "迷走神经" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心跳停止" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "心跳" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "心室颤动" } ] }, { "text": "前者为溺水后因心跳、呼吸反射性停止、意识迅速丧失,或伴有抽搐、颅脑外伤、颅内出血等;后者较为常见,原因为患儿在淹溺时出现划水和挣扎运动,引起体内儿茶酚胺大量释放。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "溺水" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "心跳、呼吸反射性停止" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "心跳" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "呼吸" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "意识迅速丧失" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "抽搐" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "颅脑外伤" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "颅内出血" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "淹溺" }, { "start_idx": 70, "end_idx": 79, "type": "sym", "entity": "体内儿茶酚胺大量释放" }, { "start_idx": 70, "end_idx": 75, "type": "bod", "entity": "体内儿茶酚胺" } ] }, { "text": "【流行病学】淹溺是意外死亡的重要原因之一。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "淹溺" } ] }, { "text": "在美国,2000年以后,所有淹溺者中,50%年龄低于20岁;儿童淹溺死亡仍排在儿童意外死亡的第3位,高峰年龄为1~4岁。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "淹溺" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "淹溺" } ] }, { "text": "【病因与发病机制】淹溺患者中,大多为淡水淹溺,包括池塘、江河、游泳池甚至浴池。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "淹溺" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "淡水淹溺" } ] }, { "text": "国外报道游泳池淹溺比率可占淡水淹溺患者的50%。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "淹溺" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "淡水淹溺" } ] }, { "text": "淹溺好发时间通常为夏季,小孩可因不熟水性或不慎由浮水板上跌入水中溺水。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" } ] }, { "text": "这些儿童发生淹溺,通常存在一些意外因素,如游泳中头颈部严重受伤、潜水中发生减压病、低温性痉挛或心律失常、水中受虐待、惊厥、中毒等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "淹溺" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "头颈部严重受伤" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "减压病" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "低温性痉挛" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "心律失常" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "惊厥" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "中毒" } ] }, { "text": "如因照看不慎,年幼儿跌入浴池内;年长儿则可因浴中癫痫发作或因被虐待而淹溺。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "癫痫发作" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "淹溺" } ] }, { "text": "此外,体温过低和体能耗竭也是水性良好的游泳者发生淹溺的重要原因。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "体温过低" }, { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "体温" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "体能耗竭" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "淹溺" } ] }, { "text": "游泳时的过度通气、照相闪光灯刺激、水淹性癫痫(waterimmersionepilepsy)和劳累均会诱发癫痫发作。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "水淹性癫痫" }, { "start_idx": 23, "end_idx": 44, "type": "dis", "entity": "waterimmersionepilepsy" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "癫痫" } ] }, { "text": "癫痫患儿游泳时的溺水发生率是正常儿童的4~5倍,即使使用抗痫药也不能达到完全保护作用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "癫痫" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "抗痫药" } ] }, { "text": "当发生淹溺时,淹溺者可出现两种反应:挣扎和反射性心跳呼吸骤停。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "淹溺" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "淹溺" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "挣扎" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "反射性心跳呼吸骤停" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "心跳" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "呼吸" } ] }, { "text": "年幼儿可表现入水后反射性屏气,直接沉入水里而不出现挣扎,在被救起后才恢复自主呼吸。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "入水后反射性屏气" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "直接沉入水里而不出现挣扎" }, { "start_idx": 28, "end_idx": 39, "type": "sym", "entity": "在被救起后才恢复自主呼吸" } ] }, { "text": "部分游泳者头面部在接触冷水后,由于迷走神经过度兴奋,反射性地出现心动过缓或骤停而发生淹溺。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "头" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "面部" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "迷走神经过度兴奋" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "迷走神经" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "心动过缓或骤停" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "淹溺" } ] }, { "text": "大部分淹溺者在淹溺时发生挣扎和呼吸运动。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "淹溺" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "淹溺" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "挣扎" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸运动" } ] }, { "text": "随着挣扎和缺氧,大量水被吞入胃内,吞水同时亦可有部分水被吸入气道。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "挣扎" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "缺氧" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "大量水被吞入胃内" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "胃内" }, { "start_idx": 17, "end_idx": 31, "type": "sym", "entity": "吞水同时亦可有部分水被吸入气道" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "气道" } ] }, { "text": "吸入肺的液体通常很少,大多数溺水者吸入气道内的水量低于20ml/kg。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "吸入肺的液体通常很少" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肺" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "溺水" }, { "start_idx": 17, "end_idx": 33, "type": "sym", "entity": "吸入气道内的水量低于20ml/kg" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "气道内" } ] }, { "text": "淹溺后,随着缺氧,溺者意识很快丧失,如未及时遇救,随即会出现心跳停止。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "缺氧" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "意识很快丧失" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "心跳停止" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "心跳" } ] }, { "text": "干溺者约占淹溺总数的7%~10%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "干溺" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "淹溺" } ] }, { "text": "窒息的结果导致低氧血症和意识丧失,最终因严重缺氧而心脏停止跳动。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "窒息" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低氧血症" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "意识丧失" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "严重缺氧" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "心脏停止跳动" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心脏" } ] }, { "text": "溺水的原发损害部位为肺脏,肺气体交换障碍所致的低氧血症是包括脑损伤在内的继发性损害的主要原因。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "溺水" }, { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "原发损害" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肺脏" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "肺气体交换障碍" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "低氧血症" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "脑损伤" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "继发性损害" } ] }, { "text": "在淹溺后初期一般无明显体液和水电解质代谢紊乱。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "淹溺" }, { "start_idx": 11, "end_idx": 21, "type": "dis", "entity": "体液和水电解质代谢紊乱" } ] }, { "text": "动物实验证实吸入20ml/kg水不会导致持续水电解质紊乱,而气道内滴入1~3ml/kg淡水或海水则足以使动物血氧饱和度下降。", "entities": [ { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "水电解质紊乱" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "气道内" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "血氧饱和度下降" }, { "start_idx": 54, "end_idx": 58, "type": "ite", "entity": "血氧饱和度" } ] }, { "text": "因海水与淡水成分不同,所致淹溺并大量吸入后的病理生理亦存在差异。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "淹溺" } ] }, { "text": "海水的渗透压约为血清的14倍,高渗盐水吸入肺泡内的结果导致毛细血管内血浆渗入肺泡内而发生肺泡性肺水肿。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血清" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺泡内" }, { "start_idx": 29, "end_idx": 40, "type": "sym", "entity": "毛细血管内血浆渗入肺泡内" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "毛细血管内血浆" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "肺泡内" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "肺泡性肺水肿" } ] }, { "text": "血浆丧失造成有效循环血量减少及血液浓缩。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "血浆丧失" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血浆" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "有效循环血量减少" }, { "start_idx": 6, "end_idx": 11, "type": "ite", "entity": "有效循环血量" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "血液浓缩" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血液" } ] }, { "text": "同时,海水中浓度较高的电解质钠、氯、镁等弥散进入血液后导致高渗和高钠血症。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "血液" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "高渗" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "高钠血症" } ] }, { "text": "淡水渗透压低于血浆和体液。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "渗透压" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血浆" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "体液" } ] }, { "text": "当大量低渗水顺渗透压梯度向肺间质及毛细血管方向移行,则引起间质性肺水肿、血管内低渗性溶血和低钠血症。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "渗透压梯度" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺间质" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "毛细血管" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "间质性肺水肿" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "血管内低渗性溶血" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "血管内" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "血" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "低钠血症" } ] }, { "text": "淡水与海水导致低氧血症的机制略有不同。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低氧血症" } ] }, { "text": "淡水可使肺表面活性物质失活和肺泡萎陷,并引起肺泡基底膜损害、肺泡炎症、蛋白渗出、肺泡透明膜形成、肺广泛水肿伴局部出血。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "sym", "entity": "肺表面活性物质失活" }, { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "肺泡萎陷" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺泡" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "肺泡基底膜损害" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "肺泡基底膜" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "肺泡炎症" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "蛋白渗出" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "蛋白" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "肺泡透明膜形成" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "肺泡透明膜" }, { "start_idx": 48, "end_idx": 57, "type": "sym", "entity": "肺广泛水肿伴局部出血" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "肺" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "血" } ] }, { "text": "海水淹溺则可使肺血管内液体向肺泡转移,使肺表面活性物质被稀释和冲洗流失,引起肺泡萎陷。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "海水淹溺" }, { "start_idx": 7, "end_idx": 17, "type": "sym", "entity": "肺血管内液体向肺泡转移" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "肺血管内液体" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺泡" }, { "start_idx": 20, "end_idx": 34, "type": "sym", "entity": "肺表面活性物质被稀释和冲洗流失" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "肺泡萎陷" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肺泡" } ] }, { "text": "动物兔实验中发现淹溺后肺血管内皮和肺泡壁的线粒体形态异常。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "淹溺" }, { "start_idx": 11, "end_idx": 27, "type": "sym", "entity": "肺血管内皮和肺泡壁的线粒体形态异常" }, { "start_idx": 11, "end_idx": 23, "type": "bod", "entity": "肺血管内皮和肺泡壁的线粒体" } ] }, { "text": "液体吸入还可引起肺内血液分流、肺顺应性下降、通气血流比值失调和支气管痉挛,这些变化均可加重低氧血症。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "肺内血液分流" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "肺内血液" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "肺顺应性下降" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "通气血流比值失调" }, { "start_idx": 22, "end_idx": 27, "type": "ite", "entity": "通气血流比值" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "支气管痉挛" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "低氧血症" } ] }, { "text": "【临床表现】淹溺的临床表现集中在肺部和中枢神经系统的缺氧损害。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "淹溺" }, { "start_idx": 16, "end_idx": 29, "type": "dis", "entity": "肺部和中枢神经系统的缺氧损害" } ] }, { "text": "部分病例在淹溺经复苏后2~6小时才出现严重症状,并可有生命体征改变。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "淹溺" } ] }, { "text": "临床症状与淹溺时间、吸水量及是否及时抢救有关。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "淹溺" } ] }, { "text": "个体间病情程度差异很大,轻者可无明显异常体征,重者需进行心肺复苏。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "心肺复苏" } ] }, { "text": "溺水1~2分钟即可有神志模糊、呼吸不规则、血压下降和心跳缓慢;并可有呛咳、呕吐,或因反射性喉痉挛而窒息死亡,亦可因呕吐物吸入呼吸道而窒息。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "溺水" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "神志模糊" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "呼吸不规则" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "呼吸" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "血压下降" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "血压" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "心跳缓慢" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "心跳" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "呛咳" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "呕吐" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "反射性喉痉挛" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "窒息" }, { "start_idx": 57, "end_idx": 64, "type": "sym", "entity": "呕吐物吸入呼吸道" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "呼吸道" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "窒息" } ] }, { "text": "溺水3~4分钟以上者常出现昏迷、惊厥、颜面青紫、水肿、血性液体经口鼻涌出、四肢冰冷、血压下降、肺部有啰音、心律失常或呼吸、心跳停止。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "溺水" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "昏迷" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "惊厥" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "颜面青紫" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "颜面" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "水肿" }, { "start_idx": 27, "end_idx": 35, "type": "sym", "entity": "血性液体经口鼻涌出" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "口" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "鼻" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "四肢冰冷" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "四肢" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "血压下降" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "血压" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "肺部有啰音" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肺部" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "心律失常" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "呼吸、心跳停止" }, { "start_idx": 58, "end_idx": 59, "type": "ite", "entity": "呼吸" }, { "start_idx": 61, "end_idx": 62, "type": "ite", "entity": "心跳" } ] }, { "text": "淹溺挣扎时吞入大量液体者可出现腹胀。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" }, { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "挣扎时吞入大量液体" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "腹胀" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "腹" } ] }, { "text": "部分患者还可在淹溺时发生脑外伤、骨折淹溺后发生的低氧血症、酸中毒、血流低灌注可影响全身各个脏器,部分患儿可继发出现吸入性肺炎、呼吸衰竭、ARDS、缺血缺氧性脑病、溶血、心律失常、休克、急性肾衰竭及DIC等。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "淹溺" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "脑外伤" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "骨折" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "淹溺" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "低氧血症" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "酸中毒" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "血流低灌注" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血流" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "全身各个脏器" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "ARDS" }, { "start_idx": 73, "end_idx": 79, "type": "dis", "entity": "缺血缺氧性脑病" }, { "start_idx": 81, "end_idx": 82, "type": "sym", "entity": "溶血" }, { "start_idx": 82, "end_idx": 82, "type": "bod", "entity": "血" }, { "start_idx": 84, "end_idx": 87, "type": "dis", "entity": "心律失常" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "休克" }, { "start_idx": 92, "end_idx": 96, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "DIC" } ] }, { "text": "其中溶血、外伤引起的血红蛋白尿和肌红蛋白尿可诱发急性肾衰竭;显著水、电解质紊乱多见于特殊液体(如海水)淹溺或液体大量吞入等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "溶血" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "血" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "外伤" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "血红蛋白尿" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "尿" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肌红蛋白尿" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "肌红蛋白" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "显著水、电解质紊乱" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "淹溺" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "液体大量吞入" } ] }, { "text": "淹溺时的病情评估见表6-6。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" } ] }, { "text": "表6-16淹溺患者的病情评估【诊断】应先仔细了解相关病史,如:溺水时间、地点,是否沉入水底,获救时意识、自主呼吸和发绀及水温和水的性质(淡水、海水、脏水)等情况,以及对心肺复苏抢救的反应等,结合体格检查,确定淹溺的性质和程度。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "淹溺" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "溺水" }, { "start_idx": 52, "end_idx": 55, "type": "ite", "entity": "自主呼吸" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "发绀" }, { "start_idx": 84, "end_idx": 89, "type": "pro", "entity": "心肺复苏抢救" }, { "start_idx": 97, "end_idx": 100, "type": "pro", "entity": "体格检查" }, { "start_idx": 104, "end_idx": 105, "type": "dis", "entity": "淹溺" } ] }, { "text": "同时,注意鉴别是否有外伤、惊厥、中毒、过度疲劳等异常因素。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "外伤" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "惊厥" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "中毒" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "过度疲劳" } ] }, { "text": "体格检查应详细,在抢救期间应重点观察下列项目:①中心体温及发绀情况;②是否存在自主心跳呼吸、呼吸急促、三凹征及喘鸣;③意识状态情况、瞳孔对光反应、是否有去大脑体位及痛觉反应;④是否有颈部受伤和其他脏器损伤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "抢救" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "中心体温" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "发绀" }, { "start_idx": 39, "end_idx": 44, "type": "ite", "entity": "自主心跳呼吸" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "呼吸" }, { "start_idx": 51, "end_idx": 53, "type": "sym", "entity": "三凹征" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "喘鸣" }, { "start_idx": 59, "end_idx": 64, "type": "ite", "entity": "意识状态情况" }, { "start_idx": 66, "end_idx": 71, "type": "ite", "entity": "瞳孔对光反应" }, { "start_idx": 76, "end_idx": 80, "type": "sym", "entity": "去大脑体位" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "大脑" }, { "start_idx": 82, "end_idx": 85, "type": "ite", "entity": "痛觉反应" }, { "start_idx": 91, "end_idx": 94, "type": "dis", "entity": "颈部受伤" }, { "start_idx": 96, "end_idx": 101, "type": "dis", "entity": "其他脏器损伤" } ] }, { "text": "如需经常复查血气,可安置一动脉插管,或辅以脉搏氧饱和度监测。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "复查血气" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "安置一动脉插管" }, { "start_idx": 21, "end_idx": 28, "type": "pro", "entity": "脉搏氧饱和度监测" } ] }, { "text": "X线胸片检查用于了解患者是否存在吸入性肺炎和气胸,观察气管插管、胃管和中心静脉导管的安放位置。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "X线胸片检查" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "气胸" }, { "start_idx": 27, "end_idx": 30, "type": "equ", "entity": "气管插管" }, { "start_idx": 32, "end_idx": 33, "type": "equ", "entity": "胃管" }, { "start_idx": 35, "end_idx": 40, "type": "equ", "entity": "中心静脉导管" } ] }, { "text": "颅内压监测有利于了解脑水肿状态和指导治疗。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "颅内压监测" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑水肿" } ] }, { "text": "患者的预后很大程度上取决于神经系统缺氧后的受损程度。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "神经系统缺氧" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "神经系统" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "受损" } ] }, { "text": "文献报道存在下列一些因素提示预后不良:年龄低于3岁,淹溺时间超过5分钟,心肺复苏开始时间超过10分钟;出现惊厥、瞳孔扩大固定、昏迷、去大脑强直;非低温状态仍需持续心肺复苏;Glasgow评分低于5分;pH<7.10;血糖>10mmol/L。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "淹溺" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "瞳孔扩大固定" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "瞳孔" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "昏迷" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "去大脑强直" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "大脑" }, { "start_idx": 72, "end_idx": 76, "type": "sym", "entity": "非低温状态" }, { "start_idx": 79, "end_idx": 84, "type": "pro", "entity": "持续心肺复苏" }, { "start_idx": 86, "end_idx": 98, "type": "sym", "entity": "Glasgow评分低于5分" }, { "start_idx": 86, "end_idx": 94, "type": "pro", "entity": "Glasgow评分" }, { "start_idx": 100, "end_idx": 106, "type": "sym", "entity": "pH<7.10" }, { "start_idx": 100, "end_idx": 101, "type": "ite", "entity": "pH" }, { "start_idx": 108, "end_idx": 118, "type": "sym", "entity": "血糖>10mmol/L" }, { "start_idx": 108, "end_idx": 109, "type": "ite", "entity": "血糖" } ] }, { "text": "【治疗】(一)入院前现场急救由于患者的预后直接与复苏的时间和效率有关,救护者须首先搜寻和找到淹溺者,将其救上岸,对心跳呼吸停止者在现场进行心肺复苏。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "入院前现场急救" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "淹溺" }, { "start_idx": 57, "end_idx": 62, "type": "sym", "entity": "心跳呼吸停止" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "心跳" }, { "start_idx": 59, "end_idx": 60, "type": "ite", "entity": "呼吸" }, { "start_idx": 64, "end_idx": 72, "type": "pro", "entity": "在现场进行心肺复苏" } ] }, { "text": "有报道入院前干预者预后较好,有效的入院前复苏可使32%心搏骤停患者恢复。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "入院前干预" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "入院前复苏" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "心搏骤停" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "心搏" } ] }, { "text": "除已明确死亡1小时以上者,其余均应进行心肺复苏,并设法通知救护站。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "设法通知救护站" } ] }, { "text": "怀疑颈部损伤者,可将其头部放正,并使颈部制动。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颈部损伤" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "将其头部放正" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "使颈部制动" } ] }, { "text": "对于已昏迷者可按常规进行心肺复苏,首先保持气道通畅,然后给予呼吸和心脏复苏。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "已昏迷" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "保持气道通畅" }, { "start_idx": 28, "end_idx": 36, "type": "pro", "entity": "给予呼吸和心脏复苏" } ] }, { "text": "倒水可用双手抱住小儿腹部方法,使其腰背向上,头脚下垂,使水自呼吸道自然流出,抢救者同时将淹溺者的两手臂不断抖动促使积水外流。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "倒水" }, { "start_idx": 2, "end_idx": 13, "type": "pro", "entity": "可用双手抱住小儿腹部方法" }, { "start_idx": 15, "end_idx": 25, "type": "pro", "entity": "使其腰背向上,头脚下垂" }, { "start_idx": 27, "end_idx": 36, "type": "pro", "entity": "使水自呼吸道自然流出" }, { "start_idx": 43, "end_idx": 60, "type": "pro", "entity": "将淹溺者的两手臂不断抖动促使积水外流" } ] }, { "text": "如气道存在梗阻可采用提颏压腭(颈椎损伤者忌)方法。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "气道存在梗阻" }, { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "气道" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "提颏压腭" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "颈椎损伤" } ] }, { "text": "如仍无效应考虑存在异物梗阻,可采用Heimlich法按压腹部。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "异物梗阻" }, { "start_idx": 15, "end_idx": 29, "type": "pro", "entity": "采用Heimlich法按压腹部" } ] }, { "text": "心肺复苏或气管插管中患者出现呕吐可压迫环状软骨(Sellick法)防止异物误吸入气道内。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "气管插管" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "呕吐" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "压迫环状软骨" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "Sellick法" }, { "start_idx": 33, "end_idx": 42, "type": "pro", "entity": "防止异物误吸入气道内" } ] }, { "text": "复苏时人工呼吸和心脏按压应同时进行。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "人工呼吸" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "心脏按压" } ] }, { "text": "如获救援,应尽可能及早给予以下处理:吸氧;对低体温者擦干皮肤,以干衣包裹和取暖,并注意心律失常;及早放置周围静脉通路,对低血压者给予输液20ml/kg;所有淹溺者在得到初步处理后,均应送医院作进一步观察和治疗。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "吸氧" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "低体温者擦干皮肤" }, { "start_idx": 31, "end_idx": 38, "type": "pro", "entity": "以干衣包裹和取暖" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "心律失常" }, { "start_idx": 48, "end_idx": 57, "type": "pro", "entity": "及早放置周围静脉通路" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "低血压" }, { "start_idx": 64, "end_idx": 74, "type": "pro", "entity": "给予输液20ml/kg" }, { "start_idx": 78, "end_idx": 79, "type": "dis", "entity": "淹溺" }, { "start_idx": 92, "end_idx": 103, "type": "pro", "entity": "送医院作进一步观察和治疗" } ] }, { "text": "如转运路途较远时,在下列情况下应予气管插管:①患儿昏迷,为保护气道防止误吸;②经一般处理不能维持气道通畅;③转运路途和时间较长,需保证稳定的开放气道;④低体温者。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "气管插管" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "昏迷" }, { "start_idx": 29, "end_idx": 36, "type": "pro", "entity": "保护气道防止误吸" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "一般处理" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "气道" }, { "start_idx": 70, "end_idx": 73, "type": "pro", "entity": "开放气道" }, { "start_idx": 76, "end_idx": 78, "type": "sym", "entity": "低体温" }, { "start_idx": 77, "end_idx": 78, "type": "ite", "entity": "体温" } ] }, { "text": "(二)医院内处理1.患者安置不论患者存在轻度发绀,还是伴有呼吸急促、呼吸窘迫、两肺喘鸣及啰音、循环不良、心动过速、意识障碍等,均应送观察室进行监护。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "医院内处理" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "轻度发绀" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "呼吸窘迫" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "两肺喘鸣及啰音" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "两肺" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "循环不良" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "心动过速" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "意识障碍" }, { "start_idx": 65, "end_idx": 72, "type": "pro", "entity": "送观察室进行监护" } ] }, { "text": "文献报道淹溺者中5%患者在数小时后可再次出现病情恶化(称为secondarydrowning),淡水淹溺的发作时间约为4小时,海水淹溺约为36小时。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "淹溺" }, { "start_idx": 29, "end_idx": 45, "type": "dis", "entity": "secondarydrowning" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "淡水淹溺" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "海水淹溺" } ] }, { "text": "2.呼吸支持对入院时存在呼吸急促者均给予鼻导管或面罩吸氧。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "呼吸支持" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "呼吸" }, { "start_idx": 18, "end_idx": 27, "type": "pro", "entity": "给予鼻导管或面罩吸氧" } ] }, { "text": "如无改善,可采用鼻塞或面罩式持续正压给氧(CPAP)法及提高吸入氧浓度。", "entities": [ { "start_idx": 8, "end_idx": 26, "type": "pro", "entity": "鼻塞或面罩式持续正压给氧(CPAP)法" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "提高吸入氧浓度" } ] }, { "text": "CPAP开始的压力可设定在5cmH2O左右,以后可增加2~5cmH2O,直至脉搏氧饱和度满意为止。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CPAP" }, { "start_idx": 60, "end_idx": 65, "type": "ite", "entity": "脉搏氧饱和度" } ] }, { "text": "对于自主呼吸较弱或呼吸停止、呼吸费力、不能稳定维持气道开放、需气道保护预防误吸、神经系统状况严重及存在低氧血症和二氧化碳潴留者均应考虑给予气管插管和机械通气。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "自主呼吸较弱" }, { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "呼吸" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "呼吸" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "呼吸费力" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "呼吸" }, { "start_idx": 19, "end_idx": 28, "type": "sym", "entity": "不能稳定维持气道开放" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "气道" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "气道保护" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "误吸" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "神经系统状况严重" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "神经系统" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "低氧血症" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "二氧化碳潴留" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "二氧化碳" }, { "start_idx": 69, "end_idx": 72, "type": "pro", "entity": "气管插管" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "机械通气" } ] }, { "text": "通气效果可结合临床表现和血气分析进行判定。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "通气" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "血气分析" } ] }, { "text": "亦有报道采用肺表面活性物质进行治疗。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dru", "entity": "肺表面活性物质" } ] }, { "text": "但目前尚无证据表明应用肾上腺皮质激素和应用抗生素与呼吸复苏成功率之间存在相关关系。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "抗生素" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "呼吸复苏" } ] }, { "text": "3.纠正低血压、维持心功能淹溺后引起休克特点为低心脏收缩力、高循环阻力和肺血管阻力;其原因为心脏存在缺血缺氧病变、体内酸中毒、中枢神经严重损伤、低体温、外伤性失血或血液分布异常以及颈椎损伤等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "纠正低血压" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "维持心功能" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "淹溺" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "休克" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "低心脏收缩力" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心脏" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "高循环阻力和肺血管阻力" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "肺血管" }, { "start_idx": 46, "end_idx": 55, "type": "sym", "entity": "心脏存在缺血缺氧病变" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "心脏" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "缺血缺氧病变" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "体内酸中毒" }, { "start_idx": 63, "end_idx": 70, "type": "dis", "entity": "中枢神经严重损伤" }, { "start_idx": 72, "end_idx": 74, "type": "sym", "entity": "低体温" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "外伤性失血" }, { "start_idx": 82, "end_idx": 87, "type": "sym", "entity": "血液分布异常" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "血液" }, { "start_idx": 90, "end_idx": 93, "type": "dis", "entity": "颈椎损伤" } ] }, { "text": "对于低血压者,可给予生理盐水或林格液10~20ml/kg1~2次,并以10ml/(kg•h)速度维持。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "低血压" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "生理盐水" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "林格液" } ] }, { "text": "如休克持续存在,可考虑放置中心静脉导管和肺动脉导管进行压力监测,指导补液。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "休克" }, { "start_idx": 13, "end_idx": 18, "type": "equ", "entity": "中心静脉导管" }, { "start_idx": 20, "end_idx": 24, "type": "equ", "entity": "肺动脉导管" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "压力监测" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "指导补液" } ] }, { "text": "如补充血容量数小时后血压仍较低,则应给予升血压药物(如多巴胺)维持,并可给予多巴酚丁胺5~20μg/(kg•min)静脉维持增强心肌收缩功能。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "补充血容量" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "血压仍较低" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "血压" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "升血压药物" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "多巴胺" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "多巴酚丁胺" }, { "start_idx": 58, "end_idx": 69, "type": "pro", "entity": "静脉维持增强心肌收缩功能" } ] }, { "text": "对出现心律失常者应注意鉴别和及时纠正。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心律失常" } ] }, { "text": "但对于脑死亡者,由于脑内体温调节功能丧失,复温治疗效果通常很差。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑死亡" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "脑内体温调节功能丧失" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "脑内" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "复温治疗" } ] }, { "text": "5.缓解脑水肿淹溺后产生严重神经系统损伤的发生率可达40%以上,目前在淹溺抢救方面特别强调注意脑复苏和维持正常颅内压。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脑水肿" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "淹溺" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "严重神经系统损伤" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "淹溺" }, { "start_idx": 47, "end_idx": 49, "type": "pro", "entity": "脑复苏" }, { "start_idx": 51, "end_idx": 57, "type": "pro", "entity": "维持正常颅内压" } ] }, { "text": "有资料显示颅内压增高与病死率有关。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "颅内压增高" } ] }, { "text": "当患者颅内压超过20mmHg、脑灌注压(CPP)低于50mmHg时,大多数患者最终导致死亡。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "颅内压超过20mmHg" }, { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "颅内压" }, { "start_idx": 15, "end_idx": 31, "type": "sym", "entity": "脑灌注压(CPP)低于50mmHg" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "脑灌注压" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "CPP" } ] }, { "text": "对有颅高压症状者可选用呋塞米1mg/kg静注以及甘露醇0.5g/kg静滴30分钟,每3~4小时输注一次,治疗中注意用药后的症状反跳现象。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "颅高压" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "呋塞米" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "静注" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "甘露醇" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "静滴" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "输注" }, { "start_idx": 55, "end_idx": 66, "type": "pro", "entity": "注意用药后的症状反跳现象" } ] }, { "text": "机械通气患者可采用过度通气,使动脉血CO2分压维持在25~30mmHg水平。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "机械通气" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "过度通气" }, { "start_idx": 14, "end_idx": 47, "type": "pro", "entity": "使动脉血CO2分压维持在25~30mmHg水平" } ] }, { "text": "积极控制惊厥亦有利于防止脑水肿和脑损害的进一步加重,对出现抽搐者可选用地西泮0.3mg/kg静注,或苯妥英钠10~20mg/kg缓慢静注。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "积极控制惊厥" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "脑水肿" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑损害" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "抽搐" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "地西泮" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "静注" }, { "start_idx": 50, "end_idx": 53, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "缓慢静注" } ] }, { "text": "脑水肿严重者可在12~24小时给予亚低温处理,使体温保持在32~34℃。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑水肿" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "亚低温处理" }, { "start_idx": 23, "end_idx": 34, "type": "pro", "entity": "使体温保持在32~34℃" } ] }, { "text": "6.其他治疗酌情选用抗生素;纠正水电解质和酸碱平衡紊乱;积极治疗淹溺并发症(如溶血、急性肾衰竭等)。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "抗生素" }, { "start_idx": 14, "end_idx": 26, "type": "pro", "entity": "纠正水电解质和酸碱平衡紊乱" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "淹溺并发症" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "溶血" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "腹胀者予放置胃管抽吸或持续胃肠吸引减压和监测胃内容物,防止反流后误吸。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹胀" }, { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "腹" }, { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "放置胃管抽吸" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "持续胃肠吸引减压" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "监测胃内容物" }, { "start_idx": 27, "end_idx": 33, "type": "pro", "entity": "防止反流后误吸" } ] }, { "text": "病情较重及尿潴留者应放置导尿管监测尿量;对于污水、氨水等淹溺时,应用清水冲洗和保护五官、清理呼吸道污物。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "尿潴留" }, { "start_idx": 10, "end_idx": 18, "type": "pro", "entity": "放置导尿管监测尿量" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "淹溺" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "清水冲洗" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "保护五官" }, { "start_idx": 44, "end_idx": 50, "type": "pro", "entity": "清理呼吸道污物" } ] }, { "text": "【预防】迄今为止,淹溺的防治重点仍为预防,其地位和重要性高于治疗和抢救。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "淹溺" } ] }, { "text": "淹溺在大多数情况下是可以做到预防的。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" } ] }, { "text": "对于可能在游泳中发生抽搐的患儿不宜下水游泳,如智能低下、近期更换抗癫痫药、癫痫未完全控制等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "抽搐" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "智能低下" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "抗癫痫药" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "癫痫" } ] }, { "text": "对于病情稳定2年以上的癫痫患儿,应在严密照看下进行游泳,以防不测。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "癫痫" } ] }, { "text": "此外,还应加强青少年及家长心肺复苏知识培训。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "心肺复苏" } ] }, { "text": "游泳区域和船舶上应配备急救设施,使淹溺后患者能得到及时复苏抢救。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "淹溺" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "复苏抢救" } ] }, { "text": "第九节碘缺乏病碘是人体不可缺少的一种营养素,是甲状腺素的必需成分。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "碘缺乏病" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "碘" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "营养素" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "甲状腺素" } ] }, { "text": "甲状腺利用碘和酪氨酸合成甲状腺激素,故当碘摄入不足时,机体会出现一系列的障碍,由于机体缺碘的程度和时期不同,机体出现障碍的严重程度也不同。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "甲状腺" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "碘" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "酪氨酸" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "碘" }, { "start_idx": 27, "end_idx": 37, "type": "sym", "entity": "机体会出现一系列的障碍" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "机体缺碘" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "碘" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "机体出现障碍" } ] }, { "text": "这些障碍,统称为碘缺乏病。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "障碍" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "碘缺乏病" } ] }, { "text": "【流行病学】碘缺乏病的最大危害就是对胎儿、新生儿、婴幼儿和儿童的脑发育造成损害,尤其是在胎儿和婴幼儿时期,即使是轻微的碘缺乏,也会引起一定程度的智力损害,造成轻度智力低下。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "碘缺乏病" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "脑发育造成损害" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脑" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "碘缺乏" }, { "start_idx": 67, "end_idx": 75, "type": "sym", "entity": "一定程度的智力损害" }, { "start_idx": 79, "end_idx": 84, "type": "sym", "entity": "轻度智力低下" } ] }, { "text": "1990年,世界儿童首脑峰会上确定了到2000年消除碘缺乏病的目标。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "碘缺乏病" } ] }, { "text": "【生理功能】碘的生理功能主要是作为甲状腺激素的合成原料,因此碘的生理功能也是通过甲状腺素的作用得以表现。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "碘" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "碘" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "甲状腺素" } ] }, { "text": "(一)促进体格生长出生后的体格生长和骨骼成熟依赖于正常量的甲状腺激素分泌。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "骨骼" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "甲状腺激素" } ] }, { "text": "在儿童发育期,促进身高、体重、骨骼和肌肉的增长和性发育,当碘供应不足时,这些都可出现延迟。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "身高" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "体重" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "骨骼" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肌肉" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "碘" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "这些都可出现延迟" } ] }, { "text": "(二)参与能量代谢甲状腺激素也主要参与机体细胞的能量代谢,最熟悉的指标就是基础代谢率。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "机体细胞" }, { "start_idx": 37, "end_idx": 41, "type": "ite", "entity": "基础代谢率" } ] }, { "text": "甲状腺激素可以增强机体基础代谢率,促进物质的分解代谢,增加氧耗量,产生能量,维持基本生命活动,保持体温。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "基础代谢率" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "氧耗量" }, { "start_idx": 49, "end_idx": 50, "type": "ite", "entity": "体温" } ] }, { "text": "(三)神经系统的发育甲状腺激素可影响脑神经细胞的生长、迁移和树突的发育。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "脑神经细胞" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "树突" } ] }, { "text": "在脑组织发育的临界期(从妊娠开始至出生后2岁),神经系统的发育依赖于甲状腺素的存在。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "脑组织" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "神经系统" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "甲状腺素" } ] }, { "text": "神经元的增殖、分化和髓鞘化,特别是树突、树突棘、突触及神经联系的建立,都需要甲状腺素的参与,它的缺乏会导致脑发育障碍,导致永久性的、不可逆转的脑功能不全。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "神经元" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "树突" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "树突棘" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "突触" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "神经" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "甲状腺素" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "脑发育障碍" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "脑功能不全" } ] }, { "text": "(四)参与垂体的调节垂体的正常生理功能有赖于甲状腺激素的支持和保证。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "垂体" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "垂体" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "甲状腺激素" } ] }, { "text": "如甲状腺激素的合成、释放不足,对垂体负反馈抑制减弱,垂体分泌促甲状腺激素(TSH)过多而导致甲状腺组织增生、腺体肿大。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "垂体" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "垂体" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "促甲状腺激素" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "TSH" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "甲状腺组织增生" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "腺体肿大" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "腺体" } ] }, { "text": "【发病机制】妊娠期间,如碘的摄入不足,孕妇血浆中无机碘离子浓度降低,尽管孕妇甲状腺处于代偿性吸碘率增高的状态,但甲状腺产生的T3、T4仍相对较少。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "碘" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血浆" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "无机碘离子浓度降低" }, { "start_idx": 24, "end_idx": 30, "type": "ite", "entity": "无机碘离子浓度" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "甲状腺" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "代偿性吸碘率增高" }, { "start_idx": 46, "end_idx": 48, "type": "ite", "entity": "吸碘率" }, { "start_idx": 56, "end_idx": 93, "type": "sym", "entity": "甲状腺产生的T3、T4仍相对较少" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "甲状腺" }, { "start_idx": 62, "end_idx": 74, "type": "bod", "entity": "T3" }, { "start_idx": 76, "end_idx": 88, "type": "bod", "entity": "T4" } ] }, { "text": "血液中的T3、T4大部分与甲状腺结合球蛋白等结合存在,仅有少量的游离T3、T4,而结合的T3、T4不能通过胎盘屏障。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 4, "end_idx": 16, "type": "bod", "entity": "T3" }, { "start_idx": 18, "end_idx": 30, "type": "bod", "entity": "T4" }, { "start_idx": 35, "end_idx": 42, "type": "bod", "entity": "甲状腺结合球蛋白" }, { "start_idx": 56, "end_idx": 68, "type": "bod", "entity": "T3" }, { "start_idx": 70, "end_idx": 82, "type": "bod", "entity": "T4" }, { "start_idx": 88, "end_idx": 100, "type": "bod", "entity": "T3" }, { "start_idx": 102, "end_idx": 114, "type": "bod", "entity": "T4" }, { "start_idx": 119, "end_idx": 122, "type": "bod", "entity": "胎盘屏障" } ] }, { "text": "孕妇雌激素分泌增加,血液中的甲状腺结合球蛋白减少,游离T3、T4减少,以致通过胎盘的T3、T4不能满足胎儿的需要,胎儿的生长发育即出现了一系列的障碍,中枢神经系统首先出现症状。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "雌激素分泌增加" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "雌激素" }, { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "血液中的甲状腺结合球蛋白减少" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "血液中" }, { "start_idx": 14, "end_idx": 21, "type": "ite", "entity": "甲状腺结合球蛋白" }, { "start_idx": 25, "end_idx": 55, "type": "sym", "entity": "游离T3、T4减少" }, { "start_idx": 27, "end_idx": 39, "type": "ite", "entity": "T3" }, { "start_idx": 41, "end_idx": 53, "type": "ite", "entity": "T4" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "胎盘" }, { "start_idx": 64, "end_idx": 76, "type": "bod", "entity": "T3" }, { "start_idx": 78, "end_idx": 90, "type": "bod", "entity": "T4" }, { "start_idx": 101, "end_idx": 117, "type": "sym", "entity": "胎儿的生长发育即出现了一系列的障碍" }, { "start_idx": 119, "end_idx": 124, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "出生以后(尤其断乳后),小儿才可直接从饮食中摄取碘,所以碘缺乏有所好转,但如饮食中缺碘严重,不能满足儿童合成甲状腺素的最低要求,儿童也可出现生长发育落后。", "entities": [ { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "碘" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "碘缺乏" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "碘" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "甲状腺素" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "生长发育落后" } ] }, { "text": "如长期缺碘,甲状腺组织发生了由代偿到病理损伤的过程。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "碘" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "甲状腺组织" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "病理损伤" } ] }, { "text": "碘不足,甲状腺激素水平降低,垂体分泌TSH增加,刺激甲状腺滤泡上皮增生。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "sym", "entity": "甲状腺激素水平降低" }, { "start_idx": 4, "end_idx": 10, "type": "ite", "entity": "甲状腺激素水平" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "垂体分泌TSH增加" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "垂体" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "TSH" }, { "start_idx": 24, "end_idx": 34, "type": "sym", "entity": "刺激甲状腺滤泡上皮增生" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "上皮增生" } ] }, { "text": "甲状腺组织中可见增生的滤泡,滤泡上皮增多,滤泡腔小,胶质储存减少,甲状腺体积增大,功能增强,如时间持续长,反复这样进行,则出现弥漫性甲状腺肿大。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "甲状腺组织中可见增生的滤泡" }, { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "甲状腺组织" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "增生的滤泡" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "滤泡上皮增多" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "滤泡上皮" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "滤泡腔小" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "滤泡腔" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "胶质储存减少" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "胶质" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "甲状腺体积增大" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "甲状腺" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "功能增强" }, { "start_idx": 63, "end_idx": 70, "type": "dis", "entity": "弥漫性甲状腺肿大" } ] }, { "text": "【碘缺乏病的病因】(一)环境因素其流行的原因是世界大部分地区的土壤中缺碘,尤其是冰川冲刷地带和洪水泛滥的平原。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "碘缺乏病" } ] }, { "text": "我国地方性甲状腺肿也多分布在山区,主要因为山区坡度大,雨水冲刷,碘从土壤中丢失所致。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "地方性甲状腺肿" } ] }, { "text": "低蛋白、低能量可使血清中T3、T4、血浆蛋白结合碘(PBI)降低,血清促甲状腺素(TSH)升高,使酪氨酸分泌减少,降低碘的有机化。", "entities": [ { "start_idx": 9, "end_idx": 53, "type": "sym", "entity": "血清中T3、T4、血浆蛋白结合碘(PBI)降低" }, { "start_idx": 9, "end_idx": 43, "type": "bod", "entity": "血清中T3、T4、血浆蛋白" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "碘" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "(PBI)" }, { "start_idx": 55, "end_idx": 61, "type": "bod", "entity": "血清促甲状腺素" }, { "start_idx": 55, "end_idx": 68, "type": "sym", "entity": "血清促甲状腺素(TSH)升高" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "TSH" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "酪氨酸" }, { "start_idx": 71, "end_idx": 77, "type": "sym", "entity": "酪氨酸分泌减少" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "碘" } ] }, { "text": "低蛋白、高碳水化合物可影响甲状腺对碘的吸收和利用。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "甲状腺" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "碘" } ] }, { "text": "人们普遍认为玉米、小米、甜薯、高粱及各种豆类中在肠道中可释放出氰化物,进而被代谢成硫氰酸盐,可抑制甲状腺摄取碘化物。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肠道中" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "氰化物" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "硫氰酸盐" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "甲状腺" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "碘化物" } ] }, { "text": "钙、磷含量高的食物可妨碍碘的吸收,抑制甲状腺素的合成,加速碘的排泄。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "碘" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "甲状腺素" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "碘" } ] }, { "text": "(三)饮水因素部分地区水中碘的含量较低,与碘缺乏病的发病率有关。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "碘缺乏病" } ] }, { "text": "(四)药物因素硫脲类抗甲状腺药物、四环素、磺胺类、咪唑类等可干扰酪氨酸的碘化过程,也有一定导致甲状腺肿作用。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "dru", "entity": "硫脲类抗甲状腺药物" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "四环素" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "磺胺类" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "咪唑类" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "酪氨酸" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "甲状腺肿" } ] }, { "text": "【临床表现】碘缺乏病的临床表现取决于缺碘的程度、缺碘时机体所处发育时期以及机体对缺碘的反应性或对缺碘的代偿适应能力。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "碘缺乏病" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "缺碘" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "碘" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "缺碘" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "碘" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "缺碘" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "碘" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "缺碘" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "碘" } ] }, { "text": "如碘缺乏是在儿童期,即可发生甲状腺肿。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "碘缺乏" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "甲状腺肿" } ] }, { "text": "(一)地方性甲状腺功能减退症的临床表现本病可分为三型:神经型、黏液性水肿型、混合型。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "地方性甲状腺功能减退症" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "神经" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "黏液性水肿" } ] }, { "text": "1.神经型智力呈中度及重度减退,甲状腺轻度肿大,身高可正常,表情淡漠,聋哑,多有精神缺陷,眼多斜视,四肢痉挛或瘫痪,膝关节屈曲,膝反射亢进,可出现病理反射,甲状腺功能正常或轻度低下。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "神经" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "智力呈中度及重度减退" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "甲状腺" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "甲状腺轻度肿大" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "身高" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "表情淡漠" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "聋哑" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "精神缺陷" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "眼多斜视" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "眼" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "四肢痉挛或瘫痪" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "四肢痉挛" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "瘫痪" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "膝关节屈曲" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "膝关节" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "膝反射亢进" }, { "start_idx": 64, "end_idx": 66, "type": "ite", "entity": "膝反射" }, { "start_idx": 70, "end_idx": 76, "type": "sym", "entity": "可出现病理反射" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "甲状腺" }, { "start_idx": 78, "end_idx": 89, "type": "sym", "entity": "甲状腺功能正常或轻度低下" } ] }, { "text": "2.黏液性水肿型轻度智力低下,有的能说话,侏儒状态明显,生长发育和性发育落后,有甲状腺肿大和严重的甲状腺功能低下表现,有典型的面容,便秘及黏液性水肿较突出,某些患者呈家族性发病。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "黏液性水肿" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "轻度智力低下" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "侏儒" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "生长发育和性发育落后" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "甲状腺" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "甲状腺肿大" }, { "start_idx": 46, "end_idx": 55, "type": "sym", "entity": "严重的甲状腺功能低下" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "甲状腺" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "面" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "便秘" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "黏液性水肿" } ] }, { "text": "两种类型地方性甲状腺功能减退症的临床表现比较见表4-4。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "dis", "entity": "地方性甲状腺功能减退症" } ] }, { "text": "表4-11两种类型地方性甲状腺功能减退症临床表现的比较(二)地方性甲状腺肿的临床表现主要表现为甲状腺肿大,甲状腺常呈轻度或中度弥漫性肿大,质地较软,无压痛。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "dis", "entity": "地方性甲状腺功能减退症" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "地方性甲状腺肿" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "甲状腺" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "甲状腺肿大" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "甲状腺" }, { "start_idx": 53, "end_idx": 67, "type": "sym", "entity": "甲状腺常呈轻度或中度弥漫性肿大" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "压痛" } ] }, { "text": "随着病情进展,甲状腺可逐渐增大,甚至引起压迫症状。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "甲状腺" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "甲状腺可逐渐增大" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "压迫症状" } ] }, { "text": "正常甲状腺呈“H”型,分左右两叶,附着于喉及气管起始部的两侧,于皮肤外较难触到或看到。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "甲状腺" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "左右两叶" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "喉" }, { "start_idx": 22, "end_idx": 29, "type": "bod", "entity": "气管起始部的两侧" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "皮肤外" } ] }, { "text": "当甲状腺肿大时,可根据临床诊断分度:正常:甲状腺看不见,摸不着。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "甲状腺" }, { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "甲状腺肿大" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "甲状腺" } ] }, { "text": "生理增大:头部保持正常位置时,甲状腺容易摸到,相当于人拇指末节,特点是“摸得着”。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "头部" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "甲状腺" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "拇指末节" } ] }, { "text": "Ⅰ度:头部保持正常位置时,甲状腺容易看到。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "头部" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "甲状腺" } ] }, { "text": "由超过本人拇指末节到相当于1/3个拳头,特点是“看得见”。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "拇指末节" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "拳头" } ] }, { "text": "甲状腺不超过本人拇指末节,但摸到结节时也算1度。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "甲状腺" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "拇指末节" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "结节" } ] }, { "text": "除了明显的甲状腺功能减退症和地方性甲状腺肿外,还存在着许多亚临床患者。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "甲状腺功能减退症" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "地方性甲状腺肿" } ] }, { "text": "DeQuarrain与Wegelin首先用类甲状腺功能减退症来描述亚临床患者。", "entities": [ { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "类甲状腺功能减退症" } ] }, { "text": "亚临床体格发育落后综合征:主要是身高和体重低于正常儿童,某些生理检查指标(如握力、肺活量和血压等)也偏低,少数人还有轻度骨龄发育落后,性发育落后一般不明显。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "亚临床体格发育落后综合征" }, { "start_idx": 16, "end_idx": 26, "type": "sym", "entity": "身高和体重低于正常儿童" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "身高" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "体重" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "生理检查" }, { "start_idx": 36, "end_idx": 51, "type": "sym", "entity": "(如握力、肺活量和血压等)也偏低" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "握力" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "肺活量" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "血压" }, { "start_idx": 58, "end_idx": 65, "type": "sym", "entity": "轻度骨龄发育落后" }, { "start_idx": 67, "end_idx": 71, "type": "sym", "entity": "性发育落后" } ] }, { "text": "【营养状况评价】1.尿碘习惯上根据尿碘的排出量来评价机体碘的营养状况,儿童尿碘低于100μg/24h,提示碘营养不良。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "尿碘" }, { "start_idx": 17, "end_idx": 22, "type": "ite", "entity": "尿碘的排出量" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "碘" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "尿碘" }, { "start_idx": 37, "end_idx": 49, "type": "sym", "entity": "尿碘低于100μg/24h" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "碘营养不良" } ] }, { "text": "其中,T4和FT4下降、TSH升高是碘缺乏的表现。", "entities": [ { "start_idx": 3, "end_idx": 32, "type": "sym", "entity": "T4和FT4下降" }, { "start_idx": 3, "end_idx": 15, "type": "ite", "entity": "T4" }, { "start_idx": 17, "end_idx": 30, "type": "ite", "entity": "FT4" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "TSH" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "TSH升高" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "碘缺乏" } ] }, { "text": "3.地区儿童甲状腺肿大率如地区儿童甲状腺肿大率大于5%,则提示该地区存在碘营养不良。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "地区儿童甲状腺肿大" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "地区儿童甲状腺肿大" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "碘营养不良" } ] }, { "text": "有些甲状腺肿可能是过去碘缺乏所造成,碘缺乏予以纠正以后,甲状腺肿可能需数月甚至数年才能消退,而此时尿碘则已在正常水平。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "甲状腺肿" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "碘缺乏" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "碘缺乏" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "甲状腺肿" }, { "start_idx": 49, "end_idx": 50, "type": "ite", "entity": "尿碘" } ] }, { "text": "【诊断】(一)地方性甲状腺功能减退症的诊断标准1.出生、居住于低碘地方性甲状腺肿病地区。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "dis", "entity": "地方性甲状腺功能减退症" }, { "start_idx": 31, "end_idx": 40, "type": "dis", "entity": "低碘地方性甲状腺肿病" } ] }, { "text": "4.辅助检查包括T3、T4、TSH的水平异常。", "entities": [ { "start_idx": 8, "end_idx": 43, "type": "sym", "entity": "T3、T4、TSH的水平异常" }, { "start_idx": 8, "end_idx": 20, "type": "ite", "entity": "T3" }, { "start_idx": 22, "end_idx": 34, "type": "ite", "entity": "T4" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "TSH" } ] }, { "text": "X线表现为骨龄落后,以成骨中心及骨骺不能按时出现为突出,颅骨脑回压迹增多,颅底短小,蝶鞍偶见增大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "X线" }, { "start_idx": 0, "end_idx": 47, "type": "sym", "entity": "X线表现为骨龄落后,以成骨中心及骨骺不能按时出现为突出,颅骨脑回压迹增多,颅底短小,蝶鞍偶见增大" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "骨中心" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨骺" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "颅骨脑回压迹" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "颅底" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "蝶鞍" } ] }, { "text": "如具有上述任何一项症状或体征,再加上一项辅助检查指标者,而又可排除分娩损伤、脑炎、脑膜炎及药物中毒等病史者,即可诊断为地方性甲状腺功能减退症。", "entities": [ { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "分娩损伤" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "脑炎" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "药物中毒" }, { "start_idx": 59, "end_idx": 69, "type": "dis", "entity": "地方性甲状腺功能减退症" } ] }, { "text": "(二)地方性甲状腺肿的诊断标准居住在地方性低碘甲状腺肿病的流行区,有甲状腺肿大的临床表现及相关的压迫症状,排除甲状腺肿大的其他甲状腺疾病,实验室检查表现为尿碘偏低,血浆中TSH可有不同程度增高,血浆中T4、T3浓度多属于正常,但严重患者T4身体低于正常,T3稍高,甲状腺扫描也可见弥漫型或结节性甲状腺肿大。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "地方性甲状腺肿" }, { "start_idx": 18, "end_idx": 27, "type": "dis", "entity": "地方性低碘甲状腺肿病" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "甲状腺肿大" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "压迫症状" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "甲状腺肿大" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "甲状腺疾病" }, { "start_idx": 69, "end_idx": 73, "type": "pro", "entity": "实验室检查" }, { "start_idx": 77, "end_idx": 78, "type": "ite", "entity": "尿碘" }, { "start_idx": 77, "end_idx": 80, "type": "sym", "entity": "尿碘偏低" }, { "start_idx": 82, "end_idx": 87, "type": "ite", "entity": "血浆中TSH" }, { "start_idx": 82, "end_idx": 95, "type": "sym", "entity": "血浆中TSH可有不同程度增高" }, { "start_idx": 97, "end_idx": 128, "type": "ite", "entity": "血浆中T4、T3浓度" }, { "start_idx": 140, "end_idx": 158, "type": "sym", "entity": "T4身体低于正常" }, { "start_idx": 140, "end_idx": 152, "type": "ite", "entity": "T4" }, { "start_idx": 160, "end_idx": 172, "type": "ite", "entity": "T3" }, { "start_idx": 160, "end_idx": 174, "type": "sym", "entity": "T3稍高" }, { "start_idx": 176, "end_idx": 180, "type": "pro", "entity": "甲状腺扫描" }, { "start_idx": 176, "end_idx": 195, "type": "sym", "entity": "甲状腺扫描也可见弥漫型或结节性甲状腺肿大" }, { "start_idx": 184, "end_idx": 195, "type": "dis", "entity": "弥漫型或结节性甲状腺肿大" } ] }, { "text": "食盐中加碘也是防治碘缺乏的重要措施,进入用户的食盐中碘比例1∶50000(含量不得低于20mg/kg)可有效地预防地方性甲状腺肿,1∶20000(含量为50mg/kg)可预防地方性甲状腺功能减退症。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "碘" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "碘缺乏" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "碘" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "地方性甲状腺肿" }, { "start_idx": 87, "end_idx": 97, "type": "dis", "entity": "地方性甲状腺功能减退症" } ] }, { "text": "现在,我国大部分地区食盐中已经加碘,明显减少了碘缺乏病的发生率。", "entities": [ { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "碘" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "碘缺乏病" } ] }, { "text": "(三)药物治疗可通过碘化油的口服或注射来满足机体对碘的需要。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "药物治疗" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "碘化油" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "口服" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "注射" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "碘" } ] }, { "text": "碘化油是一种长效、经济、方便、副作用小的防治药物,目前常用的是巴黎Guerter实验室的产品,名为LipodolUF的产品用于肌注,Oridol的产品用于口服。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "碘化油" }, { "start_idx": 49, "end_idx": 57, "type": "dru", "entity": "LipodolUF" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "肌注" }, { "start_idx": 66, "end_idx": 71, "type": "dru", "entity": "Oridol" }, { "start_idx": 77, "end_idx": 78, "type": "pro", "entity": "口服" } ] }, { "text": "如补碘后,甲状腺肿大仍不能控制,可采用甲状腺素制剂治疗,以补充内源性甲状腺激素不足,可使甲状腺减小或消失。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "碘" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "甲状腺" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "甲状腺肿大" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "甲状腺素制剂治疗" }, { "start_idx": 31, "end_idx": 40, "type": "sym", "entity": "内源性甲状腺激素不足" }, { "start_idx": 31, "end_idx": 38, "type": "bod", "entity": "内源性甲状腺激素" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "甲状腺" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "甲状腺减小或消失" } ] }, { "text": "(四)手术治疗一般不采取手术治疗,但甲状腺肿大严重、引起压迫症状且内科治疗无效者,可行手术治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "手术治疗" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "手术治疗" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "甲状腺肿大" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "甲状腺" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "压迫症状" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "内科治疗" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "手术治疗" } ] }, { "text": "十一、恶性间叶瘤恶性间叶瘤(malignantmesenchymoma)是一种来自间叶成分软组织肉瘤,儿童少见。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "恶性间叶瘤" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "恶性间叶瘤" }, { "start_idx": 14, "end_idx": 34, "type": "dis", "entity": "malignantmesenchymoma" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "间叶" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "软组织肉瘤" } ] }, { "text": "肿瘤有多种恶性软组织,以横纹肌肉瘤、血管内皮瘤、滑膜肉瘤、恶性神经鞘肉瘤和平滑肌肉瘤为最多。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "恶性软组织" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "血管内皮瘤" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "滑膜肉瘤" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "恶性神经鞘肉瘤" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "平滑肌肉瘤" } ] }, { "text": "因组织学多样性,故表现也多样性,总的来说很像横纹肌肉瘤,可发生于身体任何部位,以股部和腹膜后最多见。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "组织" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "股部" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "腹膜后" } ] }, { "text": "体征取决于肿瘤溃破和侵犯周围组织器官情况。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "溃破" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "周围组织器官" } ] }, { "text": "股部肿瘤可阻碍静脉回流下肢水肿腹膜后肿瘤常误诊为腹部肿瘤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "股部肿瘤" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "阻碍静脉回流" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "静脉" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "下肢" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "下肢水肿" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "腹膜后肿瘤" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "腹部肿瘤" } ] }, { "text": "术后放疗可延长复发的出现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "术后放疗" } ] }, { "text": "化疗方案同横纹肌肉瘤方案。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "第五节新生儿肾积水【病因和发病机制】胎儿泌尿生殖系畸形的超声检出率约为每1000个新生儿中2~9例,其中最常见的是肾积水,占50%~87%,引起肾积水的最常见原因是肾盂输尿管连接部的梗阻(UPJO),其他原因包括膀胱输尿管反流(VUR)、巨输尿管、后尿道瓣膜以及输尿管异位开口、输尿管囊肿等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "新生儿肾积水" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "胎儿泌尿生殖系畸形" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "超声" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "肾积水" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "肾积水" }, { "start_idx": 82, "end_idx": 92, "type": "dis", "entity": "肾盂输尿管连接部的梗阻" }, { "start_idx": 94, "end_idx": 97, "type": "dis", "entity": "UPJO" }, { "start_idx": 106, "end_idx": 112, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 114, "end_idx": 116, "type": "dis", "entity": "VUR" }, { "start_idx": 119, "end_idx": 122, "type": "dis", "entity": "巨输尿管" }, { "start_idx": 124, "end_idx": 128, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 131, "end_idx": 137, "type": "dis", "entity": "输尿管异位开口" }, { "start_idx": 139, "end_idx": 143, "type": "dis", "entity": "输尿管囊肿" } ] }, { "text": "以目前资料来看,绝大部分肾积水是生理性的。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肾积水" } ] }, { "text": "胎儿及新生儿生理性肾积水发生的主要原因是:①胎儿的尿流量相对比较高,可导致输尿管扩张;②胎儿肾盂及输尿管的顺应性较高,这与其肾盂和输尿管中弹力纤维、胶原纤维以及其他基质成分的含量和排列方式不同有关。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "胎儿及新生儿生理性肾积水" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "尿" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "尿流量相对比较高" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "输尿管" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "输尿管扩张" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肾盂" }, { "start_idx": 46, "end_idx": 57, "type": "sym", "entity": "肾盂及输尿管的顺应性较高" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "输尿管" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "肾盂" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "输尿管中" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "弹力纤维" }, { "start_idx": 74, "end_idx": 77, "type": "bod", "entity": "胶原纤维" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "其他基质" } ] }, { "text": "它们引起的肾积水可随出生后肾盂及输尿管的发育而得以改善。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肾积水" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肾盂" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "输尿管" } ] }, { "text": "从随访资料来看,随着患儿泌尿系器官的发育成熟,60%~70%的肾积水会消失或减轻,肾功能会改善,需手术的仅占20%左右。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "泌尿系器官" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "肾积水" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "肾功能" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "手术" } ] }, { "text": "美国的胎儿泌尿外科协会(SocietyforFetalUrology,SFU)提出了新生儿肾积水的分级标准,共4级(表12-12),主要依据B超检查的结果,这是目前最为广泛接受的分级标准。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dep", "entity": "泌尿外科" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "新生儿肾积水" }, { "start_idx": 70, "end_idx": 73, "type": "pro", "entity": "B超检查" } ] }, { "text": "但即使是Ⅲ、Ⅳ级的新生儿肾积水,仍有65%的可能其积水消失或减轻。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "dis", "entity": "Ⅲ、Ⅳ级的新生儿肾积水" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "积水" } ] }, { "text": "表12-7新生儿肾积水分级标准【诊断】新生儿肾积水目前尚缺乏准确、可靠的诊断评价方法,密切随访极其重要。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "新生儿肾积水" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "新生儿肾积水" } ] }, { "text": "临床常用的诊断及随访手段如下:(一)B超胎儿期肾积水的新生儿在出生2天后复查B超。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "B超" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "胎儿期肾积水" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "B超" } ] }, { "text": "如在头2天内即进行,可能会因此时生理性的少尿而使肾积水消失或程度减轻。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "生理性的少尿" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "尿" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "肾积水" } ] }, { "text": "(二)排尿性膀胱尿道造影(VCUG)膀胱输尿管反流导致的肾积水占20%~30%,它会造成严重的肾脏损害,且反流程度与肾积水程度并不一致,故肾积水的新生儿宜做VCUG。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "VCUG" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肾积水" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "肾脏损害" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "反流" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "肾积水" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "肾积水" }, { "start_idx": 78, "end_idx": 81, "type": "pro", "entity": "VCUG" } ] }, { "text": "当明确反流时,要预防性使用抗生素。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "反流" }, { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "预防性使用抗生素" } ] }, { "text": "VCUG也是观察膀胱和尿道的重要手段,男婴于胎儿期有双肾及双输尿管积水、膀胱扩张、膀胱壁增厚及羊水减少的情况要高度怀疑后尿道瓣膜的存在,出生后即应做VCUG以明确。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "VCUG" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "膀胱" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "尿道" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "双肾及双输尿管积水" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "双肾" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "双输尿管" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "膀胱扩张" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "膀胱" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "膀胱壁增厚" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "膀胱壁" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "羊水减少" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "羊水" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "VCUG" } ] }, { "text": "(三)利尿性同位素肾图可以明确患儿的分肾功能,判别同位素排泄的速度。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "利尿性同位素肾图" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "分肾功能" }, { "start_idx": 25, "end_idx": 32, "type": "ite", "entity": "同位素排泄的速度" } ] }, { "text": "也可观察肾脏及输尿管形态的改变,了解梗阻部位。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肾脏" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "输尿管" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "梗阻" } ] }, { "text": "利尿性同位素肾图宜在出生后1个月左右进行,是我们目前新生儿肾积水随访最重要的评估手段。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "利尿性同位素肾图" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "新生儿肾积水" } ] }, { "text": "(四)静脉尿路造影(IVU)国内开展小儿同位素肾图的地方并不多,且价格较高,可用IVU来观察其肾积水的程度、输尿管的情况以及梗阻的部位,也可在IVU时注射呋塞米,观察其造影剂排空情况,缺点是X线对身体的损害、造影剂过敏可能以及没有量化的指标判断分肾功能。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "静脉尿路造影" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "IVU" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "小儿同位素肾图" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "IVU" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "肾积水" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "输尿管" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "梗阻" }, { "start_idx": 71, "end_idx": 73, "type": "pro", "entity": "IVU" }, { "start_idx": 75, "end_idx": 76, "type": "pro", "entity": "注射" }, { "start_idx": 77, "end_idx": 79, "type": "dru", "entity": "呋塞米" }, { "start_idx": 95, "end_idx": 96, "type": "pro", "entity": "X线" }, { "start_idx": 98, "end_idx": 102, "type": "sym", "entity": "身体的损害" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "身体" }, { "start_idx": 104, "end_idx": 108, "type": "sym", "entity": "造影剂过敏" }, { "start_idx": 123, "end_idx": 125, "type": "ite", "entity": "肾功能" } ] }, { "text": "(五)磁共振尿路显像(MRU)泌尿系脏器的扩张积水在磁共振(MRI)的T2加权相显示为高信号,MRU可以根据尿路积水的范围来推断可能存在的梗阻部位,但不能提供功能指标。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "磁共振尿路显像" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "MRU" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "泌尿系脏器" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "泌尿系脏器的扩张积水" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "磁共振" }, { "start_idx": 30, "end_idx": 32, "type": "pro", "entity": "MRI" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "MRU" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "尿路积水" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "尿路" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "梗阻" } ] }, { "text": "在双肾功能不良,IVU不显影时MRU尤其有用。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "双肾功能不良" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "IVU" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "MRU" } ] }, { "text": "【处理】在排除后尿道瓣膜后,肾积水多可观察。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肾积水" } ] }, { "text": "肾积水的消失或减轻多发生在出生后的18个月内,一些严重的肾积水,可达30个月。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾积水" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肾积水" } ] }, { "text": "是否所有的肾积水于观察中要预防性使用抗生素尚无定论,对肾盂扩张达1cm以上的,有人建议口服阿莫西林(15mg/kg)或三甲氧苄氨嘧啶(2mg/kg),每天一次。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肾积水" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "预防性使用抗生素" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "肾盂扩张" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "口服" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "阿莫西林" }, { "start_idx": 59, "end_idx": 65, "type": "dru", "entity": "三甲氧苄氨嘧啶" } ] }, { "text": "目前多依据利尿性同位素肾图测定的分肾功能值来决定随访的间隔时间,选择手术的时机。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "利尿性同位素肾图" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "分肾功能值" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "手术" } ] }, { "text": "如无肾图指标,但B超和IVP随访中,肾积水持续发展,功能下降也可手术。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "肾图" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "B超" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "IVP" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肾积水" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "功能下降" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "手术" } ] }, { "text": "六、庚型肝炎【病原和流行病学】庚型肝炎病毒(hepatitisGvirus,HGV)属黄病毒科,有包膜和核衣壳,基因为单股正链RNA。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "庚型肝炎" }, { "start_idx": 15, "end_idx": 20, "type": "mic", "entity": "庚型肝炎病毒" }, { "start_idx": 22, "end_idx": 36, "type": "mic", "entity": "hepatitisGvirus" }, { "start_idx": 38, "end_idx": 40, "type": "mic", "entity": "HGV" }, { "start_idx": 43, "end_idx": 45, "type": "mic", "entity": "黄病毒" }, { "start_idx": 49, "end_idx": 50, "type": "mic", "entity": "包膜" }, { "start_idx": 52, "end_idx": 54, "type": "mic", "entity": "核衣壳" }, { "start_idx": 56, "end_idx": 57, "type": "mic", "entity": "基因" }, { "start_idx": 59, "end_idx": 65, "type": "mic", "entity": "单股正链RNA" } ] }, { "text": "HGV感染呈世界性分布,其高危人群和传播方式与丙型肝炎类似。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HGV感染" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "丙型肝炎" } ] }, { "text": "我国不同地区报告在非甲至非戊型肝炎患者中的检出率为2%~30%,在献血员、静脉药瘾者、血透或器官移植患者和肝癌患者中证实存在HGV感染,并发现HGV与HBV或HCV混合或重叠感染。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "mic", "entity": "非甲至非戊型肝炎" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "血" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "静脉药瘾" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "血透" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "器官移植" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "肝癌" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "HGV感染" }, { "start_idx": 71, "end_idx": 88, "type": "dis", "entity": "HGV与HBV或HCV混合或重叠感染" } ] }, { "text": "【临床特点】1.显性感染症状轻,黄疸少见,ALT升高不如丙型肝炎明显。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "显性感染" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "黄疸" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "ALT升高" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "ALT" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "丙型肝炎" } ] }, { "text": "急性肝炎较少发生慢性化。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性肝炎" } ] }, { "text": "2.约50%ALT正常,无临床症状和肝组织学异常,呈病毒携带状态。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "ALT" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肝组织" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "肝组织学异常" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "病毒" } ] }, { "text": "3.和HBV、HCV混合感染与单纯感染在临床上无明显差异。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "HBV、HCV混合感染" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "单纯感染" } ] }, { "text": "4.病毒血症待续时间长,>1~4年。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "病毒血症" } ] }, { "text": "【病原学诊断】(一)HGVRNA检查用RT-PCR法或加用Southern杂交法检测血清或肝组织中HGV,常作为HGV感染的验证试验或早期诊断手段。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "pro", "entity": "HGVRNA检查" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "RT-PCR法" }, { "start_idx": 29, "end_idx": 39, "type": "pro", "entity": "Southern杂交法" }, { "start_idx": 40, "end_idx": 51, "type": "ite", "entity": "检测血清或肝组织中HGV" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "HGV感染" } ] }, { "text": "(二)抗HGV测定多用于初筛试验或流行病学调查。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "抗HGV测定" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "初筛试验" } ] }, { "text": "【治疗】可用干扰素,但疗效不佳。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "干扰素" } ] }, { "text": "与HBV或HCV混同感染者干扰素治疗后易复发。", "entities": [ { "start_idx": 1, "end_idx": 11, "type": "dis", "entity": "HBV或HCV混同感染" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "干扰素治疗" } ] }, { "text": "第六章小儿惊厥惊厥(convulsion)是小儿时期常见的症状,小儿惊厥的发生率是成人的10~15倍,是儿科重要的急症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "小儿惊厥" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "惊厥" }, { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "convulsion" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "小儿惊厥" }, { "start_idx": 52, "end_idx": 53, "type": "dep", "entity": "儿科" } ] }, { "text": "50%惊厥持续状态发生于3岁以内,特别在第一年内最常见。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "惊厥持续状态" } ] }, { "text": "惊厥性癫痫持续所致的惊厥性脑损伤与癫痫发生为4%~40%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "惊厥性癫痫" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "惊厥性脑损伤" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "癫痫" } ] }, { "text": "【病因】(一)有热惊厥(感染性惊厥)感染性惊厥多数伴有发热,但严重感染以及某些寄生虫脑病可以不伴发热。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "热惊厥" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "感染性惊厥" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "感染性惊厥" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "发热" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "严重感染" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "寄生虫脑病" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "发热" } ] }, { "text": "感染性病因又分为颅内感染与颅外感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "感染" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "颅内感染" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "颅外感染" } ] }, { "text": "1.颅内感染各种病原如细菌、病毒、隐球菌、原虫和寄生虫等所致的脑膜炎、脑炎。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颅内感染" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "细菌" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "病毒" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "隐球菌" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "原虫" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "寄生虫" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "脑炎" } ] }, { "text": "惊厥反复发作,年龄越小,越易发生惊厥。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "惊厥" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "惊厥" } ] }, { "text": "体格检查可发现脑膜刺激征及锥体束征阳性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "脑膜刺激征及锥体束征阳性" }, { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "脑膜刺激征" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "锥体束征" } ] }, { "text": "2.颅外感染(1)热性惊厥:为小儿惊厥最常见的原因,其发生率约4%~8%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颅外感染" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "热性惊厥" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "小儿惊厥" } ] }, { "text": "清醒后可能有神经系统异常体征。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "神经系统异常" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "神经系统" } ] }, { "text": "惊厥停止7~10日后脑电图明显异常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "惊厥" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "脑电图明显异常" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "脑电图" } ] }, { "text": "某一患儿具有复杂性高热惊厥发作的次数越多,今后转为无热惊厥及癫痫的危险性愈大。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "复杂性高热惊厥" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "无热惊厥" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "癫痫" } ] }, { "text": "自贡会议明确指出凡发生以下疾病中的发热惊厥均不要诊断为高热惊厥:①中枢神经系统感染;②中枢神经系统疾病(颅脑外伤、出血、占位性病变、脑水肿和癫痫发作);③严重的全身性代谢紊乱,如缺氧、水和电解质紊乱、内分泌紊乱、低血糖、低血钙、低血镁、维生素缺乏及中毒等;④明显的遗传性疾病、出生缺陷、神经皮肤综合征(如结节性硬化)、先天性代谢异常(如苯丙酮尿症)及神经结节苷脂病;⑤新生儿期惊厥。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "发热惊厥" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "高热惊厥" }, { "start_idx": 33, "end_idx": 40, "type": "dis", "entity": "中枢神经系统感染" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "中枢神经系统疾病" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "颅脑外伤" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "出血" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "血" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "占位性病变" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "脑水肿" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "癫痫" }, { "start_idx": 77, "end_idx": 86, "type": "dis", "entity": "严重的全身性代谢紊乱" }, { "start_idx": 89, "end_idx": 90, "type": "sym", "entity": "缺氧" }, { "start_idx": 92, "end_idx": 98, "type": "dis", "entity": "水和电解质紊乱" }, { "start_idx": 100, "end_idx": 104, "type": "dis", "entity": "内分泌紊乱" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "低血糖" }, { "start_idx": 110, "end_idx": 112, "type": "dis", "entity": "低血钙" }, { "start_idx": 114, "end_idx": 116, "type": "dis", "entity": "低血镁" }, { "start_idx": 118, "end_idx": 122, "type": "dis", "entity": "维生素缺乏" }, { "start_idx": 124, "end_idx": 125, "type": "dis", "entity": "中毒" }, { "start_idx": 132, "end_idx": 136, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 138, "end_idx": 141, "type": "dis", "entity": "出生缺陷" }, { "start_idx": 143, "end_idx": 149, "type": "dis", "entity": "神经皮肤综合征" }, { "start_idx": 152, "end_idx": 156, "type": "dis", "entity": "结节性硬化" }, { "start_idx": 159, "end_idx": 165, "type": "dis", "entity": "先天性代谢异常" }, { "start_idx": 168, "end_idx": 172, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 175, "end_idx": 181, "type": "dis", "entity": "神经结节苷脂病" }, { "start_idx": 184, "end_idx": 189, "type": "dis", "entity": "新生儿期惊厥" } ] }, { "text": "(2)中毒性脑病:颅外感染所致中毒性脑病常见于重症肺炎、中毒性菌痢以及败血症等急性感染过程中出现类似脑炎的表现,但并非病原体直接侵入脑组织。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "中毒性脑病" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "颅外感染" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "中毒性脑病" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "重症肺炎" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "中毒性菌痢" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "败血症" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "急性感染" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "脑炎" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "脑组织" } ] }, { "text": "这种惊厥的特点是能找到原发病症,且发生在原发病的极期,惊厥发生次数多,持续时间长,常有意识障碍,脑脊液检查基本正常。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "惊厥" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "惊厥" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "意识障碍" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "脑脊液检查" } ] }, { "text": "(二)无热惊厥(非感染性惊厥)1.颅内疾病小儿时期原发性癫痫最为多见(其表现详见本篇第五章)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "无热惊厥" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "非感染性惊厥" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "颅内疾病" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "原发性癫痫" } ] }, { "text": "2.颅外疾病(1)代谢异常:如低血钙、低血糖、低血镁、低血钠、高血钠、维生素B1和维生素B6缺乏症,均是引起代谢紊乱的病因并有原发疾病表现。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颅外疾病" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "代谢异常" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "低血钙" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "低血糖" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "低血镁" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "低血钠" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "高血钠" }, { "start_idx": 35, "end_idx": 60, "type": "dis", "entity": "维生素B1和维生素B6缺乏症" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "代谢紊乱" } ] }, { "text": "(2)遗传代谢疾病:如苯丙酮尿症、半乳糖血症、肝豆状核变性以及黏多糖病等,较为少见。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "遗传代谢疾病" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "黏多糖病" } ] }, { "text": "(3)中毒性因素:如药物中毒(中枢兴奋药、氨茶碱、抗组胺类药物、山道年、异烟肼、阿司匹林、安乃近及氯丙嗪)、植物中毒(发芽马铃薯、白果、核仁、蓖麻子及地瓜子等)、农药中毒(有机磷农药如1605、1509、敌敌畏、敌百虫、乐果、666及DDT等)、杀鼠药及有害气体中毒等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "中毒" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "药物中毒" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "中枢兴奋药" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 25, "end_idx": 30, "type": "dru", "entity": "抗组胺类药物" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "山道年" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "异烟肼" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "安乃近" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "氯丙嗪" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "植物中毒" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "农药中毒" }, { "start_idx": 123, "end_idx": 132, "type": "dis", "entity": "杀鼠药及有害气体中毒" } ] }, { "text": "(4)其他:全身性疾病如高血压脑病、阿-斯综合征和尿毒症等,抗癫痫药物撤退,预防接种如百白破三联疫苗等均可发生惊厥。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "全身性疾病" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "高血压脑病" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "阿-斯综合征" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "尿毒症" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "抗癫痫药物" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "预防接种" }, { "start_idx": 43, "end_idx": 49, "type": "dru", "entity": "百白破三联疫苗" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "惊厥" } ] }, { "text": "【临床表现】小儿惊厥多表现为全身性发作,患儿意识丧失,全身骨骼肌不自主、持续地强直收缩,或有节律的阵挛性收缩;也可表现为部分性发作,神志清楚或意识丧失,局限于单个肢体、单侧肢体半身性惊厥,有时半身性惊厥后产生暂时性肢体瘫痪,称为Todd麻痹。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "小儿惊厥" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "意识丧失" }, { "start_idx": 27, "end_idx": 53, "type": "sym", "entity": "全身骨骼肌不自主、持续地强直收缩,或有节律的阵挛性收缩" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "全身骨骼肌" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "意识丧失" }, { "start_idx": 76, "end_idx": 92, "type": "sym", "entity": "局限于单个肢体、单侧肢体半身性惊厥" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "单个肢体" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "单侧肢体" }, { "start_idx": 88, "end_idx": 92, "type": "dis", "entity": "半身性惊厥" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "半身性惊厥" }, { "start_idx": 104, "end_idx": 110, "type": "dis", "entity": "暂时性肢体瘫痪" }, { "start_idx": 114, "end_idx": 119, "type": "dis", "entity": "Todd麻痹" } ] }, { "text": "小婴儿,特别是新生儿惊厥表现不典型,可表现为阵发性眨眼、眼球转动、斜视、凝视或上翻,面肌抽动似咀嚼、吸吮动作,口角抽动,也可以表现为阵发性面部发红、发绀或呼吸暂停而无明显的抽搐。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "新生儿惊厥" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "阵发性眨眼" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "眼" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "眼球转动" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "眼球" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "斜视" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "凝视" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "上翻" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "面肌抽动似咀嚼" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "面肌" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "吸吮动作" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "口角抽动" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "口角" }, { "start_idx": 66, "end_idx": 72, "type": "sym", "entity": "阵发性面部发红" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "面部" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "发绀" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "呼吸暂停" }, { "start_idx": 83, "end_idx": 87, "type": "sym", "entity": "明显的抽搐" } ] }, { "text": "【诊断】惊厥是一个症状,通过仔细的病史资料、全面的体格检查以及必要的实验室检查,以尽快明确惊厥的病因是感染性或非感染性,原发病在颅内还是在颅外。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "惊厥" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "体格检查" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "实验室检查" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "惊厥" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "感染" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "感染" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "颅内" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "颅外" } ] }, { "text": "(一)病史有无发热及感染伴随症状,了解惊厥的特点,惊厥发作是全身性还是局限性、惊厥持续时间、有否意识障碍以及大小便失禁,有否误服毒物或药物史。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "感染" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "惊厥" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "惊厥" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "惊厥" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "意识障碍" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "大小便失禁" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "大小便" } ] }, { "text": "①新生儿时期惊厥发作常见于缺血缺氧性脑病、颅内出血、颅脑畸形、低血糖、低血钙、低血镁、低血钠、高血钠、化脓性脑膜炎、破伤风以及高胆红素血症等;②婴儿时期惊厥常见于低血钙、化脓性脑膜炎、热性惊厥(4个月后)、中毒性脑病、低血糖及头部跌伤等;③幼儿及年长儿惊厥常见于癫痫、颅内感染、中毒性脑病及头部外伤等。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "新生儿时期惊厥" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "缺血缺氧性脑病" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "颅内出血" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "颅脑畸形" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "低血糖" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "低血钙" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "低血镁" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "低血钠" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "高血钠" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "破伤风" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "婴儿时期惊厥" }, { "start_idx": 81, "end_idx": 83, "type": "dis", "entity": "低血钙" }, { "start_idx": 85, "end_idx": 90, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 92, "end_idx": 95, "type": "dis", "entity": "热性惊厥" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "中毒性脑病" }, { "start_idx": 109, "end_idx": 111, "type": "dis", "entity": "低血糖" }, { "start_idx": 113, "end_idx": 116, "type": "dis", "entity": "头部跌伤" }, { "start_idx": 120, "end_idx": 127, "type": "dis", "entity": "幼儿及年长儿惊厥" }, { "start_idx": 131, "end_idx": 132, "type": "dis", "entity": "癫痫" }, { "start_idx": 134, "end_idx": 137, "type": "dis", "entity": "颅内感染" }, { "start_idx": 139, "end_idx": 143, "type": "dis", "entity": "中毒性脑病" }, { "start_idx": 145, "end_idx": 148, "type": "dis", "entity": "头部外伤" } ] }, { "text": "(二)体格检查惊厥发生时注意生命体征T、R、HR、BP、意识状态以及神经系统异常体征、头围测量。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "体格检查" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "惊厥" }, { "start_idx": 18, "end_idx": 18, "type": "ite", "entity": "T" }, { "start_idx": 20, "end_idx": 20, "type": "ite", "entity": "R" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "HR" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "BP" }, { "start_idx": 28, "end_idx": 31, "type": "ite", "entity": "意识状态" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "神经系统异常" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "神经系统" }, { "start_idx": 43, "end_idx": 46, "type": "ite", "entity": "头围测量" } ] }, { "text": "检查有否颅内压增高征(前囟是否紧张与饱满,颅缝是否增宽)、脑膜刺激征和阳性神经征,以及全身详细的体格检查,如皮肤有无瘀点、瘀斑,肝、脾是否肿大。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "前囟" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "颅缝" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "脑膜刺激征" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "阳性神经征" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "体格检查" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "皮肤" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "瘀点" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "瘀斑" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "肝" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "脾" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "肿大" } ] }, { "text": "有否牛奶咖啡斑、皮肤脱失斑或面部血管瘤;有否毛发或头部畸形;并观察患儿发育进程是否迟缓以帮助明确病因。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "牛奶咖啡斑" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "皮肤脱失斑" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "面部血管瘤" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "毛发或头部畸形" } ] }, { "text": "(三)实验室检查①血、尿、粪三大常规,有助于中毒性菌痢及尿路感染等感染性疾病诊断;②血生化检查,如钙、磷、钠、钾、肝、肾功能帮助了解有否代谢异常,所有惊厥病例均检查血糖,了解有否低血糖;③选择血、尿、粪及脑脊液等标本培养明确感染病原;④毒物及抗癫痫药物浓度测定;⑤疑颅内病变,选择腰椎穿刺、眼底检查、头颅B超及脑电图等检查。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "实验室检查" }, { "start_idx": 9, "end_idx": 17, "type": "ite", "entity": "血、尿、粪三大常规" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "中毒性菌痢" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "尿路感染" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 42, "end_idx": 46, "type": "ite", "entity": "血生化检查" }, { "start_idx": 49, "end_idx": 49, "type": "ite", "entity": "钙" }, { "start_idx": 51, "end_idx": 51, "type": "ite", "entity": "磷" }, { "start_idx": 53, "end_idx": 53, "type": "ite", "entity": "钠" }, { "start_idx": 55, "end_idx": 55, "type": "ite", "entity": "钾" }, { "start_idx": 57, "end_idx": 61, "type": "ite", "entity": "肝、肾功能" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "代谢异常" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "惊厥" }, { "start_idx": 80, "end_idx": 83, "type": "ite", "entity": "检查血糖" }, { "start_idx": 89, "end_idx": 91, "type": "dis", "entity": "低血糖" }, { "start_idx": 96, "end_idx": 96, "type": "ite", "entity": "血" }, { "start_idx": 98, "end_idx": 98, "type": "ite", "entity": "尿" }, { "start_idx": 100, "end_idx": 100, "type": "ite", "entity": "粪" }, { "start_idx": 102, "end_idx": 104, "type": "ite", "entity": "脑脊液" }, { "start_idx": 112, "end_idx": 113, "type": "dis", "entity": "感染" }, { "start_idx": 118, "end_idx": 129, "type": "ite", "entity": "毒物及抗癫痫药物浓度测定" }, { "start_idx": 133, "end_idx": 136, "type": "dis", "entity": "颅内病变" }, { "start_idx": 140, "end_idx": 143, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 145, "end_idx": 148, "type": "pro", "entity": "眼底检查" }, { "start_idx": 150, "end_idx": 153, "type": "pro", "entity": "头颅B超" }, { "start_idx": 155, "end_idx": 157, "type": "pro", "entity": "脑电图" } ] }, { "text": "神经影像学检查的指征为局灶性发作、异常神经系统体征以及怀疑颅内病变时;疑外伤颅内出血时,首选头颅CT;疑颅内肿瘤、颞叶病变、脑干及小脑病变和陈旧性出血时,首选MRI。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "神经影像学检查" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "异常神经系统体征" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "神经系统" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "颅内病变" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "外伤" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "颅内出血" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "头颅CT" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "颞叶病变" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "脑干及小脑病变" }, { "start_idx": 70, "end_idx": 74, "type": "dis", "entity": "陈旧性出血" }, { "start_idx": 79, "end_idx": 81, "type": "pro", "entity": "MRI" } ] }, { "text": "【治疗】(一)一般治疗保持气道通畅,及时清除咽喉部分泌物;头部侧向一侧,避免呕吐物及分泌物吸入呼吸道;吸氧以减少缺氧性脑损伤发生;退热,应用物理降温或药物降温;保持安静,避免过多的刺激。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "保持气道通畅" }, { "start_idx": 18, "end_idx": 27, "type": "pro", "entity": "及时清除咽喉部分泌物" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "头部侧向一侧" }, { "start_idx": 36, "end_idx": 49, "type": "pro", "entity": "避免呕吐物及分泌物吸入呼吸道" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "吸氧" }, { "start_idx": 54, "end_idx": 63, "type": "pro", "entity": "减少缺氧性脑损伤发生" }, { "start_idx": 65, "end_idx": 66, "type": "pro", "entity": "退热" }, { "start_idx": 70, "end_idx": 73, "type": "pro", "entity": "物理降温" }, { "start_idx": 75, "end_idx": 78, "type": "pro", "entity": "药物降温" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "保持安静" }, { "start_idx": 85, "end_idx": 91, "type": "pro", "entity": "避免过多的刺激" } ] }, { "text": "(二)止痉药物首选静脉或肌注途径:1.地西泮(安定,diazepam)为惊厥首选用药,1~3分钟起效,每次0.2~0.5mg/kg(最大剂量10mg),静脉推注,注入速度为1~1.5mg/min,作用时间5~15分钟,必要时每15~30分钟可重复使用2~3次。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "止痉药物" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "静脉" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "肌注" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "地西泮" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "安定" }, { "start_idx": 26, "end_idx": 33, "type": "dru", "entity": "diazepam" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "惊厥" }, { "start_idx": 76, "end_idx": 79, "type": "pro", "entity": "静脉推注" } ] }, { "text": "2.氯羟安定(劳拉西泮,lorazepam)与蛋白结合含量仅为安定的1/6,入脑量随之增大,止惊作用显著加强。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "氯羟安定" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "劳拉西泮" }, { "start_idx": 12, "end_idx": 20, "type": "dru", "entity": "lorazepam" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "蛋白" }, { "start_idx": 31, "end_idx": 32, "type": "dru", "entity": "安定" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "脑" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "止惊作用" } ] }, { "text": "首量0.05~0.1mg/kg,静脉注射,注速1mg/min(每次极量4mg),必要时可15分钟后重复一次。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "静脉注射" } ] }, { "text": "降低血压及抑制呼吸的不良反应比地西泮小而轻,为惊厥持续状态首选药。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "血压" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "地西泮" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "惊厥持续状态" } ] }, { "text": "3.氯硝西泮(clonazepam)亦为惊厥持续状态首选用药,起效快,作用比安定强5~10倍,维持时间长达24~48小时。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "氯硝西泮" }, { "start_idx": 7, "end_idx": 16, "type": "dru", "entity": "clonazepam" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "惊厥持续状态" }, { "start_idx": 38, "end_idx": 39, "type": "dru", "entity": "安定" } ] }, { "text": "剂量为每次0.03~0.1mg/kg,每次极量10mg,用原液或生理盐水稀释静脉推注,也可肌注。", "entities": [ { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "生理盐水" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "静脉推注" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "肌注" } ] }, { "text": "呼吸抑制发生较少,但有支气管分泌物增多和血压下降等不良反应。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "支气管分泌物增多" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "支气管分泌物" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "血压下降" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "血压" } ] }, { "text": "4.苯巴比妥(鲁米那,phenobarbital)脂溶性低,半衰期长,起效慢,静注15~20分钟开始见效,作用时间24~72小时。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "鲁米那" }, { "start_idx": 11, "end_idx": 23, "type": "dru", "entity": "phenobarbital" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "静注" } ] }, { "text": "多在地西泮用药后,首次剂量10mg/kg,若首选止惊用药时,应尽快饱和用药,即首次剂量15~20mg/kg,在12小时后给维持量每日4~5mg/kg,静脉(注速为每分钟0.5~1mg/kg)或肌肉注射。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "地西泮" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "止惊" }, { "start_idx": 75, "end_idx": 76, "type": "pro", "entity": "静脉" }, { "start_idx": 96, "end_idx": 99, "type": "pro", "entity": "肌肉注射" } ] }, { "text": "较易出现呼吸抑制和心血管系统异常,尤其是在合用安定时。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "心血管系统异常" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "心血管系统" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "安定" } ] }, { "text": "新生儿惊厥常常首选苯巴比妥,起效较快,疗效可靠,不良反应也较少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "新生儿惊厥" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "苯巴比妥" } ] }, { "text": "5.苯妥英钠(phenytoin)为惊厥持续状态的常见药,可单用,或一开始就与安定合用,或作为安定奏效后的维持用药,或继用于安定无效后,效果均好。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 7, "end_idx": 15, "type": "dru", "entity": "phenytoin" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "惊厥持续状态" }, { "start_idx": 39, "end_idx": 40, "type": "dru", "entity": "安定" }, { "start_idx": 47, "end_idx": 48, "type": "dru", "entity": "安定" }, { "start_idx": 62, "end_idx": 63, "type": "dru", "entity": "安定" } ] }, { "text": "宜用于部分性发作惊厥持续状态或脑外伤惊厥持续状态。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "部分性发作惊厥持续状态" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "脑外伤" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "惊厥持续状态" } ] }, { "text": "负荷量15~20mg/kg(注速每分钟0.5~1.0mg/kg),10~30分钟起效,2~3小时后方能止惊,必要时,2~3小时后可重复一次,作用维持12~24小时,12小时后给维持量每日5mg/kg,静脉注射,应密切注意心率、心律及血压,最好用药同时进行心电监护。", "entities": [ { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "止惊" }, { "start_idx": 100, "end_idx": 103, "type": "pro", "entity": "静脉注射" }, { "start_idx": 110, "end_idx": 111, "type": "ite", "entity": "心率" }, { "start_idx": 113, "end_idx": 114, "type": "ite", "entity": "心律" }, { "start_idx": 116, "end_idx": 117, "type": "ite", "entity": "血压" }, { "start_idx": 127, "end_idx": 130, "type": "pro", "entity": "心电监护" } ] }, { "text": "Fosphenytoin为新的水溶性苯妥英钠药物,在体内转化成苯妥英钠,两药剂量可换算(1.5mgFosphenytoin=1mgphenytoin),血压及心血管不良反应相近,但局部注射的反应如静脉炎和软组织损伤在应用Fosphenytoin时较少见。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dru", "entity": "Fosphenytoin" }, { "start_idx": 15, "end_idx": 23, "type": "dru", "entity": "水溶性苯妥英钠药物" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 76, "end_idx": 77, "type": "ite", "entity": "血压" }, { "start_idx": 79, "end_idx": 85, "type": "sym", "entity": "心血管不良反应" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "心血管" }, { "start_idx": 90, "end_idx": 93, "type": "pro", "entity": "局部注射" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "静脉炎" }, { "start_idx": 102, "end_idx": 106, "type": "dis", "entity": "软组织损伤" }, { "start_idx": 110, "end_idx": 121, "type": "dru", "entity": "Fosphenytoin" } ] }, { "text": "6.丙戊酸(valproicacid)目前常用为丙戊酸钠。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "丙戊酸" }, { "start_idx": 6, "end_idx": 17, "type": "dru", "entity": "valproicacid" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "丙戊酸钠" } ] }, { "text": "对各种惊厥发作均有效,脂溶性高,迅速入脑,首剂10~15mg/kg,静脉推注,以后每小时0.6~1mg/kg滴注,可维持24小时,注意肝功能随访。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "惊厥" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "脑" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "静脉推注" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "滴注" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "肝功能" } ] }, { "text": "7.灌肠药物当静脉用药及肌注无效或无条件注射时选用直肠保留灌肠:5%副醛每次0.3~0.4ml/kg;10%水合氯醛每次0.3~0.6ml/kg;其他脂溶性药物如地西泮和氯硝西泮、丙戊酸钠糖均可使用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "灌肠药物" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "静脉用药" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "肌注" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "注射" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "直肠保留灌肠" }, { "start_idx": 34, "end_idx": 35, "type": "dru", "entity": "副醛" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "水合氯醛" }, { "start_idx": 75, "end_idx": 79, "type": "dru", "entity": "脂溶性药物" }, { "start_idx": 81, "end_idx": 83, "type": "dru", "entity": "地西泮" }, { "start_idx": 85, "end_idx": 88, "type": "dru", "entity": "氯硝西泮" }, { "start_idx": 90, "end_idx": 94, "type": "dru", "entity": "丙戊酸钠糖" } ] }, { "text": "8.严重惊厥不止者考虑其他药物或全身麻醉药物①咪唑安定(midazolam)静注每次0.05~0.2mg/kg,1.5~5.0分钟起效,作用持续2~6小时,不良反应同安定;②硫喷妥钠(sodiumpentothal)每次10~20mg/kg,配制成1.25%~2.5%溶液,先按5mg/kg静脉缓注、余者静脉滴速为2mg/min,惊厥控制后递减滴速,应用时需严密监制呼吸、脉搏、瞳孔、意识水平及血压等生命体征;③异丙酚(propofol)负荷量为3mg/kg,维持量为每分钟100μg/kg,近年来治疗难治性惊厥获得成功;④对难治性惊厥持续状态,还可持续静脉滴注苯巴比妥0.5~3mg/(kg•h),或地西泮2mg/(kg•h),或咪唑安定,开始0.15mg/kg,然后0.5~1μg/(kg•min)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "严重惊厥" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "全身麻醉药物" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "咪唑安定" }, { "start_idx": 28, "end_idx": 36, "type": "dru", "entity": "midazolam" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "静注" }, { "start_idx": 83, "end_idx": 84, "type": "dru", "entity": "安定" }, { "start_idx": 87, "end_idx": 90, "type": "dru", "entity": "硫喷妥钠" }, { "start_idx": 92, "end_idx": 106, "type": "dru", "entity": "sodiumpentothal" }, { "start_idx": 145, "end_idx": 148, "type": "pro", "entity": "静脉缓注" }, { "start_idx": 152, "end_idx": 153, "type": "bod", "entity": "静脉" }, { "start_idx": 165, "end_idx": 166, "type": "dis", "entity": "惊厥" }, { "start_idx": 179, "end_idx": 203, "type": "pro", "entity": "严密监制呼吸、脉搏、瞳孔、意识水平及血压等生命体征" }, { "start_idx": 206, "end_idx": 208, "type": "dru", "entity": "异丙酚" }, { "start_idx": 210, "end_idx": 217, "type": "dru", "entity": "propofol" }, { "start_idx": 251, "end_idx": 255, "type": "dis", "entity": "难治性惊厥" }, { "start_idx": 263, "end_idx": 271, "type": "dis", "entity": "难治性惊厥持续状态" }, { "start_idx": 277, "end_idx": 280, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 281, "end_idx": 284, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 301, "end_idx": 303, "type": "dru", "entity": "地西泮" }, { "start_idx": 316, "end_idx": 319, "type": "dru", "entity": "咪唑安定" } ] }, { "text": "(三)惊厥持续状态的处理惊厥持续状态的预后不仅取决于不同的病因、年龄及惊厥状态本身的过程,还取决于可能出现的危及生命的病理生理改变,故治疗除有效选择抗惊厥药物治疗外,还强调综合性治疗措施:①20%甘露醇每次0.5~1g/kg静脉推注,每4~6小时1次;或复方甘油10~15ml/kg静滴,每日2次,纠正脑水肿。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "惊厥持续状态" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "惊厥持续状态" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "惊厥" }, { "start_idx": 74, "end_idx": 80, "type": "pro", "entity": "抗惊厥药物治疗" }, { "start_idx": 86, "end_idx": 90, "type": "pro", "entity": "综合性治疗" }, { "start_idx": 98, "end_idx": 100, "type": "dru", "entity": "甘露醇" }, { "start_idx": 112, "end_idx": 115, "type": "pro", "entity": "静脉推注" }, { "start_idx": 127, "end_idx": 130, "type": "dru", "entity": "复方甘油" }, { "start_idx": 141, "end_idx": 142, "type": "pro", "entity": "静滴" }, { "start_idx": 151, "end_idx": 153, "type": "dis", "entity": "脑水肿" } ] }, { "text": "②25%葡萄糖1~2g/kg,静脉推注或10%葡萄糖静注,纠正低血糖,保证氧和葡萄糖的充分供应,是治疗惊厥持续状态成功的基础。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "静脉推注" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "静注" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "低血糖" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "惊厥持续状态" } ] }, { "text": "③5%NaHCO35ml/kg,纠正酸中毒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "NaHCO" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "酸中毒" } ] }, { "text": "④防止多系统损害:如心肌损害、肾衰竭、急性肺水肿及肺部感染。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "多系统损害" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "心肌损害" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "急性肺水肿" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "肺部感染" } ] }, { "text": "四、珠蛋白生成障碍性贫血珠蛋白生成障碍性贫血(thalassemia)原称地中海贫血(Mediterraneananemia),是由于正常血红蛋白中一种或几种珠蛋白肽链合成障碍或完全抑制为特征的异常血红蛋白病,属较常见的常染色体不完全显性遗传性溶血性贫血。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "珠蛋白生成障碍性贫血" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "珠蛋白生成障碍性贫血" }, { "start_idx": 23, "end_idx": 33, "type": "dis", "entity": "thalassemia" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "地中海贫血" }, { "start_idx": 43, "end_idx": 61, "type": "dis", "entity": "Mediterraneananemia" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 79, "end_idx": 87, "type": "dis", "entity": "珠蛋白肽链合成障碍" }, { "start_idx": 97, "end_idx": 103, "type": "dis", "entity": "异常血红蛋白病" }, { "start_idx": 110, "end_idx": 126, "type": "dis", "entity": "常染色体不完全显性遗传性溶血性贫血" } ] }, { "text": "【分类及发病机制】珠蛋白生成障碍性贫血的分类有两种方法:①根据珠蛋白肽链基因缺陷情况分为α、β、γ和δ等。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "dis", "entity": "珠蛋白生成障碍性贫血" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "珠蛋白肽链基因缺陷" }, { "start_idx": 31, "end_idx": 37, "type": "bod", "entity": "珠蛋白肽链基因" } ] }, { "text": "人类各种血红蛋白的合成是由其相应的珠蛋白基因专一性控制。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "珠蛋白基因" } ] }, { "text": "正常人血红蛋白(Hb)中的珠蛋白有四种肽链,即α、β、γ和δ。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "bod", "entity": "血红蛋白(Hb)中的珠蛋白" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "四种肽链" } ] }, { "text": "根据珠蛋白肽链组合的不同而形成三种血红蛋白,即HbA(α2β2)、HbA2(α2δ2)和HbF(α2γ2)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "珠蛋白肽链" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 23, "end_idx": 43, "type": "bod", "entity": "HbA(α2β2)" }, { "start_idx": 45, "end_idx": 72, "type": "bod", "entity": "HbA2(α2δ2)" }, { "start_idx": 74, "end_idx": 94, "type": "bod", "entity": "HbF(α2γ2)" } ] }, { "text": "HbA是成人红细胞中的主要血红蛋白,约占Hb中的95%,HbA2约占成人Hb中的2%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "HbA" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "红细胞" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "Hb" }, { "start_idx": 28, "end_idx": 37, "type": "bod", "entity": "HbA2" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "Hb" } ] }, { "text": "HbF是胎儿期及早期新生儿期的主要血红蛋白,刚出生时约占新生儿Hb中的70%,2个月后降至50%,1岁时不超过5%,2岁时即降至正常成人水平(0~2%)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "HbF" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "Hb" } ] }, { "text": "珠蛋白生成障碍性贫血时,由于遗传缺陷,珠蛋白基因发生突变,致珠蛋白肽链合成障碍,根据合成受抑制的珠蛋白肽链的不同,可分为若干类型。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "珠蛋白生成障碍性贫血" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "遗传缺陷" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "珠蛋白基因发生突变" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "珠蛋白基因" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "珠蛋白肽链合成障碍" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "珠蛋白肽链" } ] }, { "text": "最常见的是β链合成障碍,称β-珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "β链合成障碍" }, { "start_idx": 13, "end_idx": 24, "type": "dis", "entity": "β-珠蛋白生成障碍性贫血" } ] }, { "text": "其次为α-链合成减少的α-珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "α-链合成减少" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "α-链" }, { "start_idx": 11, "end_idx": 22, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" } ] }, { "text": "还有较少见的δ链合成减少或δ与β链皆减少的δ或δβ以及γβ珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "δ链合成减少" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "δ链" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "δ与β链皆减少" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "δ与β链" }, { "start_idx": 21, "end_idx": 38, "type": "dis", "entity": "δ或δβ以及γβ珠蛋白生成障碍性贫血" } ] }, { "text": "【临床类型及特征】(一)α-珠蛋白生成障碍性贫血编码α-珠蛋白的基因位于第16号染色体短臂末端(16p13.3),每条染色体上有2个α基因即α1和α2,故二倍体细胞中共有4个α基因。", "entities": [ { "start_idx": 12, "end_idx": 23, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" }, { "start_idx": 24, "end_idx": 33, "type": "bod", "entity": "编码α-珠蛋白的基因" }, { "start_idx": 36, "end_idx": 55, "type": "bod", "entity": "第16号染色体短臂末端(16p13.3)" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "染色体" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "2个α基因" }, { "start_idx": 70, "end_idx": 77, "type": "bod", "entity": "α1" }, { "start_idx": 79, "end_idx": 86, "type": "bod", "entity": "α2" }, { "start_idx": 89, "end_idx": 94, "type": "bod", "entity": "二倍体细胞中" }, { "start_idx": 97, "end_idx": 101, "type": "bod", "entity": "4个α基因" } ] }, { "text": "根据α基因缺失的数量和功能障碍的情况不同又可分为五种类型。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "α基因" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "α基因缺失" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "功能障碍" } ] }, { "text": "1.静止型珠蛋白生成障碍性贫血又称α2-珠蛋白生成障碍性贫血或α+珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "静止型珠蛋白生成障碍性贫血" }, { "start_idx": 17, "end_idx": 35, "type": "dis", "entity": "α2-珠蛋白生成障碍性贫血" }, { "start_idx": 37, "end_idx": 54, "type": "dis", "entity": "α+珠蛋白生成障碍性贫血" } ] }, { "text": "由一条染色体上缺乏一个α-基因(-,α/α,α);其临床特点为患者无症状,红细胞形态正常,出生时血中HbBart's(γ4)约1%~2%,3个月后即消失。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "一条染色体上" }, { "start_idx": 1, "end_idx": 14, "type": "sym", "entity": "一条染色体上缺乏一个α-基因" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "一个α-基因" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "红细胞" }, { "start_idx": 48, "end_idx": 67, "type": "bod", "entity": "血中HbBart's(γ4)" } ] }, { "text": "2.α1-珠蛋白生成障碍性贫血或α-珠蛋白生成障碍性贫血本病也称标准型α-珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "dis", "entity": "α1-珠蛋白生成障碍性贫血" }, { "start_idx": 22, "end_idx": 33, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" }, { "start_idx": 38, "end_idx": 52, "type": "dis", "entity": "标准型α-珠蛋白生成障碍性贫血" } ] }, { "text": "由一条染色体上的两个α-基因缺失(-,-/α,α)或2个α+珠蛋白生成障碍性贫血基因(-,α/-,α)导致α链合成障碍。", "entities": [ { "start_idx": 1, "end_idx": 13, "type": "bod", "entity": "一条染色体上的两个α-基因" }, { "start_idx": 1, "end_idx": 24, "type": "sym", "entity": "一条染色体上的两个α-基因缺失(-,-/α,α)" }, { "start_idx": 26, "end_idx": 41, "type": "bod", "entity": "2个α+珠蛋白生成障碍性贫血基因" }, { "start_idx": 26, "end_idx": 50, "type": "sym", "entity": "2个α+珠蛋白生成障碍性贫血基因(-,α/-,α)" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "α链" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "α链合成障碍" } ] }, { "text": "其临床特点为患者无症状,红细胞形态有轻度改变;HbF正常,出生时HbBart占5%~6%,3个月后即消失;此型多见于HbBart胎儿水肿综合征患者的双亲和HbH病的双亲中一人。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "红细胞" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "红细胞形态有轻度改变" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "HbF" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "HbBart" }, { "start_idx": 58, "end_idx": 70, "type": "dis", "entity": "HbBart胎儿水肿综合征" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "HbH病" } ] }, { "text": "3.血红蛋白H病是α-珠蛋白生成障碍性贫血的中间型,由一条16号染色体上的一对α基因和另一条16号染色体上的一个α基因缺陷。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "血红蛋白H病" }, { "start_idx": 9, "end_idx": 20, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" }, { "start_idx": 27, "end_idx": 60, "type": "sym", "entity": "一条16号染色体上的一对α基因和另一条16号染色体上的一个α基因缺陷" }, { "start_idx": 29, "end_idx": 41, "type": "bod", "entity": "16号染色体上的一对α基因" }, { "start_idx": 46, "end_idx": 58, "type": "bod", "entity": "16号染色体上的一个α基因" } ] }, { "text": "临床特点为出现中等度或严重小细胞低色素性贫血,包涵体试验阳性。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "小细胞低色素性贫血" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "包涵体试验" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "包涵体试验阳性" } ] }, { "text": "新生儿期血中HbBart20%~30%。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "bod", "entity": "血中HbBart" }, { "start_idx": 4, "end_idx": 18, "type": "sym", "entity": "血中HbBart20%~30%" } ] }, { "text": "婴儿期以后才出现症状者,出现不同程度贫血、黄疸、肝脾肿大,发作性加重(尤以感染和药物为诱因);成熟红细胞形态改变明显;年长儿童则可出现HbH(β4)4%~20%,而HbA2及HbF含量正常。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "贫血" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "黄疸" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肝" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "脾" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "感染" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "成熟红细胞" }, { "start_idx": 47, "end_idx": 57, "type": "sym", "entity": "成熟红细胞形态改变明显" }, { "start_idx": 67, "end_idx": 79, "type": "bod", "entity": "HbH(β4)" }, { "start_idx": 67, "end_idx": 85, "type": "sym", "entity": "HbH(β4)4%~20%" }, { "start_idx": 88, "end_idx": 97, "type": "bod", "entity": "HbA2" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "HbF" } ] }, { "text": "4.HbBart胎儿水肿综合征是重型α-珠蛋白生成障碍性贫血,为α1的纯合子状态,所有二条16号染色体上的α基因均缺陷,其基因型为(-,-/-,-)。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "HbBart胎儿水肿综合征" }, { "start_idx": 16, "end_idx": 29, "type": "dis", "entity": "重型α-珠蛋白生成障碍性贫血" }, { "start_idx": 32, "end_idx": 39, "type": "bod", "entity": "α1" }, { "start_idx": 47, "end_idx": 64, "type": "sym", "entity": "所有二条16号染色体上的α基因均缺陷" }, { "start_idx": 49, "end_idx": 56, "type": "bod", "entity": "二条16号染色体" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "α基因" } ] }, { "text": "由于控制α-链合成的4个基因均缺失,故无α-链合成,因此不能合成含α-链的HbA、HbA2和HbF,在胎儿后期γ、β链各自形成大量的γ4(HbBart)和β4(HbH),同时胚胎早期ζ链合成代偿性增加并持续至整个胎儿期,并与α链组成HbPortland。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "bod", "entity": "控制α-链合成的4个基因" }, { "start_idx": 2, "end_idx": 16, "type": "sym", "entity": "控制α-链合成的4个基因均缺失" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "无α-链合成" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "α-链" }, { "start_idx": 28, "end_idx": 54, "type": "sym", "entity": "不能合成含α-链的HbA、HbA2和HbF" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "α-链" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "HbA" }, { "start_idx": 41, "end_idx": 50, "type": "bod", "entity": "HbA2" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "HbF" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "γ、β链" }, { "start_idx": 72, "end_idx": 79, "type": "bod", "entity": "γ4" }, { "start_idx": 81, "end_idx": 86, "type": "bod", "entity": "HbBart" }, { "start_idx": 89, "end_idx": 96, "type": "bod", "entity": "β4" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "HbH" }, { "start_idx": 109, "end_idx": 110, "type": "bod", "entity": "ζ链" }, { "start_idx": 130, "end_idx": 131, "type": "bod", "entity": "α链" }, { "start_idx": 134, "end_idx": 143, "type": "bod", "entity": "HbPortland" } ] }, { "text": "HbBart具高度氧亲和力且极不稳定,导致宫内胎儿严重的慢性溶血和组织严重缺氧、心力衰竭、水肿,造成流产、死胎,绝大多数于妊娠期30~40周时胎儿死于宫内或娩出后短期内死亡。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "HbBart" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "严重的慢性溶血" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "组织严重缺氧" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "水肿" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "流产" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "死胎" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "宫内" } ] }, { "text": "一旦胎儿有幸出生时全身重度水肿、腹水,重度贫血,轻度黄疸,肝大;HbBart含量70%~100%,可同时少量HbH(β4)。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "全身重度水肿" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "全身" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腹水" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "腹" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "重度贫血" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "轻度黄疸" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "肝大" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肝" }, { "start_idx": 32, "end_idx": 47, "type": "sym", "entity": "HbBart含量70%~100%" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "HbBart" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "HbH" }, { "start_idx": 58, "end_idx": 65, "type": "bod", "entity": "β4" } ] }, { "text": "本症双亲为α1珠蛋白生成障碍性贫血杂合子,同胞中的发病率约为1/4。", "entities": [ { "start_idx": 5, "end_idx": 22, "type": "dis", "entity": "α1珠蛋白生成障碍性贫血" } ] }, { "text": "5.非缺失型α-珠蛋白生成障碍性贫血α基因结构并未缺失,但其功能障碍,表达水平降低。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "dis", "entity": "非缺失型α-珠蛋白生成障碍性贫血" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "α基因" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "功能障碍" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "表达水平降低" } ] }, { "text": "临床出现与α-珠蛋白生成障碍性贫血类似的表现。", "entities": [ { "start_idx": 5, "end_idx": 16, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" } ] }, { "text": "α-珠蛋白生成障碍性贫血及其临床特征(表10-6)。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" } ] }, { "text": "表10-6α-珠蛋白生成障碍性贫血及其临床特征(二)β珠蛋白生成障碍性贫血β珠蛋白基因位于第11号染色体短臂1区2带(11p1.2)。", "entities": [ { "start_idx": 5, "end_idx": 16, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" }, { "start_idx": 26, "end_idx": 36, "type": "dis", "entity": "β珠蛋白生成障碍性贫血" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "β珠蛋白基因" }, { "start_idx": 45, "end_idx": 57, "type": "bod", "entity": "第11号染色体短臂1区2带" }, { "start_idx": 59, "end_idx": 64, "type": "bod", "entity": "11p1.2" } ] }, { "text": "本病除少数几种为几个核苷酸缺失外,绝大部分由点突变所致。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "核苷酸" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "核苷酸缺失" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "点突变" } ] }, { "text": "目前已发现β基因突变有数十种。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "β基因突变" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "β基因" } ] }, { "text": "β链合成部分受抑制者称为β+珠蛋白生成障碍性贫血,β链合成完全受阻者称为β°珠蛋白生成障碍性贫血,肽链合成抑制涉及δ链者称为δβ珠蛋白生成障碍性贫血(δβ+或δβ°)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "β链" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "β链合成部分受抑制" }, { "start_idx": 12, "end_idx": 29, "type": "dis", "entity": "β+珠蛋白生成障碍性贫血" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "β链" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "β链合成完全受阻" }, { "start_idx": 42, "end_idx": 53, "type": "dis", "entity": "β°珠蛋白生成障碍性贫血" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "肽链" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "肽链合成抑制涉及δ链" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "δ链" }, { "start_idx": 68, "end_idx": 79, "type": "dis", "entity": "δβ珠蛋白生成障碍性贫血" }, { "start_idx": 81, "end_idx": 89, "type": "dis", "entity": "δβ+" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "δβ°" } ] }, { "text": "染色体上的两个等位基因突变点相同者称为纯合子;同源染色体上只有一个突变点者称为杂合子;等位基因的突变点不同者称为双重杂合子。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "bod", "entity": "染色体上的两个等位基因" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "染色体上的两个等位基因突变点" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "纯合子" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "同源染色体" }, { "start_idx": 23, "end_idx": 35, "type": "sym", "entity": "同源染色体上只有一个突变点" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "杂合子" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "等位基因" }, { "start_idx": 43, "end_idx": 52, "type": "sym", "entity": "等位基因的突变点不同" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "双重杂合子" } ] }, { "text": "我国β珠蛋白生成障碍性贫血的发生率为0.67%,以广东、广西、云南、贵州等省地为高。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "β珠蛋白生成障碍性贫血" } ] }, { "text": "根据β肽链基因的缺失程度和β链合成受阻(部分或完成抑制)程度,β-海洋性贫血主要类型的临床及血液学特征可分为以下几种类型(表10-10)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "β肽链基因" }, { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "β肽链基因的缺失" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "β链" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "β链合成受阻" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "部分或完成抑制" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "β-海洋性贫血" } ] }, { "text": "表10-7β-海洋性贫血主要类型的临床及血液学特征根据临床表现的轻重,可将β珠蛋白生成障碍性贫血临床上分为轻型、重型及中间型三型。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "β-海洋性贫血" }, { "start_idx": 37, "end_idx": 47, "type": "dis", "entity": "β珠蛋白生成障碍性贫血" } ] }, { "text": "1.轻型是正常基因和珠蛋白生成障碍性贫血β°、β+、δβ基因的杂合子状态。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "珠蛋白生成障碍性贫血" }, { "start_idx": 20, "end_idx": 41, "type": "sym", "entity": "β°、β+、δβ基因的杂合子状态" }, { "start_idx": 20, "end_idx": 35, "type": "bod", "entity": "β°、β+、δβ基因" } ] }, { "text": "其临床特点是多数为轻度贫血(Hb100~120g/L),尤其婴儿期,可有轻度黄疸和脾大;红细胞呈大小不等、中央浅染、异形(椭圆形或靶形)、嗜碱性点彩现象等;红细胞渗透脆性降低;HbA23.5%~8.0%;HbF正常或轻度升高(<5%);预后良好;多在重型患者家族调查中被发现。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "轻度贫血" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "轻度黄疸" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "脾大" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "脾" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "红细胞" }, { "start_idx": 44, "end_idx": 67, "type": "sym", "entity": "红细胞呈大小不等、中央浅染、异形(椭圆形或靶形)" }, { "start_idx": 69, "end_idx": 75, "type": "sym", "entity": "嗜碱性点彩现象" }, { "start_idx": 78, "end_idx": 86, "type": "sym", "entity": "红细胞渗透脆性降低" }, { "start_idx": 78, "end_idx": 84, "type": "ite", "entity": "红细胞渗透脆性" }, { "start_idx": 88, "end_idx": 106, "type": "sym", "entity": "HbA23.5%~8.0%" }, { "start_idx": 88, "end_idx": 97, "type": "ite", "entity": "HbA2" }, { "start_idx": 108, "end_idx": 122, "type": "sym", "entity": "HbF正常或轻度升高(<5%)" }, { "start_idx": 108, "end_idx": 110, "type": "ite", "entity": "HbF" } ] }, { "text": "2.重型也称库理(Cooley)贫血,为β°、β+基因纯合子或双重杂合子状态。", "entities": [ { "start_idx": 6, "end_idx": 17, "type": "dis", "entity": "库理(Cooley)贫血" }, { "start_idx": 20, "end_idx": 43, "type": "sym", "entity": "β°、β+基因纯合子或双重杂合子状态" }, { "start_idx": 20, "end_idx": 32, "type": "bod", "entity": "β°、β+基因" } ] }, { "text": "常并发感染。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "感染" } ] }, { "text": "成熟红细胞形态改变明显,出现红细胞大小不等,梨形、泪滴状、小球形、三角形、靶形红细胞多见;网织红细胞增高(通常≤10%),红细胞渗透脆性降低(0.3%~0.2%以下才完全溶血),HbF含量明显增高(20%~99.6%),HbA2正常或增高;骨骼X线检查,骨髓腔增宽,皮质变薄和骨质疏松,颅骨的内外板变薄,板障加宽和短发样骨刺形成;预后差,多于5岁前死于心力衰竭和感染,经治疗者常于20岁前死于心律不齐或心力衰竭。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "成熟红细胞" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "成熟红细胞形态改变明显" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "红细胞" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "红细胞大小不等" }, { "start_idx": 22, "end_idx": 43, "type": "sym", "entity": "梨形、泪滴状、小球形、三角形、靶形红细胞多见" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "红细胞" }, { "start_idx": 45, "end_idx": 49, "type": "ite", "entity": "网织红细胞" }, { "start_idx": 45, "end_idx": 59, "type": "sym", "entity": "网织红细胞增高(通常≤10%)" }, { "start_idx": 61, "end_idx": 67, "type": "ite", "entity": "红细胞渗透脆性" }, { "start_idx": 61, "end_idx": 69, "type": "sym", "entity": "红细胞渗透脆性降低" }, { "start_idx": 71, "end_idx": 86, "type": "sym", "entity": "0.3%~0.2%以下才完全溶血" }, { "start_idx": 89, "end_idx": 93, "type": "ite", "entity": "HbF含量" }, { "start_idx": 89, "end_idx": 108, "type": "sym", "entity": "HbF含量明显增高(20%~99.6%)" }, { "start_idx": 110, "end_idx": 119, "type": "ite", "entity": "HbA2" }, { "start_idx": 110, "end_idx": 124, "type": "sym", "entity": "HbA2正常或增高" }, { "start_idx": 126, "end_idx": 131, "type": "pro", "entity": "骨骼X线检查" }, { "start_idx": 133, "end_idx": 137, "type": "sym", "entity": "骨髓腔增宽" }, { "start_idx": 133, "end_idx": 135, "type": "bod", "entity": "骨髓腔" }, { "start_idx": 139, "end_idx": 142, "type": "sym", "entity": "皮质变薄" }, { "start_idx": 139, "end_idx": 140, "type": "bod", "entity": "皮质" }, { "start_idx": 144, "end_idx": 147, "type": "sym", "entity": "骨质疏松" }, { "start_idx": 149, "end_idx": 156, "type": "sym", "entity": "颅骨的内外板变薄" }, { "start_idx": 149, "end_idx": 154, "type": "bod", "entity": "颅骨的内外板" }, { "start_idx": 158, "end_idx": 161, "type": "sym", "entity": "板障加宽" }, { "start_idx": 158, "end_idx": 159, "type": "bod", "entity": "板障" }, { "start_idx": 163, "end_idx": 169, "type": "sym", "entity": "短发样骨刺形成" }, { "start_idx": 182, "end_idx": 185, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 187, "end_idx": 188, "type": "dis", "entity": "感染" }, { "start_idx": 202, "end_idx": 205, "type": "dis", "entity": "心律不齐" }, { "start_idx": 207, "end_idx": 210, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "3.中间型是β+基因纯合子状态(高F)或δβ°珠蛋白生成障碍性贫血,其临床表现介于重型与轻型之间。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "bod", "entity": "β+基因" }, { "start_idx": 6, "end_idx": 20, "type": "sym", "entity": "β+基因纯合子状态" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "高F" }, { "start_idx": 26, "end_idx": 38, "type": "dis", "entity": "δβ°珠蛋白生成障碍性贫血" } ] }, { "text": "临床特点为发病年龄较晚(常于4~5岁);中度贫血;肝轻度大,脾轻度到中度大,常有黄疸;红细胞形态与重型类似;HbF含量约40%~70%,HbA2含量正常或降低;不需治疗,可活至成人期。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "中度贫血" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肝" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "肝轻度大" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "脾" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "脾轻度到中度大" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "黄疸" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "红细胞" }, { "start_idx": 43, "end_idx": 52, "type": "sym", "entity": "红细胞形态与重型类似" }, { "start_idx": 54, "end_idx": 58, "type": "ite", "entity": "HbF含量" }, { "start_idx": 54, "end_idx": 66, "type": "sym", "entity": "HbF含量约40%~70%" }, { "start_idx": 68, "end_idx": 84, "type": "sym", "entity": "HbA2含量正常或降低" }, { "start_idx": 68, "end_idx": 79, "type": "ite", "entity": "HbA2含量" } ] }, { "text": "【实验室检查】1.血象由于Hb合成下降而呈小细胞低色素性贫血,外周血片可见红细胞大小不等,中央浅染区扩大,出现异形、靶形、碎片红细胞和有核红细胞、点彩红细胞、嗜多染性红细胞、豪-周小体等,网织红细胞正常或增多,通常≤10%。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "血象" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "Hb" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "Hb合成下降" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "小细胞低色素性贫血" }, { "start_idx": 31, "end_idx": 43, "type": "sym", "entity": "外周血片可见红细胞大小不等" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "外周血片" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "红细胞" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "中央浅染区扩大" }, { "start_idx": 53, "end_idx": 65, "type": "sym", "entity": "出现异形、靶形、碎片红细胞" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "红细胞" }, { "start_idx": 67, "end_idx": 71, "type": "sym", "entity": "有核红细胞" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "红细胞" }, { "start_idx": 73, "end_idx": 77, "type": "sym", "entity": "点彩红细胞" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "红细胞" }, { "start_idx": 79, "end_idx": 85, "type": "sym", "entity": "嗜多染性红细胞" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "红细胞" }, { "start_idx": 87, "end_idx": 91, "type": "sym", "entity": "豪-周小体" }, { "start_idx": 94, "end_idx": 98, "type": "ite", "entity": "网织红细胞" }, { "start_idx": 94, "end_idx": 110, "type": "sym", "entity": "网织红细胞正常或增多,通常≤10%" } ] }, { "text": "2.红细胞渗透脆性试验减低,0.40%~0.38%NaCl溶液开始溶血,在0.20%或更低的低渗盐水中才完全溶血,轻型病例可正常。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "sym", "entity": "红细胞渗透脆性试验减低" }, { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "红细胞渗透脆性试验" }, { "start_idx": 14, "end_idx": 30, "type": "dru", "entity": "0.40%~0.38%NaCl溶液" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "溶血" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "血" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "低渗盐水" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "完全溶血" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "血" } ] }, { "text": "3.HbF测定这是诊断重型β珠蛋白生成障碍性贫血的重要依据。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "HbF测定" }, { "start_idx": 11, "end_idx": 23, "type": "dis", "entity": "重型β珠蛋白生成障碍性贫血" } ] }, { "text": "含量增加可达20%~99.6%,HbA2常降低、正常或中度增高。", "entities": [ { "start_idx": 16, "end_idx": 25, "type": "bod", "entity": "HbA2" }, { "start_idx": 16, "end_idx": 36, "type": "sym", "entity": "HbA2常降低、正常或中度增高" } ] }, { "text": "4.血红蛋白电泳分离出HbH或HbBart’s是确诊α珠蛋白生成障碍性贫血的重要依据。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "血红蛋白电泳" }, { "start_idx": 2, "end_idx": 22, "type": "sym", "entity": "血红蛋白电泳分离出HbH或HbBart’s" }, { "start_idx": 26, "end_idx": 36, "type": "dis", "entity": "α珠蛋白生成障碍性贫血" } ] }, { "text": "5.肽链分析采用高效液相层析分析法可测定α、β、γ、δ肽链的含量,Cooley贫血时,β/α比值<0.1(正常值为1.0~1.1)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "肽链分析" }, { "start_idx": 8, "end_idx": 16, "type": "pro", "entity": "高效液相层析分析法" }, { "start_idx": 20, "end_idx": 20, "type": "ite", "entity": "α" }, { "start_idx": 22, "end_idx": 22, "type": "ite", "entity": "β" }, { "start_idx": 24, "end_idx": 24, "type": "ite", "entity": "γ" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "δ肽链" }, { "start_idx": 33, "end_idx": 40, "type": "dis", "entity": "Cooley贫血" }, { "start_idx": 43, "end_idx": 64, "type": "sym", "entity": "β/α比值<0.1(正常值为1.0~1.1)" }, { "start_idx": 43, "end_idx": 47, "type": "ite", "entity": "β/α比值" } ] }, { "text": "6.异丙醇试验呈阳性。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "异丙醇试验" }, { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "异丙醇试验呈阳性" } ] }, { "text": "如同热不稳定试验一样,可鉴别不稳定Hb和α-珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "热不稳定试验" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "不稳定Hb" }, { "start_idx": 20, "end_idx": 31, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血" } ] }, { "text": "7.包涵体生成试验红细胞包涵体和Heinz小体可呈阳性。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "包涵体生成试验" }, { "start_idx": 2, "end_idx": 26, "type": "sym", "entity": "包涵体生成试验红细胞包涵体和Heinz小体可呈阳性" }, { "start_idx": 9, "end_idx": 14, "type": "ite", "entity": "红细胞包涵体" }, { "start_idx": 16, "end_idx": 22, "type": "ite", "entity": "Heinz小体" } ] }, { "text": "8.骨髓象红系增生明显活跃,以中、晚幼红细胞占多数,成熟红细胞形态改变与外周血相同。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "骨髓象" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "红系增生明显活跃" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "红系" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "以中、晚幼红细胞占多数" }, { "start_idx": 15, "end_idx": 21, "type": "ite", "entity": "中、晚幼红细胞" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "成熟红细胞" }, { "start_idx": 26, "end_idx": 40, "type": "sym", "entity": "成熟红细胞形态改变与外周血相同" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "外周血" } ] }, { "text": "但α-珠蛋白生成障碍性贫血静止型骨髓象可正常。", "entities": [ { "start_idx": 1, "end_idx": 15, "type": "dis", "entity": "α-珠蛋白生成障碍性贫血静止型" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "骨髓象" } ] }, { "text": "9.核酸分析测定Hb肽键的mRNA含量或通过DNA分子杂交及限制性内切酶技术鉴定患者的珠蛋白基因是否缺失。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "核酸分析" }, { "start_idx": 6, "end_idx": 18, "type": "pro", "entity": "测定Hb肽键的mRNA含量" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "DNA分子杂交" }, { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "限制性内切酶技术" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "珠蛋白基因" } ] }, { "text": "近年来应用限制性片段长度多态性(RFLP)连续分析,人工合成的寡核苷酸探针杂交及基因体外扩增(PCR)技术间接或直接进行基因诊断,可检测和鉴定突变基因。", "entities": [ { "start_idx": 5, "end_idx": 24, "type": "pro", "entity": "限制性片段长度多态性(RFLP)连续分析" }, { "start_idx": 26, "end_idx": 38, "type": "pro", "entity": "人工合成的寡核苷酸探针杂交" }, { "start_idx": 40, "end_idx": 52, "type": "pro", "entity": "基因体外扩增(PCR)技术" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "基因诊断" }, { "start_idx": 66, "end_idx": 74, "type": "pro", "entity": "检测和鉴定突变基因" } ] }, { "text": "【诊断和鉴别诊断】根据婴儿期开始呈慢性进行性贫血、脾大和特殊面容,外周血红细胞形态改变,红细胞渗透脆性减低和(或)阳性家庭史,应高度怀疑本病,进一步作血红蛋白电泳、基因分析等检查,可以确诊,但须与缺铁性贫血、遗传性球形细胞增多症、病毒性肝炎或肝硬化等病鉴别。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "慢性进行性贫血" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "脾大" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "脾" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "特殊面容" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "外周血红细胞形态改变" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "外周血红细胞" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "红细胞渗透脆性减低" }, { "start_idx": 44, "end_idx": 50, "type": "ite", "entity": "红细胞渗透脆性" }, { "start_idx": 75, "end_idx": 80, "type": "pro", "entity": "血红蛋白电泳" }, { "start_idx": 82, "end_idx": 85, "type": "pro", "entity": "基因分析" }, { "start_idx": 98, "end_idx": 102, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 104, "end_idx": 113, "type": "dis", "entity": "遗传性球形细胞增多症" }, { "start_idx": 115, "end_idx": 119, "type": "dis", "entity": "病毒性肝炎" }, { "start_idx": 121, "end_idx": 123, "type": "dis", "entity": "肝硬化" } ] }, { "text": "预防感染可防止贫血加剧。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "预防感染" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "贫血" } ] }, { "text": "(二)对症治疗1.输血是重型β-珠蛋白生成障碍性贫血的有效疗法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "对症治疗" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "输血" }, { "start_idx": 12, "end_idx": 25, "type": "dis", "entity": "重型β-珠蛋白生成障碍性贫血" } ] }, { "text": "国外采用高量输血,开始每周输一次,可输新鲜或经等渗盐水洗涤过的红细胞或压缩红细胞,反复多次使Hb升至120~140g/L。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "高量输血" }, { "start_idx": 18, "end_idx": 39, "type": "pro", "entity": "输新鲜或经等渗盐水洗涤过的红细胞或压缩红细胞" }, { "start_idx": 41, "end_idx": 59, "type": "pro", "entity": "反复多次使Hb升至120~140g/L" } ] }, { "text": "然后定期每隔4~5周1次,每次输入20ml/kg或压缩红细胞15ml/kg,维持Hb>80g/L。", "entities": [ { "start_idx": 13, "end_idx": 36, "type": "pro", "entity": "每次输入20ml/kg或压缩红细胞15ml/kg" }, { "start_idx": 38, "end_idx": 47, "type": "pro", "entity": "维持Hb>80g/L" } ] }, { "text": "这样使病人保持较高的Hb水平,以消除贫血症状,防止严重并发症的发生。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "pro", "entity": "使病人保持较高的Hb水平" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "贫血" } ] }, { "text": "2.铁螯合剂反复输血可导致继发性含铁血黄素沉着症,如反复输血>100次,血清铁饱和度>80%者应给予螯合剂去铁胺(deferoxamine)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "铁螯合剂" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "反复输血" }, { "start_idx": 13, "end_idx": 23, "type": "dis", "entity": "继发性含铁血黄素沉着症" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "反复输血" }, { "start_idx": 36, "end_idx": 41, "type": "ite", "entity": "血清铁饱和度" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "血清铁饱和度>80%" }, { "start_idx": 50, "end_idx": 55, "type": "dru", "entity": "螯合剂去铁胺" }, { "start_idx": 57, "end_idx": 68, "type": "dru", "entity": "deferoxamine" } ] }, { "text": "去铁胺可与体内过量三价铁形成大分子复合物从尿中排出。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "去铁胺" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "体内过量三价铁" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "大分子复合物" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "尿中" } ] }, { "text": "剂量每日20~40mg/kg,加在注射用水或生理盐水中静滴,可于晚上睡眠时给予,每次维持8小时,每周连用5~6晚;也可采用肌注或便携式输液泵作腹壁皮下注射,8~12小时给予。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "静滴" }, { "start_idx": 61, "end_idx": 62, "type": "pro", "entity": "肌注" }, { "start_idx": 64, "end_idx": 69, "type": "equ", "entity": "便携式输液泵" }, { "start_idx": 71, "end_idx": 76, "type": "pro", "entity": "腹壁皮下注射" } ] }, { "text": "主要不良反应为皮疹,药物过量可致长骨生长障碍、白内障等。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "皮疹" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "皮" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "长骨生长障碍" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "白内障" } ] }, { "text": "口服螯合剂去铁酮(deferiprone)也可应用,可促进铁的排出。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" }, { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "螯合剂去铁酮" }, { "start_idx": 9, "end_idx": 19, "type": "dru", "entity": "deferiprone" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "铁" } ] }, { "text": "主要不良反应为粒细胞减少、关节炎、肌肉痛等,少部分病例出现胃肠道反应,严重者应停药。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "粒细胞减少" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "粒细胞" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "关节炎" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "肌肉痛" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肌肉" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "胃肠道反应" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "胃肠道" } ] }, { "text": "有报道认为该药长期应用能增加肝脏内铁贮存,因此,长期单独应用似不足于有效预防慢性铁中毒。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "肝脏内铁" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "慢性铁中毒" } ] }, { "text": "铁螯合剂应用时,宜用大剂量维生素C口服以增加尿铁的排出。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "铁螯合剂" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "维生素C" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "口服" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "尿铁" } ] }, { "text": "3.抗氧化剂治疗可长期口服维生素E50~150mg/d,有抗红细胞膜脂质的过氧化损伤,减少溶血的作用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "抗氧化剂" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "长期口服" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "维生素E" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "抗红细胞膜脂质的过氧化损伤" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "抗红细胞膜脂质" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "溶血" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "血" } ] }, { "text": "4.脾切除或脾栓塞术可改善贫血症状和减少输血次数。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "脾切除" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "脾栓塞术" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "贫血" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "输血" } ] }, { "text": "适用于:①输血日益增多者;②巨脾引起压迫症状者;③合并脾功能亢进者。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "输血" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "巨脾引起压迫症状" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "脾" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "脾功能亢进" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "脾功能" } ] }, { "text": "脾切除一般于5~6岁后施行。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "脾切除" } ] }, { "text": "本疗法可减轻贫血症状和减少输血量。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "贫血" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "输血" } ] }, { "text": "(三)基因活化疗法应用化学药物重新活化γ珠蛋白的基因,与过量α珠蛋白结合使HbF的合成增加,改善β珠蛋白生成障碍性贫血的症状。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "基因活化疗法" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "化学药物" }, { "start_idx": 19, "end_idx": 25, "type": "bod", "entity": "γ珠蛋白的基因" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "α珠蛋白" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "HbF" }, { "start_idx": 48, "end_idx": 58, "type": "dis", "entity": "β珠蛋白生成障碍性贫血" } ] }, { "text": "化学药物有羟基脲每日15mg/kg,每3周为一疗程。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "羟基脲" } ] }, { "text": "此外可用阿糖胞苷、白消安、长春新碱等。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "阿糖胞苷" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "白消安" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "长春新碱" } ] }, { "text": "也有应用异烟肼每日15~20mg/kg,每3周为一疗程,可使过量的α珠蛋白减少。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "异烟肼" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "α珠蛋白" } ] }, { "text": "(四)造血干细胞移植异基因骨髓或脐血干细胞移植成功后的五年无病生存率达80%以上。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "造血干细胞移植" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "异基因骨髓" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "脐血干细胞移植" } ] }, { "text": "可采用HLA匹配的同胞姊妹、父母或者非亲缘供者的骨髓、G-CSF动员的外周血以及脐血造血干细胞。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "HLA" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "骨髓" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "G-CSF" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "外周血" }, { "start_idx": 40, "end_idx": 46, "type": "bod", "entity": "脐血造血干细胞" } ] }, { "text": "近年来采用新生儿脐血作pH8.34不连续醋纤膜微量电泳,可作为一种早期筛查α珠蛋白生成障碍性贫血的方法,尤其是可检出无临床症状的静止型和标准型α珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "脐血" }, { "start_idx": 11, "end_idx": 26, "type": "pro", "entity": "pH8.34不连续醋纤膜微量电泳" }, { "start_idx": 37, "end_idx": 47, "type": "dis", "entity": "α珠蛋白生成障碍性贫血" }, { "start_idx": 64, "end_idx": 81, "type": "dis", "entity": "静止型和标准型α珠蛋白生成障碍性贫血" } ] }, { "text": "开展产前诊断,用胎儿血测定α/β链比例或从胎血白细胞或绒毛细胞提取DNA制作基因图可及早确诊本病,及时终止妊娠,可减轻家庭和社会的负担,提高民族素质。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "产前诊断" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胎儿血" }, { "start_idx": 13, "end_idx": 18, "type": "ite", "entity": "α/β链比例" }, { "start_idx": 20, "end_idx": 40, "type": "pro", "entity": "从胎血白细胞或绒毛细胞提取DNA制作基因图" }, { "start_idx": 49, "end_idx": 54, "type": "pro", "entity": "及时终止妊娠" } ] }, { "text": "儿童的意外伤害事故常与居住和玩耍的周围环境密切相关,也与患儿年龄有关,新生儿容易发生窒息冻伤;婴幼儿和年长儿易发生溺水、溺粪、烫伤、呼吸道异物、电击伤和各种咬伤;至于成人的自杀,虽在儿童极少见,但近年来也有报道。", "entities": [ { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "窒息" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "冻伤" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "溺水" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "溺粪" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "烫伤" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "呼吸道异物" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "呼吸道" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "电击伤" }, { "start_idx": 78, "end_idx": 79, "type": "dis", "entity": "咬伤" } ] }, { "text": "一、淹溺参见第十二章。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "淹溺" } ] }, { "text": "五、其他革兰阴性杆菌肺炎常见的革兰阴性杆菌包括大肠埃希菌、肺炎克雷伯杆菌、铜绿假单胞菌等。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "其他革兰阴性杆菌肺炎" }, { "start_idx": 15, "end_idx": 20, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "大肠埃希菌" }, { "start_idx": 29, "end_idx": 35, "type": "mic", "entity": "肺炎克雷伯杆菌" }, { "start_idx": 37, "end_idx": 42, "type": "mic", "entity": "铜绿假单胞菌" } ] }, { "text": "本病临床过程难以与其他细菌性肺炎鉴别。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "细菌性肺炎" } ] }, { "text": "原有肺炎经适当治疗好转后又见恶化,或原发病迁延不愈,应怀疑此类肺部感染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肺炎" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "肺部感染" } ] }, { "text": "诊断主要依靠气管吸出物、血或胸水培养结果。", "entities": [ { "start_idx": 6, "end_idx": 17, "type": "pro", "entity": "气管吸出物、血或胸水培养" } ] }, { "text": "多数革兰阴性杆菌耐药率较高,一旦诊断此类感染,宜首选第三代头孢霉素或复合β-内酰胺类(含β-内酰胺酶抑制剂)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "感染" }, { "start_idx": 26, "end_idx": 32, "type": "dru", "entity": "第三代头孢霉素" }, { "start_idx": 34, "end_idx": 41, "type": "dru", "entity": "复合β-内酰胺类" }, { "start_idx": 44, "end_idx": 52, "type": "dru", "entity": "β-内酰胺酶抑制剂" } ] }, { "text": "三、呼吸机的管理(一)电源和气源1.呼吸机的电源一般为220V电压供电。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "呼吸机" }, { "start_idx": 19, "end_idx": 21, "type": "equ", "entity": "呼吸机" } ] }, { "text": "呼吸机、空气压缩机和管道加温湿化器的三个电源插头,应该分别接入墙壁插座。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "呼吸机" }, { "start_idx": 4, "end_idx": 8, "type": "equ", "entity": "空气压缩机" }, { "start_idx": 10, "end_idx": 16, "type": "equ", "entity": "管道加温湿化器" } ] }, { "text": "如果需要接线板加长,该接线板上不应再接入其他电器插头,尤其应该严格禁止再接入床旁X线摄片机,避免由于大电流变化导致呼吸机保险丝熔断。", "entities": [ { "start_idx": 40, "end_idx": 44, "type": "equ", "entity": "X线摄片机" }, { "start_idx": 57, "end_idx": 59, "type": "equ", "entity": "呼吸机" } ] }, { "text": "呼吸机本身的保险丝不可用高于3A的,以免强电流损坏呼吸机内的主控制器件。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "呼吸机" }, { "start_idx": 25, "end_idx": 27, "type": "equ", "entity": "呼吸机" } ] }, { "text": "2.呼吸机的气源气压缩泵作为驱动压力气源,其工作压力为0.4mPa1mPa=1000kPa,101.33kPa=1大气压=760mmHg,1kPa=10.2cmH2O=7.5mmHg,英制1PSI(poundspersquareinch)=6.89kPa,相当于4个大气压。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" }, { "start_idx": 8, "end_idx": 11, "type": "equ", "entity": "气压缩泵" } ] }, { "text": "在呼吸机进气接口有气水分离器,应经常检查将积水释放,防止水分进入空气-氧气混合器内导致呼吸机损坏。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "equ", "entity": "呼吸机进气接口" }, { "start_idx": 9, "end_idx": 13, "type": "equ", "entity": "气水分离器" }, { "start_idx": 32, "end_idx": 39, "type": "equ", "entity": "空气-氧气混合器" }, { "start_idx": 43, "end_idx": 45, "type": "equ", "entity": "呼吸机" } ] }, { "text": "(二)湿化器常用的加温湿化装置保证在30~33℃时有30~35mg/L(液体/气体)的蒸发量(相当于100%的相对湿度)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "湿化器" }, { "start_idx": 9, "end_idx": 14, "type": "equ", "entity": "加温湿化装置" } ] }, { "text": "但管道内容易因室内温度变化出现水凝聚,需要及时将积水瓶中的水倒出。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "equ", "entity": "积水瓶" } ] }, { "text": "对于有供气管道加温的湿化器,要求将湿化温度设置在37℃。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "equ", "entity": "供气管道" }, { "start_idx": 10, "end_idx": 12, "type": "equ", "entity": "湿化器" } ] }, { "text": "湿化罐加温至35℃,然后管道电热丝加温提高到37℃。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "湿化罐" }, { "start_idx": 12, "end_idx": 16, "type": "equ", "entity": "管道电热丝" } ] }, { "text": "此种湿化器的湿化效果最好,在供气管道末端和湿化罐上有两个温度探头,将温度反馈到湿化器控制主板,使湿化罐和管道间歇加温,保持管道温度的恒定。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "湿化器" }, { "start_idx": 14, "end_idx": 19, "type": "equ", "entity": "供气管道末端" }, { "start_idx": 21, "end_idx": 23, "type": "equ", "entity": "湿化罐" }, { "start_idx": 28, "end_idx": 31, "type": "equ", "entity": "温度探头" }, { "start_idx": 39, "end_idx": 45, "type": "equ", "entity": "湿化器控制主板" }, { "start_idx": 48, "end_idx": 50, "type": "equ", "entity": "湿化罐" } ] }, { "text": "新湿化罐要求高压灭菌消毒,对化学消毒液有严格限制,应该参考有关说明书。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "新湿化罐" }, { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "高压灭菌消毒" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "化学消毒液" } ] }, { "text": "如果出现湿化器故障且不能立即排除,应将其关闭,以避免过度加热导致患儿气道灼伤。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "equ", "entity": "湿化器" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "气道" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "气道灼伤" } ] }, { "text": "(三)呼吸机的定期检查1.日常维护呼吸机应该有专人管理,定期通电开机,检查其状态,发现问题和故障,及时排除,保持随时进入工作状态。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "呼吸机" }, { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "呼吸机" } ] }, { "text": "一般要求对患者通气回路管道及时清洗消毒。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "equ", "entity": "通气回路管道" } ] }, { "text": "日常工作中要及时清洁空气滤网,排除供气管道积水。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "equ", "entity": "空气滤网" }, { "start_idx": 17, "end_idx": 20, "type": "equ", "entity": "供气管道" } ] }, { "text": "通气回路管道老化、开裂、严重污染,应该弃去不用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "通气回路管道" } ] }, { "text": "日常工作应该保持至少二套管道。", "entities": [] }, { "text": "在呼吸机使用完毕关机前,要用21%氧(空气)通气5~10分钟,将管道系统内的高氧排除。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "呼吸机" } ] }, { "text": "第二节解脲脲原体感染【病原和流行病学】解脲脲原体是人类泌尿生殖道的致病菌。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "解脲脲原体感染" }, { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "泌尿生殖道" }, { "start_idx": 33, "end_idx": 35, "type": "mic", "entity": "致病菌" } ] }, { "text": "解脲脲原体有14个血清型,对红霉素敏感。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血清" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "红霉素" } ] }, { "text": "解脲脲原体定居于青春期后的男性和女性生殖泌尿道,主要定植于女性的阴道,男性的主要定居部位为尿道。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "生殖泌尿道" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴道" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "尿道" } ] }, { "text": "通过感染的羊水或经阴道分娩传播是新生儿获得定居的途径,定植可发生于羊膜完整和剖宫产的新生儿。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "羊水" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "阴道" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "羊膜" }, { "start_idx": 38, "end_idx": 40, "type": "pro", "entity": "剖宫产" } ] }, { "text": "【临床表现】(一)尿道炎解脲脲原体是非淋球菌性尿道炎的病原。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "尿道炎" }, { "start_idx": 12, "end_idx": 16, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "非淋球菌性尿道炎" } ] }, { "text": "由脲原体引起的尿道炎症状和体征无特异性,典型症状是尿道口有少许白色黏液样分泌物、尿痛或阴茎不适感。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "mic", "entity": "脲原体" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "尿道炎" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "尿道口" }, { "start_idx": 25, "end_idx": 38, "type": "sym", "entity": "尿道口有少许白色黏液样分泌物" }, { "start_idx": 31, "end_idx": 38, "type": "bod", "entity": "白色黏液样分泌物" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "尿痛" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "阴茎" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "阴茎不适感" } ] }, { "text": "女性尿道炎较少见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "女性尿道炎" } ] }, { "text": "(二)宫内和新生儿感染无临床表现的解脲脲原体绒毛膜炎使死胎和早产增加8倍,解脲脲原体培养阳性与严重的呼吸道功能不全、需要辅助呼吸和死亡有关。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "宫内和新生儿感染" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "解脲脲原体绒毛膜炎" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "死胎" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "早产" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "解脲脲原体培养阳性" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "解脲脲原体培养" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "呼吸道功能不全" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "辅助呼吸" } ] }, { "text": "解脲脲原体在支气管肺发育不良的发生或出生伴呼吸窘迫综合征的早产婴儿的慢性肺部疾病发生中起重要作用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "支气管肺发育不良" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "早产婴儿" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "慢性肺部疾病" } ] }, { "text": "某些研究显示,从早产儿或足月儿的脑脊液中分离到解脲脲原体,绝大多数婴儿无明显中枢神经系统感染的体征。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "早产儿" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "脑脊液" }, { "start_idx": 16, "end_idx": 27, "type": "sym", "entity": "脑脊液中分离到解脲脲原体" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "中枢神经系统感染" } ] }, { "text": "解脲脲原体引起新生儿结膜炎、肺炎、淋巴结炎、咽炎、骨髓炎亦均有报道。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "新生儿结膜炎" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肺炎" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "淋巴结炎" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "咽炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "骨髓炎" } ] }, { "text": "【诊断】对患肺炎、局部脓肿或中枢神经系统疾病的早产儿,特别是有进行性脑积水,脑脊液细胞增多或正常,血培养细菌阴性和用标准抗生素治疗无效时均应考虑进行解脲脲原体的培养。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肺炎" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "局部脓肿" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "中枢神经系统疾病" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "早产" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "进行性脑积水" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "脑脊液细胞增多或正常" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "脑脊液细胞" }, { "start_idx": 49, "end_idx": 55, "type": "sym", "entity": "血培养细菌阴性" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "血培养" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "抗生素" }, { "start_idx": 74, "end_idx": 78, "type": "mic", "entity": "解脲脲原体" } ] }, { "text": "分离需特殊培养基,标本必须立即培养或-80℃冻存,可长出典型的“油煎蛋”样菌落。", "entities": [ { "start_idx": 26, "end_idx": 38, "type": "sym", "entity": "长出典型的“油煎蛋”样菌落" }, { "start_idx": 37, "end_idx": 38, "type": "mic", "entity": "菌落" } ] }, { "text": "由于阴道和泌尿道的定植率高,故生殖道感染的诊断困难。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "阴道" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "泌尿道" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "生殖道感染" } ] }, { "text": "非淋球菌性尿道炎可进行尿道分泌物革兰染色,如每个油镜视野见到3个以上的多形核白细胞而无革兰染色阴性双球菌可确定诊断。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "非淋球菌性尿道炎" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "尿道分泌物革兰染色" }, { "start_idx": 24, "end_idx": 25, "type": "equ", "entity": "油镜" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "3个以上的多形核白细胞" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "多形核白细胞" }, { "start_idx": 43, "end_idx": 51, "type": "mic", "entity": "革兰染色阴性双球菌" } ] }, { "text": "【治疗】解脲脲原体可用红霉素,或根据培养及药物敏感结果选择用药。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "解脲脲原体" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "红霉素" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "培养及药物敏感" } ] }, { "text": "第二章遗传性疾病的诊断基础第一节染色体检查由于各种原因引起染色体的数目和结构发生改变,称染色体异常,又称之为染色体畸变综合征(chromosomalaberrationsyndrome),分为常染色体病和性染色体病两大类。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "染色体检查" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "染色体" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "染色体的数目和结构发生改变" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "染色体异常" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "染色体畸变综合征" }, { "start_idx": 63, "end_idx": 91, "type": "dis", "entity": "chromosomalaberrationsyndrome" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "常染色体病" }, { "start_idx": 102, "end_idx": 106, "type": "dis", "entity": "性染色体病" } ] }, { "text": "正常染色体数目和染色体上的基因之间的一定排列顺序,决定着人体正常发育。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "染色体" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "染色体上的基因" } ] }, { "text": "染色体异常为人类遗传性疾病的重要组成部分,包括大部分的死胎、流产、先天性畸形、生长发育障碍和性发育障碍、行为异常和智力低下。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "染色体异常" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "死胎" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "流产" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "先天性畸形" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "生长发育障碍" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "性发育障碍" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "行为异常" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "智力低下" } ] }, { "text": "染色体异常在恶性肿瘤的发病中起到重要作用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "染色体异常" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "由于染色体病的遗传物质改变较多,通常累及数个甚至上百个基因。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "染色体病" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "遗传物质改变" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "遗传物质" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "基因" } ] }, { "text": "因此,染色体疾病大多是影响多器官、多系统、表现复杂的临床综合征。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "染色体疾病" } ] }, { "text": "将一个细胞的全部染色体按标准配对排列进行分析诊断,即是核型分析。", "entities": [ { "start_idx": 0, "end_idx": 23, "type": "pro", "entity": "将一个细胞的全部染色体按标准配对排列进行分析诊断" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "核型分析" } ] }, { "text": "染色体病的发病率在新生儿约占1/200。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "染色体病" } ] }, { "text": "然而,它们仅占所有胎儿染色体异常中的一小部分,大部分的胎儿染色体异常是致死性的,在妊娠期就流产或未着床,或在围生期死亡。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "染色体异常" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "染色体异常" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "流产" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "未着床" } ] }, { "text": "妊娠头3个月的流产胎儿中大约50%~60%有染色体异常。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "流产" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "染色体异常" } ] }, { "text": "围生期死亡者有6%是染色体异常。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "染色体异常" } ] }, { "text": "孕妇年龄>35岁,且在妊娠中期进行羊膜穿刺检查的孕母中,大约2%的胎儿有染色体异常。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "羊膜穿刺检查" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "染色体异常" } ] }, { "text": "在人群中有智力低下者,也有一定数量是由染色体异常引起,如果存在其他畸形,则比例更大。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "智力低下" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "染色体异常" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "畸形" } ] }, { "text": "在表型正常的不育症夫妇中经常发现潜在染色体的异常。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "不育症" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "潜在染色体的异常" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "染色体" } ] }, { "text": "一、染色体和细胞分裂人类染色体由脱氧核糖核酸和特异蛋白质组成,它们紧密包裹在未分裂细胞的细胞核中。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "染色体" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "细胞" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "染色体" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "脱氧核糖核酸" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "特异蛋白质" }, { "start_idx": 38, "end_idx": 46, "type": "bod", "entity": "未分裂细胞的细胞核" } ] }, { "text": "除了配子以外,所有正常人类细胞核包含有23对(46条)染色体,其中22对称常染色体,另一对称性染色体。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "配子" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "细胞核" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "染色体" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "常染色体" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "对称性染色体" } ] }, { "text": "这两条性染色体,女性为两条X染色体,男性为一条X和一条Y染色体。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "两条性染色体" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "两条X染色体" }, { "start_idx": 21, "end_idx": 30, "type": "bod", "entity": "一条X和一条Y染色体" } ] }, { "text": "同一对染色体中的这两条染色体称同源染色体。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "一对染色体" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "这两条染色体" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "同源染色体" } ] }, { "text": "同源染色体中,一条来源于母方,另一条来源于父方。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "同源染色体" } ] }, { "text": "人体细胞的染色体数目是恒定的,称为二倍体(2n)。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "染色体" } ] }, { "text": "卵子和精子各含有二倍体数目的一半(即23条染色体),称为单倍体(n)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "卵子" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "精子" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "染色体" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "单倍体" } ] }, { "text": "为保持这种规律,细胞的分裂有两种形式:一为有丝分裂,当机体组织生长和修复时发生的细胞分裂;二为减数分裂,系形成生殖细胞时细胞分裂的特殊形式。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "细胞" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "细胞" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "细胞" } ] }, { "text": "在核分裂间期细胞分裂停止,染色体难以看到。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "细胞" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "染色体" } ] }, { "text": "当进入分裂前期时,染色体浓缩增厚,每条染色体纵向分裂成两个子染色体。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "染色体" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "染色体" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "子染色体" } ] }, { "text": "在分裂中期,核膜消失,染色体在赤道板上朝向核中央排列,同时,形成纺锤体并移动染色体,在这个时期,染色体最好观察,每条染色体类似于字母“X”的形状。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "核膜" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "染色体" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "核中央" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "纺锤体" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "染色体" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "染色体" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "染色体" } ] }, { "text": "分裂末期,两组子染色体分开由核膜包裹,两个新细胞分开,它们的核又进入核分裂间期。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "染色体" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "核膜" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "新细胞" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "核" } ] }, { "text": "减数分裂仅发生在生殖细胞中,并且在一次生殖中只有一次。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "生殖细胞" } ] }, { "text": "减数分裂Ⅰ是减少染色体数目分裂,即有46条染色体的母细胞分裂成两个带23条染色体的子细胞。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "染色体" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "染色体" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "母细胞" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "染色体" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "子细胞" } ] }, { "text": "减数分裂Ⅱ与有丝分裂类似,产生另外的子细胞,每个核只含有单倍体数目的染色体,在减数分裂结束时,形成四个为单倍体数目染色体的子细胞。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "子细胞" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "核" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "单倍体" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "染色体" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "单倍体" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "染色体" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "子细胞" } ] }, { "text": "在男性,每个初级精母细胞产生四个精细胞,精细胞发展成为精子;而在女性,每个卵母细胞仅形成一个卵子,剩余的产物变成无功能的极体。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "初级精母细胞" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "精细胞" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "精细胞" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "精子" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "卵母细胞" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "卵子" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "极体" } ] }, { "text": "在减数分裂过程中,每一对染色体的分配是随机的,因此,每个生殖细胞接受到母系或父系染色体的不同组合,23对染色体能够在每个配子中发生223种不同的染色体组合,加上人类基因的易变性,子代可能产生数目庞大的多样性。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "染色体" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "染色体" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "染色体" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "染色体" }, { "start_idx": 86, "end_idx": 89, "type": "bod", "entity": "人类基因" } ] }, { "text": "二、疼痛缓解与姑息治疗策略疼痛处理以全身体检及疼痛评估为基础,包括评估疼痛感觉的特征(如部位、性质、强度、持续时间)、疼痛的原发因素和继发的因素。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "疼痛缓解" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "姑息治疗" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "疼痛处理" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "全身体检" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "疼痛评估" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "疼痛" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "疼痛" } ] }, { "text": "为了使疼痛得到有效的缓解,治疗必须同时针对疼痛的原发和各种继发因素。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "疼痛" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "疼痛" } ] }, { "text": "(一)疼痛评估疼痛处理的第一步是对疼痛作评估,应将疼痛的部位、严重度、性质及持续时间视作重要的临床症状。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "疼痛评估" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "疼痛处理" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "对疼痛作评估" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "疼痛" } ] }, { "text": "婴幼儿只能通过对他们的表情、动作的观察来评估他们的疼痛程度。", "entities": [] }, { "text": "对年长儿可采用简便的疼痛量表,如0至10分数表,0为无疼痛,10为最严重的疼痛,从小到大不同的数值代表不同的疼痛严重度,他们可以告知医务人员自己评估疼痛的数值。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "疼痛量表" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "疼痛" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "疼痛" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "疼痛" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "疼痛" } ] }, { "text": "体格检查应包括对所有可能有疼痛的部位的全身综合检查,体格检查时患儿的反应——痛苦面容、退缩、强直等等可能提示疼痛。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "疼痛" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "全身综合检查" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "体格检查" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "痛苦面容" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "退缩" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "强直" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "疼痛" } ] }, { "text": "在治疗过程中应有规律地评价疼痛干预措施的有效性,必要时修改治疗计划,直至患儿疼痛缓解或至最轻程度。", "entities": [ { "start_idx": 11, "end_idx": 22, "type": "pro", "entity": "评价疼痛干预措施的有效性" }, { "start_idx": 24, "end_idx": 32, "type": "pro", "entity": "必要时修改治疗计划" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "疼痛" } ] }, { "text": "疼痛的缓解程度可用于评估生活质量和衡量其他治愈性或姑息性治疗的有益性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "疼痛" } ] }, { "text": "(二)非药物性疼痛缓解疗法是儿童癌痛治疗整体中的一部分,从诊断起即开始,贯穿整个治疗过程。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "非药物性疼痛缓解疗法" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "儿童癌痛治疗" } ] }, { "text": "这些疗法在不同环境下均可实施,它可明显地改变加剧疼痛的许多因素,如激活阻断疼痛信号的感觉系统,触发体内的疼痛抑制系统。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "疼痛" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "疼痛" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "感觉系统" }, { "start_idx": 52, "end_idx": 57, "type": "bod", "entity": "疼痛抑制系统" } ] }, { "text": "非药物性疗法作为补充而不是替代适当的药物治疗;可分为支持性、认知性、行为性和物理性。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "非药物性疗法" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "药物治疗" } ] }, { "text": "支持疗法主要促进对患儿的良好的社会心理护理,这些护理以家庭为中心,基于家庭及儿童的需要,提供与他们文化程度相适应的信息,教会他们如何协助医务人员处理患儿的疼痛和焦虑等技巧。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "支持疗法" }, { "start_idx": 6, "end_idx": 20, "type": "pro", "entity": "促进对患儿的良好的社会心理护理" }, { "start_idx": 44, "end_idx": 58, "type": "pro", "entity": "提供与他们文化程度相适应的信息" }, { "start_idx": 60, "end_idx": 84, "type": "pro", "entity": "教会他们如何协助医务人员处理患儿的疼痛和焦虑等技巧" } ] }, { "text": "认知疗法通过引导来影响患儿的思维及想象,使患儿集中注意力想象愉快的、有趣的经历,主动地分散患儿对疼痛的注意力。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "认知疗法" }, { "start_idx": 4, "end_idx": 18, "type": "pro", "entity": "通过引导来影响患儿的思维及想象" }, { "start_idx": 20, "end_idx": 38, "type": "pro", "entity": "使患儿集中注意力想象愉快的、有趣的经历" }, { "start_idx": 40, "end_idx": 53, "type": "pro", "entity": "主动地分散患儿对疼痛的注意力" } ] }, { "text": "行为疗法中深呼吸是帮助患儿减轻疼痛和自我控制疼痛的一个简单办法,它使注意力集中于深呼吸、减低肌肉紧张度、放松膈肌、增加全身供氧。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "行为疗法" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "深呼吸" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "疼痛" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "疼痛" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肌肉" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "膈肌" } ] }, { "text": "这种技术最好从教患儿从呼气开始,随着每次呼吸,紧张和恐惧感逐渐消失。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "教患儿从呼气开始" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "紧张" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "恐惧感" } ] }, { "text": "躺下后渐进性、顺序性收缩和放松肌群是对青少年有用的技术。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "pro", "entity": "躺下后渐进性、顺序性收缩和放松肌群" } ] }, { "text": "结合暗示和深呼吸,能减轻可以预见到的焦虑,辅助减轻恶心、呕吐。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "暗示" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "深呼吸" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "焦虑" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "恶心" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "呕吐" } ] }, { "text": "物理疗法包括按摩、抚触(touch)等,抚触必须合适于患儿的需要,包括抚摸、拥抱、轻摇、关怀、按摩手、背、脚、头和腹部。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "物理疗法" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "按摩" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "抚触" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "touch" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "抚触" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "抚摸" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "拥抱" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "轻摇" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "关怀" }, { "start_idx": 47, "end_idx": 58, "type": "pro", "entity": "按摩手、背、脚、头和腹部" } ] }, { "text": "冷疗和热疗也有一定疗效,冰枕可减轻癌性疼痛和炎性疼痛,也可减轻肌注等操作性疼痛。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "冷疗" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "热疗" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "冰枕" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "癌性疼痛" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "炎性疼痛" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "肌注" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "操作性疼痛" } ] }, { "text": "热敷对肌肉疼痛也有用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "热敷" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肌肉疼痛" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肌肉" } ] }, { "text": "无论是冷疗还是热疗对婴儿均不合适,因有损伤的危险。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "冷疗" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "热疗" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "损伤" } ] }, { "text": "经皮神经电刺激(TENS)仪通过置于皮肤的电极传递电刺激,刺激粗的皮下神经纤维起作用,在脊髓水平减少疼痛传递。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "equ", "entity": "经皮神经电刺激(TENS)仪" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" }, { "start_idx": 31, "end_idx": 38, "type": "bod", "entity": "粗的皮下神经纤维" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "脊髓" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "疼痛" } ] }, { "text": "在接受TENS治疗时患儿有麻木、痒感,但无痛感。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "TENS治疗" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "麻木" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "痒感" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "痛感" } ] }, { "text": "(三)药物镇痛疗法原则药物镇痛主要原则为按阶梯、按时、按合适的给药途径、按个体。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "药物镇痛疗法" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "药物镇痛" } ] }, { "text": "正确应用镇痛药物能使多数患儿疼痛缓解。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "镇痛药物" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "疼痛" } ] }, { "text": "1.“按阶梯”将疼痛分为轻、中、重度,根据疼痛严重度分三步选择相应的镇痛药,三步镇痛方法称为镇痛剂阶梯,根据患儿的疼痛水平、顺序性应用镇痛剂。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "疼痛" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "疼痛" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "镇痛药" }, { "start_idx": 38, "end_idx": 43, "type": "pro", "entity": "三步镇痛方法" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "镇痛剂" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "疼痛" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "镇痛剂" } ] }, { "text": "控制疼痛的第一步是用非阿片类镇痛剂如对乙酰氨基酚。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "疼痛" }, { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "非阿片类镇痛剂" }, { "start_idx": 18, "end_idx": 23, "type": "dru", "entity": "对乙酰氨基酚" } ] }, { "text": "第二步,假如疼痛持续可给针对轻、中度疼痛的阿片类药物如可待因,患儿可继续应用对乙酰氨基酚或一个其他的非甾体素抗炎药(NSAID)作为补充镇痛剂。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "疼痛" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "轻、中度疼痛" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "可待因" }, { "start_idx": 38, "end_idx": 43, "type": "dru", "entity": "对乙酰氨基酚" }, { "start_idx": 50, "end_idx": 56, "type": "dru", "entity": "非甾体素抗炎药" }, { "start_idx": 58, "end_idx": 62, "type": "dru", "entity": "NSAID" }, { "start_idx": 68, "end_idx": 70, "type": "dru", "entity": "镇痛剂" } ] }, { "text": "第三步,当针对轻、中度疼痛阿片类镇痛剂联合非阿片类镇痛剂失败时,应换用针对中、重度疼痛的阿片类镇痛剂如吗啡,同样可继续应用对乙酰氨基酚(或NSAID),有特殊指征时加用辅助性药物。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "轻、中度疼痛" }, { "start_idx": 21, "end_idx": 27, "type": "dru", "entity": "非阿片类镇痛剂" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "中、重度疼痛" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "阿片类镇痛剂" }, { "start_idx": 51, "end_idx": 52, "type": "dru", "entity": "吗啡" }, { "start_idx": 61, "end_idx": 66, "type": "dru", "entity": "对乙酰氨基酚" }, { "start_idx": 69, "end_idx": 73, "type": "dru", "entity": "NSAID" }, { "start_idx": 84, "end_idx": 88, "type": "dru", "entity": "辅助性药物" } ] }, { "text": "当应用针对中、重度疼痛阿片类镇痛剂时,剂量一直可以增加至疼痛缓解或出现毒性,然后可更换为同类药物中的另一个药物。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "中、重度疼痛" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "阿片类镇痛剂" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "疼痛" } ] }, { "text": "2.“按时”慢性或持续疼痛应根据计划常规定时给药,而不是需要时(p.r.n)或病人要求时给药。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "慢性或持续疼痛" }, { "start_idx": 14, "end_idx": 23, "type": "pro", "entity": "根据计划常规定时给药" } ] }, { "text": "预防疼痛反复的阿片类药物剂量低于疼痛发作时的治疗剂量,因此患儿应该有规律地接受镇痛剂,对间隙性和突发性疼痛再增加解救剂量。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "疼痛" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "疼痛" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "镇痛剂" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "间隙性和突发性疼痛" }, { "start_idx": 54, "end_idx": 59, "type": "pro", "entity": "增加解救剂量" } ] }, { "text": "给药间隙应根据疼痛的严重度和药物作用时间来决定。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "疼痛" } ] }, { "text": "3.“按合适的给药途径”应采用最简单、最有效、最无痛的给药途径,同时也应考虑到疼痛的严重度、类型、药物的强度和给药间隙。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "按合适的给药途径" }, { "start_idx": 12, "end_idx": 30, "type": "pro", "entity": "应采用最简单、最有效、最无痛的给药途径" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "疼痛" } ] }, { "text": "可依次选择口服、静脉、皮下、经皮给药。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "口服" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "静脉" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "皮下" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "经皮给药" } ] }, { "text": "肌肉注射较痛,应尽量避免。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "肌肉注射" }, { "start_idx": 5, "end_idx": 5, "type": "sym", "entity": "痛" } ] }, { "text": "病人自我控制镇痛法(PCA)是一种静脉或皮下给药的新方法,它允许7岁以上患儿自己控制给药按钮将事先设定剂量的镇痛药通过电脑控制的输液泵给药。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "自我控制镇痛法" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "PCA" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "静脉或皮下给药" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "镇痛药" }, { "start_idx": 64, "end_idx": 66, "type": "equ", "entity": "输液泵" } ] }, { "text": "为安全起见,输液泵可设定一个不应期,在不应期内输液泵拒绝给予另一重复剂量。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "输液泵" }, { "start_idx": 23, "end_idx": 25, "type": "equ", "entity": "输液泵" } ] }, { "text": "PCA可单独应用,也可和静脉持续滴注同时应用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "PCA" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "静脉持续滴注" } ] }, { "text": "4.“按个体”所有药物的剂量必须基于患儿的个体情况,能有效缓解疼痛的阿片类药物剂量在不同的患儿和同一患儿的不同疾病阶段差异很大。", "entities": [ { "start_idx": 7, "end_idx": 24, "type": "pro", "entity": "所有药物的剂量必须基于患儿的个体情况" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "疼痛" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "应常规地评估患儿的疼痛,需要时调整剂量以达到疼痛控制。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "pro", "entity": "常规地评估患儿的疼痛" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "需要时调整剂量" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "疼痛控制" } ] }, { "text": "疼痛控制满意而副作用轻微并能被辅助药处理的剂量即是合理的剂量。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "疼痛控制" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "辅助药" } ] }, { "text": "如有不能接受的副作用如嗜睡和呼吸抑制时,应试用另一种阿片类药物。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "嗜睡" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "呼吸抑制" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "各种阿片类药物之间有不完全交叉耐药性,因此当一个药物失效或毒性太大时,另一个药物可能在低剂量时即有效而副作用轻微。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "(四)缓解疼痛的特殊药物1.非阿片类镇痛剂非阿片类镇痛剂用于缓解轻度的疼痛,和阿片类药物合用可缓解中重度疼痛。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "疼痛" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "特殊药物" }, { "start_idx": 14, "end_idx": 20, "type": "dru", "entity": "非阿片类镇痛剂" }, { "start_idx": 21, "end_idx": 27, "type": "dru", "entity": "非阿片类镇痛剂" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "轻度的疼痛" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "中重度疼痛" } ] }, { "text": "对乙酰氨基酚对儿童疗效较好,无胃肠道和血液学方面的副作用,与Reye综合征亦无关,新生儿和小婴儿也能很好地耐受,推荐剂量为每4~6小时口服10~15mg/kg。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "对乙酰氨基酚" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "胃肠道" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血液" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "Reye综合征" }, { "start_idx": 67, "end_idx": 68, "type": "pro", "entity": "口服" } ] }, { "text": "也可选用布洛芬(10mg/kg,口服,q6~8h)。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "布洛芬" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "口服" } ] }, { "text": "阿司匹林和其他的NSAIDs有胃肠道副作用及抗凝作用,因此在儿童癌症患者中应用要谨慎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "NSAIDs" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "胃肠道" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "儿童癌症" } ] }, { "text": "非阿片类镇痛剂超出推荐剂量时产生“天花板”效应,此时镇痛效应不再增加,但毒副作用却明显加大。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "非阿片类镇痛剂" }, { "start_idx": 27, "end_idx": 27, "type": "sym", "entity": "痛" } ] }, { "text": "所以当一个非阿片类镇痛剂合用或不合用一个辅助药物不足以缓解、轻中度疼痛时,应加用轻、中度疼痛的阿片类药物。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dru", "entity": "非阿片类镇痛剂" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "辅助药物" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "轻中度疼痛" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "轻、中度疼痛" }, { "start_idx": 47, "end_idx": 51, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "假如疼痛为重度,应给予针对中、重度疼痛的阿片类药物。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "疼痛" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "中、重度疼痛" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "2.治疗轻、中度疼痛的阿片类镇痛剂主要有可待因,对6个月以上的患儿推荐起始剂量为0.5~1mg/kg,口服,q3~4h。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "轻、中度疼痛" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "阿片类镇痛剂" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "可待因" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "口服" } ] }, { "text": "与更强的阿片类药物一样,小于6个月患儿的推荐起始剂量为年长儿的1/4~1/3mg/kg。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "通常可待因和非阿片类药物(常为对乙酰氨基酚)固定搭配应用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "可待因" }, { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "非阿片类药物" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "对乙酰氨基酚" } ] }, { "text": "不推荐肠道外给药。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "肠道外给药" } ] }, { "text": "假如推荐剂量不能缓解疼痛,则应换用另一个更强效的阿片类药物。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "疼痛" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "表10-30无阿片类用药史病人的阿片类镇痛剂指导剂量续表a等效剂量基于成人的单剂量研究b起始剂量通常是常用的标准剂量,但并不总是基于等效原则(如氢吗啡酮口服与非肠道给药的起始剂量未按它的非肠道给药与口服等效剂量比例1∶5)。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "阿片类" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "阿片类镇痛剂" }, { "start_idx": 84, "end_idx": 87, "type": "dru", "entity": "氢吗啡酮" }, { "start_idx": 88, "end_idx": 89, "type": "pro", "entity": "口服" }, { "start_idx": 91, "end_idx": 95, "type": "pro", "entity": "非肠道给药" }, { "start_idx": 105, "end_idx": 109, "type": "pro", "entity": "非肠道给药" }, { "start_idx": 111, "end_idx": 112, "type": "pro", "entity": "口服" } ] }, { "text": "对小于6个月的婴儿起始剂量为提示(推荐)剂量的1/4~1/3,并调整至有效剂量c因为哌替啶半衰期长和其毒性产物在体内积蓄的可能,因此不推荐长期使用d每小时100μg芬太尼持续滴注接近等同于每小时2.5mg吗啡e美沙酮皮下注射时有刺激作用。", "entities": [ { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "哌替啶" }, { "start_idx": 94, "end_idx": 96, "type": "dru", "entity": "芬太尼" }, { "start_idx": 97, "end_idx": 100, "type": "pro", "entity": "持续滴注" }, { "start_idx": 114, "end_idx": 115, "type": "dru", "entity": "吗啡" }, { "start_idx": 123, "end_idx": 125, "type": "dru", "entity": "美沙酮" }, { "start_idx": 126, "end_idx": 129, "type": "pro", "entity": "皮下注射" } ] }, { "text": "开始应用美沙酮和增加剂量时均需特别谨慎,因为它的生物半衰期特别长注意:1.对不同体重采用不同药物剂量计算方法,<50kg儿童以mg/kg计算,>50kg的儿童给予“成人常用剂量”。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "美沙酮" } ] }, { "text": "2.阿片类药物耐受病人换用短半衰期阿片类药物时,新换用药物的剂量应是原药等效剂量的50%(因为不完全交叉耐药),然后调整至有效剂量。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "阿片类药物耐受" }, { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 13, "end_idx": 21, "type": "dru", "entity": "短半衰期阿片类药物" } ] }, { "text": "3.IV:静脉注射,SC:皮下3.治疗中、重度疼痛的阿片类镇痛剂缓解重度疼痛需强效阿片类镇痛剂,这些药物使用简便,多数患儿能得到有效的疼痛控制。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "IV" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "静脉注射" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "SC" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "皮下" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "中、重度疼痛" }, { "start_idx": 26, "end_idx": 31, "type": "dru", "entity": "阿片类镇痛剂" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "重度疼痛" }, { "start_idx": 39, "end_idx": 46, "type": "dru", "entity": "强效阿片类镇痛剂" }, { "start_idx": 67, "end_idx": 70, "type": "pro", "entity": "疼痛控制" } ] }, { "text": "根据疼痛原因,它们可单独应用或和非阿片类镇痛剂和(或)辅助性药物联合应用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "疼痛" }, { "start_idx": 16, "end_idx": 22, "type": "dru", "entity": "非阿片类镇痛剂" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "辅助性药物" } ] }, { "text": "强效阿片类药物无镇痛剂“天花板”效应,因此无固定上限剂量,达到满意的疼痛缓解且副作用轻微的剂量就是正确剂量。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "强效阿片类药物" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "镇痛剂" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "疼痛" } ] }, { "text": "儿童可能需要极大的剂量才能达到疼痛缓解,有时高达1000倍于标准剂量。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "疼痛" } ] }, { "text": "在疾病扩散或药物耐受增加时常需要增加剂量才能维持满意的疼痛控制效果。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "增加剂量" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "疼痛控制" } ] }, { "text": "应用阿片类药物超过7天会引起心理依赖,停药时需逐渐减量,以避免戒断症状。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心理依赖" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "停药时需逐渐减量" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "戒断症状" } ] }, { "text": "经典的减量方案是先减50%剂量维持2天,然后每2天减量25%,直至等量于口服吗啡剂量0.6mg/(kg•d)(体重低于50kg)或30mg/d(体重超过50kg)时可停药。", "entities": [ { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "口服" }, { "start_idx": 38, "end_idx": 39, "type": "dru", "entity": "吗啡" } ] }, { "text": "患儿重度营养不良、肝肾功能不全或多脏器衰竭,或已用镇静剂,其阿片类药物起始剂量应减低。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "重度营养不良" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "肝肾功能不全" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "多脏器衰竭" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "镇静剂" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "WHO推荐的强阿片类药物为吗啡,其他有氢吗啡酮、美沙酮和芬太尼。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "强阿片类药物" }, { "start_idx": 13, "end_idx": 14, "type": "dru", "entity": "吗啡" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "氢吗啡酮" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "美沙酮" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "芬太尼" } ] }, { "text": "哌替啶的毒性代谢产物会在体内积蓄,因此不推荐长期应用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "哌替啶" } ] }, { "text": "(1)吗啡:重度疼痛患儿可选用吗啡。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "吗啡" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "重度疼痛" }, { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "吗啡" } ] }, { "text": "吗啡是一个标准镇痛药物,可用它衡量其他药物镇痛特性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "吗啡" }, { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "标准镇痛药物" }, { "start_idx": 22, "end_idx": 22, "type": "sym", "entity": "痛" } ] }, { "text": "推荐起始剂量为0.15~0.3mg/kg,口服,q4h,个体化调整至疼痛缓解。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "口服" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "疼痛" } ] }, { "text": "小婴儿的吗啡药代动力学与年长儿不同,小于6个月婴儿的起始剂量(以mg/kg为基础)应为年长儿的1/4~1/3剂量。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "吗啡" } ] }, { "text": "给婴儿应用吗啡时应具备持续观察、必要时可急救的条件,以防出现延迟性呼吸抑制的副作用。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "吗啡" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "延迟性呼吸抑制" } ] }, { "text": "如采用持续静脉或皮下滴注,起始剂量为0.03mg/(kg/h),可获得恒定的镇痛效果。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "持续静脉或皮下滴注" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "镇痛" } ] }, { "text": "也可间歇性通过固定的皮下或静脉输液管给药,起始剂量为0.05~0.1mg/kg,q2~4h。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "pro", "entity": "皮下或静脉输液管给药" } ] }, { "text": "长期给药时口服吗啡(mg/kg)剂量接近于非肠道给药剂量的三倍。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "口服" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "吗啡" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "非肠道给药" } ] }, { "text": "若估计疼痛会持续较长时间,可用口服控释吗啡,8~12小时给药一次,由于服用次数少,可不打扰患儿睡眠。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "疼痛" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "口服" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "控释吗啡" } ] }, { "text": "推荐起始剂量为0.6mg/kg,q8h,或0.9mg/kg,q12h,但这种制剂的有效剂量比标准吗啡更难于调整。", "entities": [ { "start_idx": 48, "end_idx": 49, "type": "dru", "entity": "吗啡" } ] }, { "text": "为调整至合适的剂量可先口服即释吗啡q4h,经24小时剂量调整,至疼痛控制剂量,然后用控释吗啡替代,将有效控制疼痛的24小时口服吗啡总量分为q8h或q12h。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "口服" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "即释吗啡" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "疼痛" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "控释吗啡" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "疼痛" }, { "start_idx": 61, "end_idx": 62, "type": "pro", "entity": "口服" }, { "start_idx": 63, "end_idx": 64, "type": "dru", "entity": "吗啡" } ] }, { "text": "(2)氢吗啡酮:氢吗啡酮的药代动力学、有效性、毒性相似于吗啡,但非肠道给药时它的效力比吗啡要强大约6倍,口服时强8倍。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "氢吗啡酮" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "氢吗啡酮" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "吗啡" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "非肠道给药" }, { "start_idx": 43, "end_idx": 44, "type": "dru", "entity": "吗啡" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "口服" } ] }, { "text": "它可口服、直肠和非肠道给药。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "口服" }, { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "直肠和非肠道给药" } ] }, { "text": "口服与非肠道剂量比为5∶1。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" }, { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "非肠道" } ] }, { "text": "(3)美沙酮:美沙酮是一种合成的、长效的阿片类镇痛剂,当患儿由于副作用(如恶心、嗜睡)而不能耐受吗啡和氢吗啡酮时推荐应用。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "美沙酮" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "美沙酮" }, { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "阿片类镇痛剂" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "恶心" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "嗜睡" }, { "start_idx": 48, "end_idx": 49, "type": "dru", "entity": "吗啡" }, { "start_idx": 51, "end_idx": 54, "type": "dru", "entity": "氢吗啡酮" } ] }, { "text": "美沙酮半衰期长,因此需极谨慎地调整剂量以达到疼痛控制效果。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "美沙酮" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "疼痛控制" } ] }, { "text": "虽患儿在开始几天对美沙酮耐受很好,但药物缓慢地积蓄,以致在以后几天内可能出现过量症状。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "美沙酮" } ] }, { "text": "因此其他强效阿片类药物定时给药,而美沙酮则以需要时每4小时给药开始。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "强效阿片类药物" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "美沙酮" } ] }, { "text": "对任何一个应用美沙酮的患儿,在开始用药的几天和增加剂量时,均应仔细监护,经24~48小时,很好地建立了患儿的所需剂量后,可开始定时给药。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "美沙酮" } ] }, { "text": "若发生昏睡或呼吸变浅,应停用美沙酮直至患儿清醒、呼吸正常,然后将原美沙酮剂量减少50%后重新开始或延长给药间期。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "昏睡" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "呼吸变浅" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "美沙酮" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "美沙酮" } ] }, { "text": "(4)芬太尼:是一种合成阿片类药物,单剂静脉注射时作用时间比吗啡短。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "芬太尼" }, { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "合成阿片类药物" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉注射" }, { "start_idx": 30, "end_idx": 31, "type": "dru", "entity": "吗啡" } ] }, { "text": "它可应用于简短的有创性操作引起的重度疼痛,持续疼痛时可滴注。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "重度疼痛" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "持续疼痛" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "滴注" } ] }, { "text": "对于简短的操作术,术前5分钟静脉注射1~2μg/kg。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "简短的操作术" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "静脉注射" } ] }, { "text": "静脉持续滴注的推荐首剂剂量为1~2μg/(kg•h)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "静脉持续滴注" } ] }, { "text": "相对于吗啡而言,芬太尼较少引起组胺释放,因此它也可用于伴有抗组胺治疗无效的严重瘙痒症的疼痛患儿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "吗啡" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "芬太尼" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "组胺" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "抗组胺治疗" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "严重瘙痒症" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "疼痛" } ] }, { "text": "快速给药3μg/kg可引起胸壁强直和严重的换气困难。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "快速给药" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "胸壁强直" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胸壁" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "严重的换气困难" } ] }, { "text": "阿片类拮抗剂纳氯酮可逆转这一并发症。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dru", "entity": "阿片类拮抗剂纳氯酮" } ] }, { "text": "芬太尼还有经皮吸收的药贴,这种贴剂不宜用于急性疼痛。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "芬太尼" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "经皮吸收" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "急性疼痛" } ] }, { "text": "也不宜用于无阿片类药物用药史或还在调整剂量的病人。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "芬太尼药贴可应用于>12岁,体重超过50kg的慢性疼痛患儿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "芬太尼药贴" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "慢性疼痛" } ] }, { "text": "4.阿片类药物的副作用及处理所有阿片类药物引起相似的副作用,给患儿阿片类药物时应预见到这些问题并给予治疗。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "有些副作用如恶心、呕吐、嗜睡,可在开始治疗的一周内得到解决,但另一些需要积极治疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "恶心" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "嗜睡" } ] }, { "text": "如果已给合适的干预措施,但副作用持续,应试用另一种不同的阿片类药物,患儿有可能较好地耐受其副作用。", "entities": [ { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "通常阿片类药物间有不完全交叉耐药性,因此当一个药物替代另一个药物时,新药的剂量应为原药等效剂量的50%,然后再调整建立有效剂量。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "常见的副作用如下:(1)便秘:是意料中的阿片类药物副作用,尚未完全得到解决。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "便秘" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "便" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "采用适当的饮食疗法(如增加水分和食物容积),每日给予大便软化剂,结合肠道刺激剂如番泻叶,可避免或减轻便秘。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "饮食疗法" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "增加水分和食物容积" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "大便软化剂" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "肠道刺激剂" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "番泻叶" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "便秘" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "便" } ] }, { "text": "(2)恶心和(或)呕吐:当阿片类药物引起恶心和(或)呕吐时可给止吐剂如甲氧氯普胺。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "恶心" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "恶心" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "呕吐" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "止吐剂" }, { "start_idx": 35, "end_idx": 39, "type": "dru", "entity": "甲氧氯普胺" } ] }, { "text": "在少数病人中,这类药物会引起锥体外系副作用,如肌张力障碍。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "锥体外系" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肌张力障碍" } ] }, { "text": "抗组胺药可治疗肌张力障碍,如苯海拉明,它也可用于止吐。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "抗组胺药" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肌张力障碍" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "苯海拉明" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "止吐" } ] }, { "text": "(3)瘙痒:抗组胺药苯海拉明和羟嗪特别适应于阿片类药物相关的瘙痒。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "瘙痒" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "抗组胺药" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "苯海拉明" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "羟嗪特别" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "瘙痒" } ] }, { "text": "也可将阿片类药物更换为芬太尼或氧吗啡酮,这些药物引起组胺释放较少,很少引起严重的瘙痒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "芬太尼" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "氧吗啡酮" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "组胺" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "组胺释放较少" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "严重的瘙痒" } ] }, { "text": "(4)呼吸抑制:出现呼吸抑制时,应该根据患儿的个体健康状况和治疗目的来处理。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "呼吸抑制" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "如患儿处于疾病终末期,呼吸不全是濒死过程的一部分,或治疗只能加重和延长病痛折磨,那么治疗呼吸困难的企图就欠合理。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "呼吸不全" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "当呼吸抑制轻微时,简单的方法即可逆转呼吸,如刺激患儿以提醒患儿呼吸,停用下一剂阿片类药物,此后的阿片类剂量先减少50%,然后再调整至保持疼痛缓解而无呼吸抑制的剂量。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "呼吸抑制轻微" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "逆转呼吸" }, { "start_idx": 22, "end_idx": 32, "type": "pro", "entity": "刺激患儿以提醒患儿呼吸" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "阿片类" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "疼痛" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "很少需要用阿片类拮抗剂来取得药理学上的逆转。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "阿片类拮抗剂" } ] }, { "text": "但对严重的呼吸抑制应持续气道支持、给氧、给纳氯酮至呼吸抑制逆转,最好不要影响疼痛控制。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "严重的呼吸抑制" }, { "start_idx": 10, "end_idx": 23, "type": "pro", "entity": "持续气道支持、给氧、给纳氯酮" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "呼吸抑制" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "疼痛" } ] }, { "text": "纳氯酮剂量一般每1~2分钟给0.5~2μg/kg,静注。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "纳氯酮" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "静注" } ] }, { "text": "患儿对阿片类耐受越大,对纳氯酮的敏感性亦越强,有出现十分痛苦的戒断症状的风险。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "阿片类" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "纳氯酮" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "十分痛苦的戒断症状" } ] }, { "text": "因此在阿片耐药病人中,必须通过很小的剂量增减来调整纳氯酮剂量以避免预料中的戒断症状。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "阿片" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "纳氯酮" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "戒断症状" } ] }, { "text": "患儿用纳氯酮后必需持续接受监护,因为阿片类药物的效应比拮抗剂持续时间长,即纳氯酮的半衰期比任何一个阿片类药物的半衰期要短得多。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "纳氯酮" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "持续接受监护" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "拮抗剂" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "纳氯酮" }, { "start_idx": 49, "end_idx": 53, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "(5)错乱和(或)幻觉:有许多原因可引起肿瘤患儿中枢神经系统副作用表现。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "错乱" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "幻觉" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肿瘤" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "中枢神经系统副作用" } ] }, { "text": "假如通过仔细的调查发现错乱和(或)幻觉显然是与阿片类药物相关,那么应该换用其他阿片类药物或加用精神抑制类药物如氟哌啶醇(0.01~0.1mg/kg口服或静注,q8h,至最大剂量为30mg/d)。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "错乱" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "幻觉" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 47, "end_idx": 53, "type": "dru", "entity": "精神抑制类药物" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 73, "end_idx": 74, "type": "pro", "entity": "口服" }, { "start_idx": 76, "end_idx": 77, "type": "pro", "entity": "静注" } ] }, { "text": "应用精神抑制药时应谨慎,因有潜在的锥体外系副作用可能。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "精神抑制药" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "锥体外系" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "锥体外系副作用" } ] }, { "text": "(6)嗜睡:开始应用阿片类药物后由于疼痛缓解患儿入睡,如一周内嗜睡未逐渐好转或太严重,可给予精神兴奋剂如右苯丙胺或利他林(0.1mg/kg,早晨和中午各一次,这样可不影响夜间睡眠)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "嗜睡" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "疼痛" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "嗜睡" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "精神兴奋剂" }, { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "右苯丙胺" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "利他林" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "影响夜间睡眠" } ] }, { "text": "(7)阿片类药物依赖及耐药:重度癌痛患儿未能接受适当镇痛剂的主要原因通常是恐惧阿片成瘾,这种恐惧已被极大地夸张。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "重度癌痛" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "镇痛剂" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "阿片成瘾" } ] }, { "text": "当某人为了获得欣快感而不惜一切手段寻觅并使用一种药物时称之为成瘾,这与接受阿片类药物以控制疼痛的癌症患儿完全不同。", "entities": [ { "start_idx": 0, "end_idx": 25, "type": "sym", "entity": "当某人为了获得欣快感而不惜一切手段寻觅并使用一种药物" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "成瘾" }, { "start_idx": 37, "end_idx": 41, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "疼痛" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "癌症" } ] }, { "text": "躯体性依赖和耐受是一种生理现象,长期应用阿片类药物的人均有这一现象,因此需要持续用药,突然停药时患儿会出现激惹、焦虑、失眠、大量出汗、流涕、恶心、呕吐、腹部痉挛性疼痛和腹泻等症状,因此当患儿用药一周以上而不再需要阿片类药物控制疼痛时,应逐渐减量停药以避免戒断症状。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "激惹" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "焦虑" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "失眠" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "大量出汗" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "汗" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "流涕" }, { "start_idx": 68, "end_idx": 68, "type": "bod", "entity": "涕" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "恶心" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "呕吐" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "腹部" }, { "start_idx": 76, "end_idx": 82, "type": "sym", "entity": "腹部痉挛性疼痛" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "腹泻" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "腹" }, { "start_idx": 106, "end_idx": 110, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 113, "end_idx": 114, "type": "sym", "entity": "疼痛" }, { "start_idx": 127, "end_idx": 130, "type": "sym", "entity": "戒断症状" } ] }, { "text": "反复应用阿片类药物后产生耐药。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "阿片类药物" } ] }, { "text": "疼痛又可能是疾病进展的信号,因此当患儿需要增加剂量才能缓解原已控制的疼痛时,应仔细地评估患儿以确定是否有疾病进展。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "疼痛" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "疼痛" } ] }, { "text": "家长常对孩子应用阿片类药物感到担忧,特别是在需要增加剂量时,因此医务人员需向家长说明阿片类药物依赖和耐药是正常现象,并不意味患儿“成瘾”。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 42, "end_idx": 46, "type": "dru", "entity": "阿片类药物" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "成瘾" } ] }, { "text": "三、抽动-秽语综合征抽动-秽语综合征是由法国医生Tourette(1885年)对该病的起始和发展作了详细地描述,故又命名为Tourette综合征。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 62, "end_idx": 72, "type": "dis", "entity": "Tourette综合征" } ] }, { "text": "本症除抽动症状之外,可不同程度地干扰损害儿童的认知功能和发育,影响社会适应能力,近年来,引起儿科较广泛的重视和研究。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "抽动" }, { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "损害儿童的认知功能和发育" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "影响社会适应能力" }, { "start_idx": 46, "end_idx": 47, "type": "dep", "entity": "儿科" } ] }, { "text": "【流行病学】抽动秽语综合征在男孩中的发病率为0.1%~0.8%,女孩为0.01%~0.4%(Peterson,1996)。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "抽动秽语综合征" }, { "start_idx": 46, "end_idx": 53, "type": "dis", "entity": "Peterson" } ] }, { "text": "【病因病理】自首次报道本征以来,其病因至今不甚清楚,但大多数学者认为,该症是器质性疾病,一般认为与下列因素有关。", "entities": [ { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "器质性疾病" } ] }, { "text": "Cornings等(1996年)发现抽动秽语综合征为多基因遗传病,并发现在遗传家系的病人中有3种多巴胺能系的基因,即多巴胺D2受体、多巴胺羟化酶和多巴胺转运蛋白1(DAT1)的基因与此症有关联。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "抽动秽语综合征" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "多基因遗传病" }, { "start_idx": 46, "end_idx": 55, "type": "bod", "entity": "3种多巴胺能系的基因" }, { "start_idx": 58, "end_idx": 95, "type": "bod", "entity": "多巴胺D2受体、多巴胺羟化酶和多巴胺转运蛋白1(DAT1)的基因" } ] }, { "text": "(二)神经生化因素1.多巴胺本征主要病理部位可能是在纹状体多巴胺能系统的靶细胞受体,由于多巴胺活动过度或是突触后多巴胺受体超敏所致,患儿服用选择性阻滞中枢多巴胺D2受体的抗精神病药如氟哌啶醇、泰必利等能使症状减轻,而多巴胺能受体激动剂可使症状加重。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "神经" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "多巴胺" }, { "start_idx": 26, "end_idx": 40, "type": "bod", "entity": "纹状体多巴胺能系统的靶细胞受体" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "多巴胺活动过度" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "多巴胺" }, { "start_idx": 53, "end_idx": 62, "type": "sym", "entity": "突触后多巴胺受体超敏" }, { "start_idx": 53, "end_idx": 60, "type": "bod", "entity": "突触后多巴胺受体" }, { "start_idx": 73, "end_idx": 95, "type": "dru", "entity": "阻滞中枢多巴胺D2受体的抗精神病药" }, { "start_idx": 97, "end_idx": 100, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 102, "end_idx": 104, "type": "dru", "entity": "泰必利" }, { "start_idx": 114, "end_idx": 122, "type": "dru", "entity": "多巴胺能受体激动剂" } ] }, { "text": "此外,患儿脑脊液中多巴胺代谢产物高香草酸的减少程度与症状严重程度呈明显的相关,说明中枢神经系统多巴胺的功能改变与本症之间有一定的关系。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脑脊液" }, { "start_idx": 9, "end_idx": 19, "type": "bod", "entity": "多巴胺代谢产物高香草酸" }, { "start_idx": 41, "end_idx": 49, "type": "bod", "entity": "中枢神经系统多巴胺" }, { "start_idx": 41, "end_idx": 54, "type": "sym", "entity": "中枢神经系统多巴胺的功能改变" } ] }, { "text": "2.去甲肾上腺素有报道此症与去甲肾上腺素系统受累有关。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "去甲肾上腺素" }, { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "去甲肾上腺素系统" } ] }, { "text": "盐酸可乐定是一种α肾上腺素能受体阻滞剂,患儿服用此药可使症状缓解或症状减轻。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "盐酸可乐定" }, { "start_idx": 8, "end_idx": 18, "type": "dru", "entity": "α肾上腺素能受体阻滞剂" } ] }, { "text": "据研究小剂量的盐酸可乐定刺激突触前α受体,反馈抑制中枢蓝斑区去甲肾上腺素的合成释放,故使抽动症状减轻。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "盐酸可乐定" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "突触前α受体" }, { "start_idx": 26, "end_idx": 35, "type": "bod", "entity": "枢蓝斑区去甲肾上腺素" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "抽动" } ] }, { "text": "3.5-羟色胺有人认为本症与5-羟色胺代谢紊乱有关。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "5-羟色胺" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "5-羟色胺代谢紊乱" } ] }, { "text": "5-羟色胺能的纤维从中缝核放射到纹状体、黑质及皮质。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "5-羟色胺能的纤维" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "中缝核" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "纹状体" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "黑质" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "皮质" } ] }, { "text": "据动物实验表明氯丙米嗪可以增加脑内5-羟色胺水平,临床使用氯丙米嗪治疗本症伴有强迫症患儿效果较好,但也有报告未见效。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "氯丙米嗪" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "脑内5-羟色胺" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "氯丙米嗪" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "强迫症" } ] }, { "text": "4.γ-氨基丁酸(GABA)GABA能细胞存在于大脑皮质及基底节各区,属于中枢抑制性神经元。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "γ-氨基丁酸" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "GABA" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "GABA" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "细胞" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "大脑皮质" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "基底节" }, { "start_idx": 37, "end_idx": 44, "type": "bod", "entity": "中枢抑制性神经元" } ] }, { "text": "抽动-秽语综合征可能是脑内GABA抑制功能降低,引起皮质谷氨酸能兴奋性增加。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "脑内GABA抑制功能降低" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "脑内GABA" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "皮质谷氨酸" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "皮质谷氨酸能兴奋性增加" } ] }, { "text": "临床上用苯二氮类药物具有加强GABA抑制作用,从而减轻抽动症状。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "苯二氮类药物" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "GABA" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "抽动" } ] }, { "text": "近年来的研究认为该症与脑基底神经节及其与皮质、丘脑和中脑的联系功能障碍有关,患儿的行为运动改变与杏仁核纹状体通路障碍有关,而不自主发声则可能与扣带回基底节及脑干不规则放电有关。", "entities": [ { "start_idx": 11, "end_idx": 34, "type": "dis", "entity": "脑基底神经节及其与皮质、丘脑和中脑的联系功能障碍" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "行为运动改变" }, { "start_idx": 48, "end_idx": 57, "type": "dis", "entity": "杏仁核纹状体通路障碍" }, { "start_idx": 62, "end_idx": 66, "type": "sym", "entity": "不自主发声" }, { "start_idx": 71, "end_idx": 84, "type": "sym", "entity": "扣带回基底节及脑干不规则放电" }, { "start_idx": 71, "end_idx": 76, "type": "bod", "entity": "扣带回基底节" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "脑干" } ] }, { "text": "(四)精神因素患儿多有焦虑、紧张等精神因素所致的抽动,久而久之成为习惯。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "焦虑" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "紧张" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "抽动" } ] }, { "text": "这些因素多为对患儿的学习要求过高、家庭冲突、遭遇意外事件等,有人认为本病可能与患儿的神经类型有关,神经质、胆怯、不稳定的儿童易出现抽动症状。", "entities": [ { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "神经" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "神经质" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "胆怯" }, { "start_idx": 56, "end_idx": 58, "type": "sym", "entity": "不稳定" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "抽动" } ] }, { "text": "(五)药源性因素不恰当地长期或大剂量应用抗精神病药或中枢兴奋剂如氯氮平、卡马西平、利他林等,可能产生抽动-秽语综合征。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "抗精神病药" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "中枢兴奋剂" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "氯氮平" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "卡马西平" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "利他林" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "抽动-秽语综合征" } ] }, { "text": "【临床表现】(一)抽动症状1.运动抽动简单性发声抽动的特点是清嗓、咳嗽、哼声、吠叫声等;复杂性发声抽动可发出完整的或省略的词语,具有语言的意义,如模仿言语和秽语等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "抽动" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "运动抽动" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "简单性发声抽动" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "清嗓" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "咳嗽" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "哼声" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "吠叫声" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "复杂性发声抽动" } ] }, { "text": "(二)抽动形式通常起病多从眼、面开始,如眨眼、点头、努嘴等,然后逐步向肢体近端发展,甚至波及全身多部位肌肉抽动。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "抽动" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "眼" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "面" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "眨眼" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "眼" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "点头" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "头" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "努嘴" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "嘴" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "肢体近端" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "全身多部位肌肉抽动" }, { "start_idx": 46, "end_idx": 52, "type": "bod", "entity": "全身多部位肌肉" } ] }, { "text": "(三)常伴发其他病症1.伴发注意缺陷多动障碍(ADHD)大约50%的抽动-秽语综合征患儿同时被诊断为ADHD。", "entities": [ { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "伴发注意缺陷多动障碍" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "ADHD" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "ADHD" } ] }, { "text": "2.伴发强迫障碍(OCD)大约40%~50%的抽动-秽语综合征患儿同时有OCD。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "伴发强迫障碍" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "OCD" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "OCD" } ] }, { "text": "遗传研究表明抽动秽语综合征患儿一级亲属中强迫症的发生率较正常群体高9~13倍。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "抽动秽语综合征" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "强迫症" } ] }, { "text": "1995年,Como指出抽动-秽语综合征是常染色体显性表现型,而OCD是该基因的另一种表现型。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "常染色体" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "OCD" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "基因" } ] }, { "text": "男性常表现为抽动-秽语综合征;而女性常表现为无抽动的强迫症。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "无抽动的强迫症" } ] }, { "text": "3.伴发学习不能症、焦虑和抑郁约25%的患儿同时伴有学习不能症,而25%~50%的患儿可有焦虑和抑郁。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "学习不能症" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "焦虑" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "抑郁" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "学习不能症" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "焦虑" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "抑郁" } ] }, { "text": "【诊断】(一)诊断标准CCMD-3提出的Tourette综合征诊断标准为:1.症状标准表现为多种运动抽动和一种或多种发声抽动,多为复杂性抽动,两者多同时出现。", "entities": [ { "start_idx": 20, "end_idx": 30, "type": "dis", "entity": "Tourette综合征" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "多种运动抽动" }, { "start_idx": 53, "end_idx": 61, "type": "sym", "entity": "一种或多种发声抽动" }, { "start_idx": 65, "end_idx": 69, "type": "sym", "entity": "复杂性抽动" } ] }, { "text": "抽动可在短时间内受意志控制,在应激下加剧,睡眠时消失。", "entities": [ { "start_idx": 0, "end_idx": 25, "type": "sym", "entity": "抽动可在短时间内受意志控制,在应激下加剧,睡眠时消失" } ] }, { "text": "(3)排除小舞蹈症、肝豆状核变性、癫痫肌阵挛发作、药源性不自主运动及其他锥体外系病变。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "小舞蹈症" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "癫痫肌阵挛发作" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "癫痫肌阵挛" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "药源性不自主运动" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "锥体外系病变" } ] }, { "text": "【鉴别诊断】(一)小舞蹈症这是风湿性感染所致,通常也多发生于5~15岁的儿童少年,以舞蹈样异常运动为特征,并有其他风湿热(rheumaticfever)的症状,实验室检查血沉增快,C反应蛋白阳性,ASO升高,经抗风湿治疗有效。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "小舞蹈症" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "风湿性感染" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "舞蹈样异常运动" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "风湿热" }, { "start_idx": 61, "end_idx": 74, "type": "dis", "entity": "rheumaticfever" }, { "start_idx": 80, "end_idx": 84, "type": "pro", "entity": "实验室检查" }, { "start_idx": 85, "end_idx": 86, "type": "ite", "entity": "血沉" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "血沉增快" }, { "start_idx": 90, "end_idx": 94, "type": "ite", "entity": "C反应蛋白" }, { "start_idx": 90, "end_idx": 96, "type": "sym", "entity": "C反应蛋白阳性" }, { "start_idx": 98, "end_idx": 100, "type": "ite", "entity": "ASO" }, { "start_idx": 98, "end_idx": 102, "type": "sym", "entity": "ASO升高" }, { "start_idx": 105, "end_idx": 109, "type": "pro", "entity": "抗风湿治疗" } ] }, { "text": "(二)急性运动性障碍表现为突然不自主运动、震颤、张力障碍、扭转痉挛或舞蹈样动作,常由某些药物如中枢兴奋剂、抗精神病药物引起,停药后症状可消失。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "急性运动性障碍" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "突然不自主运动" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "震颤" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "张力障碍" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "扭转痉挛" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "舞蹈样动作" }, { "start_idx": 47, "end_idx": 51, "type": "dru", "entity": "中枢兴奋剂" }, { "start_idx": 53, "end_idx": 58, "type": "dru", "entity": "抗精神病药物" } ] }, { "text": "(三)手足徐动症(athetosis)此症表现为缓慢蠕动样、联合性的不自主运动,是脑性瘫痪的一种类型,根据病史、肌张力和腱反射的改变等,不难鉴别。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "手足徐动症" }, { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "athetosis" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "缓慢蠕动样" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "联合性的不自主运动" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "脑性瘫痪" }, { "start_idx": 56, "end_idx": 65, "type": "sym", "entity": "肌张力和腱反射的改变" }, { "start_idx": 56, "end_idx": 58, "type": "ite", "entity": "肌张力" }, { "start_idx": 60, "end_idx": 62, "type": "ite", "entity": "腱反射" } ] }, { "text": "(四)儿童精神分裂症(childschizophrenia)该病具有思维和情感障碍,异常的感知、幻觉妄想和怪异行为,这些精神特征是抽动-秽语综合征所不具备的。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "儿童精神分裂症" }, { "start_idx": 11, "end_idx": 28, "type": "dis", "entity": "childschizophrenia" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "思维和情感障碍" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "异常的感知" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "幻觉" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "妄想" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "怪异行为" }, { "start_idx": 65, "end_idx": 72, "type": "dis", "entity": "抽动-秽语综合征" } ] }, { "text": "【治疗】(一)心理治疗教育患儿不要过于担忧和紧张,建立信心,积极配合治疗。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "心理治疗" }, { "start_idx": 11, "end_idx": 23, "type": "pro", "entity": "教育患儿不要过于担忧和紧张" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "建立信心" }, { "start_idx": 30, "end_idx": 35, "type": "pro", "entity": "积极配合治疗" } ] }, { "text": "(二)消除诱因包括躯体诱因如扁桃体炎、上呼吸道感染、结膜炎、喉炎等可能的诱因进行治疗;心理社会诱因如家庭矛盾的调整,父母、教师的过度要求所致的精神矛盾和紧张情绪的解决,给予支持性心理治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "消除诱因" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "躯体" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "结膜炎" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "喉炎" }, { "start_idx": 50, "end_idx": 56, "type": "pro", "entity": "家庭矛盾的调整" }, { "start_idx": 58, "end_idx": 82, "type": "pro", "entity": "父母、教师的过度要求所致的精神矛盾和紧张情绪的解决" }, { "start_idx": 86, "end_idx": 92, "type": "pro", "entity": "支持性心理治疗" } ] }, { "text": "(三)药物治疗1.抽动的治疗(1)氟哌啶醇:系一种高效较多巴胺受体阻滞剂,从1961年开始,此药治疗抽动-秽语综合征,是最有效的药物之一,可作首选,由小剂量开始,0.05mg/(kg•d)口服,然后调整剂量,使症状获得控制而无不良反应。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "药物治疗" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "抽动" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 25, "end_idx": 35, "type": "dru", "entity": "高效较多巴胺受体阻滞剂" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "抽动-秽语综合征" }, { "start_idx": 94, "end_idx": 95, "type": "pro", "entity": "口服" } ] }, { "text": "一般初次给0.5mg,口服,每日2次,逐渐加量,直至满意效果。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "口服" } ] }, { "text": "对于反应严重者可肌肉注射东莨菪碱0.3mg,每日1~2次。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "肌肉注射" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "东莨菪碱" } ] }, { "text": "(2)硫必利(泰必利):此药与多巴胺D2受体结合,抑制中脑边缘系统多巴胺能亢进。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "硫必利" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "泰必利" }, { "start_idx": 15, "end_idx": 27, "type": "bod", "entity": "多巴胺D2受体" }, { "start_idx": 33, "end_idx": 41, "type": "bod", "entity": "中脑边缘系统多巴胺" } ] }, { "text": "开始剂量为每次50mg,口服,每日3次,连服1~2周后若症状控制欠佳,可逐渐加量至每日300~400mg。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "口服" } ] }, { "text": "该药不良反应为头昏、无力和嗜睡。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "头昏" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "头" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "无力" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "嗜睡" } ] }, { "text": "(3)匹莫齐特(哌迷清):其作用与氟哌啶醇类似,但镇静作用轻。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "匹莫齐特" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "哌迷清" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "镇静作用" } ] }, { "text": "此药不良反应为引起心电图的变化如T波倒置、Q-T间期延长的心率缓慢,故在服药过程中须监测心电图的变化。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "心电图" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "T波倒置" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "Q-T间期延长的心率缓慢" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "监测心电图" } ] }, { "text": "有报道匹莫齐特与丁苯那嗪或可乐定合用能较好地控制抽动症状。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "匹莫齐特" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "丁苯那嗪" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "可乐定" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "抽动" } ] }, { "text": "(4)可乐定:是一种中枢性α2受体阻滞剂,能减少中枢去甲肾上腺素能系统的活动。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "可乐定" }, { "start_idx": 10, "end_idx": 25, "type": "dru", "entity": "中枢性α2受体阻滞剂" }, { "start_idx": 30, "end_idx": 40, "type": "bod", "entity": "中枢去甲肾上腺素能系统" } ] }, { "text": "2.伴发行为的治疗(1)伴发注意缺陷伴多动症的治疗:过去认为这类患儿不宜用中枢兴奋剂,新近观点认为,服用中枢兴奋性药物引起抽动的风险不像过去认为的那样大,用药后不久出现抽动症可能反映患儿具有抽动症的遗传素质,即使这些患儿不服用该类药物,以后也常常会出现抽动症状。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "伴发行为的治疗" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "注意缺陷伴多动症" }, { "start_idx": 37, "end_idx": 41, "type": "dru", "entity": "中枢兴奋剂" }, { "start_idx": 52, "end_idx": 58, "type": "dru", "entity": "中枢兴奋性药物" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "抽动" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "抽动症" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "抽动症" }, { "start_idx": 126, "end_idx": 127, "type": "sym", "entity": "抽动" } ] }, { "text": "哌醋甲酯最佳剂量为每日0.1mg/kg,这一剂量不伴抽动症状的加重,因此,可间歇和适量应用此药。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "哌醋甲酯" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "抽动症状的加重" } ] }, { "text": "(2)伴发强迫观念和行为的治疗:氟西汀(百忧解,fluoxetine)是有效的药物之一,剂量为10~40mg/d,晨服,疗程4~20周。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "pro", "entity": "伴发强迫观念和行为的治疗" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "氟西汀" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "百忧解" }, { "start_idx": 24, "end_idx": 33, "type": "dru", "entity": "fluoxetine" }, { "start_idx": 57, "end_idx": 58, "type": "pro", "entity": "晨服" } ] }, { "text": "第三节快速诊断技术在感染性疾病中的地位从某种意义上说,病原学是诊断之本。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "快速诊断技术" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "临床上感染性疾病首先要明确是何种病原体感染,这样才能做到有的放矢。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "病原体感染" } ] }, { "text": "及时获得病原学结果,有助于降低和控制滥用抗生素。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "抗生素" } ] }, { "text": "细菌培养结果,尤其是抗生素药敏试验结果,对指导临床用药意义很大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "细菌培养" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "抗生素药敏试验" } ] }, { "text": "不少感染性疾病都是传染病,有在一个单位、地区传播和流行的可能,早期的病原学诊断是及时采取预防的基础。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "传染病" } ] }, { "text": "一旦发现像流脑、伤寒、传染性肝炎、霍乱等急性传染病,就应对患者采取隔离措施,对危险人群进行预防性给药,对周围环境进行消毒处理等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "流脑" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "伤寒" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "传染性肝炎" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "霍乱" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "急性传染病" }, { "start_idx": 28, "end_idx": 36, "type": "pro", "entity": "对患者采取隔离措施" }, { "start_idx": 38, "end_idx": 49, "type": "pro", "entity": "对危险人群进行预防性给药" }, { "start_idx": 51, "end_idx": 61, "type": "pro", "entity": "对周围环境进行消毒处理" } ] }, { "text": "我国感染性疾病仍然是威胁儿童健康的主要疾病,没有病原学依据,就不能确切了解这些疾病的流行病学资料,如发病率、病死率、年龄和季节分布,更无从了解其危险因素,无法提出切实可行的预防措施。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "第六章先天性肾上腺皮质增生症【概述】先天性肾上腺皮质增生症(congenitaladrenalhyperplasia,CAH)是一组常染色体隐性遗传性疾病,因类固醇激素合成过程中某种酶的先天性缺陷,导致肾上腺皮质合成的皮质醇完全或部分受阻,皮质醇缺乏,对下丘脑-垂体的负反馈作用消除,促使下丘脑-垂体分泌的促肾上腺皮质激素释放激素(corticotrophic-relieasinghormone,CRH)和促肾上腺皮质激素(adrenocorticotrophichormone,ACTH)分泌增加,导致肾上腺皮质增生,有些酶的缺乏同时可导致盐皮质激素和性激素合成障碍。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "先天性肾上腺皮质增生症" }, { "start_idx": 18, "end_idx": 28, "type": "dis", "entity": "先天性肾上腺皮质增生症" }, { "start_idx": 30, "end_idx": 57, "type": "dis", "entity": "congenitaladrenalhyperplasia" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "CAH" }, { "start_idx": 66, "end_idx": 76, "type": "dis", "entity": "常染色体隐性遗传性疾病" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "类固醇激素" }, { "start_idx": 89, "end_idx": 97, "type": "sym", "entity": "某种酶的先天性缺陷" }, { "start_idx": 91, "end_idx": 91, "type": "bod", "entity": "酶" }, { "start_idx": 101, "end_idx": 105, "type": "bod", "entity": "肾上腺皮质" }, { "start_idx": 101, "end_idx": 118, "type": "sym", "entity": "肾上腺皮质合成的皮质醇完全或部分受阻" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "皮质醇" }, { "start_idx": 120, "end_idx": 122, "type": "bod", "entity": "皮质醇" }, { "start_idx": 120, "end_idx": 124, "type": "sym", "entity": "皮质醇缺乏" }, { "start_idx": 127, "end_idx": 132, "type": "bod", "entity": "下丘脑-垂体" }, { "start_idx": 144, "end_idx": 149, "type": "bod", "entity": "下丘脑-垂体" }, { "start_idx": 153, "end_idx": 164, "type": "bod", "entity": "促肾上腺皮质激素释放激素" }, { "start_idx": 166, "end_idx": 197, "type": "bod", "entity": "corticotrophic-relieasinghormone" }, { "start_idx": 199, "end_idx": 201, "type": "bod", "entity": "CRH" }, { "start_idx": 204, "end_idx": 211, "type": "bod", "entity": "促肾上腺皮质激素" }, { "start_idx": 213, "end_idx": 239, "type": "bod", "entity": "adrenocorticotrophichormone" }, { "start_idx": 241, "end_idx": 244, "type": "bod", "entity": "ACTH" }, { "start_idx": 253, "end_idx": 259, "type": "dis", "entity": "肾上腺皮质增生" }, { "start_idx": 263, "end_idx": 263, "type": "bod", "entity": "酶" }, { "start_idx": 263, "end_idx": 266, "type": "sym", "entity": "酶的缺乏" }, { "start_idx": 272, "end_idx": 284, "type": "dis", "entity": "盐皮质激素和性激素合成障碍" } ] }, { "text": "根据类固醇激素合成途径中发生缺陷的酶的不同,临床症状、体征和实验室也各不相同。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "类固醇激素" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "缺陷" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "酶" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "实验" } ] }, { "text": "临床主要特点为肾上腺皮质功能不全、水盐代谢失调以及性腺发育异常。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "肾上腺皮质功能不全" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "水盐代谢失调" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "水" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "盐" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "性腺发育异常" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "性腺" } ] }, { "text": "【流行病学】CAH是常染色体隐性遗传病,患者为纯合子,父母为杂合子,每生育一胎,1/4的可能性为CAH纯合子患儿。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "CAH" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "常染色体隐性遗传病" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "CAH" } ] }, { "text": "21-羟化酶缺乏症是CAH中最常见的类型,约占90%~95%。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "CAH" } ] }, { "text": "根据新生儿筛查统计,全世界21-羟化酶缺乏症发生率约为1/10000~1/15000,北美1/15000,欧洲国家为1/14000~1/10000,日本为1/21000,菲律宾1/8437,台湾1/11690,泰国1/29281。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "21-羟化酶缺乏症" } ] }, { "text": "非典型21-羟化酶缺乏症在白种人的发生率约1/500~1/1000;日本新生儿筛查统计,非典型者为1/100000。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "非典型21-羟化酶缺乏症" } ] }, { "text": "【病因机制和病理生理】肾上腺由皮质和髓质两个功能不同的内分泌器官组成,皮质分泌肾上腺皮质激素,髓质分泌儿茶酚胺激素。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肾上腺" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮质" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "髓质" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "内分泌器官" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "皮质" }, { "start_idx": 39, "end_idx": 45, "type": "bod", "entity": "肾上腺皮质激素" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "髓质" }, { "start_idx": 51, "end_idx": 56, "type": "bod", "entity": "儿茶酚胺激素" } ] }, { "text": "肾上腺皮质又可分为3个区带:①球状带,位于肾上腺皮质最外层,占皮质的5%~10%,主要合成和分泌盐皮质激素;②束状带,位于中间层,约占皮质的75%,是储存胆固醇的重要场所,主要合成糖皮质激素,如皮质醇及少量去氧皮质酮(DOC)、脱氧皮质醇(S)和皮质酮(B);③网状带,位于肾上腺皮质最内层,主要合成肾上腺雄激素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肾上腺皮质" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "球状带" }, { "start_idx": 21, "end_idx": 28, "type": "bod", "entity": "肾上腺皮质最外层" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "皮质" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "盐皮质激素" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "束状带" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "中间层" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "皮质" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "胆固醇" }, { "start_idx": 90, "end_idx": 94, "type": "bod", "entity": "糖皮质激素" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "皮质醇" }, { "start_idx": 103, "end_idx": 107, "type": "bod", "entity": "去氧皮质酮" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "DOC" }, { "start_idx": 114, "end_idx": 118, "type": "bod", "entity": "脱氧皮质醇" }, { "start_idx": 120, "end_idx": 120, "type": "bod", "entity": "S" }, { "start_idx": 123, "end_idx": 125, "type": "bod", "entity": "皮质酮" }, { "start_idx": 127, "end_idx": 127, "type": "bod", "entity": "B" }, { "start_idx": 131, "end_idx": 133, "type": "bod", "entity": "网状带" }, { "start_idx": 137, "end_idx": 144, "type": "bod", "entity": "肾上腺皮质最内层" }, { "start_idx": 150, "end_idx": 155, "type": "bod", "entity": "肾上腺雄激素" } ] }, { "text": "诸类肾上腺皮质激素均为胆固醇的衍生物,其合成过程极为复杂,必须经过一系列的酶促反应加工而成。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "肾上腺皮质激素" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "胆固醇的衍生物" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "酶" } ] }, { "text": "在诸多类固醇激素合成酶中,除3β-羟类固醇脱氢酶(3β-HSD)外,均为细胞色素P450(cytochromeP450)蛋白超家族成员。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "类固醇激素合成酶" }, { "start_idx": 14, "end_idx": 23, "type": "bod", "entity": "3β-羟类固醇脱氢酶" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "3β-HSD" }, { "start_idx": 35, "end_idx": 64, "type": "bod", "entity": "为细胞色素P450(cytochromeP450)蛋白超家族" } ] }, { "text": "类固醇激素的生物合成途径(图15-15)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "类固醇激素" } ] }, { "text": "在肾上腺皮质发育过程中有两个重要转录因子:类固醇生成因子-1(SF-1)和DAX-1。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "肾上腺皮质" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "转录因子" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "类固醇生成因子-1" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "SF-1" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "DAX-1" } ] }, { "text": "SF-1基因定位于染色体9q33,参与类固醇合成过程中的一些酶的编码基因的转录调节,该因子的缺乏将导致肾上腺和性腺的发育不全DAX-1基因位于Xq21,该基因的突变可造成先天性肾上腺发育不全和低促性腺素功能减退症。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "SF-1基因" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "染色体9q33" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "类固醇" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "酶的编码基因" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "因子的缺乏" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "因子" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "肾上腺" }, { "start_idx": 51, "end_idx": 61, "type": "sym", "entity": "肾上腺和性腺的发育不全" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "性腺" }, { "start_idx": 62, "end_idx": 68, "type": "bod", "entity": "DAX-1基因" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "Xq21" }, { "start_idx": 77, "end_idx": 81, "type": "sym", "entity": "基因的突变" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "基因" }, { "start_idx": 85, "end_idx": 94, "type": "dis", "entity": "先天性肾上腺发育不全" }, { "start_idx": 96, "end_idx": 105, "type": "dis", "entity": "低促性腺素功能减退症" } ] }, { "text": "另外,DAX-1还参与类固醇合成的调节。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "DAX-1" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "类固醇" } ] }, { "text": "在正常情况下,下丘脑分泌的CRH通过垂体分泌的ACTH能促进肾上腺皮质细胞增生、激素合成和分泌。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "bod", "entity": "下丘脑分泌的CRH" }, { "start_idx": 18, "end_idx": 26, "type": "bod", "entity": "垂体分泌的ACTH" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "肾上腺皮质细胞增生" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "激素" } ] }, { "text": "当血中皮质醇达到一定浓度时,即通过负反馈机制使CRH和ACTH分泌减少。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "血中皮质醇" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "CRH和ACTH分泌减少" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "CRH" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "ACTH" } ] }, { "text": "若在类固醇激素合成途径中任何一个酶发生缺陷时,都会使血中皮质醇浓度降低负反馈作用消失,以致ACTH分泌增加,刺激肾上腺皮质增生酶缺陷导致前体中间代谢产物增多,经旁路代谢可致雄激素产生过多。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "类固醇激素" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "酶" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "酶发生缺陷" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "血中皮质醇浓度降低" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "血中皮质醇" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "负反馈作用消失" }, { "start_idx": 45, "end_idx": 52, "type": "sym", "entity": "ACTH分泌增加" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "ACTH" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "肾上腺皮质增生" }, { "start_idx": 63, "end_idx": 65, "type": "sym", "entity": "酶缺陷" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "酶" }, { "start_idx": 68, "end_idx": 77, "type": "sym", "entity": "前体中间代谢产物增多" }, { "start_idx": 68, "end_idx": 75, "type": "bod", "entity": "前体中间代谢产物" }, { "start_idx": 86, "end_idx": 92, "type": "sym", "entity": "雄激素产生过多" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "雄激素" } ] }, { "text": "由于醛固酮合成和分泌在常见类型的CAH中亦大多同时受到影响,故常引起血浆肾素活性(PRA)增高CAH主要包括21-羟化酶缺乏症(21-OHD)、11β-羟化酶缺乏症(11β-OHD)、3β-羟类固醇脱氢酶(3β-HSD)缺乏症、17-α羟化酶缺乏症(17α-OHD)以及类脂性肾上腺增生症(类固醇合成急性调节蛋白缺乏,StAR基因缺陷)等类型。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "醛固酮" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "CAH" }, { "start_idx": 34, "end_idx": 46, "type": "sym", "entity": "血浆肾素活性(PRA)增高" }, { "start_idx": 34, "end_idx": 39, "type": "ite", "entity": "血浆肾素活性" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "PRA" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "CAH" }, { "start_idx": 54, "end_idx": 62, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 64, "end_idx": 69, "type": "dis", "entity": "21-OHD" }, { "start_idx": 72, "end_idx": 81, "type": "dis", "entity": "11β-羟化酶缺乏症" }, { "start_idx": 83, "end_idx": 89, "type": "dis", "entity": "11β-OHD" }, { "start_idx": 92, "end_idx": 111, "type": "dis", "entity": "3β-羟类固醇脱氢酶(3β-HSD)缺乏" }, { "start_idx": 114, "end_idx": 123, "type": "dis", "entity": "17-α羟化酶缺乏症" }, { "start_idx": 125, "end_idx": 131, "type": "dis", "entity": "17α-OHD" }, { "start_idx": 135, "end_idx": 143, "type": "dis", "entity": "类脂性肾上腺增生症" }, { "start_idx": 145, "end_idx": 157, "type": "dis", "entity": "类固醇合成急性调节蛋白缺乏" }, { "start_idx": 159, "end_idx": 166, "type": "dis", "entity": "StAR基因缺陷" } ] }, { "text": "其中21-OHD是最常见的CAH,约占CAH总数的90%以上,11β-OHD次之,约占5%~8%,再次为3β-HSD缺乏症,17α-OHD和类脂性肾上腺增生症则十分罕见,约占1%。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "21-OHD" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "CAH" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "CAH" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "11β-OHD" }, { "start_idx": 52, "end_idx": 60, "type": "dis", "entity": "3β-HSD缺乏症" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "17α-OHD" }, { "start_idx": 70, "end_idx": 78, "type": "dis", "entity": "类脂性肾上腺增生症" } ] }, { "text": "21-羟化酶基因定位于第6号染色体短臂(6p21.3),与HLA基因族紧密连锁。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "21-羟化酶基因" }, { "start_idx": 11, "end_idx": 18, "type": "bod", "entity": "第6号染色体短臂" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "6p21.3" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "HLA基因" } ] }, { "text": "由A1、A2两个基因座构成,A1基因(CYP21A1)是假基因,A2基因(CYP21A2)是编码21-OH的功能基因,两者高度同源。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "A1" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "A2" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "两个基因座" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "A1基因" }, { "start_idx": 19, "end_idx": 25, "type": "bod", "entity": "CYP21A1" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "基因" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "A2基因" }, { "start_idx": 37, "end_idx": 43, "type": "bod", "entity": "CYP21A2" }, { "start_idx": 46, "end_idx": 57, "type": "bod", "entity": "编码21-OH的功能基因" } ] }, { "text": "CYP21A1和CYP21A2各由10个外显子及9个内含子组成,基因全长为3463bp。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "CYP21A1" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "CYP21A2" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "基因" } ] }, { "text": "CYP21A2基因突变是导致21-OHD的根本原因,包括基因缺失、转换和点突变等。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "CYP21A2基因突变" }, { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "CYP21A2基因" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "21-OHD" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "基因缺失、转换和点突变" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "基因" } ] }, { "text": "编码11β-羟化酶的基因为CYP11B1,定位于第8号染色体长臂(8q21)。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "bod", "entity": "编码11β-羟化酶的基因" }, { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "CYP11B1" }, { "start_idx": 24, "end_idx": 31, "type": "bod", "entity": "第8号染色体长臂" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "8q21" } ] }, { "text": "基因突变热点在外显子2、6、7和8,至今已发现20种基因点突变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "基因突变" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 7, "end_idx": 16, "type": "bod", "entity": "外显子2、6、7和8" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "基因点突变" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "基因" } ] }, { "text": "17-羟化酶基因定位于第10号染色体长臂(10q24.3),包含8个外显子和7个内含子,基因全长6.6kb。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "17-羟化酶基因" }, { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "第10号染色体长臂" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "10q24.3" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "基因" } ] }, { "text": "基因缺陷包括小片段缺失、重复及点突变,迄今未见大片段缺失报道。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "基因缺陷" }, { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "小片段缺失、重复" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "点突变" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "大片段缺失" } ] }, { "text": "3β-羟类固醇脱氢酶主要由HSD3B2基因编码表达,与HSD3B1同工酶基因的同源序列高达93%,均定位于第1号染色体短臂(1p11-11),由4个外显子和3个内含子组成,基因全长约7.8kb。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "3β-羟类固醇脱氢酶" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "HSD3B2基因" }, { "start_idx": 27, "end_idx": 37, "type": "bod", "entity": "HSD3B1同工酶基因" }, { "start_idx": 53, "end_idx": 60, "type": "bod", "entity": "第1号染色体短臂" }, { "start_idx": 62, "end_idx": 68, "type": "bod", "entity": "1p11-11" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "基因" } ] }, { "text": "目前已报道的基因缺陷不少于17种,主要包括移码突变、无义突变和错义突变。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "基因缺陷" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "基因" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "移码突变" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "无义突变" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "错义突变" } ] }, { "text": "【临床表现】因21-羟化酶缺乏症缺乏程度不同,可分为典型的单纯男性化型、失盐型和非典型(迟发型或轻型)三种。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "21-羟化酶缺乏症" } ] }, { "text": "1.典型的21-羟化酶缺乏症的临床表现(1)单纯男性化型:21-羟化酶缺乏症部分性缺乏,占21-羟化酶缺乏症患者总数25%。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "典型的21-羟化酶缺乏症" }, { "start_idx": 29, "end_idx": 42, "type": "dis", "entity": "21-羟化酶缺乏症部分性缺乏" }, { "start_idx": 45, "end_idx": 53, "type": "dis", "entity": "21-羟化酶缺乏症" } ] }, { "text": "血醛固酮和皮质醇合成部分受阻,在反馈性ACTH分泌增加情况下,尚能维持醛固酮,皮质醇接近正常水平或低于正常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "血醛固酮" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "血醛固酮和皮质醇合成部分受阻" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "皮质醇" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "反馈性ACTH" }, { "start_idx": 16, "end_idx": 26, "type": "sym", "entity": "反馈性ACTH分泌增加" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "醛固酮" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "皮质醇" } ] }, { "text": "临床上无明显的失盐症状出现,主要的临床表现为雄激素增高的症状和体征。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "失盐" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "盐" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "雄激素增高" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "雄激素" } ] }, { "text": "无论男女21-羟化酶缺乏症,由于雄激素异常增高,一般在4~7岁可明显出现胡须、阴毛及腋毛,有的甚至在婴儿期出现阴毛发育体臭、秃发及痤疮等。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "雄激素异常增高" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "雄激素" }, { "start_idx": 26, "end_idx": 43, "type": "sym", "entity": "在4~7岁可明显出现胡须、阴毛及腋毛" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "胡须" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "阴毛" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "腋毛" }, { "start_idx": 50, "end_idx": 58, "type": "sym", "entity": "婴儿期出现阴毛发育" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "阴毛" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "体臭" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "秃发" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "发" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "痤疮" } ] }, { "text": "由于ACTH增高,在皮肤皱褶处有不同程度色素沉着。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "ACTH增高" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "ACTH" }, { "start_idx": 9, "end_idx": 23, "type": "sym", "entity": "在皮肤皱褶处有不同程度色素沉着" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "皮肤皱褶处" } ] }, { "text": "由于雄激素增高,患儿早期身高增长加速,超过同年龄、同性别正常小儿,身体强壮,似“小大力士”,以后随着骨骺成熟提前,早闭,导致最终成人身高明显低于正常。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "雄激素增高" }, { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "雄激素" }, { "start_idx": 12, "end_idx": 31, "type": "sym", "entity": "身高增长加速,超过同年龄、同性别正常小儿" }, { "start_idx": 33, "end_idx": 44, "type": "sym", "entity": "身体强壮,似“小大力士”" }, { "start_idx": 50, "end_idx": 58, "type": "sym", "entity": "骨骺成熟提前,早闭" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "骨骺" }, { "start_idx": 62, "end_idx": 73, "type": "sym", "entity": "最终成人身高明显低于正常" }, { "start_idx": 66, "end_idx": 67, "type": "ite", "entity": "身高" } ] }, { "text": "图15-1类固醇激素的生物合成途径(2)失盐型:21-羟化酶完全缺乏,约占21-羟化酶缺乏症患者总数的75%。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "类固醇激素" }, { "start_idx": 24, "end_idx": 33, "type": "dis", "entity": "21-羟化酶完全缺乏" }, { "start_idx": 37, "end_idx": 45, "type": "dis", "entity": "21-羟化酶缺乏症" } ] }, { "text": "临床上除出现单纯男性化型的一系列临床表现外,还可因醛固酮严重缺乏导致失盐。", "entities": [ { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "醛固酮严重缺乏" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "醛固酮" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "失盐" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "盐" } ] }, { "text": "随着年龄增大,一般在4岁后,机体对失盐的耐受性有所增加,失盐现象逐渐改善。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "失盐" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "盐" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "失盐" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "盐" } ] }, { "text": "因失盐型比单纯男性化型CAH更易引起注意,且能得到更早治疗。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "单纯男性化型CAH" } ] }, { "text": "有调查发现,失盐型者男性成年最终身高约(156.8±6.6)cm,单纯男性化型者最终身高约(153.3±5.4)cm;约1/3CAH者最终成人身高均低于正常标准身高第3百分位数或明显低于其父母平均身高。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "身高" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "身高" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "CAH" }, { "start_idx": 67, "end_idx": 99, "type": "sym", "entity": "最终成人身高均低于正常标准身高第3百分位数或明显低于其父母平均身高" }, { "start_idx": 71, "end_idx": 72, "type": "ite", "entity": "身高" } ] }, { "text": "一些未治疗的或治疗不当的男性患儿,由于增高的雄激素水平对下丘脑-垂体-性腺轴的成熟抑制,导致睾丸发育欠佳和成年后患无精子或少精子症;部分男性患者伴有真性性早熟。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "雄激素" }, { "start_idx": 28, "end_idx": 37, "type": "bod", "entity": "下丘脑-垂体-性腺轴" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "睾丸发育欠佳" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "睾丸" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "无精子" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "少精子症" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "真性性早熟" } ] }, { "text": "(3)非典型型(迟发型或轻型):21-羟化酶缺乏症酶活性为正常人20%~50%,皮质醇和醛固酮分泌影响轻微,临床表现各异,发病年龄不一,多在肾上腺功能初现年龄阶段出现症状。", "entities": [ { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "酶" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "皮质醇" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "醛固酮" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "肾上腺" } ] }, { "text": "男女患者均有阴毛早现,腋毛也较早出现。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "阴毛早现" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "阴毛" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "腋毛也较早出现" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "腋毛" } ] }, { "text": "女性患者出生时外生殖器正常,可表现为初潮延迟、原发性闭经、多毛症及不孕症等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "外生殖器" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "初潮延迟" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "原发性闭经" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "多毛症" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "不孕症" } ] }, { "text": "2.11β-羟化酶缺乏症临床可分为典型与非典型型。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "11β-羟化酶缺乏症" } ] }, { "text": "但一般女孩男性化体征较轻,男孩出生后外生殖器多正常,至儿童期方出现性早熟体征。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "外生殖器" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "性早熟" } ] }, { "text": "非典型型临床表现差异较大,部分患儿可至青春发育期因多毛、痤疮和月经不规则而就诊,大多血压正常,男孩有时仅表现为生长加速和阴毛早现,临床较难与21-羟化酶缺乏症的非典型患者区别。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "多毛" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "毛" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "痤疮" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "月经不规则" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "血压" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "生长加速" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "阴毛早现" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "阴毛" }, { "start_idx": 70, "end_idx": 78, "type": "dis", "entity": "21-羟化酶缺乏症" } ] }, { "text": "3.3β-羟类固醇脱氢酶缺乏症典型病例出生后即可出现失盐和肾上腺皮质功能不全的症状,如厌食、呕吐、脱水、低血钠、高血钾及酸中毒等,严重者因循环衰竭而死亡。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "3β-羟类固醇脱氢酶缺乏症" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "失盐" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "盐" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "肾上腺皮质功能不全" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "厌食" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "呕吐" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "脱水" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "低血钠" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "高血钾" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "酸中毒" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "循环衰竭" } ] }, { "text": "男性可有不同程度的外生殖器发育不良,女性则出现不同程度男性化。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "外生殖器发育不良" }, { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "女性则出现不同程度男性化" } ] }, { "text": "非典型病例约占本症10%~15%,出生时往往无异常,至青春发育期前后出现轻度雄激素增高体征,如女孩阴毛早现、多毛、痤疮、月经量少及多囊卵巢等。", "entities": [ { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "轻度雄激素增高" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "雄激素" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "阴毛早现" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "阴毛" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "多毛" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "毛" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "痤疮" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "月经量少" }, { "start_idx": 65, "end_idx": 68, "type": "dis", "entity": "多囊卵巢" } ] }, { "text": "4.17-羟化酶缺乏症由于皮质醇和性激素合成受阻,而DOC和皮质酮分泌增多低钾性碱中毒和高血压,女性青春期呈幼稚型性征和原发性闭经;男性则表现男性假两性畸形。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "17-羟化酶缺乏症" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "皮质醇" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "皮质醇和性激素合成受阻" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "性激素" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "DOC和皮质酮分泌增多" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "DOC" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "皮质酮" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "低钾性碱中毒" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "高血压" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "幼稚型性征" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "原发性闭经" }, { "start_idx": 71, "end_idx": 77, "type": "dis", "entity": "男性假两性畸形" } ] }, { "text": "5.先天性类脂质性肾上腺增生症以前曾认为先天性类脂质性肾上腺增生症是由于胆固醇向黄体酮转换过程中20、22-碳链裂解酶(P450scc)缺乏所致,但近来研究发现该病并非由于P450scc基因突变所致,而是由于类固醇生成急性调控蛋白(steroidogenicacuteregulatoryprotein,StAR)基因突变所致。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "先天性类脂质性肾上腺增生症" }, { "start_idx": 20, "end_idx": 32, "type": "dis", "entity": "先天性类脂质性肾上腺增生症" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "胆固醇" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "黄体酮" }, { "start_idx": 48, "end_idx": 58, "type": "bod", "entity": "20、22-碳链裂解酶" }, { "start_idx": 48, "end_idx": 69, "type": "sym", "entity": "20、22-碳链裂解酶(P450scc)缺乏" }, { "start_idx": 60, "end_idx": 66, "type": "bod", "entity": "P450scc" }, { "start_idx": 86, "end_idx": 96, "type": "sym", "entity": "P450scc基因突变" }, { "start_idx": 86, "end_idx": 94, "type": "bod", "entity": "P450scc基因" }, { "start_idx": 104, "end_idx": 158, "type": "bod", "entity": "类固醇生成急性调控蛋白(steroidogenicacuteregulatoryprotein,StAR)基因" }, { "start_idx": 104, "end_idx": 160, "type": "sym", "entity": "类固醇生成急性调控蛋白(steroidogenicacuteregulatoryprotein,StAR)基因突变" } ] }, { "text": "StAR失活导致类固醇激素生成严重受阻,胆固醇堆积于肾上腺皮质细胞并对其产生毒性作用致病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "StAR" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "类固醇激素生成严重受阻" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "类固醇激素" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "胆固醇" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "肾上腺皮质细胞" } ] }, { "text": "典型的临床表现有男性外生殖器完全女性化,皮肤色素沉着,血糖皮质激素、盐皮质激素、性激素及其代谢物水平明显降低死亡。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "男性外生殖器完全女性化" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "外生殖器" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "皮肤色素沉着" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "血糖" }, { "start_idx": 27, "end_idx": 53, "type": "sym", "entity": "血糖皮质激素、盐皮质激素、性激素及其代谢物水平明显降低" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "皮质激素" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "盐皮质激素" }, { "start_idx": 40, "end_idx": 47, "type": "ite", "entity": "性激素及其代谢物" } ] }, { "text": "【实验室.血17-OHP、ACTH及睾酮水平测定21-羟化酶缺乏症均增高,其中17-OHP可增高达正常的几十倍,是21-羟化酶缺乏症较可靠的诊断依据。", "entities": [ { "start_idx": 5, "end_idx": 23, "type": "ite", "entity": "血17-OHP、ACTH及睾酮水平测定" }, { "start_idx": 24, "end_idx": 32, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 39, "end_idx": 44, "type": "ite", "entity": "17-OHP" }, { "start_idx": 39, "end_idx": 54, "type": "sym", "entity": "17-OHP可增高达正常的几十倍" }, { "start_idx": 57, "end_idx": 65, "type": "dis", "entity": "21-羟化酶缺乏症" } ] }, { "text": "2.血浆肾素、血管紧张素及醛固酮水平测定失盐型者血醛固酮早期可升高以代偿失盐倾向,严重失代偿后,其水平下降;单纯男性化型者大多正常或轻度增高,但所有患儿其血浆肾素及血管紧张素均有不同程度增高。", "entities": [ { "start_idx": 2, "end_idx": 19, "type": "ite", "entity": "血浆肾素、血管紧张素及醛固酮水平测定" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "血醛固酮" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "失盐" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "盐" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "血浆肾素" }, { "start_idx": 77, "end_idx": 94, "type": "sym", "entity": "血浆肾素及血管紧张素均有不同程度增高" }, { "start_idx": 82, "end_idx": 86, "type": "bod", "entity": "血管紧张素" } ] }, { "text": "3.血皮质醇测定典型失盐型CAH,皮质醇水平低于正常,单纯男性化型其水平可在正常范围或稍低于正常。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "ite", "entity": "血皮质醇测定" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "典型失盐型CAH" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "皮质醇" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "皮质醇水平低于正常" } ] }, { "text": "4.血电解质水平测定21-羟化酶缺乏症患者出现低血钠、高血钾及代谢性酸中毒。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "ite", "entity": "血电解质水平测定" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "低血钠" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "高血钾" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "5.对于外生殖器两性难辨者,进一步可作染色体核型检查以明确遗传性别。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "外生殖器两性难辨" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "外生殖器" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "染色体核型检查" } ] }, { "text": "6.基因诊断基因诊断是遗传病诊断最可靠的方法。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "基因诊断" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "基因诊断" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "遗传病" } ] }, { "text": "可对21-羟化酶缺乏症的致病基因CYP21A2或者其他相应致病基因进行DNA序列分析。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "致病基因" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "CYP21A2" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "致病基因" }, { "start_idx": 35, "end_idx": 41, "type": "pro", "entity": "DNA序列分析" } ] }, { "text": "CYP21A2基因异常分三大类:基因缺失、基因转换及点突变。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "CYP21A2基因异常" }, { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "CYP21A2基因" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "基因缺失" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "基因" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "基因转换" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "基因" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "点突变" } ] }, { "text": "【诊断及鉴别诊断】各种类型CAH可导致性发育异常,首先要询问病史和通过全面的体格检查,确定生殖器的解剖结构,尿道口的开口部位,分辨阴囊或阴唇,睾丸是否位于腹股沟(如在该部位触及睾丸组织,则可确定患者的性别为男性)以及是否存在其他畸形。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "CAH" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "性发育异常" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "体格检查" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "生殖器" }, { "start_idx": 54, "end_idx": 61, "type": "bod", "entity": "尿道口的开口部位" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "阴囊" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "阴唇" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "睾丸" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "腹股沟" }, { "start_idx": 88, "end_idx": 91, "type": "bod", "entity": "睾丸组织" }, { "start_idx": 114, "end_idx": 115, "type": "dis", "entity": "畸形" } ] }, { "text": "B超检查可确定患者是否有子宫和卵巢。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "B超检查" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "子宫" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "卵巢" } ] }, { "text": "核型分析能确定患者的遗传性别,血17-OHP、ACTH及睾酮水平测定可提供类固醇激素代谢异常的诊断依据。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "核型分析" }, { "start_idx": 15, "end_idx": 33, "type": "ite", "entity": "血17-OHP、ACTH及睾酮水平测定" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "类固醇激素代谢异常" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "类固醇激素" } ] }, { "text": "儿童期患儿应与性早熟、真两性畸形、男(或女)性化肾上腺皮质肿瘤以及性腺肿瘤等相鉴别。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "性早熟" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "真两性畸形" }, { "start_idx": 17, "end_idx": 30, "type": "dis", "entity": "男(或女)性化肾上腺皮质肿瘤" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "性腺肿瘤" } ] }, { "text": "临床各种类型CAH的特征(表15-6)。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "CAH" } ] }, { "text": "新生儿期失盐型患儿应与幽门狭窄及食道闭锁等症相鉴别;患者血清17-羟孕酮水平升高,需要鉴别是否早产儿、低体重儿或者是否有感染等,并且需要复查随访。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "幽门狭窄" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "食道闭锁" }, { "start_idx": 28, "end_idx": 39, "type": "sym", "entity": "血清17-羟孕酮水平升高" }, { "start_idx": 28, "end_idx": 37, "type": "ite", "entity": "血清17-羟孕酮水平" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "早产儿" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "低体重儿" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "感染" } ] }, { "text": "表15-6各种类型CAH临床特征【治疗】诊断一经明确应立即治疗,治疗药物剂量因人、因病情轻重而异。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "CAH" } ] }, { "text": "1.糖皮质激素首选氢化可的松(HC)或醋酸可的松治疗,按每日10~20mg/m2计算,总量一般分2~3次,每8~12小时服用1次。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "氢化可的松" }, { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "HC" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "醋酸可的松" } ] }, { "text": "新生儿开始治疗剂量宜大些,以抑制ACTH分泌和纠正水、电解质紊乱。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "ACTH" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "水、电解质紊乱" } ] }, { "text": "在应激情况下,如感染或手术,剂量需加倍(2~3倍)。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "感染" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "手术" } ] }, { "text": "糖皮质激素治疗剂量应该个体化。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "糖皮质激素治疗" } ] }, { "text": "生长快于正常同龄者为治疗量不足,而生长慢于正常同龄者为治疗量过度。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "生长快于正常同龄者" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "生长慢于正常同龄者" } ] }, { "text": "药物剂量过度时,体重亦增加明显。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "体重亦增加明显" }, { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "体重" } ] }, { "text": "定期的体格检查可以监测性发育情况,定期手腕部位的X线片可以判断骨骼发育情况。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "定期的体格检查" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "监测性发育情况" }, { "start_idx": 17, "end_idx": 26, "type": "pro", "entity": "定期手腕部位的X线片" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "骨骼" } ] }, { "text": "用药剂量应根据生长速率、骨成熟度、17-OHP、睾酮以及ACTH等指标综合分析调整。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "生长速率" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "骨成熟度" }, { "start_idx": 17, "end_idx": 22, "type": "ite", "entity": "17-OHP" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "睾酮" }, { "start_idx": 28, "end_idx": 31, "type": "ite", "entity": "ACTH" } ] }, { "text": "大多数治疗有效的女性患者,可在正常年龄出现初潮,当控制欠佳时,初潮延迟。", "entities": [ { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "初潮延迟" } ] }, { "text": "单纯男性化型患者,有些男孩要到3~7岁才能明确诊断,他们的骨龄可比实际年龄提前5岁或更多,并且提前开始青春发育,启动下丘脑-垂体-性腺轴功能。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "骨龄" }, { "start_idx": 47, "end_idx": 69, "type": "sym", "entity": "提前开始青春发育,启动下丘脑-垂体-性腺轴功能" }, { "start_idx": 58, "end_idx": 67, "type": "bod", "entity": "下丘脑-垂体-性腺轴" } ] }, { "text": "对于这类真性性早熟,可以用促性腺激素释放激素类似物治疗,例如醋酸亮丙瑞林。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "真性性早熟" }, { "start_idx": 13, "end_idx": 26, "type": "pro", "entity": "促性腺激素释放激素类似物治疗" }, { "start_idx": 30, "end_idx": 35, "type": "dru", "entity": "醋酸亮丙瑞林" } ] }, { "text": "2.盐皮质激素21-羟化酶缺乏症患儿无论是否失盐,其血浆肾素活性都很活跃,应用9α-氟氢可的松(9α-fludrocortisone)可协同糖皮质激素作用,使ACTH分泌进一步减少。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "盐皮质激素" }, { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "失盐" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "盐" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "血浆肾素" }, { "start_idx": 39, "end_idx": 46, "type": "dru", "entity": "9α-氟氢可的松" }, { "start_idx": 48, "end_idx": 65, "type": "dru", "entity": "9α-fludrocortisone" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "糖皮质激素" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "ACTH" } ] }, { "text": "一般口服9α-氟氢可的松的剂量0.05~0.1mg/d,失盐难纠正者可加大9α-氟氢可的松至0.2~0.3mg/d,每日饮食中加入1~2g盐。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "口服" }, { "start_idx": 4, "end_idx": 11, "type": "dru", "entity": "9α-氟氢可的松" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "失盐" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "盐" }, { "start_idx": 37, "end_idx": 44, "type": "dru", "entity": "9α-氟氢可的松" } ] }, { "text": "盐皮质激素使用过量时会出现心动过速和高血压。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "盐皮质激素" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "心动过速" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "高血压" } ] }, { "text": "婴儿早期,应该定期复查血清电解质浓度。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "pro", "entity": "定期复查血清电解质浓度" } ] }, { "text": "血浆肾素活性测定是检测疗效的有效手段。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "血浆肾素活性测定" } ] }, { "text": "3.急性肾上腺皮质功能衰竭处理①纠正脱水:轻、中度脱水,在最初2小时内静滴5%~10%葡萄糖生理盐水20~40ml/kg。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "急性肾上腺皮质功能衰竭" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "纠正脱水" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "轻、中度脱水" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "静滴" }, { "start_idx": 43, "end_idx": 49, "type": "dru", "entity": "葡萄糖生理盐水" } ] }, { "text": "②纠正低血钠:补钠量(mmol/L)按(135-测得值)×0.6×体重计算,初8~12小时给予总量的一半,余半量放入维持量中补给;可用9α-氟氢可的松0.05~0.1mg/d口服。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "纠正低血钠" }, { "start_idx": 67, "end_idx": 74, "type": "dru", "entity": "9α-氟氢可的松" }, { "start_idx": 87, "end_idx": 88, "type": "pro", "entity": "口服" } ] }, { "text": "③纠正严重高血钾:按葡萄糖0.5g/kg加胰岛素0.3U/kg静滴。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "纠正严重高血钾" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "胰岛素" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "静滴" } ] }, { "text": "④补充HC100~200mg/(m2•d)或醋酸可的松125~250mg/(m2•d),分3次口服,1周后减量,3~4周后减至维持量。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "补充HC" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "醋酸可的松" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "口服" } ] }, { "text": "4.外科治疗女性患者呈现明显男性化时,在药物控制前提下可行外阴矫治术,一般在4~12个月可行外生殖器矫形手术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "外科治疗" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "女性患者呈现明显男性化" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "药物控制" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "外阴矫治术" }, { "start_idx": 46, "end_idx": 53, "type": "pro", "entity": "外生殖器矫形手术" } ] }, { "text": "手术切除肥大部分,保留神经血管束。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "手术切除肥大部分" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "保留神经血管束" } ] }, { "text": "【预防】1.新生儿筛查目前许多国家,包括上海地区已经开展了针对21-羟化酶缺乏症的新生儿筛查。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "新生儿筛查" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "21-羟化酶缺乏症" }, { "start_idx": 41, "end_idx": 45, "type": "pro", "entity": "新生儿筛查" } ] }, { "text": "具体方法是新生儿生后3天,在脚跟部位采血数滴于滤纸片上,测定干血滤纸片中17-羟孕酮的水平,同时还可测定干血中促甲状腺素和苯丙氨酸水平,进行先天性甲状腺功能减退症和苯丙酮尿症的新生儿筛查。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "脚跟部位采血" }, { "start_idx": 28, "end_idx": 44, "type": "ite", "entity": "测定干血滤纸片中17-羟孕酮的水平" }, { "start_idx": 50, "end_idx": 66, "type": "ite", "entity": "测定干血中促甲状腺素和苯丙氨酸水平" }, { "start_idx": 70, "end_idx": 80, "type": "dis", "entity": "先天性甲状腺功能减退症" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 88, "end_idx": 92, "type": "pro", "entity": "新生儿筛查" } ] }, { "text": "干血滴纸片法作为初筛,如结果异常,需要招回,再次采血测定17-羟孕酮,以及测定血电解质、ACTH及睾酮等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "干血滴纸片法" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "采血" }, { "start_idx": 26, "end_idx": 33, "type": "pro", "entity": "测定17-羟孕酮" }, { "start_idx": 37, "end_idx": 50, "type": "ite", "entity": "测定血电解质、ACTH及睾酮" } ] }, { "text": "新生儿筛查是早期诊断,目的是①预防危及生命的肾上腺皮质危象及盐皮质功能不足而导致的死亡;②预防女性患儿由于外生殖器男性化造成性别判断错误;③预防过多雄激素造成患儿日后身材矮小及心理生理发育等障碍。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "新生儿筛查" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "肾上腺皮质危象" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "盐皮质功能不足" }, { "start_idx": 53, "end_idx": 59, "type": "sym", "entity": "外生殖器男性化" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "外生殖" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "雄激素" }, { "start_idx": 83, "end_idx": 86, "type": "sym", "entity": "身材矮小" }, { "start_idx": 88, "end_idx": 96, "type": "dis", "entity": "心理生理发育等障碍" } ] }, { "text": "2.产前诊断患儿家庭再生育要进行遗传咨询。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "产前诊断" }, { "start_idx": 6, "end_idx": 19, "type": "pro", "entity": "患儿家庭再生育要进行遗传咨询" } ] }, { "text": "第五节腺样体肥大腺样体又称增殖体或咽扁桃体,系鼻咽顶后壁的淋巴组织。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "腺样体肥大" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "腺样体" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "增殖体" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "咽扁桃体" }, { "start_idx": 24, "end_idx": 33, "type": "bod", "entity": "鼻咽顶后壁的淋巴组织" } ] }, { "text": "腺样体肥大(adenoidalhypertrophy)是指腺样体由于受鼻咽部炎症的反复刺激而发生的病理性增生。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "腺样体肥大" }, { "start_idx": 6, "end_idx": 25, "type": "dis", "entity": "adenoidalhypertrophy" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "腺样体" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "鼻咽部炎症" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "病理性增生" } ] }, { "text": "多见于儿童,常与慢性扁桃体炎同时存在。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "慢性扁桃体炎" } ] }, { "text": "肥大的腺样体可堵塞后鼻孔及咽鼓管咽口,影响通气和鼻分泌物的清除。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "肥大的腺样体可堵塞后鼻孔及咽鼓管咽口" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "腺样体" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "后鼻孔" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "咽鼓管咽口" }, { "start_idx": 19, "end_idx": 30, "type": "sym", "entity": "影响通气和鼻分泌物的清除" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "鼻分泌物" } ] }, { "text": "说话时带闭塞性鼻音。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "说话时带闭塞性鼻音" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "鼻" } ] }, { "text": "婴幼儿可通过手指鼻咽触诊,扪及鼻咽顶部和后壁柔软块状物。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "手指鼻咽触诊" }, { "start_idx": 13, "end_idx": 26, "type": "sym", "entity": "扪及鼻咽顶部和后壁柔软块状物" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "鼻咽顶部和后壁" } ] }, { "text": "X线鼻咽侧位拍片,有助于诊断。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "X线鼻咽侧位拍片" } ] }, { "text": "腺样体肥大伴明显症状者,宜施行手术切除。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "腺样体肥大" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "手术切除" } ] }, { "text": "除非有明确扁桃体切除指征,一般不同时做扁桃体切除。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "扁桃体切除" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "扁桃体切除" } ] }, { "text": "四、异体和自身移植前必要条件1.符合移植指征。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "异体和自身移植" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "移植" } ] }, { "text": "2.异体移植时找到合适的供体。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "异体移植" } ] }, { "text": "3.受体的心、肺、肝、肾等各脏器功能正常,全身无活动性感染灶。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "心" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肺" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肝" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肾" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "脏器" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "活动性感染灶" } ] }, { "text": "4.恶性肿瘤患者,自身移植时骨髓必须无肿瘤细胞,异体移植则在完全CR后进行较合适。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "自身移植" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "骨髓" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "异体移植" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "完全CR" } ] }, { "text": "第八章感染性心内膜炎心内膜炎(endocarditis)指各种原因引起的心内膜炎症病变,常累及心脏瓣膜,也可累及室间隔缺损处、心内壁内膜或未闭动脉导管、动静脉瘘等处,按原因可分为感染性和非感染性两大类,非感染性心内膜炎包括:风湿性心内膜炎、类风湿性心内膜炎、系统性红斑狼疮性心内膜炎、新生儿急性症状性心内膜炎等,本章主要阐述感染性心内膜炎。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 15, "end_idx": 26, "type": "dis", "entity": "endocarditis" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "心内膜炎症病变" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "心脏瓣膜" }, { "start_idx": 56, "end_idx": 61, "type": "bod", "entity": "室间隔缺损处" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "心内壁内膜" }, { "start_idx": 69, "end_idx": 74, "type": "bod", "entity": "未闭动脉导管" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "动静脉瘘" }, { "start_idx": 101, "end_idx": 108, "type": "dis", "entity": "非感染性心内膜炎" }, { "start_idx": 112, "end_idx": 118, "type": "dis", "entity": "风湿性心内膜炎" }, { "start_idx": 120, "end_idx": 127, "type": "dis", "entity": "类风湿性心内膜炎" }, { "start_idx": 129, "end_idx": 140, "type": "dis", "entity": "系统性红斑狼疮性心内膜炎" }, { "start_idx": 142, "end_idx": 153, "type": "dis", "entity": "新生儿急性症状性心内膜炎" }, { "start_idx": 162, "end_idx": 168, "type": "dis", "entity": "感染性心内膜炎" } ] }, { "text": "感染性心内膜炎(infectiveendocarditis)在过去常分为急性和亚急性两个类型。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 8, "end_idx": 28, "type": "dis", "entity": "infectiveendocarditis" } ] }, { "text": "急性者多发生于原无心脏病的患儿,侵入细菌毒力较强,起病急骤,进展迅速,病程在6周以内。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "心脏病" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "细菌" } ] }, { "text": "亚急性者多在原有心脏病的基础上感染毒力较弱的细菌,起病潜隐,进展相对缓慢,病程超过6周。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "心脏病" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "感染毒力较弱的细菌" } ] }, { "text": "由于抗生素的广泛应用,本病的病程已延长,临床急性和亚急性难以截然划分,致病微生物除了最常见的细菌外,尚有真菌、衣原体、立克次体及病毒等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "抗生素" }, { "start_idx": 46, "end_idx": 47, "type": "mic", "entity": "细菌" }, { "start_idx": 52, "end_idx": 53, "type": "mic", "entity": "真菌" }, { "start_idx": 55, "end_idx": 57, "type": "mic", "entity": "衣原体" }, { "start_idx": 59, "end_idx": 62, "type": "mic", "entity": "立克次体" }, { "start_idx": 64, "end_idx": 65, "type": "mic", "entity": "病毒" } ] }, { "text": "近年来随着新型抗生素的不断出现,外科手术的进步,感染性心内膜炎死亡率已显著下降,但由于致病微生物的变迁,心脏手术和心导管检查的广泛开展,长期静脉插管输液的增多等因素,本病的发病率并无显著下降。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "抗生素" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "外科手术" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 52, "end_idx": 55, "type": "pro", "entity": "心脏手术" }, { "start_idx": 57, "end_idx": 61, "type": "pro", "entity": "心导管检查" }, { "start_idx": 70, "end_idx": 75, "type": "pro", "entity": "静脉插管输液" } ] }, { "text": "【病因】(一)心脏的原发病变92%的感染性心内膜炎患者均有原发心脏病变,其中以先天性心脏病最为多见,约占78%,室间隔缺损最易合并感染性心内膜炎,其他依次为法洛四联症、动脉导管未闭、肺动脉瓣狭窄、主动脉瓣狭窄、主动脉瓣二叶畸形、房间隔缺损等;后天性心脏病如风湿性瓣膜病、二尖瓣脱垂综合征等也可并发感染性心内膜炎,随着小儿心脏外科技术的发展,越来越多的小儿心脏病得以纠正、根治,但因此而留置在心腔内的装置或材料(如心内补片、人造心脏瓣等)是近年来感染性心内膜炎常见的易患因素。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "心脏的原发病变" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "原发心脏病变" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 65, "end_idx": 71, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 84, "end_idx": 89, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 91, "end_idx": 96, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 98, "end_idx": 103, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 105, "end_idx": 112, "type": "dis", "entity": "主动脉瓣二叶畸形" }, { "start_idx": 114, "end_idx": 118, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 121, "end_idx": 126, "type": "dis", "entity": "后天性心脏病" }, { "start_idx": 128, "end_idx": 133, "type": "dis", "entity": "风湿性瓣膜病" }, { "start_idx": 135, "end_idx": 142, "type": "dis", "entity": "二尖瓣脱垂综合征" }, { "start_idx": 148, "end_idx": 154, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 158, "end_idx": 165, "type": "pro", "entity": "小儿心脏外科技术" }, { "start_idx": 175, "end_idx": 179, "type": "dis", "entity": "小儿心脏病" }, { "start_idx": 195, "end_idx": 197, "type": "bod", "entity": "心腔内" }, { "start_idx": 206, "end_idx": 207, "type": "bod", "entity": "心内" }, { "start_idx": 213, "end_idx": 215, "type": "bod", "entity": "心脏瓣" }, { "start_idx": 222, "end_idx": 228, "type": "dis", "entity": "感染性心内膜炎" } ] }, { "text": "(二)病原体几乎所有种类的细菌均可导致感染性心内膜炎,草绿色链球菌仍为最常见的致病菌,但所占比例已显著下降,近年来金黄色葡萄球菌、白色葡萄球菌、肠球菌、产气杆菌等革兰阴性杆菌引起的感染性心内膜炎显著增多,真菌性心内膜炎极少见。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "细菌" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 27, "end_idx": 32, "type": "mic", "entity": "草绿色链球菌" }, { "start_idx": 39, "end_idx": 41, "type": "mic", "entity": "致病菌" }, { "start_idx": 57, "end_idx": 63, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 65, "end_idx": 70, "type": "mic", "entity": "白色葡萄球菌" }, { "start_idx": 72, "end_idx": 74, "type": "mic", "entity": "肠球菌" }, { "start_idx": 76, "end_idx": 79, "type": "mic", "entity": "产气杆菌" }, { "start_idx": 81, "end_idx": 86, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 90, "end_idx": 96, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 102, "end_idx": 108, "type": "dis", "entity": "真菌性心内膜炎" } ] }, { "text": "立克次体及病毒感染所致的心内膜炎甚罕见,少数情况下,感染性心内膜炎由一种以上的病原体引起,常见于人工瓣膜手术者。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "立克次体及病毒感染" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 48, "end_idx": 53, "type": "pro", "entity": "人工瓣膜手术" } ] }, { "text": "其他致病因素如长期应用抗生素、皮质激素或免疫抑制剂等。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "抗生素" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "皮质激素" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "(三)诱发因素约1/3的患儿在病史中可找到诱发因素,常见的诱发因素为矫治牙病和扁桃体摘除术。", "entities": [ { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "矫治牙病" }, { "start_idx": 39, "end_idx": 44, "type": "pro", "entity": "扁桃体摘除术" } ] }, { "text": "近年来心导管检查和介入性治疗、人工瓣膜置换、心内直视手术的广泛开展,也是感染性心内膜炎的重要诱发因素之一,其他诱发因素如长期使用抗生素、肾上腺皮质激素、免疫抑制剂等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心导管检查" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "介入性治疗" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "人工瓣膜置换" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "心内直视手术" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 64, "end_idx": 66, "type": "dru", "entity": "抗生素" }, { "start_idx": 68, "end_idx": 74, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 76, "end_idx": 80, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "【病理及病理生理】正常人口腔和上呼吸道常聚集一些细菌,一般不会致病,只有在机体防御功能低下时可侵入血流,特别是口腔感染、拔牙、扁桃体摘除术时易侵入血流。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "口腔" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "细菌" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "防御功能低下" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "血流" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "口腔感染" }, { "start_idx": 60, "end_idx": 61, "type": "pro", "entity": "拔牙" }, { "start_idx": 63, "end_idx": 68, "type": "pro", "entity": "扁桃体摘除术" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "血流" } ] }, { "text": "当心腔内膜,特别是心瓣膜存在病理改变或先天性缺损时,细菌易在心瓣膜、心内膜和动脉内膜表面粘着、繁殖,从而形成心内膜炎;但若形成一种病变尚需下列条件,即双侧心室或大血管之间有较大的压力差,能够产生高速的血流,经常冲击心内膜面,使之损伤,心内膜下胶原组织暴露,血小板和纤维蛋白聚积形成无菌性赘生物,当有菌血症时,细菌易在上述部位黏附、定居,并繁殖,形成有菌赘生物。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "心腔内膜" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "心瓣膜" }, { "start_idx": 9, "end_idx": 23, "type": "sym", "entity": "心瓣膜存在病理改变或先天性缺损" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "细菌" }, { "start_idx": 30, "end_idx": 43, "type": "bod", "entity": "心瓣膜、心内膜和动脉内膜表面" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "双侧心室" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "大血管" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "血流" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "心内膜面" }, { "start_idx": 114, "end_idx": 115, "type": "dis", "entity": "损伤" }, { "start_idx": 117, "end_idx": 124, "type": "bod", "entity": "心内膜下胶原组织" }, { "start_idx": 128, "end_idx": 130, "type": "bod", "entity": "血小板" }, { "start_idx": 132, "end_idx": 135, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 140, "end_idx": 145, "type": "sym", "entity": "无菌性赘生物" }, { "start_idx": 149, "end_idx": 151, "type": "dis", "entity": "菌血症" }, { "start_idx": 154, "end_idx": 155, "type": "mic", "entity": "细菌" }, { "start_idx": 175, "end_idx": 178, "type": "sym", "entity": "菌赘生物" }, { "start_idx": 175, "end_idx": 175, "type": "mic", "entity": "菌" } ] }, { "text": "在病理上,受累部位多在压力低的一侧,如室间隔缺损感染性赘生物常见于缺损的右缘、三尖瓣的隔叶及肺动脉瓣;动脉导管在肺动脉侧;主动脉关闭不全在左心室等。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "感染性赘生物" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "缺损的右缘" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "三尖瓣的隔叶" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "动脉导管" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "肺动脉侧" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "主动脉关闭不全" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "左心室" } ] }, { "text": "当狭窄瓣孔及异常通道两侧心室或管腔之间的压力差越大时,湍流越明显,在压力低的一侧越易形成血栓和赘生物。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "狭窄瓣孔" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "异常通道两侧" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心室" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "管腔" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "血栓" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "赘生物" } ] }, { "text": "当房间隔缺损、大型室间隔缺损、并发心力衰竭等时,由于异常通道两侧压力差减小,血流速度减慢,湍流相对不明显,一般较少并发感染性心内膜炎。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "大型室间隔缺损" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "异常通道两侧压力差减小" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "异常通道" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "血流速度减慢" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "血流" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "湍流" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "感染性心内膜炎" } ] }, { "text": "本病的基本病理改变是心瓣膜、心内膜及大血管内膜面附着疣状感染性赘生物。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "心瓣膜" }, { "start_idx": 10, "end_idx": 33, "type": "sym", "entity": "心瓣膜、心内膜及大血管内膜面附着疣状感染性赘生物" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "心内膜" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "大血管内膜面" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "疣状感染性赘生物" } ] }, { "text": "赘生物由血小板、白细胞、红细胞、纤维蛋白、胶原组织和致病微生物等组成,心脏瓣膜的赘生物可致瓣膜溃疡、穿孔,若累及腱索和乳头肌,可使腱索缩短及断裂瓣环和心肌时,可致心肌脓疡室间隔穿孔动脉瘤等,大的或多量的赘生物可堵塞瓣膜口或肺动脉,致急性循环障碍。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "赘生物" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "血小板" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "白细胞" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "红细胞" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "胶原组织" }, { "start_idx": 26, "end_idx": 30, "type": "mic", "entity": "致病微生物" }, { "start_idx": 35, "end_idx": 42, "type": "bod", "entity": "心脏瓣膜的赘生物" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "瓣膜溃疡、穿孔" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "瓣膜" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "腱索" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "乳头肌" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "腱索" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "腱索缩短及断裂" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "瓣环" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "心肌" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "心肌脓疡" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "室间隔穿孔" }, { "start_idx": 90, "end_idx": 92, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 95, "end_idx": 103, "type": "bod", "entity": "大的或多量的赘生物" }, { "start_idx": 95, "end_idx": 113, "type": "sym", "entity": "大的或多量的赘生物可堵塞瓣膜口或肺动脉" }, { "start_idx": 107, "end_idx": 109, "type": "bod", "entity": "瓣膜口" }, { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "肺动脉" }, { "start_idx": 116, "end_idx": 121, "type": "dis", "entity": "急性循环障碍" } ] }, { "text": "赘生物受高速血流冲击可有血栓脱落,随血流散布到全身血管导致器官栓塞。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "赘生物" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血流" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "血栓脱落" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "血" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血流" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "全身血管" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "器官栓塞" } ] }, { "text": "右心的栓子引起肺栓塞;左心的栓子引起肾、脑、脾、四肢、肠系膜等动脉栓塞,微小栓子栓塞毛细血管出现皮肤瘀点,即欧氏小结(Oslernode)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "右心的栓子" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肺栓塞" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "左心的栓子" }, { "start_idx": 18, "end_idx": 34, "type": "dis", "entity": "肾、脑、脾、四肢、肠系膜等动脉栓塞" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "微小栓子" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "微小栓子栓塞毛细血管" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "毛细血管" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "皮肤瘀点" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "皮肤" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "欧氏小结" }, { "start_idx": 59, "end_idx": 67, "type": "sym", "entity": "Oslernode" } ] }, { "text": "肾栓塞时可致梗死,局灶性肾炎,或弥漫性肾小球肾炎;脑栓塞时可发生脑膜、脑实质、脊髓、脑神经等弥漫性炎症,产生出血、水肿、脑软化、脑脓疡、颅内动脉瘤破裂等病变,后者破裂可引起颅内各部位的出血如脑出血、蜘蛛膜下腔出血等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾栓塞" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "梗死" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "局灶性肾炎" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "弥漫性肾小球肾炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "脑栓塞" }, { "start_idx": 32, "end_idx": 50, "type": "dis", "entity": "脑膜、脑实质、脊髓、脑神经等弥漫性炎症" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "出血" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "血" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "水肿" }, { "start_idx": 60, "end_idx": 62, "type": "sym", "entity": "脑软化" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "脑" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "脑脓疡" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "颅内动脉瘤破裂" }, { "start_idx": 81, "end_idx": 82, "type": "sym", "entity": "破裂" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "颅内各部位" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "出血" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "血" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "脑出血" }, { "start_idx": 99, "end_idx": 105, "type": "dis", "entity": "蜘蛛膜下腔出血" } ] }, { "text": "【临床表现】大多数患者有器质性心脏病,部分病人发病前有龋齿、扁桃体炎、静脉插管、介入治疗或心内手术史,临床症状可归纳为三方面:①全身感染症状;②心脏症状;③栓塞及血管症状。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "器质性心脏病" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "龋齿" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "静脉插管" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "介入治疗" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "心内手术" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "全身感染" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "心脏" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "心脏症状" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "栓塞" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "血管" }, { "start_idx": 81, "end_idx": 84, "type": "sym", "entity": "血管症状" } ] }, { "text": "(一)感染症状发热是最常见的症状,几乎所有的病例都有过不同程度的发热,热型不规则,热程较长,个别病例无发热,此外患者有疲乏、盗汗、食欲减退、体重减轻、关节痛、皮肤苍白等表现,病情进展较慢。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "感染" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "发热" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "发热" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "疲乏" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "盗汗" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "食欲减退" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "体重减轻" }, { "start_idx": 75, "end_idx": 77, "type": "sym", "entity": "关节痛" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "关节" }, { "start_idx": 79, "end_idx": 82, "type": "sym", "entity": "皮肤苍白" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "皮肤" } ] }, { "text": "(二)心脏方面的症状原有的心脏杂音可因心脏瓣膜的赘生物而发生改变,出现粗糙、响亮、呈海鸥鸣样或音乐样的杂音。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "心脏方面的症状" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "心脏杂音" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 19, "end_idx": 26, "type": "bod", "entity": "心脏瓣膜的赘生物" }, { "start_idx": 35, "end_idx": 52, "type": "sym", "entity": "粗糙、响亮、呈海鸥鸣样或音乐样的杂音" } ] }, { "text": "原无心脏杂音者可出现音乐样杂音,约一半患儿由于心瓣膜病变、中毒性心肌炎等导致充血性心力衰竭,出现心音低钝、奔马律等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心脏" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "心脏杂音" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "音乐样杂音" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "心瓣膜病变" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "中毒性心肌炎" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "心音低钝" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "心" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "奔马律" } ] }, { "text": "(三)栓塞症状视栓塞部位的不同而出现不同的临床表现,一般发生于病程后期,但约1/3的患者为首发症状,皮肤栓塞可见散在的小瘀点,指(趾)的腹面可触到隆起的紫红色的小结节,略有触痛,此即欧氏小结。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "栓塞" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "栓塞" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "皮肤栓塞" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "散在的小瘀点" }, { "start_idx": 63, "end_idx": 82, "type": "sym", "entity": "指(趾)的腹面可触到隆起的紫红色的小结节" }, { "start_idx": 63, "end_idx": 69, "type": "bod", "entity": "指(趾)的腹面" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "小结节" }, { "start_idx": 84, "end_idx": 87, "type": "sym", "entity": "略有触痛" }, { "start_idx": 91, "end_idx": 94, "type": "sym", "entity": "欧氏小结" } ] }, { "text": "病程久者可见杵状指、趾,但无发绀。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "杵状指、趾" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "指" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "趾" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "发绀" } ] }, { "text": "【实验室检查】(一)血培养血细菌培养阳性是确诊感染性心内膜炎的重要依据,凡原因未明的发热、体温持续在1周以上,且原有心脏病者,均应积极反复多次进行血培养,以提高阳性率,若血培养阳性,尚应做药物敏感试验。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "血培养" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "血细菌培养阳性" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "血细菌培养" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "发热" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "心脏病" }, { "start_idx": 73, "end_idx": 75, "type": "pro", "entity": "血培养" }, { "start_idx": 85, "end_idx": 89, "type": "sym", "entity": "血培养阳性" }, { "start_idx": 85, "end_idx": 87, "type": "pro", "entity": "血培养" }, { "start_idx": 94, "end_idx": 99, "type": "pro", "entity": "药物敏感试验" } ] }, { "text": "(二)超声心动图超声心动图检查能够检出直径大于2mm以上的赘生物,因此对诊断感染性心内膜炎很有帮助,此外在治疗过程中超声心动图还可动态观察赘生物大小、形态、活动和瓣膜功能状态,了解瓣膜损害程度,对决定是否做换瓣手术有参考价值。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "赘生物" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 58, "end_idx": 62, "type": "pro", "entity": "超声心动图" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "赘生物" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "瓣膜" }, { "start_idx": 90, "end_idx": 93, "type": "dis", "entity": "瓣膜损害" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "换瓣手术" } ] }, { "text": "该检查还可发现原有的心脏病。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "心脏病" } ] }, { "text": "(三)CT对怀疑有颅内病变者应及时做CT,了解病变的部位范围。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "CT" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "颅内病变" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "CT" } ] }, { "text": "(四)其他血常规可见进行性贫血,多为正细胞性贫血,白细胞计数增高和中性粒细胞升高,血沉快C反应蛋白阳性,血清球蛋白常常增多,免疫球蛋白升高,循环免疫复合物及类风湿因子阳性,尿常规有红细胞发热期可出现蛋白尿。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "血常规" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "进行性贫血" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "正细胞性贫血" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "白细胞计数增高" }, { "start_idx": 25, "end_idx": 29, "type": "ite", "entity": "白细胞计数" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "中性粒细胞升高" }, { "start_idx": 33, "end_idx": 37, "type": "ite", "entity": "中性粒细胞" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "血沉" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "血沉快" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "C反应蛋白阳性" }, { "start_idx": 44, "end_idx": 48, "type": "ite", "entity": "C反应蛋白" }, { "start_idx": 52, "end_idx": 60, "type": "sym", "entity": "血清球蛋白常常增多" }, { "start_idx": 52, "end_idx": 56, "type": "ite", "entity": "血清球蛋白" }, { "start_idx": 62, "end_idx": 68, "type": "sym", "entity": "免疫球蛋白升高" }, { "start_idx": 62, "end_idx": 66, "type": "ite", "entity": "免疫球蛋白" }, { "start_idx": 70, "end_idx": 84, "type": "sym", "entity": "循环免疫复合物及类风湿因子阳性" }, { "start_idx": 70, "end_idx": 76, "type": "ite", "entity": "循环免疫复合物" }, { "start_idx": 78, "end_idx": 82, "type": "ite", "entity": "类风湿因子" }, { "start_idx": 86, "end_idx": 88, "type": "ite", "entity": "尿常规" }, { "start_idx": 86, "end_idx": 92, "type": "sym", "entity": "尿常规有红细胞" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "红细胞" }, { "start_idx": 93, "end_idx": 94, "type": "sym", "entity": "发热" }, { "start_idx": 99, "end_idx": 101, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 99, "end_idx": 100, "type": "bod", "entity": "蛋白" }, { "start_idx": 101, "end_idx": 101, "type": "bod", "entity": "尿" } ] }, { "text": "【诊断】对原有心脏病的患儿,如出现1周以上不明原因的发热应想到本病的可能,诊断除了病史、临床表现外,血培养是确诊的关键,超声心动图对判断赘生物的数目、大小、形态、位置和瓣膜的功能有重要的价值,但结果阴性不能排除本病的诊断。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "心脏病" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" }, { "start_idx": 50, "end_idx": 52, "type": "pro", "entity": "血培养" }, { "start_idx": 60, "end_idx": 64, "type": "pro", "entity": "超声心动图" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "赘生物" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "瓣膜" } ] }, { "text": "【治疗】总的原则是积极抗感染、加强支持疗法,但在应用抗生素之前必须先做几次血培养和药物敏感试验,以期对选用抗生素及剂量提供指导。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "积极抗感染" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "加强支持疗法" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "抗生素" }, { "start_idx": 37, "end_idx": 39, "type": "pro", "entity": "血培养" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "药物敏感试验" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "抗生素" } ] }, { "text": "(一)抗生素应用原则是早期、联合应用、剂量足、选用敏感的杀菌药,疗程要长。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "抗生素" }, { "start_idx": 25, "end_idx": 30, "type": "dru", "entity": "敏感的杀菌药" } ] }, { "text": "在具体应用时,对不同的病原菌感染选用不同的抗生素:①草绿色链球菌:首选青霉素G2000万U/d,分4次,每6小时1次,静脉滴注,疗程4~6周;加庆大霉素4~6mg/(kg•d),每8小时1次,疗程2周;对青霉素过敏者可选用头孢菌素类或万古霉素。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "病原菌感染" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "抗生素" }, { "start_idx": 26, "end_idx": 31, "type": "mic", "entity": "草绿色链球菌" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "青霉素G" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 72, "end_idx": 75, "type": "dru", "entity": "庆大霉素" }, { "start_idx": 102, "end_idx": 104, "type": "dru", "entity": "青霉素" }, { "start_idx": 102, "end_idx": 106, "type": "sym", "entity": "青霉素过敏" }, { "start_idx": 111, "end_idx": 115, "type": "dru", "entity": "头孢菌素类" }, { "start_idx": 117, "end_idx": 120, "type": "dru", "entity": "万古霉素" } ] }, { "text": "②金黄色葡萄球菌:对青霉素敏感者选用青霉素G2000万U/d,加庆大霉素,用法同上;青霉素耐药才选用新青霉素Ⅱ或新青霉素Ⅲ200~300mg/(kg•d),分4次,每6小时1次静脉滴注。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "青霉素" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "青霉素G" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "庆大霉素" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "青霉素" }, { "start_idx": 50, "end_idx": 54, "type": "dru", "entity": "新青霉素Ⅱ" }, { "start_idx": 56, "end_idx": 60, "type": "dru", "entity": "新青霉素Ⅲ" }, { "start_idx": 88, "end_idx": 91, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "④真菌:应停用抗生素,选用二性霉素B0.1~0.25mg/(kg•d),以后每日逐渐增加至1mg/(kg•d),静脉滴注1次,可合用5-氟胞嘧啶50~150mg/(kg•d),分3~4次服用;⑤病原菌不明或术后者:选用新青霉素Ⅲ加氨苄西林及庆大霉素,或头孢菌素类;或万古霉素。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "真菌" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "抗生素" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "二性霉素B" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 66, "end_idx": 71, "type": "dru", "entity": "5-氟胞嘧啶" }, { "start_idx": 97, "end_idx": 99, "type": "mic", "entity": "病原菌" }, { "start_idx": 109, "end_idx": 113, "type": "dru", "entity": "新青霉素Ⅲ" }, { "start_idx": 115, "end_idx": 118, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 120, "end_idx": 123, "type": "dru", "entity": "庆大霉素" }, { "start_idx": 126, "end_idx": 130, "type": "dru", "entity": "头孢菌素类" }, { "start_idx": 133, "end_idx": 136, "type": "dru", "entity": "万古霉素" } ] }, { "text": "上述抗感染药物应连用4~8周,用至体温正常,栓塞现象消失,血象、血沉恢复正常,血培养阴性后逐渐停药。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "抗感染药物" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "体温" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "栓塞现象" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "血象" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "血沉" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "血培养" } ] }, { "text": "(二)一般治疗包括细心护理,保证病人充足的热量供应,可少量多次输新鲜血或血浆,也可输注丙种球蛋白。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "细心护理" }, { "start_idx": 14, "end_idx": 24, "type": "pro", "entity": "保证病人充足的热量供应" }, { "start_idx": 27, "end_idx": 37, "type": "pro", "entity": "少量多次输新鲜血或血浆" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "输注" }, { "start_idx": 43, "end_idx": 47, "type": "dru", "entity": "丙种球蛋白" } ] }, { "text": "(三)手术治疗近年来早期外科治疗感染性心内膜炎取得了良好效果。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "手术治疗" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "外科治疗" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "感染性心内膜炎" } ] }, { "text": "对心脏赘生物和污染的人造代用品清创、修复或置换损害的瓣膜,挽救了严重病人,提高了治愈率,手术指征:①瓣膜功能不全引起的中、重度心力衰竭;②赘生物阻塞瓣膜口;③反复发生栓塞;④真菌感染;⑤经最佳抗生素治疗无效;⑥新发生的心脏传导阻滞。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "心脏赘生物" }, { "start_idx": 15, "end_idx": 27, "type": "pro", "entity": "清创、修复或置换损害的瓣膜" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "手术" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "瓣膜功能不全" }, { "start_idx": 59, "end_idx": 66, "type": "dis", "entity": "中、重度心力衰竭" }, { "start_idx": 69, "end_idx": 76, "type": "sym", "entity": "赘生物阻塞瓣膜口" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "赘生物" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "瓣膜口" }, { "start_idx": 79, "end_idx": 84, "type": "sym", "entity": "反复发生栓塞" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "真菌感染" }, { "start_idx": 94, "end_idx": 100, "type": "pro", "entity": "最佳抗生素治疗" }, { "start_idx": 109, "end_idx": 114, "type": "dis", "entity": "心脏传导阻滞" } ] }, { "text": "【预后和预防】在应用抗生素治疗前本病的死亡率几乎为100%。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "经合理应用抗生素治疗以来,近年病死率已下降为20%~25%。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "因此预防感染性心内膜炎发生显得极为重要。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "感染性心内膜炎" } ] }, { "text": "有先天性或风湿性心脏病患儿平时应注意口腔卫生,防止齿龈炎、龋齿;预防感染;若施行口腔手术、扁桃体摘除术、心导管和心脏手术时,可于术前1~2小时及术后48小时内肌注青霉素80万U/d,或长效青霉素120万U1剂。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "先天性或风湿性心脏病" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "注意口腔卫生" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "齿龈炎" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "龋齿" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "感染" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "口腔手术" }, { "start_idx": 45, "end_idx": 50, "type": "pro", "entity": "扁桃体摘除术" }, { "start_idx": 52, "end_idx": 59, "type": "pro", "entity": "心导管和心脏手术" }, { "start_idx": 79, "end_idx": 80, "type": "pro", "entity": "肌注" }, { "start_idx": 81, "end_idx": 83, "type": "dru", "entity": "青霉素" }, { "start_idx": 92, "end_idx": 96, "type": "dru", "entity": "长效青霉素" } ] }, { "text": "青霉素过敏者,可选用头孢菌素类或万古霉素静脉注射一次,然后改口服红霉素30mg/(kg•d),分4次服用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "青霉素" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "青霉素过敏" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "头孢菌素类" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "万古霉素" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉注射" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "口服" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "红霉素" } ] }, { "text": "四、哮喘的长期管理计划长期管理是哮喘防治的重要环节之一,由于哮喘是一种慢性呼吸道疾病,治疗时间长,而且大部分时间在家中治疗,因而对患儿进行病情的随访、监控,及时接受患儿及家长的咨询,对于控制疾病尤为重要。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "哮喘" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "哮喘" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "哮喘" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "慢性呼吸道疾病" }, { "start_idx": 64, "end_idx": 76, "type": "pro", "entity": "对患儿进行病情的随访、监控" }, { "start_idx": 78, "end_idx": 89, "type": "pro", "entity": "及时接受患儿及家长的咨询" } ] }, { "text": "哮喘的长期管理计划包括以下六个部分:1.教育患者与医生发展成伙伴关系。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "哮喘" } ] }, { "text": "2.尽可能应用肺功能评估和监测哮喘的症状的严重程度。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "肺功能评估" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "哮喘" } ] }, { "text": "3.避免和控制哮喘的触发因素。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "pro", "entity": "避免和控制哮喘的触发因素" } ] }, { "text": "四、气道异常(一)后鼻孔闭锁有膜性及骨性两类,单侧和双侧,X线检查可将水溶性造影剂注入鼻腔,如造影剂未进入咽部即可确诊。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "气道异常" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "后鼻孔闭锁" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "膜" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "骨" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "X线检查" }, { "start_idx": 35, "end_idx": 40, "type": "dru", "entity": "水溶性造影剂" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "鼻腔" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "造影剂未进入咽部" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "咽部" } ] }, { "text": "CT扫描可鉴别膜性及骨性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CT扫描" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "膜" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "骨" } ] }, { "text": "(二)气管软化原发性气管软化见于各种软骨综合征及全身性疾病,继发性见于大血管畸形和气管外伤等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "气管软化" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "原发性气管软化" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "软骨综合征" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "大血管畸形" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "气管外伤" } ] }, { "text": "X线胸片检查以高千伏为好,CT扫描有助于诊断,主要表现气管的过度塌陷(三)支气管异物常为单侧,右侧居多。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "X线胸片检查" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "CT扫描" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "气管" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "气管的过度塌陷" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "支气管异物" } ] }, { "text": "胸片表现以呼气时明显,吸气时两侧肺野透亮度则对称,透视见纵隔摆动,CT扫描可显示支气管内异物。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "胸片" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "两侧肺野" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "透视" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "透视见纵隔摆动" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "纵隔" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "CT扫描" }, { "start_idx": 33, "end_idx": 45, "type": "sym", "entity": "CT扫描可显示支气管内异物" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "支气管内" } ] }, { "text": "(四)外伤性支气管断裂单侧多见,开始表现为大量气胸,引流后气体不能减少。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "外伤性支气管断裂" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "大量气胸" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "胸" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "引流" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "气体不能减少" } ] }, { "text": "当断裂两端闭塞时,则肺野萎陷不张,纵隔心脏向病侧移位,CT扫描可显示支气管不连续。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "断裂两端闭塞" }, { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "断裂两端" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "肺野萎陷不张" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肺野" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "纵隔" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "纵隔心脏向病侧移位" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心脏" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "CT扫描" }, { "start_idx": 27, "end_idx": 39, "type": "sym", "entity": "CT扫描可显示支气管不连续" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "支气管" } ] }, { "text": "三、腹腔结核病腹腔结核病包括胃、肝、脾、肠、腹膜及肠系膜淋巴结结核。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "腹腔结核病" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "腹腔结核病" }, { "start_idx": 14, "end_idx": 32, "type": "dis", "entity": "胃、肝、脾、肠、腹膜及肠系膜淋巴结结核" } ] }, { "text": "其中以肠、腹膜及肠系膜淋巴结结核为多见。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "肠、腹膜及肠系膜淋巴结结核" } ] }, { "text": "三者之间有密切联系,多同时存在,但临床上亦可表现为以某一脏器为主,成为单独的病型。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "脏器" } ] }, { "text": "(一)传染途径最常见于消化道传染,饮用带结核杆菌的牛奶,食入和舔啃被结核杆菌污染过的食具或物品,以及吞咽带菌痰液可使结核杆菌侵入肠道;也可血行播散,腹部病变为全身粟粒结核病的一部分;另外,胸腔内淋巴结结核经淋巴管逆流可侵犯腹腔内淋巴结。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "消化道" }, { "start_idx": 20, "end_idx": 23, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 34, "end_idx": 37, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "带菌痰液" }, { "start_idx": 58, "end_idx": 61, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "肠道" }, { "start_idx": 69, "end_idx": 69, "type": "bod", "entity": "血" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "腹部病变" }, { "start_idx": 79, "end_idx": 85, "type": "dis", "entity": "全身粟粒结核病" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "胸腔" }, { "start_idx": 97, "end_idx": 101, "type": "bod", "entity": "淋巴结结核" }, { "start_idx": 103, "end_idx": 105, "type": "bod", "entity": "淋巴管" }, { "start_idx": 111, "end_idx": 116, "type": "bod", "entity": "腹腔内淋巴结" } ] }, { "text": "肠系膜淋巴结结核可为肠道原发综合征的一部分,肠原发灶可很快吸收,但肿大且干酪样变的肠系膜淋巴结则可长期存在。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "肠系膜淋巴结结核" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肠道原发综合征" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肠" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "肿大" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "干酪样变" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "肠系膜淋巴结" } ] }, { "text": "也可由淋巴或血行播散而来,多与胸腔内淋巴结结核或全身粟粒结核病同时存在。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "淋巴" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "血" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "胸腔内淋巴结结核" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "全身粟粒结核病" } ] }, { "text": "结核性腹膜炎可能是全身血行播散的一部分,但更多见的是由肠结核、肠系膜淋巴结结核或由泌尿生殖系统结核直接蔓延而来。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "结核性腹膜炎" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肠结核" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "肠系膜淋巴结结核" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "泌尿生殖系统结核" } ] }, { "text": "(二)临床表现可有不规则发热和消化道功能障碍,包括食欲减退、消化不良、恶心、呕吐、腹胀、腹泻或腹泻与便秘相交替。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "不规则发热" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "消化道功能障碍" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "食欲减退" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "消化不良" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "恶心" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "呕吐" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "腹胀" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "腹" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "腹泻" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "腹" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "腹泻与便秘相交替" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "腹" } ] }, { "text": "肠道结核病变可发生于肠道的任何部分,但好发部位为回盲部。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "肠道结核病变" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肠道" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "回盲部" } ] }, { "text": "肠道狭窄时可出现阵发性绞痛,常伴有肠鸣,腹部出现肠型和蠕动波溃疡型肠结核可大便带血,有时是脓血便。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肠道狭窄" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "阵发性绞痛" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "肠鸣" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肠" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "腹部" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "腹部出现肠型和蠕动波" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "溃疡型肠结核" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "大便带血" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "大便" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "血" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "有时是脓血便" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "脓血" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "便" } ] }, { "text": "大便镜检与细菌培养以及发现身体其他部位的结核病变可协助诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "大便镜检" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "细菌培养" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "结核病变" } ] }, { "text": "肠系膜淋巴结结核触诊时可发现典型的压痛点,常在右下腹相当于阑尾炎点处或在左上腹腔带相当于第2腰椎水平即肠系膜根处。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "肠系膜淋巴结结核" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "触诊" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "触诊时可发现典型的压痛点" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "右下腹" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "阑尾炎" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "左上腹腔" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "第2腰椎水平" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "肠系膜根处" } ] }, { "text": "肿大淋巴结X线检查在肠结核,口服钡剂或钡灌肠后,X线检查早期可见肠蠕动亢进钡剂通过迅速及肠管痉挛肠段激惹性增强、盲肠钡影残缺肠黏膜褶襞僵直及增厚黏膜充盈缺损、粘连肠道狭窄腹腔淋巴结结核,腹部X线平片可发现钙化灶。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肿大淋巴结" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "淋巴结" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "X线检查" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肠结核" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "口服" }, { "start_idx": 16, "end_idx": 17, "type": "dru", "entity": "钡剂" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "钡灌肠" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "X线检查" }, { "start_idx": 24, "end_idx": 36, "type": "sym", "entity": "X线检查早期可见肠蠕动亢进" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "肠" }, { "start_idx": 37, "end_idx": 38, "type": "dru", "entity": "钡剂" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "肠管痉挛" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "肠段激惹性增强" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "肠段" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "盲肠" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "盲肠钡影残缺" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "肠黏膜" }, { "start_idx": 62, "end_idx": 71, "type": "sym", "entity": "肠黏膜褶襞僵直及增厚" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "黏膜" }, { "start_idx": 72, "end_idx": 80, "type": "sym", "entity": "黏膜充盈缺损、粘连" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "肠道狭窄" }, { "start_idx": 85, "end_idx": 91, "type": "dis", "entity": "腹腔淋巴结结核" }, { "start_idx": 93, "end_idx": 98, "type": "pro", "entity": "腹部X线平片" }, { "start_idx": 93, "end_idx": 104, "type": "sym", "entity": "腹部X线平片可发现钙化灶" } ] }, { "text": "2.腹部CT平扫和增强扫描,对诊断有重要价值。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "腹部CT平扫" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "增强扫描" } ] }, { "text": "CT平扫可以明确腹腔淋巴结受累的范围及分布情况,还能发现钙化的淋巴结CT增强扫描淋巴结环形强化或花环状强化腹腔淋巴结结核典型且较为常见的CT表现,是诊断该病的主要依据之一。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CT平扫" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "腹腔淋巴结" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "钙化的淋巴结" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "淋巴结" }, { "start_idx": 34, "end_idx": 39, "type": "pro", "entity": "CT增强扫描" }, { "start_idx": 34, "end_idx": 52, "type": "sym", "entity": "CT增强扫描淋巴结环形强化或花环状强化" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "淋巴结" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "腹腔淋巴结结核" }, { "start_idx": 68, "end_idx": 69, "type": "pro", "entity": "CT" } ] }, { "text": "(四)治疗一般处理与肺结核相同。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肺结核" } ] }, { "text": "应特别重视营养疗法,食用丰富的少渣软性食物,其中,应多含蛋白质、维生素及铁质。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "pro", "entity": "特别重视营养疗法" }, { "start_idx": 10, "end_idx": 37, "type": "pro", "entity": "食用丰富的少渣软性食物,其中,应多含蛋白质、维生素及铁质" } ] }, { "text": "发生肠狭窄及肠梗阻时应禁食,视需要而行胃及十二指肠减压,注意维持水及电解质的平衡。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肠狭窄" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "禁食" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "胃及十二指肠减压" }, { "start_idx": 30, "end_idx": 39, "type": "pro", "entity": "维持水及电解质的平衡" } ] }, { "text": "发生肠穿孔时宜开腹探查并施行肠段切除。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "开腹探查" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "肠段切除" } ] }, { "text": "术后仍应进行有效的抗结核药物治疗。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "pro", "entity": "术后仍应进行有效的抗结核药物治疗" } ] }, { "text": "肿大的淋巴结内科治疗无效且产生持久性压迫症状时,可考虑外科手术切除。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "肿大的淋巴结" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "淋巴结" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "内科治疗" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "持久性压迫症状" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "外科手术切除" } ] }, { "text": "对于渗出性腹膜炎,加用肾上腺皮质激素治疗可促进腹水吸收及减少粘连的发生,效果良好。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "渗出性腹膜炎" }, { "start_idx": 11, "end_idx": 17, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "腹水" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "腹" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "粘连" } ] }, { "text": "三、正常新生儿的护理新生儿病房应宽敞明亮,阳光充足,应按每人2~4m2的面积配备。", "entities": [] }, { "text": "新生儿病房要做到空气流通,有条件的单位可以使用层流空气。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "新生儿病房要做到空气流通" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "使用层流空气" } ] }, { "text": "新生儿病房的室温应保持在16~23℃为宜,在冬季可以适当提高室温,同时要保证新生儿病房55%~65%的湿度。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "pro", "entity": "新生儿病房的室温应保持在16~23℃为宜" }, { "start_idx": 21, "end_idx": 31, "type": "pro", "entity": "在冬季可以适当提高室温" }, { "start_idx": 35, "end_idx": 52, "type": "pro", "entity": "要保证新生儿病房55%~65%的湿度" } ] }, { "text": "新生儿病房应每天予以清洁,定期大扫除和消毒,并对病室进行紫外线消毒,定期空气菌落培养。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "新生儿病房应每天予以清洁" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "定期大扫除和消毒" }, { "start_idx": 23, "end_idx": 32, "type": "pro", "entity": "对病室进行紫外线消毒" }, { "start_idx": 34, "end_idx": 41, "type": "pro", "entity": "定期空气菌落培养" } ] }, { "text": "进入新生儿病房的医护人员应当做好消毒隔离工作,严格按照消毒隔离制度执行:①手卫生:每检查一位新生儿必须先规范洗手或用消毒液擦拭;切忌在检查患者的过程中用手接触自己的鼻孔、面部和口腔。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "pro", "entity": "进入新生儿病房的医护人员应当做好消毒隔离工作" }, { "start_idx": 23, "end_idx": 34, "type": "pro", "entity": "严格按照消毒隔离制度执行" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "手" }, { "start_idx": 41, "end_idx": 62, "type": "pro", "entity": "每检查一位新生儿必须先规范洗手或用消毒液擦拭" }, { "start_idx": 64, "end_idx": 89, "type": "pro", "entity": "切忌在检查患者的过程中用手接触自己的鼻孔、面部和口腔" } ] }, { "text": "②更换专用的消毒隔离衣和消毒隔离鞋,留长发的医护人员必须将长发盘起,如近期正患上呼吸道感染等疾病的医护人员必须戴一次性口罩。", "entities": [ { "start_idx": 1, "end_idx": 16, "type": "pro", "entity": "更换专用的消毒隔离衣和消毒隔离鞋" }, { "start_idx": 18, "end_idx": 32, "type": "pro", "entity": "留长发的医护人员必须将长发盘起" }, { "start_idx": 35, "end_idx": 60, "type": "pro", "entity": "近期正患上呼吸道感染等疾病的医护人员必须戴一次性口罩" } ] }, { "text": "③物品分开使用和消毒,所有用于新生儿的听诊器、皮尺等物品不能从外界带入,必须使用新生儿病房内已经消毒后的相应物品,尽最大可能减少医源性感染的发生。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "pro", "entity": "物品分开使用和消毒" }, { "start_idx": 11, "end_idx": 34, "type": "pro", "entity": "所有用于新生儿的听诊器、皮尺等物品不能从外界带入" }, { "start_idx": 36, "end_idx": 55, "type": "pro", "entity": "必须使用新生儿病房内已经消毒后的相应物品" }, { "start_idx": 57, "end_idx": 71, "type": "pro", "entity": "尽最大可能减少医源性感染的发生" } ] }, { "text": "④医护人员在为新生儿检查时必须注意不能将自己的身体依靠在小床或检查台上,要养成先看病历再检查患者的习惯。", "entities": [ { "start_idx": 1, "end_idx": 34, "type": "pro", "entity": "医护人员在为新生儿检查时必须注意不能将自己的身体依靠在小床或检查台上" }, { "start_idx": 36, "end_idx": 50, "type": "pro", "entity": "要养成先看病历再检查患者的习惯" } ] }, { "text": "⑤每次出入新生儿病房,必须重新更衣和清洗,新生儿病房内所有用品必须定期消毒,及时更换。", "entities": [ { "start_idx": 1, "end_idx": 19, "type": "pro", "entity": "每次出入新生儿病房,必须重新更衣和清洗" }, { "start_idx": 21, "end_idx": 41, "type": "pro", "entity": "新生儿病房内所有用品必须定期消毒,及时更换" } ] }, { "text": "⑥应该允许健康的父母进入新生儿病房探视新生儿,给新生儿以母乳喂养,但是必须执行更衣、清洗和物品使用的规范。", "entities": [ { "start_idx": 1, "end_idx": 51, "type": "pro", "entity": "应该允许健康的父母进入新生儿病房探视新生儿,给新生儿以母乳喂养,但是必须执行更衣、清洗和物品使用的规范" } ] }, { "text": "谢绝非相关人员进入新生儿病房,包括新生儿的其他亲属。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "pro", "entity": "谢绝非相关人员进入新生儿病房,包括新生儿的其他亲属" } ] }, { "text": "新生儿出生后的即刻护理:新生儿出生前,应及时准备好相应的标记牌,写明母亲的姓名和床号,准备给新生儿使用的床号;新生儿出生后立即填写新生儿的性别和出生时间。", "entities": [] }, { "text": "气道清理:胎儿娩出后,应立即为其做气道清理,保持呼吸道的通畅。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "气道清理" }, { "start_idx": 5, "end_idx": 20, "type": "pro", "entity": "胎儿娩出后,应立即为其做气道清理" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "呼吸道" } ] }, { "text": "保暖:新生儿娩出后,应立即将其放在已经预热的远红外暖床上,用干的、温暖的消毒纱布将新生儿身上的羊水擦拭干净,以减少由于水分蒸发造成的降温,并防止皮肤皱褶处潮湿、糜烂甚至感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "保暖" }, { "start_idx": 3, "end_idx": 27, "type": "pro", "entity": "新生儿娩出后,应立即将其放在已经预热的远红外暖床上" }, { "start_idx": 29, "end_idx": 52, "type": "pro", "entity": "用干的、温暖的消毒纱布将新生儿身上的羊水擦拭干净" }, { "start_idx": 72, "end_idx": 76, "type": "bod", "entity": "皮肤皱褶处" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "糜烂" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "感染" } ] }, { "text": "脐带:新生儿娩出后应在1分钟内结扎脐带,脐带结扎过迟可能造成新生儿红细胞增多症,尤其对低出生体重儿。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脐带" }, { "start_idx": 3, "end_idx": 18, "type": "pro", "entity": "新生儿娩出后应在1分钟内结扎脐带" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "脐带结扎" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "新生儿红细胞增多症" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "低出生体重儿" } ] }, { "text": "脐带结扎后,可以留有3~5cm的残端,用无菌纱布包扎,现一般不主张使用药品保护脐残端。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "脐带结扎" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "无菌纱布包扎" }, { "start_idx": 30, "end_idx": 41, "type": "pro", "entity": "不主张使用药品保护脐残端" } ] }, { "text": "在娩出时注意将胎儿保持与母亲相同水平高度,防止胎-母间输血新生儿贫血或新生儿红细胞增多症的发生。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "pro", "entity": "在娩出时注意将胎儿保持与母亲相同水平高度" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "胎-母间输血" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "新生儿贫血" }, { "start_idx": 35, "end_idx": 43, "type": "dis", "entity": "新生儿红细胞增多症" } ] }, { "text": "眼部:我国疾病预防和控制中心推荐,新生儿娩出后可以使用1%硝酸银眼药水或0.25%氯霉素滴眼,防止感染,但是这些措施对预防新生儿沙眼衣原体和疱疹病毒感染无效,同时注意硝酸银眼药水对眼部可能造成化学性结膜炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "眼部" }, { "start_idx": 27, "end_idx": 34, "type": "dru", "entity": "1%硝酸银眼药水" }, { "start_idx": 36, "end_idx": 45, "type": "dru", "entity": "0.25%氯霉素滴眼" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "感染" }, { "start_idx": 61, "end_idx": 75, "type": "dis", "entity": "新生儿沙眼衣原体和疱疹病毒感染" }, { "start_idx": 83, "end_idx": 88, "type": "dru", "entity": "硝酸银眼药水" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "眼部" }, { "start_idx": 96, "end_idx": 101, "type": "dis", "entity": "化学性结膜炎" } ] }, { "text": "新生儿日常护理:其重点在于保暖、喂养和预防感染。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "保暖" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "喂养" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "预防感染" } ] }, { "text": "保暖:新生儿病房应保持一定的温度,要防止温度的骤升骤降,尤其在冬季。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "保暖" }, { "start_idx": 3, "end_idx": 32, "type": "pro", "entity": "新生儿病房应保持一定的温度,要防止温度的骤升骤降,尤其在冬季" } ] }, { "text": "新生儿病房可以保持通风,但是切忌将新生儿放置在通风口;夏季高热时,切忌将新生儿直接放置在空调器的出风口。", "entities": [ { "start_idx": 0, "end_idx": 25, "type": "pro", "entity": "新生儿病房可以保持通风,但是切忌将新生儿放置在通风口" }, { "start_idx": 27, "end_idx": 50, "type": "pro", "entity": "夏季高热时,切忌将新生儿直接放置在空调器的出风口" } ] }, { "text": "喂养:生后即可直接给新生儿喂母乳,以后按需喂养。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "母乳" } ] }, { "text": "在生后2~3天内,可能母乳量较少,可以适当喂些糖水,但应以5%的浓度为宜。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "母乳" } ] }, { "text": "若暂时母亲没有乳汁分泌,可以人乳库的人乳喂养,也可以配方奶作人工或混合喂养。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "乳汁" }, { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "人乳库的人乳喂养" }, { "start_idx": 30, "end_idx": 36, "type": "pro", "entity": "人工或混合喂养" } ] }, { "text": "使用人工喂养时,应注意对奶瓶、奶头和配方奶调制器的消毒,已经配置好的配方奶可以放在冰箱内保存,但是不能超过24小时,而且从冰箱内取出的配方奶应加热后再给新生儿食用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "人工喂养" }, { "start_idx": 9, "end_idx": 26, "type": "pro", "entity": "注意对奶瓶、奶头和配方奶调制器的消毒" }, { "start_idx": 28, "end_idx": 56, "type": "pro", "entity": "已经配置好的配方奶可以放在冰箱内保存,但是不能超过24小时" }, { "start_idx": 60, "end_idx": 80, "type": "pro", "entity": "从冰箱内取出的配方奶应加热后再给新生儿食用" } ] }, { "text": "母乳喂养过程中要注意的问题是:人类免疫缺陷病毒(HIV)阳性的母亲不应母乳喂养;乙型肝炎抗原阳性的产妇根据本人的肝功能及乙型肝炎标记物化验结果新生儿出生后的主动、被动免疫情况而决定可否母乳喂养;活动性肺结核母亲,不应禁止其对新生儿的母乳喂养,诚然后者应注意隔离;母乳喂养前,母亲应注意乳房的清洁,近期正患传染性疾病的母亲应戴口罩。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "母乳喂养" }, { "start_idx": 15, "end_idx": 38, "type": "pro", "entity": "人类免疫缺陷病毒(HIV)阳性的母亲不应母乳喂养" }, { "start_idx": 60, "end_idx": 68, "type": "pro", "entity": "乙型肝炎标记物化验" }, { "start_idx": 97, "end_idx": 129, "type": "pro", "entity": "活动性肺结核母亲,不应禁止其对新生儿的母乳喂养,诚然后者应注意隔离" }, { "start_idx": 131, "end_idx": 163, "type": "pro", "entity": "母乳喂养前,母亲应注意乳房的清洁,近期正患传染性疾病的母亲应戴口罩" } ] }, { "text": "预防感染:新生儿预防感染的重点在于皮肤护理、脐部护理和空气传播途径。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "预防感染" }, { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "新生儿预防感染" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "皮肤护理" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "脐部护理" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "空气传播途径" } ] }, { "text": "新生儿沐浴时,应先洗头部,再洗躯干和四肢,尤其对会阴部的清洗过程中要注意水流方向应是从前往后,即从尿道口往肛门,不能逆转。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "头部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "躯干" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "四肢" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "会阴部" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "尿道口" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肛门" } ] }, { "text": "新生儿使用的皂液应是对新生儿皮肤无刺激的,所用的毛巾应是柔软的;对皮肤上的水滴应用毛巾吸干,而不是用毛巾揩擦,否则容易造成新生儿皮肤损伤。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "pro", "entity": "新生儿使用的皂液应是对新生儿皮肤无刺激的" }, { "start_idx": 21, "end_idx": 30, "type": "pro", "entity": "所用的毛巾应是柔软的" }, { "start_idx": 32, "end_idx": 53, "type": "pro", "entity": "对皮肤上的水滴应用毛巾吸干,而不是用毛巾揩擦" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "新生儿皮肤损伤" } ] }, { "text": "新生儿沐浴完成后,可以使用新生儿专用扑粉,在新生儿的颈部、腋下、腹股沟及会阴等皮肤皱褶较多处使用,但是切忌直接向新生儿撒扑粉。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "颈部" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "腋下" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "腹股沟" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "会阴" }, { "start_idx": 39, "end_idx": 45, "type": "bod", "entity": "皮肤皱褶较多处" } ] }, { "text": "注意新生儿耳道和鼻孔的清洁,但切忌用硬物挖耳道和鼻孔,可以使用卫生棉签轻轻地擦拭耳道和鼻孔。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "pro", "entity": "注意新生儿耳道和鼻孔的清洁" }, { "start_idx": 15, "end_idx": 25, "type": "pro", "entity": "切忌用硬物挖耳道和鼻孔" }, { "start_idx": 29, "end_idx": 44, "type": "pro", "entity": "使用卫生棉签轻轻地擦拭耳道和鼻孔" } ] }, { "text": "一般情况下,对新生儿不做常规的口腔护理,除非新生儿患有鹅口疮,方可以使用棉签蘸取1%的碳酸氢钠溶液擦拭新生儿的口腔黏膜。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "口腔护理" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 40, "end_idx": 48, "type": "dru", "entity": "1%的碳酸氢钠溶液" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "口腔黏膜" } ] }, { "text": "新生儿解大便后,应将会阴部擦拭干净,有条件处应予以水洗,然后在会阴部涂以鞣酸软膏,防止尿布疹。", "entities": [ { "start_idx": 0, "end_idx": 39, "type": "pro", "entity": "新生儿解大便后,应将会阴部擦拭干净,有条件处应予以水洗,然后在会阴部涂以鞣酸软膏" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "尿布疹" } ] }, { "text": "沐浴后要将新生儿脐部用洁净的卫生棉签拭净,必要时可以用75%的乙醇或苯扎溴铵酊擦拭。", "entities": [ { "start_idx": 0, "end_idx": 40, "type": "pro", "entity": "沐浴后要将新生儿脐部用洁净的卫生棉签拭净,必要时可以用75%的乙醇或苯扎溴铵酊擦拭" } ] }, { "text": "新生儿衣物清洗后可以使用太阳照晒,有条件者可以使用高温灭菌。", "entities": [ { "start_idx": 0, "end_idx": 28, "type": "pro", "entity": "新生儿衣物清洗后可以使用太阳照晒,有条件者可以使用高温灭菌" } ] }, { "text": "新生儿居室内应减少化学性空气清洁剂的使用。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "pro", "entity": "新生儿居室内应减少化学性空气清洁剂的使用" } ] }, { "text": "母亲的心跳可以给新生儿以安全和舒适的感受,让新生儿充分休息;母亲的话语可以开发新生儿的语言能力和智力。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "心" } ] }, { "text": "正常新生儿离开产院前应完成卡介苗和乙肝疫苗的接种。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "卡介苗" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "乙肝疫苗" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "接种" } ] }, { "text": "第九节射频消融术儿童快速性心律失常是儿科心血管疾病的重要组成部分,最常见的是室上性心动过速,包括房室折返性心动过速、房室结折返性心动过速、心房异位性心动过速、心房肌折返性心动过速,室性心动过速、房性扑动、房性颤动等在儿科少见。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "射频消融术" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "儿童快速性心律失常" }, { "start_idx": 18, "end_idx": 19, "type": "dep", "entity": "儿科" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "心血管疾病" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 48, "end_idx": 56, "type": "dis", "entity": "房室折返性心动过速" }, { "start_idx": 58, "end_idx": 67, "type": "dis", "entity": "房室结折返性心动过速" }, { "start_idx": 69, "end_idx": 77, "type": "dis", "entity": "心房异位性心动过速" }, { "start_idx": 79, "end_idx": 88, "type": "dis", "entity": "心房肌折返性心动过速" }, { "start_idx": 90, "end_idx": 95, "type": "dis", "entity": "室性心动过速" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "房性扑动" }, { "start_idx": 102, "end_idx": 105, "type": "dis", "entity": "房性颤动" }, { "start_idx": 108, "end_idx": 109, "type": "dep", "entity": "儿科" } ] }, { "text": "这种类型的心律失常如不及时诊治可发展为心功能不全、心动过速介导的心肌病,甚至死亡。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心律失常" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "心功能不全" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "心动过速" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "心肌病" } ] }, { "text": "快速型心律失常的治疗以往主要依靠药物,但由于药物治疗往往意味着终身,且药物有时也不能完全控制发作,药物本身的不良反应及某些抗心律失常药物的致心律失常作用等,致使儿童快速型心律失常的诊治一直成为儿科心血管疾病治疗的难点。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "快速型心律失常" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "药物治疗" }, { "start_idx": 61, "end_idx": 67, "type": "dru", "entity": "抗心律失常药物" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "心律失常" }, { "start_idx": 80, "end_idx": 88, "type": "dis", "entity": "儿童快速型心律失常" }, { "start_idx": 96, "end_idx": 97, "type": "dep", "entity": "儿科" }, { "start_idx": 98, "end_idx": 102, "type": "dis", "entity": "心血管疾病" } ] }, { "text": "近10年来成人与儿童的心律失常治疗发生了一场革命,这场革命主要是由导管消融技术的成熟引起的,这一技术以已使心律失常的诊断与“治愈”在心导管实验室内一次完成。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "心律失常" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "导管消融技术" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "心律失常" }, { "start_idx": 66, "end_idx": 68, "type": "equ", "entity": "心导管" } ] }, { "text": "某些抗心律失常药物的致心律失常作用也促进了这一治疗手段的发展。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "抗心律失常药物" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "心律失常" } ] }, { "text": "因此,心律失常的药物治疗,以前是心律失常治疗的主要手段,现在在许多情况下已退居二线。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心律失常" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "药物治疗" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "心律失常" } ] }, { "text": "(一)频消融术的基本原理射频导管消融术(radiofrequencycatherizationablation,RFCA)通常与侵入性电生理检查结合进行。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "频消融术" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "射频导管消融术" }, { "start_idx": 20, "end_idx": 54, "type": "pro", "entity": "radiofrequencycatherizationablation" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "RFCA" }, { "start_idx": 64, "end_idx": 71, "type": "pro", "entity": "侵入性电生理检查" } ] }, { "text": "先行电生理检查识别与定位致病理性心动过速的基质。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "电生理检查" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "病理性心动过速" } ] }, { "text": "这一基质可以是旁道(可引起房室折返性心动过速或预激综合征);心房或心室的异常兴奋灶(房性异位性心动过速或室性心动过速);心房或心室的一个瘢痕,形成折返性心动过速环路的一个成分(房扑或室性心动过速);或房室结双径路(房室结折返性心过动速)。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "旁道" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "房室折返性心动过速" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "预激综合征" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心房" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "心房或心室的异常兴奋灶" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "心室" }, { "start_idx": 42, "end_idx": 50, "type": "dis", "entity": "房性异位性心动过速" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "室性心动过速" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "心房" }, { "start_idx": 60, "end_idx": 69, "type": "sym", "entity": "心房或心室的一个瘢痕" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "心室" }, { "start_idx": 73, "end_idx": 79, "type": "dis", "entity": "折返性心动过速" }, { "start_idx": 88, "end_idx": 89, "type": "dis", "entity": "房扑" }, { "start_idx": 91, "end_idx": 96, "type": "dis", "entity": "室性心动过速" }, { "start_idx": 100, "end_idx": 105, "type": "dis", "entity": "房室结双径路" }, { "start_idx": 107, "end_idx": 116, "type": "dis", "entity": "房室结折返性心过动速" } ] }, { "text": "然后通过导管头端释放的能量破坏心律失常基质的关键成分(图9-11、9-9)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "导管" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心律失常" } ] }, { "text": "目前,临床上最常使用的能量是射频电流。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "射频电流" } ] }, { "text": "射频电流是一种高频交流电,其频率范围在100~1000kHz之间。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "射频电流" } ] }, { "text": "有关如何根据心腔内电图判断与定位致病理性心动过速的基质不是本书涉及的内容,本节不予叙述。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "心腔内电图" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "病理性心动过速" } ] }, { "text": "图9-11图示左侧游离壁旁道(AP)激动自心室经旁道逆传回心房,再经房室结下传至心室,如此周而复始,形成旁道介导的房室折返性心动过速图9-12消融导管到达相应旁道位置,释放能量,旁道破坏,该旁道介导的房室折返性心动过速治愈图9-13本图箭头内区域为射频电流在组织内产生边界清楚的损伤区,下方为射频消融导管头端(二)常见室上性心动过速一些常见室上性心动过速(supraventriculartachycardia,SVT)的自然史:室上性心动过速的自然史与患儿的发病年龄及心律失常的机制有关。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "左侧游离壁旁道" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "AP" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心室" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "旁道" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "心房" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "房室结" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "心室" }, { "start_idx": 52, "end_idx": 65, "type": "dis", "entity": "旁道介导的房室折返性心动过速" }, { "start_idx": 71, "end_idx": 74, "type": "equ", "entity": "消融导管" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "旁道" }, { "start_idx": 89, "end_idx": 92, "type": "sym", "entity": "旁道破坏" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "旁道" }, { "start_idx": 95, "end_idx": 108, "type": "dis", "entity": "旁道介导的房室折返性心动过速" }, { "start_idx": 129, "end_idx": 140, "type": "dis", "entity": "组织内产生边界清楚的损伤" }, { "start_idx": 146, "end_idx": 151, "type": "equ", "entity": "射频消融导管" }, { "start_idx": 159, "end_idx": 165, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 168, "end_idx": 176, "type": "dis", "entity": "常见室上性心动过速" }, { "start_idx": 178, "end_idx": 204, "type": "dis", "entity": "supraventriculartachycardia" }, { "start_idx": 206, "end_idx": 208, "type": "dis", "entity": "SVT" }, { "start_idx": 215, "end_idx": 221, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 235, "end_idx": 238, "type": "dis", "entity": "心律失常" } ] }, { "text": "旁道参与的房室折返性心动过速房室折返性心动过速(A-Vreentrytachycardia),如在婴儿期发作,至12个月时约30%完全缓解,无需终身服药或介入治疗。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "dis", "entity": "旁道参与的房室折返性心动过速" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "房室折返性心动过速" }, { "start_idx": 24, "end_idx": 44, "type": "dis", "entity": "A-Vreentrytachycardia" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "终身服药" }, { "start_idx": 77, "end_idx": 80, "type": "pro", "entity": "介入治疗" } ] }, { "text": "房室结折返性心动过速一般在5岁以上发作,且随着年龄的增长,发病率增加,婴儿期很少发作。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "房室结折返性心动过速" } ] }, { "text": "这也是为什么儿童心动过速的射频消融治疗建议在5岁以上进行的原因之一。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "儿童心动过速" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "射频消融治疗" } ] }, { "text": "(三)儿童射频消融术的成功率与并发症儿童射频消融术的疗效与心律失常的类型有关。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "儿童射频消融术" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "儿童射频消融术" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "心律失常" } ] }, { "text": "据美国多中心注册登记资料显示旁路介导的房室折返性心动过速,成功率92.2%,房室结折返性心动过速的成功率为97.4%,心房异位性心动过速的成功率为86.7%,但如心动过速合并先天性心脏病,则总体成功率可能在80%左右,而房内折返性心内过速的疗效只有50%。", "entities": [ { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "旁路介导的房室折返性心动过速" }, { "start_idx": 38, "end_idx": 47, "type": "dis", "entity": "房室结折返性心动过速" }, { "start_idx": 59, "end_idx": 67, "type": "dis", "entity": "心房异位性心动过速" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "心动过速" }, { "start_idx": 87, "end_idx": 92, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 110, "end_idx": 118, "type": "dis", "entity": "房内折返性心内过速" } ] }, { "text": "心动过速射频消融后有一定的复发率,由于目前儿童射频消融术的长期随访资料不多,确切的复发率还不是很清楚,一般认为在10%~15%左右,复发率的高低也与心律失常的类型有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心动过速" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "射频消融" }, { "start_idx": 21, "end_idx": 27, "type": "pro", "entity": "儿童射频消融术" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "心律失常" } ] }, { "text": "儿童射频消融术的并发症大概在7.2%左右,而严重并发症则不到3%。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "儿童射频消融术" } ] }, { "text": "儿童患儿最常见的严重并发症为:Ⅱ度或Ⅲ度房室传导阻滞(atrialcapture)、穿孔、心包压塞、栓塞、臂丛神经损伤及气胸。", "entities": [ { "start_idx": 15, "end_idx": 25, "type": "dis", "entity": "Ⅱ度或Ⅲ度房室传导阻滞" }, { "start_idx": 27, "end_idx": 39, "type": "dis", "entity": "atrialcapture" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "穿孔" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "心包压塞" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "栓塞" }, { "start_idx": 53, "end_idx": 58, "type": "dis", "entity": "臂丛神经损伤" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "气胸" } ] }, { "text": "(四)儿童射频导管消融术的指征随着经验的积累和技术的改进,儿童RFCA的指征已扩大。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "儿童射频导管消融术" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "儿童RFCA" } ] }, { "text": "国外儿童RFCA术注册资料显示RFCA术正越来越多地成为儿童长期抗心律失常药物治疗的替代选择。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "儿童RFCA术" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "RFCA术" }, { "start_idx": 32, "end_idx": 40, "type": "pro", "entity": "抗心律失常药物治疗" } ] }, { "text": "北美起搏电生理学会(NASPE)在21届年会上组织有关专家就儿童射频消融术举行了专门会议,会议讨论的内容发表在2002年6月的PACE杂志上。", "entities": [ { "start_idx": 30, "end_idx": 36, "type": "pro", "entity": "儿童射频消融术" } ] }, { "text": "本文摘录其建议的儿童RFCA指征供参考。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "儿童RFCA" } ] }, { "text": "该指征基于心律失常的自然史、已发表的儿童RFCA术结果、先前发表或建议的指征和该次会议的资料及讨论。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心律失常" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "儿童RFCA术" } ] }, { "text": "该指征的分类方式为:第一类为一致同意和(或)有支持性的资料RFCA对患儿可能有益或有帮助;第二类为对RFCA的益处或必要性有分歧,其中a类为大多数的意见或资料倾向于RFCA,b类为对手术的需要有明显分歧;第三类为认同无RFCA指征或手术的危险大于益处。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "RFCA" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "RFCA" }, { "start_idx": 82, "end_idx": 85, "type": "pro", "entity": "RFCA" }, { "start_idx": 91, "end_idx": 92, "type": "pro", "entity": "手术" }, { "start_idx": 109, "end_idx": 112, "type": "pro", "entity": "RFCA" }, { "start_idx": 116, "end_idx": 117, "type": "pro", "entity": "手术" } ] }, { "text": "1.第一类(1)WPW综合征心源性猝死存活者。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "WPW综合征" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "心源性猝死" } ] }, { "text": "(2)WPW综合征伴晕厥,房颤时预激的RR间期小于250ms或程控电刺激时旁道前传有效不应期小于250ms。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "WPW综合征" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "晕厥" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "房颤" } ] }, { "text": "(3)慢性或反复发作性室上性心动过速伴心室功能异常。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "dis", "entity": "慢性或反复发作性室上性心动过速" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "心室功能异常" } ] }, { "text": "(4)反复发作性室性心动过速伴血流动力学损害并可接受导管消融者。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "反复发作性室性心动过速" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "血流动力学损害" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "导管消融" } ] }, { "text": "(2)即将行先天性心脏病手术治疗,术后血管或心腔的进入可能限制者。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "手术治疗" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血管" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心腔" } ] }, { "text": "(3)慢性(首次事件后发作大于6至12个月)或无休止性室上性心动过速心室功能正常。", "entities": [ { "start_idx": 3, "end_idx": 33, "type": "dis", "entity": "慢性(首次事件后发作大于6至12个月)或无休止性室上性心动过速" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心室" } ] }, { "text": "(4)房内折返性心动过速慢性或频繁发作。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "房内折返性心动过速" } ] }, { "text": "(5)心悸伴电生理检查时可诱发持续性室上性心动过速者。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "心悸" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "心" }, { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "电生理检查" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "持续性室上性心动过速" } ] }, { "text": "3.第二b类(1)无症状性预激(心电图显示WPW图形),年龄大于5岁,无心动过速依据,当手术与心律失常的危险及益处已清晰解释时。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "心电图" }, { "start_idx": 16, "end_idx": 25, "type": "sym", "entity": "心电图显示WPW图形" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "心动过速" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "手术" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "心律失常" } ] }, { "text": "(2)室上性心动过速,年龄大于5岁,RFCA作为长期抗心律失常药物治疗的替代选择,而后者可有效控制心律失常。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "RFCA" }, { "start_idx": 26, "end_idx": 34, "type": "pro", "entity": "抗心律失常药物治疗" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "心律失常" } ] }, { "text": "(3)室上性心动过速,年龄小于5岁(包括婴儿),抗心律失常药物(包括索他洛尔和胺碘酮)无效或伴有不能耐受的不良反应。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 24, "end_idx": 30, "type": "dru", "entity": "抗心律失常药物" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "索他洛尔" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "胺碘酮" } ] }, { "text": "(4)房内折返性心动过速,每年发作1至3次,需药物干预。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "房内折返性心动过速" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "药物干预" } ] } ]