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两性霉素B脂质体毒性较小,每日剂量可增到4mg/(kg•d)。
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如窦率显著缓慢又无结性逸搏出现,可出现眩晕或晕厥。
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有时窦缓可与室上速交替出现(快慢综合征),引起心悸、运动不耐受或眩晕。
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如有些快慢综合征的患儿用药物(普萘洛尔、奎尼丁、普鲁卡因胺)控制心动过速时可出现症状性心动过缓。
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此时可能需药物治疗的同时安置起搏器。
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在年长儿,房扑通常发生在有先天性心脏病的基础上。
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房扑可发生在急性感染期间,但最常见于心房扩大患儿,如二尖瓣或三尖瓣的长期关闭不全,三尖瓣闭锁,Ebstein畸形或风湿性二尖瓣狭窄。
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房扑也可发生在姑息性及纠治性房内手术后。
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提高迷走张力的方法(如颈动脉窦压迫、将脸浸于冰水)或给予腺苷通常可使心率暂时减慢。
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Ⅲ类药物如胺碘酮、索他洛尔可用于对Ⅰ类无效的患儿。
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如药物治疗无效,可予以射频消融或外科消融治疗。
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心脏正常的新生儿患儿,如对地高辛有效,应用药6~12个月,然后停药。
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本病是儿童时期常见的继发性肾小球疾病之一,以肾病综合征或蛋白尿、血尿为突出表现,病理上则以膜性肾病最为多见。
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以往本病的命名不统一,如乙型肝炎相关性肾炎、乙肝免疫复合物肾炎以及乙肝病毒抗原相关性肾炎等,自1989年《中华内科杂志》召开乙肝肾炎座谈会后开始统一命名为“乙型肝炎病毒相关肾炎”。
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膜性肾病是儿童HBV-GN最常见的病理类型。
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对表现为大量蛋白尿的HBV-GN,有人试用泼尼松短程疗法以减轻蛋白尿,但近年来研究认为HBV-GN对皮质激素多无任何效应,而且远期临床观察表明肾上腺皮质激素有弊无利,因此,应禁止单用肾上腺皮质激素治疗。
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据统计,全世界每年约有100万~200万新生儿死于新生儿肺炎。
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国内可能仍以大肠杆菌为主要致病菌。
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另外,克雷伯杆菌、李斯特菌也可引起新生儿宫内、出生时感染性肺炎。
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巨细胞病毒、单纯疱疹病毒、风疹病毒等,以及原虫(如弓形虫)、衣原体、支原体(如解脲支原体)等也可引起新生儿感染性肺炎。
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生后立即进行胃液涂片查找白细胞与抗原;或取血样、气管分泌物等进行涂片、培养、对流免疫电泳等检测,有助于病原学诊断。
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新生儿出生后一旦发现呼吸增快即开始抗生素治疗:大肠埃希菌等肠道杆菌肺炎可用氨苄西林和阿米卡星,耐药者可选用第三代头孢菌素;GBS肺炎可选用青霉素和氨苄西林治疗3天,以后用大剂量青霉素,疗程10~14天;李斯特菌肺炎可选用氨苄西林;衣原体肺炎首选红霉素;单纯疱疹病毒性肺炎可用阿昔洛韦静脉滴注。
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导致肾盂输尿管连接部内源性梗阻的其他原因尚包括:①瓣膜样的黏膜皱襞;②胎儿期输尿管扭曲折叠的持续存在;③输尿管起始段息肉。
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患儿可出现血尿,或阵发性腹痛,在UPJO中发现的比率有升高之势,从随访来看,术后少有复发。
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由迷走血管导致的PUJO的发生率在15%~52%之间,在成人比较多见,小儿很少。
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有报道在手术中采用高选择性动脉造影,将其供应动脉栓塞,使病变逐渐萎缩。
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断续进展型其次,症状在数小时至数天内断续进展,神志异常或呆滞或激惹,肌张力低下,动作减少,呼吸不规则。
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急剧恶化型最为少见,但临床症状最严重,患儿可在数分钟至数小时内迅速恶化,出现意识障碍、呼吸困难或暂停、抽搐、瞳孔光反射消失、四肢肌张力低下、前囟紧张,伴失血性贫血、血压下降、心动过缓。
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可连续腰椎穿刺以防止脑室的迅速扩大,但往往效果不理想。
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如腰椎穿刺治疗无效,可考虑暂时直接脑室外部引流,少数患儿由于脑脊液吸收旁路重建而得以恢复。
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1986年在美国,化脑的平均发病年龄为15个月。
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有密切接触史、居住拥挤、贫穷、小婴儿缺乏母乳喂养都是诱发因素。
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传播方式是经接触呼吸道分泌物和飞沫传播,脾功能不全如镰状细胞贫血、无脾的患者易患肺炎链球菌脑膜炎,有时也易患Hib脑膜炎。
[ { "id": 0, "entity": "呼吸道分泌物", "start_offset": 8, "end_offset": 14, "label": "bod" }, { "id": 1, "entity": "脾", "start_offset": 20, "end_offset": 21, "label": "bod" }, { "id": 2, "entity": "脾功能不全", "start_offset": 20, "end_offset": 25, "label": "sym" }, { "id": 3, "entity": "镰状细胞", "start_offset": 26, "end_offset": 30, "label": "bod" }, { "id": 4, "entity": "镰状细胞贫血", "start_offset": 26, "end_offset": 32, "label": "sym" }, { "id": 5, "entity": "脾", "start_offset": 34, "end_offset": 35, "label": "bod" }, { "id": 6, "entity": "无脾", "start_offset": 33, "end_offset": 35, "label": "sym" }, { "id": 7, "entity": "肺炎链球菌脑膜炎", "start_offset": 40, "end_offset": 48, "label": "dis" }, { "id": 8, "entity": "Hib脑膜炎", "start_offset": 54, "end_offset": 60, "label": "dis" } ]
化脑一般发病急,有高热、头痛、呕吐、食欲缺乏及精神委靡等症状。
[ { "id": 0, "entity": "化脑", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "高热", "start_offset": 9, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "头痛", "start_offset": 12, "end_offset": 14, "label": "sym" }, { "id": 3, "entity": "呕吐", "start_offset": 15, "end_offset": 17, "label": "sym" }, { "id": 4, "entity": "食欲缺乏", "start_offset": 18, "end_offset": 22, "label": "sym" }, { "id": 5, "entity": "精神委靡", "start_offset": 23, "end_offset": 27, "label": "sym" } ]
起病时神志一般清醒,病情进展可发生嗜睡、谵妄、惊厥和昏迷。
[ { "id": 0, "entity": "神志一般清醒", "start_offset": 3, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "嗜睡", "start_offset": 17, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "谵妄", "start_offset": 20, "end_offset": 22, "label": "sym" }, { "id": 3, "entity": "惊厥", "start_offset": 23, "end_offset": 25, "label": "sym" }, { "id": 4, "entity": "昏迷", "start_offset": 26, "end_offset": 28, "label": "sym" } ]
体检可见意识障碍、昏迷、颈强直、克氏征与布氏征阳性。
[ { "id": 0, "entity": "意识障碍", "start_offset": 4, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "昏迷", "start_offset": 9, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "颈", "start_offset": 12, "end_offset": 13, "label": "bod" }, { "id": 3, "entity": "颈强直", "start_offset": 12, "end_offset": 15, "label": "sym" }, { "id": 4, "entity": "克氏征与布氏征阳性", "start_offset": 16, "end_offset": 25, "label": "sym" } ]
如未及时治疗,颈强直加重、头后仰、背肌僵硬甚至角弓反张。
[ { "id": 0, "entity": "颈", "start_offset": 7, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "颈强直", "start_offset": 7, "end_offset": 10, "label": "sym" }, { "id": 2, "entity": "头", "start_offset": 13, "end_offset": 14, "label": "bod" }, { "id": 3, "entity": "头后仰", "start_offset": 13, "end_offset": 16, "label": "sym" }, { "id": 4, "entity": "背肌", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 5, "entity": "背肌僵硬", "start_offset": 17, "end_offset": 21, "label": "sym" }, { "id": 6, "entity": "角弓反张", "start_offset": 23, "end_offset": 27, "label": "sym" } ]
婴幼儿期化脑起病急缓不一。
[ { "id": 0, "entity": "婴幼儿期化脑", "start_offset": 0, "end_offset": 6, "label": "dis" } ]
常先以易激惹、烦躁不安、面色苍白、食欲减低开始,然后出现发热及呼吸系统或消化系统症状,如呕吐、腹泻、轻微咳嗽,继之嗜睡、头向后仰、感觉过敏、哭声尖锐、眼神发呆、双目凝视,有时用手打头、摇头。
[ { "id": 0, "entity": "易激惹", "start_offset": 3, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "烦躁不安", "start_offset": 7, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "面色苍白", "start_offset": 12, "end_offset": 16, "label": "sym" }, { "id": 3, "entity": "食欲减低", "start_offset": 17, "end_offset": 21, "label": "sym" }, { "id": 4, "entity": "发热", "start_offset": 28, "end_offset": 30, "label": "sym" }, { "id": 5, "entity": "呼吸系统", "start_offset": 31, "end_offset": 35, "label": "bod" }, { "id": 6, "entity": "消化系统", "start_offset": 36, "end_offset": 40, "label": "bod" }, { "id": 7, "entity": "呼吸系统或消化系统症状", "start_offset": 31, "end_offset": 42, "label": "sym" }, { "id": 8, "entity": "呕吐", "start_offset": 44, "end_offset": 46, "label": "sym" }, { "id": 9, "entity": "腹泻", "start_offset": 47, "end_offset": 49, "label": "sym" }, { "id": 10, "entity": "轻微咳嗽", "start_offset": 50, "end_offset": 54, "label": "sym" }, { "id": 11, "entity": "嗜睡", "start_offset": 57, "end_offset": 59, "label": "sym" }, { "id": 12, "entity": "头", "start_offset": 60, "end_offset": 61, "label": "bod" }, { "id": 13, "entity": "头向后仰", "start_offset": 60, "end_offset": 64, "label": "sym" }, { "id": 14, "entity": "感觉过敏", "start_offset": 65, "end_offset": 69, "label": "sym" }, { "id": 15, "entity": "哭声尖锐", "start_offset": 70, "end_offset": 74, "label": "sym" }, { "id": 16, "entity": "眼神发呆", "start_offset": 75, "end_offset": 79, "label": "sym" }, { "id": 17, "entity": "双目凝视", "start_offset": 80, "end_offset": 84, "label": "sym" }, { "id": 18, "entity": "摇头", "start_offset": 92, "end_offset": 94, "label": "sym" } ]
往往在发生惊厥后才引起家长注意和就诊。
[ { "id": 0, "entity": "惊厥", "start_offset": 5, "end_offset": 7, "label": "sym" } ]
较常见的情况是出生时婴儿正常,数日后出现肌张力低下、少动、哭声微弱、吸吮力差、拒食、呕吐、黄疸、发绀、呼吸不规则等非特异性症状。
[ { "id": 0, "entity": "肌张力低下", "start_offset": 20, "end_offset": 25, "label": "sym" }, { "id": 1, "entity": "少动", "start_offset": 26, "end_offset": 28, "label": "sym" }, { "id": 2, "entity": "哭声微弱", "start_offset": 29, "end_offset": 33, "label": "sym" }, { "id": 3, "entity": "吸吮力差", "start_offset": 34, "end_offset": 38, "label": "sym" }, { "id": 4, "entity": "拒食", "start_offset": 39, "end_offset": 41, "label": "sym" }, { "id": 5, "entity": "呕吐", "start_offset": 42, "end_offset": 44, "label": "sym" }, { "id": 6, "entity": "黄疸", "start_offset": 45, "end_offset": 47, "label": "sym" }, { "id": 7, "entity": "发绀", "start_offset": 48, "end_offset": 50, "label": "sym" }, { "id": 8, "entity": "呼吸不规则", "start_offset": 51, "end_offset": 56, "label": "sym" } ]
有些病儿直到尸检时才发现其为化脑。
[ { "id": 0, "entity": "化脑", "start_offset": 14, "end_offset": 16, "label": "dis" } ]
此时须争取及早作脑脊液检查,以免延误诊断。
[ { "id": 0, "entity": "脑脊液检查", "start_offset": 8, "end_offset": 13, "label": "pro" } ]
糖、氯化物均明显减低。
[ { "id": 0, "entity": "糖", "start_offset": 0, "end_offset": 1, "label": "bod" }, { "id": 1, "entity": "氯化物", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 2, "entity": "糖、氯化物均明显减低", "start_offset": 0, "end_offset": 10, "label": "sym" } ]
脑脊液留膜涂片可找到结核杆菌。
[ { "id": 0, "entity": "脑脊液留膜涂片", "start_offset": 0, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "结核杆菌", "start_offset": 10, "end_offset": 14, "label": "mic" } ]
化脑的疗程根据病原菌而异。
[ { "id": 0, "entity": "化脑", "start_offset": 0, "end_offset": 2, "label": "dis" } ]
肺炎链球菌脑膜炎和流感嗜血杆菌脑膜炎,疗程2~3周,或热退后继续用药10~14天。
[ { "id": 0, "entity": "肺炎链球菌脑膜炎", "start_offset": 0, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "流感嗜血杆菌脑膜炎", "start_offset": 9, "end_offset": 18, "label": "dis" } ]
体格检查见前囟膨隆、颅骨缝裂开、头围增大、叩诊有破壶音。
[ { "id": 0, "entity": "前囟膨隆", "start_offset": 5, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "颅骨缝裂开", "start_offset": 10, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "头围增大", "start_offset": 16, "end_offset": 20, "label": "sym" } ]
主要临床表现为呼吸不规则、瞳孔不等大或扩大、视神经乳头水肿、前囟隆起或紧张、高血压、昏迷、惊厥和四肢张力明显增高、呕吐、头痛等。
[ { "id": 0, "entity": "呼吸不规则", "start_offset": 7, "end_offset": 12, "label": "sym" }, { "id": 1, "entity": "瞳孔", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "瞳孔不等大或扩大", "start_offset": 13, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "视神经乳头", "start_offset": 22, "end_offset": 27, "label": "bod" }, { "id": 4, "entity": "视神经乳头水肿", "start_offset": 22, "end_offset": 29, "label": "sym" }, { "id": 5, "entity": "前囟隆起", "start_offset": 30, "end_offset": 34, "label": "sym" }, { "id": 6, "entity": "紧张", "start_offset": 35, "end_offset": 37, "label": "sym" }, { "id": 7, "entity": "高血压", "start_offset": 38, "end_offset": 41, "label": "sym" }, { "id": 8, "entity": "昏迷", "start_offset": 42, "end_offset": 44, "label": "sym" }, { "id": 9, "entity": "惊厥", "start_offset": 45, "end_offset": 47, "label": "sym" }, { "id": 10, "entity": "四肢", "start_offset": 48, "end_offset": 50, "label": "bod" }, { "id": 11, "entity": "四肢张力明显增高", "start_offset": 48, "end_offset": 56, "label": "sym" }, { "id": 12, "entity": "呕吐", "start_offset": 57, "end_offset": 59, "label": "sym" }, { "id": 13, "entity": "头痛", "start_offset": 60, "end_offset": 62, "label": "sym" } ]
当化脑患儿有以下情况出现应考虑有脑室膜炎存在的可能:病情危重、频繁惊厥、呼吸衰竭、按正规治疗疗效不佳;脑超声波或CT检查有明显脑室扩大等。
[ { "id": 0, "entity": "化脑", "start_offset": 1, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "脑室膜炎", "start_offset": 16, "end_offset": 20, "label": "dis" }, { "id": 2, "entity": "惊厥", "start_offset": 33, "end_offset": 35, "label": "sym" }, { "id": 3, "entity": "呼吸衰竭", "start_offset": 36, "end_offset": 40, "label": "sym" }, { "id": 4, "entity": "脑超声波", "start_offset": 51, "end_offset": 55, "label": "pro" }, { "id": 5, "entity": "CT检查", "start_offset": 56, "end_offset": 60, "label": "pro" }, { "id": 6, "entity": "脑室扩大", "start_offset": 63, "end_offset": 67, "label": "sym" } ]
对暂时性脑积水可进行内科治疗,包括限制饮水或给予利尿剂等。
[ { "id": 0, "entity": "暂时性脑积水", "start_offset": 1, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "内科", "start_offset": 10, "end_offset": 12, "label": "dep" } ]
暴发型流脑可伴发DIC、休克。
[ { "id": 0, "entity": "暴发型流脑", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "DIC", "start_offset": 8, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "休克", "start_offset": 12, "end_offset": 14, "label": "dis" } ]
肺炎链球菌所致化脑病死率可达15%~25%,且易于复发、再发,甚至出现智力低下、耳聋及肢体瘫痪等后遗症。
[ { "id": 0, "entity": "肺炎链球菌", "start_offset": 0, "end_offset": 5, "label": "mic" }, { "id": 1, "entity": "化脑", "start_offset": 7, "end_offset": 9, "label": "dis" }, { "id": 2, "entity": "智力低下", "start_offset": 35, "end_offset": 39, "label": "sym" }, { "id": 3, "entity": "耳聋", "start_offset": 40, "end_offset": 42, "label": "sym" }, { "id": 4, "entity": "肢体瘫痪", "start_offset": 43, "end_offset": 47, "label": "sym" } ]
化脓性脑膜炎可发生神经系统后遗症,年幼者更常见,多因延误诊断和治疗不当所致。
[ { "id": 0, "entity": "化脓性脑膜炎", "start_offset": 0, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "神经系统", "start_offset": 9, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "神经系统后遗症", "start_offset": 9, "end_offset": 16, "label": "sym" } ]
化脑急性期如有严重抽搐;长时间神志不清和其他明显脑损伤表现,均有可能发生后遗症。
[ { "id": 0, "entity": "化脑", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "严重抽搐", "start_offset": 7, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "长时间神志不清", "start_offset": 12, "end_offset": 19, "label": "sym" }, { "id": 3, "entity": "脑", "start_offset": 24, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "脑损伤", "start_offset": 24, "end_offset": 27, "label": "sym" } ]
根据其发生原因分为先天性(婴儿多为支气管软骨发育缺陷,年长儿多为支气管肌肉及弹力纤维发育缺陷)和后天性两类,后者多由慢性肺部感染引起,如麻疹、百日咳和重症肺炎等。
[ { "id": 0, "entity": "支气管软骨发育缺陷", "start_offset": 17, "end_offset": 26, "label": "dis" }, { "id": 1, "entity": "支气管肌肉及弹力纤维发育缺陷", "start_offset": 32, "end_offset": 46, "label": "dis" }, { "id": 2, "entity": "慢性肺部感染", "start_offset": 58, "end_offset": 64, "label": "dis" }, { "id": 3, "entity": "麻疹", "start_offset": 68, "end_offset": 70, "label": "dis" }, { "id": 4, "entity": "百日咳", "start_offset": 71, "end_offset": 74, "label": "dis" }, { "id": 5, "entity": "重症肺炎", "start_offset": 75, "end_offset": 79, "label": "dis" } ]
免疫缺陷的患儿,尤其是低免疫球蛋白血症者反复细菌性肺炎和支气管炎后可发生支气管扩张。
[ { "id": 0, "entity": "免疫缺陷", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "低免疫球蛋白血症", "start_offset": 11, "end_offset": 19, "label": "dis" }, { "id": 2, "entity": "细菌性肺炎", "start_offset": 22, "end_offset": 27, "label": "dis" }, { "id": 3, "entity": "支气管炎", "start_offset": 28, "end_offset": 32, "label": "dis" }, { "id": 4, "entity": "支气管扩张", "start_offset": 36, "end_offset": 41, "label": "dis" } ]
其确切机制尚不明确,一般认为感染和支气管阻塞是支气管扩张的两个根本致病因素,感染后剧烈咳嗽、管腔内分泌物的淤滞等可促使损伤软化的支气管壁扩张。
[ { "id": 0, "entity": "感染", "start_offset": 14, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "支气管阻塞", "start_offset": 17, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "支气管扩张", "start_offset": 23, "end_offset": 28, "label": "dis" }, { "id": 3, "entity": "感染", "start_offset": 38, "end_offset": 40, "label": "dis" }, { "id": 4, "entity": "剧烈咳嗽", "start_offset": 41, "end_offset": 45, "label": "sym" }, { "id": 5, "entity": "管腔内分泌物的淤滞", "start_offset": 46, "end_offset": 55, "label": "sym" }, { "id": 6, "entity": "支气管壁扩张", "start_offset": 64, "end_offset": 70, "label": "dis" } ]
根据支气管扩张的形态分为4型:①圆柱状:较局限,常见于轻症;②囊状:分布范围较广,多为重症;③核状:介于两者之间;④混合型:兼有以上两种形态,较常见。
[ { "id": 0, "entity": "支气管扩张", "start_offset": 2, "end_offset": 7, "label": "dis" } ]
主要症状为咳嗽,多由变换体位时引起,急性感染时伴大量黏液脓痰。
[ { "id": 0, "entity": "咳嗽", "start_offset": 5, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "急性感染", "start_offset": 18, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "大量黏液脓痰", "start_offset": 24, "end_offset": 30, "label": "sym" } ]
发热少见。
[ { "id": 0, "entity": "发热少见", "start_offset": 0, "end_offset": 4, "label": "sym" } ]
杵状指(趾)的出现提示病程常在1年以上。
[ { "id": 0, "entity": "杵状指(趾)", "start_offset": 0, "end_offset": 6, "label": "sym" } ]
轻症患儿胸片仅见肺纹理增粗,病变明显时可见中下肺大小不等的环状透光阴影,呈卷发状或蜂窝状,常伴肺段、肺叶不张影及周围炎性浸润影。
[ { "id": 0, "entity": "胸片", "start_offset": 4, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "肺纹理增粗", "start_offset": 8, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "中下肺", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 3, "entity": "中下肺大小不等的环状透光阴影,呈卷发状或蜂窝状", "start_offset": 21, "end_offset": 44, "label": "sym" }, { "id": 4, "entity": "肺段", "start_offset": 47, "end_offset": 49, "label": "bod" }, { "id": 5, "entity": "肺叶", "start_offset": 50, "end_offset": 52, "label": "bod" }, { "id": 6, "entity": "肺段、肺叶不张影及周围炎性浸润影", "start_offset": 47, "end_offset": 63, "label": "sym" } ]
根据上述典型症状和X线表现,结合支气管造影不难诊断。
[ { "id": 0, "entity": "X线", "start_offset": 9, "end_offset": 11, "label": "pro" }, { "id": 1, "entity": "支气管造影", "start_offset": 16, "end_offset": 21, "label": "pro" } ]
近年来支气管造影在很大程度上已被CT、尤其是高分辨CT替代。
[ { "id": 0, "entity": "支气管造影", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "CT", "start_offset": 16, "end_offset": 18, "label": "pro" }, { "id": 2, "entity": "高分辨CT", "start_offset": 22, "end_offset": 27, "label": "pro" } ]
柱状支气管扩张症,CT表现为“轨道征”或“印戒征”。
[ { "id": 0, "entity": "柱状支气管扩张症", "start_offset": 0, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "CT", "start_offset": 9, "end_offset": 11, "label": "pro" }, { "id": 2, "entity": "轨道征", "start_offset": 15, "end_offset": 18, "label": "sym" }, { "id": 3, "entity": "印戒征", "start_offset": 21, "end_offset": 24, "label": "sym" } ]
囊状支气管扩张症,呈簇集蜂窝状分布,诊断准确率较高。
[ { "id": 0, "entity": "囊状支气管扩张症", "start_offset": 0, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "簇集蜂窝状分布", "start_offset": 10, "end_offset": 17, "label": "sym" } ]
支气管扩张患儿的治疗应重视体位引流,以排除气道分泌物;雾化吸入疗法对稀化黏痰有良好效果。
[ { "id": 0, "entity": "支气管扩张", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "体位引流", "start_offset": 13, "end_offset": 17, "label": "pro" }, { "id": 2, "entity": "气道分泌物", "start_offset": 21, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "雾化吸入", "start_offset": 27, "end_offset": 31, "label": "pro" }, { "id": 4, "entity": "黏痰", "start_offset": 36, "end_offset": 38, "label": "bod" } ]
急性恶化期全身应用抗生素2~3周,囊性纤维化患儿适当延长疗程,但盲目延长可能会增加耐药性和耐药菌株的产生。
[ { "id": 0, "entity": "抗生素", "start_offset": 9, "end_offset": 12, "label": "dru" }, { "id": 1, "entity": "囊性纤维化", "start_offset": 17, "end_offset": 22, "label": "dis" } ]
最好根据痰培养和药物敏感试验结果选择相应抗生素。
[ { "id": 0, "entity": "痰培养", "start_offset": 4, "end_offset": 7, "label": "ite" }, { "id": 1, "entity": "药物敏感试验", "start_offset": 8, "end_offset": 14, "label": "ite" }, { "id": 2, "entity": "抗生素", "start_offset": 20, "end_offset": 23, "label": "dru" } ]
若一侧或一叶肺组织大部分或全部被多发的囊肿占据,称为多囊肺。
[ { "id": 0, "entity": "肺组织", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "多囊肺", "start_offset": 26, "end_offset": 29, "label": "dis" } ]
囊肿大者可压迫气管或主支气管引起阵发性咳嗽、气促、喘息甚至发绀等症状,伴反复肺部感染。
[ { "id": 0, "entity": "气管", "start_offset": 7, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "主支气管", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "阵发性咳嗽", "start_offset": 16, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "气促", "start_offset": 22, "end_offset": 24, "label": "sym" }, { "id": 4, "entity": "喘息", "start_offset": 25, "end_offset": 27, "label": "sym" }, { "id": 5, "entity": "发绀", "start_offset": 29, "end_offset": 31, "label": "sym" }, { "id": 6, "entity": "反复肺部感染", "start_offset": 36, "end_offset": 42, "label": "sym" } ]
肺部叩诊鼓音或实音,听诊呼吸音减低或消失。
[ { "id": 0, "entity": "肺部", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "鼓音或实音", "start_offset": 4, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "呼吸音减低或消失", "start_offset": 12, "end_offset": 20, "label": "sym" } ]
胸部X线检查是诊断本病的主要依据。
[ { "id": 0, "entity": "胸部X线检查", "start_offset": 0, "end_offset": 6, "label": "pro" } ]
本病治疗以手术切除为主。
[ { "id": 0, "entity": "手术切除", "start_offset": 5, "end_offset": 9, "label": "pro" } ]
较大囊肿应尽早切除,早期手术可预防气胸、肺动脉高压、呼吸道感染等并发症。
[ { "id": 0, "entity": "较大囊肿", "start_offset": 0, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "尽早切除", "start_offset": 5, "end_offset": 9, "label": "pro" }, { "id": 2, "entity": "气胸", "start_offset": 17, "end_offset": 19, "label": "dis" }, { "id": 3, "entity": "肺动脉高压", "start_offset": 20, "end_offset": 25, "label": "dis" }, { "id": 4, "entity": "呼吸道感染", "start_offset": 26, "end_offset": 31, "label": "dis" } ]
如有胎儿肺囊肿伴纵隔移位,有必要进行胎儿胸腔穿刺,防止心血管功能不全。
[ { "id": 0, "entity": "胎儿肺囊肿", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "胎儿胸腔穿刺", "start_offset": 18, "end_offset": 24, "label": "pro" } ]
因此,如轻度溶血时,外周血中的红细胞数能被骨髓造血功能的增加所完全代偿,此时,临床上虽有溶血存在但并不发生贫血;如果红细胞的破坏超过了骨髓造血的代偿能力,则发生溶血性贫血。
[ { "id": 0, "entity": "轻度溶血", "start_offset": 4, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "外周血", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "红细胞数", "start_offset": 15, "end_offset": 19, "label": "ite" }, { "id": 3, "entity": "骨髓", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 4, "entity": "溶血", "start_offset": 44, "end_offset": 46, "label": "dis" }, { "id": 5, "entity": "贫血", "start_offset": 53, "end_offset": 55, "label": "dis" }, { "id": 6, "entity": "红细胞", "start_offset": 58, "end_offset": 61, "label": "bod" }, { "id": 7, "entity": "骨髓", "start_offset": 67, "end_offset": 69, "label": "bod" }, { "id": 8, "entity": "溶血性贫血", "start_offset": 80, "end_offset": 85, "label": "dis" } ]
(3)遗传性口形细胞增多症。
[ { "id": 0, "entity": "遗传性口形细胞增多症", "start_offset": 3, "end_offset": 13, "label": "dis" } ]
(4)遗传性畸形细胞增多症。
[ { "id": 0, "entity": "遗传性畸形细胞增多症", "start_offset": 3, "end_offset": 13, "label": "dis" } ]
2)磷酸葡萄糖异构酶缺乏。
[ { "id": 0, "entity": "磷酸葡萄糖异构酶缺乏", "start_offset": 2, "end_offset": 12, "label": "dis" } ]
3)腺苷三磷酸酶缺乏。
[ { "id": 0, "entity": "腺苷三磷酸酶缺乏", "start_offset": 2, "end_offset": 10, "label": "dis" } ]
4)谷胱甘肽还原酶缺乏。
[ { "id": 0, "entity": "谷胱甘肽还原酶缺乏", "start_offset": 2, "end_offset": 11, "label": "dis" } ]
(2)镰状细胞贫血。
[ { "id": 0, "entity": "镰状细胞贫血", "start_offset": 3, "end_offset": 9, "label": "dis" } ]
(3)血红蛋白H病。
[ { "id": 0, "entity": "血红蛋白H病", "start_offset": 3, "end_offset": 9, "label": "dis" } ]
(2)新生儿溶血症(Rh、ABO不相合)。
[ { "id": 0, "entity": "新生儿溶血症", "start_offset": 3, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "Rh", "start_offset": 10, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "ABO不相合", "start_offset": 13, "end_offset": 19, "label": "dis" } ]
3)与免疫现象有关的贫血(移植物排斥,免疫复合物等)。
[ { "id": 0, "entity": "贫血", "start_offset": 10, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "移植物排斥", "start_offset": 13, "end_offset": 18, "label": "sym" } ]
(3)热损伤(如烧伤、烫伤等)。
[ { "id": 0, "entity": "热损伤", "start_offset": 3, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "烧伤", "start_offset": 8, "end_offset": 10, "label": "dis" }, { "id": 2, "entity": "烫伤", "start_offset": 11, "end_offset": 13, "label": "dis" } ]
(4)弥漫性血管内凝血(DIC)。
[ { "id": 0, "entity": "血管", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "凝血", "start_offset": 9, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "DIC", "start_offset": 12, "end_offset": 15, "label": "dis" } ]
(2)无β脂蛋白血症。
[ { "id": 0, "entity": "无β脂蛋白血症", "start_offset": 3, "end_offset": 10, "label": "dis" } ]
可有黄疸、气喘、心率加快、食欲缺乏、头昏、怕冷等症。
[ { "id": 0, "entity": "黄疸、气喘、心率加快、食欲缺乏、头昏、怕冷", "start_offset": 2, "end_offset": 23, "label": "sym" } ]
急性溶血时,患儿可突然发病,感胸闷、腰酸、发热、背痛、头痛,甚至发生周围循环衰竭,出现少尿或无尿,乃至急性肾衰竭。
[ { "id": 0, "entity": "急性溶血", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "感胸闷、腰酸、发热、背痛、头痛,甚至发生周围循环衰竭,出现少尿或无尿,乃至急性肾衰竭", "start_offset": 14, "end_offset": 56, "label": "sym" } ]
慢性溶血时,患儿可出现黄疸及肝、脾大。
[ { "id": 0, "entity": "慢性溶血", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "黄疸", "start_offset": 11, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "肝、脾大", "start_offset": 14, "end_offset": 18, "label": "sym" } ]
3.血清间接胆红素增高。
[ { "id": 0, "entity": "血清", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "胆红素", "start_offset": 6, "end_offset": 9, "label": "bod" } ]
4.粪胆原定量增加。
[ { "id": 0, "entity": "粪胆原", "start_offset": 2, "end_offset": 5, "label": "bod" } ]
6.含铁血黄素尿试验阳性。
[ { "id": 0, "entity": "尿试验", "start_offset": 7, "end_offset": 10, "label": "pro" } ]
2.血清结合珠蛋白减少。
[ { "id": 0, "entity": "血清", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "结合珠蛋白", "start_offset": 4, "end_offset": 9, "label": "bod" } ]
有溶血诱因者应尽早尽快去除诱因,如冷抗体型自身免疫性溶血性贫血,应立即保暖,特别是使四肢温暖;由蚕豆病引起的溶血性贫血应避免食用蚕豆和氧化性药物;某药引起的溶血性贫血应立即停止用该药,输血引起溶血者应立即停止输血;感染引起的溶血性贫血应积极控制感染等。
[ { "id": 0, "entity": "溶血", "start_offset": 1, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "冷抗体型自身免疫性溶血性贫血", "start_offset": 17, "end_offset": 31, "label": "dis" }, { "id": 2, "entity": "四肢", "start_offset": 42, "end_offset": 44, "label": "bod" }, { "id": 3, "entity": "蚕豆病", "start_offset": 48, "end_offset": 51, "label": "dis" }, { "id": 4, "entity": "溶血性贫血", "start_offset": 54, "end_offset": 59, "label": "dis" }, { "id": 5, "entity": "氧化性药物", "start_offset": 67, "end_offset": 72, "label": "dru" }, { "id": 6, "entity": "溶血性贫血", "start_offset": 78, "end_offset": 83, "label": "dis" }, { "id": 7, "entity": "输血", "start_offset": 92, "end_offset": 94, "label": "pro" }, { "id": 8, "entity": "输血", "start_offset": 104, "end_offset": 106, "label": "pro" }, { "id": 9, "entity": "感染", "start_offset": 107, "end_offset": 109, "label": "sym" }, { "id": 10, "entity": "溶血性贫血", "start_offset": 112, "end_offset": 117, "label": "dis" }, { "id": 11, "entity": "感染", "start_offset": 122, "end_offset": 124, "label": "sym" } ]
肾上腺皮质激素能抑制免疫反应,对免疫性溶血性贫血有效。
[ { "id": 0, "entity": "肾上腺皮质激素", "start_offset": 0, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "免疫性溶血性贫血", "start_offset": 16, "end_offset": 24, "label": "dis" } ]
雄激素或蛋白合成激素能刺激造血,增加代偿功能。
[ { "id": 0, "entity": "激素", "start_offset": 8, "end_offset": 10, "label": "bod" } ]