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②神经系统症状:轻者无明显神经系统症状,重者与发生的部位及受损的程度有关,可表现智力低下、抽搐和不同程度的上运动神经元瘫痪等。
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部分患儿成年后有慢性腰痛。
[ { "id": 0, "entity": "慢性腰痛", "start_offset": 8, "end_offset": 12, "label": "sym" } ]
脊髓脊膜膨出可有不同程度的双下肢瘫痪及大小便失禁等。
[ { "id": 0, "entity": "脊髓脊膜膨出", "start_offset": 0, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "双下肢瘫痪", "start_offset": 13, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "大小便失禁", "start_offset": 19, "end_offset": 24, "label": "sym" } ]
关于脑膜膨出或脑脑膜膨出的诊断,根据囊性包块的部位、大小和外观,透光试验阳性,加上相应的病史及临床表现,一般作出正确诊断并不难。
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脊膜膨出与脊髓脊膜膨出的诊断是依据患儿出生后即发现背部中线有膨胀性的包块,并随着年龄增长而扩大,以及相应的神经损害症状。
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脊膜膨出和脊髓脊膜膨出的治疗原则是早期手术治疗。
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切除脊膜膨出囊和修补软组织缺损,单纯性脊膜膨出经此手术可以治愈。
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增补叶酸方法是妇女从怀孕前1个月至怀孕后3个月每日服用一粒叶酸增补剂,可以减少70%以上NTDs的发生。
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当前市场上叶酸制剂有两类:一类是单纯的叶酸制剂,为孕妇生产的每片含400μg,如斯利安片。
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如玛特纳片,其中包括叶酸在内,共含有13种维生素和另外13种矿物质及微量元素。
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胎儿有NTDs可使羊水AFP水平明显升高,同时母亲血清AFP水平也升高。
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而脑脑膜膨出一般均合并有神经功能障碍、智力低下和其他部位畸形,手术不能解决此类问题,预后差。
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而脊髓脊膜膨出者,手术治疗疗效差,一般预后不良,即使病人能够存活下来,也是终身残疾。
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阴道出血应与“假月经”鉴别。
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对消化道出血者,要暂时禁食,从肠道外补充营养。
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位于幕上者,主要为间变性星形细胞瘤及多形性胶质母细胞瘤,少见的有少突胶质细胞瘤、神经节胶质瘤及星形细胞、少突胶质细胞混合型瘤。
[ { "id": 0, "entity": "幕上", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "间变性星形细胞瘤", "start_offset": 9, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "多形性胶质母细胞瘤", "start_offset": 18, "end_offset": 27, "label": "dis" }, { "id": 3, "entity": "少突胶质细胞瘤", "start_offset": 32, "end_offset": 39, "label": "dis" }, { "id": 4, "entity": "神经节胶质瘤", "start_offset": 40, "end_offset": 46, "label": "dis" }, { "id": 5, "entity": "星形细胞、少突胶质细胞混合型瘤", "start_offset": 47, "end_offset": 62, "label": "dis" } ]
肿瘤呈浸润性生长,约25%~50%的肿瘤可向肺、淋巴结、肝及骨等远处转移。
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临床表现及辅助检查相似于低度胶质瘤。
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因肿瘤恶性程度高,且呈浸润性生长,很难获得全切除,原则上应在不造成严重神经功能障碍前提下尽可能多的切除肿瘤。
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术后辅以放疗。
[ { "id": 0, "entity": "术", "start_offset": 0, "end_offset": 1, "label": "pro" }, { "id": 1, "entity": "放疗", "start_offset": 4, "end_offset": 6, "label": "pro" } ]
目前多主张行局部放疗,全脑放疗对生存率并无明显影响。
[ { "id": 0, "entity": "局部放疗", "start_offset": 6, "end_offset": 10, "label": "pro" }, { "id": 1, "entity": "全脑放疗", "start_offset": 11, "end_offset": 15, "label": "pro" } ]
根据出血部位的不同,ICH可分为脑出血、蛛网膜下腔出血和硬膜下出血等。
[ { "id": 0, "entity": "ICH", "start_offset": 10, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "脑出血", "start_offset": 16, "end_offset": 19, "label": "dis" }, { "id": 2, "entity": "蛛网膜", "start_offset": 20, "end_offset": 23, "label": "bod" }, { "id": 3, "entity": "蛛网膜下腔出血", "start_offset": 20, "end_offset": 27, "label": "sym" }, { "id": 4, "entity": "硬膜", "start_offset": 28, "end_offset": 30, "label": "bod" }, { "id": 5, "entity": "硬膜下出血", "start_offset": 28, "end_offset": 33, "label": "sym" } ]
先天性脑血管畸形包括血管瘤和动静脉瘘,前者系因血管壁中层发育缺陷所致,见于末梢小动脉分叉处,直径达6~15mm的动脉瘤易发生破裂出血;后者系因动、静脉系统间毛细血管发育缺陷使动、静脉间直接吻合而成短路,以致病区动脉扩大而成动脉瘤样畸形,并压迫其周围脑组织,易破裂出血,以Galen静脉畸形多见。
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感染性脑动静脉畸形如颅内细菌性或真菌性动脉瘤,系感染性心内膜炎的感染栓子所致;人类免疫缺陷病毒感染也可导致小儿颅内动脉瘤的发生。
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外伤性脑动静脉畸形较少见,仅发生于海绵窦,因颈内动脉位于此处,故外伤可致颈动脉-海绵窦瘘。
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其他类型的脑血管畸形有毛细血管扩张、海绵状血管瘤、软脑膜静脉及毛细血管的畸形、脑底异常血管网(Moyamoya病)等。
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小儿特发性血小板减少性紫癜病例中发生ICH者占10%。
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其他如白血病、再生障碍性贫血、溶血性贫血、弥散性血管内凝血、凝血障碍等血液病,以及抗凝疗法的并发症,均可发生ICH。
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找不到病因的脑出血称为小儿特发性脑出血。
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继发性缺乏可由吸收障碍或使用药物等引起,消耗过多和代谢活动增加也可引起缺乏。
[ { "id": 0, "entity": "吸收障碍", "start_offset": 7, "end_offset": 11, "label": "dis" } ]
溃疡好发于十二指肠和胃,但也可发生于食管、小肠及胃肠吻合口处,极少数发生于异位的胃黏膜,如Meckel憩室。
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呕血一般见于胃溃疡,吐出物呈咖啡样,而黑便较多见于十二指肠溃疡。
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当出血量较多时,任何一种溃疡可同时表现呕血与黑便,在小儿胃内引流物呈血性多提示胃出血;但引流物阴性者,不能排除十二指肠溃疡合并出血的可能(因为血液可不经幽门反流入胃)。
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幽门螺杆菌感染与NSAIDs/ASA诱发的胃炎是消化性溃疡的两大潜在因素,所以对幽门螺杆菌阳性的溃疡患者亦予以幽门螺杆菌根除疗法;如果可能,停用ASA/NSAIDs。
[ { "id": 0, "entity": "幽门螺杆菌感染", "start_offset": 0, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "NSAIDs", "start_offset": 8, "end_offset": 14, "label": "dis" }, { "id": 2, "entity": "ASA", "start_offset": 15, "end_offset": 18, "label": "dis" }, { "id": 3, "entity": "胃炎", "start_offset": 21, "end_offset": 23, "label": "dis" }, { "id": 4, "entity": "消化性溃疡", "start_offset": 24, "end_offset": 29, "label": "dis" }, { "id": 5, "entity": "幽门螺杆菌阳性", "start_offset": 40, "end_offset": 47, "label": "sym" }, { "id": 6, "entity": "幽门螺杆菌根除疗法", "start_offset": 55, "end_offset": 64, "label": "pro" }, { "id": 7, "entity": "ASA", "start_offset": 72, "end_offset": 75, "label": "dis" }, { "id": 8, "entity": "NSAIDs", "start_offset": 76, "end_offset": 82, "label": "dis" } ]
(2)特异性胆碱能神经阻滞剂:哌仑西平(pirenzepine)50~100mg每日2次,治疗4~6周,PU愈合率70%~94%(成人)。
[ { "id": 0, "entity": "特异性胆碱能神经阻滞剂", "start_offset": 3, "end_offset": 14, "label": "dru" }, { "id": 1, "entity": "哌仑西平", "start_offset": 15, "end_offset": 19, "label": "dru" }, { "id": 2, "entity": "pirenzepine", "start_offset": 20, "end_offset": 31, "label": "dru" }, { "id": 3, "entity": "PU", "start_offset": 52, "end_offset": 54, "label": "dis" } ]
(1)西咪替丁(cimetidine):儿童20~40mg/(kg•d),3~4次/日,亦有主张2次/日。
[ { "id": 0, "entity": "西咪替丁", "start_offset": 3, "end_offset": 7, "label": "dru" }, { "id": 1, "entity": "cimetidine", "start_offset": 8, "end_offset": 18, "label": "dru" } ]
(4)其他:尼扎替丁、罗沙替丁。
[ { "id": 0, "entity": "尼扎替丁", "start_offset": 6, "end_offset": 10, "label": "dru" }, { "id": 1, "entity": "罗沙替丁", "start_offset": 11, "end_offset": 15, "label": "dru" } ]
(3)胶体铋制剂:为溃疡隔离剂,保护黏膜,促进前列腺素合成,与表皮生长因子形成复合物,聚集于溃疡部位,促进上皮的再生和溃疡愈合,此外有杀灭幽门螺杆菌及抑制胃蛋白酶活性的作用。
[ { "id": 0, "entity": "胶体铋制剂", "start_offset": 3, "end_offset": 8, "label": "dru" }, { "id": 1, "entity": "溃疡隔离剂", "start_offset": 10, "end_offset": 15, "label": "dru" }, { "id": 2, "entity": "黏膜", "start_offset": 18, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "前列腺素", "start_offset": 23, "end_offset": 27, "label": "bod" }, { "id": 4, "entity": "溃疡", "start_offset": 46, "end_offset": 48, "label": "dis" }, { "id": 5, "entity": "上皮", "start_offset": 53, "end_offset": 55, "label": "bod" }, { "id": 6, "entity": "溃疡", "start_offset": 59, "end_offset": 61, "label": "dis" }, { "id": 7, "entity": "幽门螺杆菌", "start_offset": 69, "end_offset": 74, "label": "mic" }, { "id": 8, "entity": "胃蛋白酶", "start_offset": 77, "end_offset": 81, "label": "bod" } ]
(4)前列腺素E(PGE):人工合成的类似物有米索前列醇(misoprostol)等。
[ { "id": 0, "entity": "前列腺素E", "start_offset": 3, "end_offset": 8, "label": "dru" }, { "id": 1, "entity": "PGE", "start_offset": 9, "end_offset": 12, "label": "dru" }, { "id": 2, "entity": "米索前列醇", "start_offset": 23, "end_offset": 28, "label": "dru" }, { "id": 3, "entity": "misoprostol", "start_offset": 29, "end_offset": 40, "label": "dru" } ]
肿瘤多位于鞍内及鞍上,球形或不规则形,边界清楚,实质性及部分囊性,囊液黄褐色,并有胆固醇结晶,囊壁及实质性部分可有钙化。
[ { "id": 0, "entity": "肿瘤", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "鞍内", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "鞍上", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 3, "entity": "胆固醇结晶", "start_offset": 41, "end_offset": 46, "label": "bod" }, { "id": 4, "entity": "囊壁", "start_offset": 47, "end_offset": 49, "label": "bod" } ]
颅咽管瘤组织学上属良性肿瘤,但由于在鞍区以及对周围重要结构的浸润,临床上呈现为恶性肿瘤的表现。
[ { "id": 0, "entity": "颅咽管瘤", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "鞍区", "start_offset": 18, "end_offset": 20, "label": "bod" } ]
化学治疗目前尚无肯定疗效的药物。
[ { "id": 0, "entity": "化学治疗", "start_offset": 0, "end_offset": 4, "label": "pro" } ]
囊性肿瘤的预后比实质肿瘤或囊性、实质性混合肿瘤好,肿瘤完整切除比部分切除预后好,部分切除的肿瘤若辅以放疗,仍能获得较长的生存期。
[ { "id": 0, "entity": "囊性肿瘤", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "实质肿瘤", "start_offset": 8, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "囊性、实质性混合肿瘤", "start_offset": 13, "end_offset": 23, "label": "dis" }, { "id": 3, "entity": "肿瘤", "start_offset": 25, "end_offset": 27, "label": "dis" }, { "id": 4, "entity": "肿瘤", "start_offset": 45, "end_offset": 47, "label": "dis" }, { "id": 5, "entity": "放疗", "start_offset": 50, "end_offset": 52, "label": "pro" } ]
有人统计完整切除肿瘤10年生存率达24%~100%;部分切除肿瘤仅达31%~52%;若辅以放疗则可达到62%~84%。
[ { "id": 0, "entity": "肿瘤", "start_offset": 8, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "肿瘤", "start_offset": 30, "end_offset": 32, "label": "dis" }, { "id": 2, "entity": "放疗", "start_offset": 45, "end_offset": 47, "label": "pro" } ]
该肿瘤好发于颈部,特别是颈后三角,其次可见于腋窝。
[ { "id": 0, "entity": "肿瘤", "start_offset": 1, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "颈部", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 2, "entity": "颈后三角", "start_offset": 12, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "腋窝", "start_offset": 22, "end_offset": 24, "label": "bod" } ]
还有人报道用OK-432,系一种经青霉素G钾盐处理而取得的人源性A簇链球菌Ⅲ型低毒菌株的冻干培养混合物。
[ { "id": 0, "entity": "OK-432", "start_offset": 6, "end_offset": 12, "label": "dru" }, { "id": 1, "entity": "青霉素G钾盐", "start_offset": 17, "end_offset": 23, "label": "dru" }, { "id": 2, "entity": "人源性A簇链球菌Ⅲ型低毒菌株", "start_offset": 29, "end_offset": 43, "label": "mic" } ]
(2)上消化道出血:原因不明的黑便,呕血。
[ { "id": 0, "entity": "上消化道出血", "start_offset": 3, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "原因不明的黑便,呕血", "start_offset": 10, "end_offset": 20, "label": "sym" } ]
(4)消化性溃疡病:食管溃疡、胃溃疡及十二指肠溃疡。
[ { "id": 0, "entity": "消化性溃疡病", "start_offset": 3, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "食管溃疡", "start_offset": 10, "end_offset": 14, "label": "dis" }, { "id": 2, "entity": "胃溃疡", "start_offset": 15, "end_offset": 18, "label": "dis" }, { "id": 3, "entity": "十二指肠溃疡", "start_offset": 19, "end_offset": 25, "label": "dis" } ]
(6)原因不明贫血:尤为低血色素小细胞性贫血。
[ { "id": 0, "entity": "贫血", "start_offset": 7, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "低血色素小细胞性贫血", "start_offset": 12, "end_offset": 22, "label": "sym" } ]
食管炎在胃镜下表现充血、水肿、糜烂及溃疡,根据其主要表现,在镜下可分为三类:卡他型、糜烂型与溃疡型。
[ { "id": 0, "entity": "食管炎", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "充血", "start_offset": 9, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "水肿", "start_offset": 12, "end_offset": 14, "label": "sym" }, { "id": 3, "entity": "糜烂", "start_offset": 15, "end_offset": 17, "label": "sym" }, { "id": 4, "entity": "溃疡", "start_offset": 18, "end_offset": 20, "label": "sym" }, { "id": 5, "entity": "卡他型", "start_offset": 38, "end_offset": 41, "label": "dis" }, { "id": 6, "entity": "糜烂型", "start_offset": 42, "end_offset": 45, "label": "dis" }, { "id": 7, "entity": "溃疡型", "start_offset": 46, "end_offset": 49, "label": "dis" } ]
3)黏液增多:黏膜与黏液附着牢固,若用水冲去,可见黏膜表面发红或糜烂剥脱。
[ { "id": 0, "entity": "黏液增多:黏膜与黏液附着牢固,若用水冲去,可见黏膜表面发红或糜烂剥脱", "start_offset": 2, "end_offset": 36, "label": "sym" } ]
内镜部分同样要求明确炎症的部分(全胃炎、胃窦炎及胃体胃炎);对内镜所见进行分级,并根据其异常表现将胃炎分成7种基本类型:即充血渗出型、平坦糜烂型、隆起糜烂型、萎缩型、出血型、反流型以及皱襞增生型。
[ { "id": 0, "entity": "全胃炎", "start_offset": 16, "end_offset": 19, "label": "dis" }, { "id": 1, "entity": "胃窦炎", "start_offset": 20, "end_offset": 23, "label": "dis" }, { "id": 2, "entity": "胃体胃炎", "start_offset": 24, "end_offset": 28, "label": "dis" }, { "id": 3, "entity": "胃炎", "start_offset": 49, "end_offset": 51, "label": "dis" }, { "id": 4, "entity": "充血渗出型", "start_offset": 61, "end_offset": 66, "label": "dis" }, { "id": 5, "entity": "平坦糜烂型", "start_offset": 67, "end_offset": 72, "label": "dis" }, { "id": 6, "entity": "隆起糜烂型", "start_offset": 73, "end_offset": 78, "label": "dis" }, { "id": 7, "entity": "萎缩型", "start_offset": 79, "end_offset": 82, "label": "dis" }, { "id": 8, "entity": "出血型", "start_offset": 83, "end_offset": 86, "label": "dis" }, { "id": 9, "entity": "反流型", "start_offset": 87, "end_offset": 90, "label": "dis" }, { "id": 10, "entity": "皱襞增生型", "start_offset": 92, "end_offset": 97, "label": "dis" } ]
HSPN好发于学龄儿童,男多于女,是儿童最常见的继发性肾小球疾病。
[ { "id": 0, "entity": "HSPN", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "继发性肾小球疾病", "start_offset": 24, "end_offset": 32, "label": "dis" } ]
其基本病变是肾小球系膜区IgA沉积、系膜细胞增生伴或不伴新月体形成。
[ { "id": 0, "entity": "肾小球系膜区", "start_offset": 6, "end_offset": 12, "label": "bod" }, { "id": 1, "entity": "肾小球系膜区IgA沉积", "start_offset": 6, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "系膜细胞", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "系膜细胞增生伴或不伴新月体形成", "start_offset": 18, "end_offset": 33, "label": "sym" } ]
肾损害多发生于出现皮肤紫癜的3个月内(95%),尽管有报道肾损害可出现在皮肤紫癜之前以及在皮肤紫癜1年之后,但在6个月后出现肾损害一般不应轻易视之为紫癜性肾炎。
[ { "id": 0, "entity": "肾损害", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "皮肤紫癜", "start_offset": 9, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "肾损害", "start_offset": 29, "end_offset": 32, "label": "dis" }, { "id": 3, "entity": "皮肤紫癜", "start_offset": 36, "end_offset": 40, "label": "dis" }, { "id": 4, "entity": "皮肤紫癜", "start_offset": 45, "end_offset": 49, "label": "dis" }, { "id": 5, "entity": "肾损害", "start_offset": 62, "end_offset": 65, "label": "dis" }, { "id": 6, "entity": "紫癜性肾炎", "start_offset": 74, "end_offset": 79, "label": "dis" } ]
补体系统的激活,产生一系列炎症介质,导致局部炎性改变,继之发生凝血和纤溶系统障碍,出现小血管内血栓形成和纤维蛋白的沉积,最终导致肾小球损伤。
[ { "id": 0, "entity": "补体系统", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "局部炎性改变", "start_offset": 20, "end_offset": 26, "label": "sym" }, { "id": 2, "entity": "凝血", "start_offset": 31, "end_offset": 33, "label": "sym" }, { "id": 3, "entity": "纤溶系统", "start_offset": 34, "end_offset": 38, "label": "bod" }, { "id": 4, "entity": "纤溶系统障碍", "start_offset": 34, "end_offset": 40, "label": "sym" }, { "id": 5, "entity": "小血管", "start_offset": 43, "end_offset": 46, "label": "bod" }, { "id": 6, "entity": "小血管内血栓形成", "start_offset": 43, "end_offset": 51, "label": "sym" }, { "id": 7, "entity": "纤维蛋白的沉积", "start_offset": 52, "end_offset": 59, "label": "sym" }, { "id": 8, "entity": "肾小球损伤", "start_offset": 64, "end_offset": 69, "label": "dis" } ]
皮疹初为鲜红色,略高出皮面,可伴痒感及风团,并反复成批出现。
[ { "id": 0, "entity": "皮疹初为鲜红色", "start_offset": 0, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "略高出皮面", "start_offset": 8, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "痒感及风团", "start_offset": 16, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "反复成批出现", "start_offset": 23, "end_offset": 29, "label": "sym" } ]
3.急性肾炎型。
[ { "id": 0, "entity": "急性肾炎型", "start_offset": 2, "end_offset": 7, "label": "dis" } ]
4.肾病综合征型。
[ { "id": 0, "entity": "肾病综合征型", "start_offset": 2, "end_offset": 8, "label": "dis" } ]
6.慢性肾炎型。
[ { "id": 0, "entity": "慢性肾炎型", "start_offset": 2, "end_offset": 7, "label": "dis" } ]
由于HSPN在急性期有特征性出血性皮疹、腹痛、肠出血、关节炎和肾炎等特点,因此不难诊断。
[ { "id": 0, "entity": "HSPN", "start_offset": 2, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "特征性出血性皮疹", "start_offset": 11, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "腹痛", "start_offset": 20, "end_offset": 22, "label": "sym" }, { "id": 3, "entity": "肠", "start_offset": 23, "end_offset": 24, "label": "bod" }, { "id": 4, "entity": "肠出血", "start_offset": 23, "end_offset": 26, "label": "sym" }, { "id": 5, "entity": "关节炎", "start_offset": 27, "end_offset": 30, "label": "dis" }, { "id": 6, "entity": "肾炎", "start_offset": 31, "end_offset": 33, "label": "dis" } ]
当临床表现不典型时,应与急性肾小球肾炎、IgA肾病、狼疮性肾炎和急性间质性肾炎相鉴别。
[ { "id": 0, "entity": "急性肾小球肾炎", "start_offset": 12, "end_offset": 19, "label": "dis" }, { "id": 1, "entity": "IgA肾病", "start_offset": 20, "end_offset": 25, "label": "dis" }, { "id": 2, "entity": "狼疮性肾炎", "start_offset": 26, "end_offset": 31, "label": "dis" }, { "id": 3, "entity": "急性间质性肾炎", "start_offset": 32, "end_offset": 39, "label": "dis" } ]
HSP肾炎和IgA肾病尽管免疫发病机制相似,但临床上有明显区别,IgA肾病缺乏HSP肾炎的肾外表现。
[ { "id": 0, "entity": "HSP肾炎", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "IgA肾病", "start_offset": 6, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "IgA肾病", "start_offset": 32, "end_offset": 37, "label": "dis" }, { "id": 3, "entity": "HSP肾炎", "start_offset": 39, "end_offset": 44, "label": "dis" }, { "id": 4, "entity": "肾", "start_offset": 45, "end_offset": 46, "label": "bod" } ]
HSP肾炎呈急性发病过程,临床表现轻重不一,病程较短,其肾损伤的程度取决于肾小球新月体的多少。
[ { "id": 0, "entity": "HSP肾炎", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "肾损伤", "start_offset": 28, "end_offset": 31, "label": "dis" } ]
而IgA肾病呈慢性持续性发展,较易发展为肾功能不全,新月体形成不甚明显,而节段性肾小球硬化较为突出。
[ { "id": 0, "entity": "IgA肾病", "start_offset": 1, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "肾功能", "start_offset": 20, "end_offset": 23, "label": "ite" }, { "id": 2, "entity": "新月体形成不甚明显", "start_offset": 26, "end_offset": 35, "label": "sym" }, { "id": 3, "entity": "节段性肾小球硬化", "start_offset": 37, "end_offset": 45, "label": "bod" } ]
无明确过敏原者,应注意有无感染或隐性感染,可同时抗过敏治疗。
[ { "id": 0, "entity": "感染", "start_offset": 13, "end_offset": 15, "label": "sym" }, { "id": 1, "entity": "隐性感染", "start_offset": 16, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "抗过敏治疗", "start_offset": 24, "end_offset": 29, "label": "pro" } ]
一般用于紫癜性肾炎临床上大量蛋白尿的病人,对于病理Ⅲ级以上病人也应积极应用肾上腺皮质激素治疗,可显著改善预后,可选用泼尼松,剂量为2mg/(kg•d),疗程1~2周。
[ { "id": 0, "entity": "紫癜性肾炎", "start_offset": 4, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "蛋白尿", "start_offset": 14, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "肾上腺皮质激素", "start_offset": 37, "end_offset": 44, "label": "dru" }, { "id": 3, "entity": "泼尼松", "start_offset": 58, "end_offset": 61, "label": "dru" } ]
维生素E也有部分治疗作用,Ⅳ~Ⅴ期病人还可试用血浆置换及免疫吸附,有望获得肾功能的改善。
[ { "id": 0, "entity": "维生素E", "start_offset": 0, "end_offset": 4, "label": "dru" }, { "id": 1, "entity": "血浆置换", "start_offset": 23, "end_offset": 27, "label": "pro" }, { "id": 2, "entity": "免疫吸附", "start_offset": 28, "end_offset": 32, "label": "pro" }, { "id": 3, "entity": "肾功能", "start_offset": 37, "end_offset": 40, "label": "ite" } ]
临床上表现为肾病综合征和/或肾炎综合征的患儿,病理上Ⅲb级以上患儿,常遗留下持续的肾损害,并最终导致肾功能不全。
[ { "id": 0, "entity": "肾病", "start_offset": 6, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "肾病综合征", "start_offset": 6, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "肾炎", "start_offset": 14, "end_offset": 16, "label": "dis" }, { "id": 3, "entity": "肾炎综合征", "start_offset": 14, "end_offset": 19, "label": "sym" }, { "id": 4, "entity": "肾损害", "start_offset": 41, "end_offset": 44, "label": "dis" }, { "id": 5, "entity": "肾功能不全", "start_offset": 50, "end_offset": 55, "label": "dis" } ]
近年的研究认为幽门螺杆菌的胃内感染是引起慢性胃炎最重要的因素,其产生的机制与黏膜的破坏和保护因素之间失去平衡有关。
[ { "id": 0, "entity": "幽门螺杆菌的胃内感染", "start_offset": 7, "end_offset": 17, "label": "dis" }, { "id": 1, "entity": "慢性胃炎", "start_offset": 20, "end_offset": 24, "label": "dis" }, { "id": 2, "entity": "黏膜", "start_offset": 38, "end_offset": 40, "label": "bod" } ]
感染幽门螺杆菌后,胃部病理形态改变主要是胃窦黏膜小结节,小颗粒隆起,组织学显示淋巴细胞增多,淋巴滤泡形成,用药物将幽门螺杆菌清除后胃黏膜炎症明显改善。
[ { "id": 0, "entity": "幽门螺杆菌", "start_offset": 2, "end_offset": 7, "label": "mic" }, { "id": 1, "entity": "胃窦黏膜小结节", "start_offset": 20, "end_offset": 27, "label": "sym" }, { "id": 2, "entity": "小颗粒隆起", "start_offset": 28, "end_offset": 33, "label": "sym" }, { "id": 3, "entity": "淋巴细胞增多", "start_offset": 39, "end_offset": 45, "label": "sym" }, { "id": 4, "entity": "淋巴滤泡形成", "start_offset": 46, "end_offset": 52, "label": "sym" }, { "id": 5, "entity": "幽门螺杆菌", "start_offset": 57, "end_offset": 62, "label": "mic" }, { "id": 6, "entity": "胃黏膜炎症", "start_offset": 65, "end_offset": 70, "label": "dis" } ]
此外成人健康志愿者口服幽门螺杆菌证实可引发胃黏膜的慢性炎症,并出现上腹部痛、恶心及呕吐等症状;用幽门螺杆菌感染动物的动物模型也获得了成功,因此幽门螺杆菌是慢性胃炎的一个重要病因。
[ { "id": 0, "entity": "幽门螺杆菌", "start_offset": 11, "end_offset": 16, "label": "mic" }, { "id": 1, "entity": "胃黏膜", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "慢性炎症", "start_offset": 25, "end_offset": 29, "label": "dis" }, { "id": 3, "entity": "上腹部痛", "start_offset": 33, "end_offset": 37, "label": "sym" }, { "id": 4, "entity": "恶心", "start_offset": 38, "end_offset": 40, "label": "sym" }, { "id": 5, "entity": "呕吐", "start_offset": 41, "end_offset": 43, "label": "sym" }, { "id": 6, "entity": "幽门螺杆菌", "start_offset": 48, "end_offset": 53, "label": "mic" }, { "id": 7, "entity": "幽门螺杆菌", "start_offset": 71, "end_offset": 76, "label": "mic" }, { "id": 8, "entity": "慢性胃炎", "start_offset": 77, "end_offset": 81, "label": "dis" } ]
有报道40%的慢性扁桃腺炎患者其胃内有卡他性改变。
[ { "id": 0, "entity": "慢性扁桃腺炎", "start_offset": 7, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "胃内有卡他性改变", "start_offset": 16, "end_offset": 24, "label": "sym" } ]
急性胃炎之后胃黏膜损伤经久不愈,反复发作亦可发展为慢性胃炎。
[ { "id": 0, "entity": "急性胃炎", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "胃黏膜损伤", "start_offset": 6, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "慢性胃炎", "start_offset": 25, "end_offset": 29, "label": "dis" } ]
胆盐可减低胃黏膜屏障对氢离子的通透性,并使胃窦部G细胞释放胃泌素,增加胃酸分泌,氢离子通过损伤的黏膜屏障并弥散进入胃黏膜引起炎症变化、血管扩张及炎性渗出增多,使慢性胃炎持续存在。
[ { "id": 0, "entity": "胆盐", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "减低胃黏膜屏障对氢离子的通透性", "start_offset": 3, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "胃窦部G细胞释放胃泌素", "start_offset": 21, "end_offset": 32, "label": "sym" }, { "id": 3, "entity": "增加胃酸分泌", "start_offset": 33, "end_offset": 39, "label": "sym" }, { "id": 4, "entity": "损伤的黏膜屏障", "start_offset": 45, "end_offset": 52, "label": "bod" }, { "id": 5, "entity": "胃黏膜", "start_offset": 57, "end_offset": 60, "label": "bod" }, { "id": 6, "entity": "炎症变化", "start_offset": 62, "end_offset": 66, "label": "sym" }, { "id": 7, "entity": "血管扩张", "start_offset": 67, "end_offset": 71, "label": "sym" }, { "id": 8, "entity": "炎性渗出增多", "start_offset": 72, "end_offset": 78, "label": "sym" }, { "id": 9, "entity": "慢性胃炎", "start_offset": 80, "end_offset": 84, "label": "dis" } ]
幼儿腹痛可仅表现不安和正常进食行为改变,年长儿症状似成人,常诉上腹痛,其次有嗳气、早饱、恶心、上腹部不适及泛酸。
[ { "id": 0, "entity": "腹痛", "start_offset": 2, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "表现不安", "start_offset": 6, "end_offset": 10, "label": "sym" }, { "id": 2, "entity": "正常进食行为改变", "start_offset": 11, "end_offset": 19, "label": "sym" }, { "id": 3, "entity": "上腹痛", "start_offset": 31, "end_offset": 34, "label": "sym" }, { "id": 4, "entity": "嗳气", "start_offset": 38, "end_offset": 40, "label": "sym" }, { "id": 5, "entity": "早饱", "start_offset": 41, "end_offset": 43, "label": "sym" }, { "id": 6, "entity": "恶心", "start_offset": 44, "end_offset": 46, "label": "sym" }, { "id": 7, "entity": "上腹部不适", "start_offset": 47, "end_offset": 52, "label": "sym" }, { "id": 8, "entity": "泛酸", "start_offset": 53, "end_offset": 55, "label": "sym" } ]
部分患儿可有食欲缺乏、乏力、消瘦及头晕,伴有胃糜烂者可出现黑便。
[ { "id": 0, "entity": "食欲缺乏", "start_offset": 6, "end_offset": 10, "label": "sym" }, { "id": 1, "entity": "乏力", "start_offset": 11, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "消瘦", "start_offset": 14, "end_offset": 16, "label": "sym" }, { "id": 3, "entity": "头晕", "start_offset": 17, "end_offset": 19, "label": "sym" }, { "id": 4, "entity": "胃糜烂", "start_offset": 22, "end_offset": 25, "label": "dis" }, { "id": 5, "entity": "黑便", "start_offset": 29, "end_offset": 31, "label": "sym" } ]
虽然过去多数放射学者认为,胃紧张度的障碍、蠕动的改变及空腹胃内的胃液,可作为诊断胃炎的依据,但近年胃镜检查发现,这种现象系胃动力异常而并非胃炎所致。
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根据有无腺体萎缩诊断为慢性浅表性胃炎或慢性萎缩性胃炎。
[ { "id": 0, "entity": "腺体", "start_offset": 4, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "慢性浅表性胃炎", "start_offset": 11, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "慢性萎缩性胃炎", "start_offset": 19, "end_offset": 26, "label": "dis" } ]
鉴别诊断:在慢性胃炎发作期时,可通过胃镜、B超、24小时pH监测综合检查,排除肝、胆、胰、消化性溃疡及反流性食管炎。
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在胃炎发作期,应注意与胃穿孔或阑尾炎早期鉴别。
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慢性胃炎尚无特殊疗法,无症状者无需治疗。
[ { "id": 0, "entity": "慢性胃炎", "start_offset": 0, "end_offset": 4, "label": "dis" } ]
3.有腹胀、恶心、呕吐者,给予胃动力药物,如多潘立酮及西沙比利等。
[ { "id": 0, "entity": "腹胀", "start_offset": 3, "end_offset": 5, "label": "sym" }, { "id": 1, "entity": "恶心", "start_offset": 6, "end_offset": 8, "label": "sym" }, { "id": 2, "entity": "呕吐", "start_offset": 9, "end_offset": 11, "label": "sym" }, { "id": 3, "entity": "多潘立酮", "start_offset": 22, "end_offset": 26, "label": "dru" }, { "id": 4, "entity": "西沙比利", "start_offset": 27, "end_offset": 31, "label": "dru" } ]
有头晕、乏力、衰弱、食欲减退和长达数月至数年的贫血症,部分病例体重减轻。
[ { "id": 0, "entity": "头晕", "start_offset": 1, "end_offset": 3, "label": "sym" }, { "id": 1, "entity": "乏力", "start_offset": 4, "end_offset": 6, "label": "sym" }, { "id": 2, "entity": "衰弱", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 3, "entity": "食欲减退", "start_offset": 10, "end_offset": 14, "label": "sym" }, { "id": 4, "entity": "贫血症", "start_offset": 23, "end_offset": 26, "label": "dis" } ]
并发症以出血和感染多见,在未转变为急性白血病的病例中,大多死于这两个原因,两者的发生率约分别为20%和39%。
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感染中以下呼吸道感染为多见,约占60%~70%,其他可表现为肛门、会阴部感染,脓疱症和败血症等。
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MDS有原发和继发于治疗相关MDS之分,儿童原发性MDS可进一步分为难治性血细胞减少症(RC)、难治性贫血伴幼稚细胞增多(RAEB)、难治性贫血伴幼稚细胞增多向白细胞转化(RAEBT)。
[ { "id": 0, "entity": "难治性血细胞减少症", "start_offset": 34, "end_offset": 43, "label": "dis" }, { "id": 1, "entity": "RC", "start_offset": 44, "end_offset": 46, "label": "dis" }, { "id": 2, "entity": "难治性贫血", "start_offset": 48, "end_offset": 53, "label": "dis" }, { "id": 3, "entity": "幼稚细胞增多", "start_offset": 54, "end_offset": 60, "label": "sym" }, { "id": 4, "entity": "RAEB", "start_offset": 61, "end_offset": 65, "label": "sym" }, { "id": 5, "entity": "难治性贫血", "start_offset": 67, "end_offset": 72, "label": "dis" }, { "id": 6, "entity": "幼稚细胞", "start_offset": 73, "end_offset": 77, "label": "bod" }, { "id": 7, "entity": "白细胞", "start_offset": 80, "end_offset": 83, "label": "bod" } ]
皮肤损害症状明显,特别是面部皮疹是常见而重要的体征之一,多数患儿脾大,部分患儿肝脏和淋巴结增大。
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外周血白细胞和单核细胞增多,贫血,血小板减少,常见幼稚红细胞和幼稚粒细胞,骨髓呈增生性特征。
[ { "id": 0, "entity": "外周血", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "白细胞", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 2, "entity": "单核细胞", "start_offset": 7, "end_offset": 11, "label": "bod" }, { "id": 3, "entity": "贫血", "start_offset": 14, "end_offset": 16, "label": "dis" }, { "id": 4, "entity": "幼稚粒细胞", "start_offset": 31, "end_offset": 36, "label": "bod" } ]
造血干细胞移植是目前唯一可以根治MDS的治疗方法。
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不良反应为皮肤黏膜干燥,ALT增高,颅压增高等。
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目前认为,HHV-6是该病的主要病因,但并不是唯一的病原。
[ { "id": 0, "entity": "HHV-6", "start_offset": 5, "end_offset": 10, "label": "mic" } ]
此期除有食欲减退、不安或轻咳外,体征不明显,仅有咽部和扁桃体轻度充血和头颈部浅表淋巴结轻度肿大。
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首现于躯干,然后迅速波及颈、上肢、脸和下肢。
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皮疹持续24~48小时很快消退,无色素沉着,也不脱皮。
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也可随后出现白细胞总数增多。
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确诊有赖于肺活检。
[ { "id": 0, "entity": "肺活检", "start_offset": 5, "end_offset": 8, "label": "ite" } ]
病毒主要通过空气飞沫传播。
[ { "id": 0, "entity": "病毒", "start_offset": 0, "end_offset": 2, "label": "mic" } ]
常有高热、中度中毒征象和流涕。
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此外,可见腹泻和皮疹。
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