儿童非典型溶血尿毒综合征15例临床分析

付 　阳1, 廖 盼丽2, 祝 高红2, 戚 　畅2, 王筱 雯*2
1、江汉大学医学院;华中科技大学同济医学院附属武汉儿童医院（武汉市妇幼保健院）肾内科
2、华中科技大学同济医学院附属武汉儿童医院（武汉市妇幼保健院）肾内科

摘要


目的：回顾性分析儿童非典型溶血性尿毒症综合征（atypical hemolytic uremic syndrome，aHUS）早期临床
特点及治疗情况，以提高该病的诊疗水平。方法：回顾性分析15例aHUS患儿的临床资料，包括临床表现、实验室
检查、辅助检查、治疗及预后。结果：患儿首发症状有腹痛或呕吐、发热、咳嗽、非产志贺毒素大肠杆菌腹泻等。
15例患儿均表现为溶血性贫血、血小板计数减少、肾损伤和乳酸脱氢酶（LDH）升高，8例C3水平降低，3例肾穿
刺病理结果提示血栓性微血管病变；5例患儿进行了基因检测及补体因子检测，3例存在基因突变（2例CFH突变、1
例CD46/CFHR3突变），1例H因子抗体阳性；H因子检测及I因子检测均正常范围；ADAMTS13活性均在正常范围
内；13例患儿行血浆置换和糖皮质激素治疗，其他治疗包括输注洗涤红细胞纠正贫血、输注血小板、补液纠正电解
质紊乱和抗感染等。治疗前后血红蛋白、血小板、肌酐、乳酸脱氢酶比较具有统计学意义；结论：aHUS患儿早期临
床表现多样，前驱期大多有感染诱因，早期往往表现为多系统损伤，基因检测或补体因子检测有助于明确病因诊断，
及时给予有效治疗，改善预后。

关键词


非典型溶血性尿毒症综合征；儿童；基因；预后

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参考


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