Complement disorders and hemolytic uremic syndrome.
Complement disorders and hemolytic uremic syndrome.
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DOI：
10.1097/mop.0b013e32835df48a
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发表时间：
2013-04
影响因子：
3.6
通讯作者：
Gattineni J
中科院分区：
文献类型：
--
作者：
Joseph C;Gattineni J
补体介导的溶血性尿毒症综合征(AHUS)在非志贺毒素尿毒症中占有相当大的比例。本综述的目的是概述aHUS的病理生理学、临床特征和治疗选择。在过去的十年里，在识别补体调节蛋白的几个新的致病突变方面取得了很大进展。补体介导的HUS(AHUS)与志贺毒素介导的HUS相比预后较差，常导致终末期肾脏疾病。早期识别aHUS是启动血浆治疗的关键。肾移植后，疾病复发和移植物丢失的风险非常高。Eculizumab和肝肾联合移植有望改善预后。
Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS. In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins. Complement mediated HUS (aHUS) has a worse prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease. Early identification of aHUS is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver–kidney transplantation offer promise for improved prognosis.