Abstract

儿童遗传性血栓性血小板减少性紫癜(hTTP)是一种少见但严重,甚至可致命的儿童血栓性微血管病,其发病机制为血管性血友病因子裂解酶(ADAMTS13)基因突变所导致的ADAMTS13活性持续严重缺乏。相较于成人,儿童期起病的hTTP更易出现脑或肾远期并发症,对预防性替代治疗的需求更迫切。本共识涵盖hTTP的定义、诊断、鉴别诊断及治疗,旨在为中国儿科医师开展儿童hTTP规范化诊治提供参考。.

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