儿童血栓性微血管病的诊断及治疗进展

Progress of diagnosis and treatment in children with thrombotic microangiopathy

血栓性微血管病是由各种原因引起的一组以溶血性贫血、血小板减少和多器官功能障碍为主要特征的临床病理综合征。血栓性微血管病中的经典类型包括溶血尿毒综合征，血栓性血小板减少性紫癜。溶血尿毒综合征是危重症肾小球疾病，90％见于儿童。根据发病机制的不同，目前分为典型溶血尿毒综合征和非典型溶血尿毒综合征两大类。非典型溶血尿毒综合征起病急骤，病情凶险，易反复，急性期病死率高。补体旁路途径的过度激活在非典型溶血尿毒综合征发病机制中的重要地位已得到广泛关注。现就血栓性微血管病的病因、诊断及治疗研究进展介绍如下。

Thrombotic microangiopathy is a group of clinical and pathological syndrome, characterized mainly by hemolytic anemia, thromboeytopenia, and multiple organ dysfunctions, caused by a variety of reasons. Classic types of thrombotic microangiopathy include hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Hemolytic uremic syndrome is a critically ill glomerular disease, of which 90% found in children. Depending on the pathogenesis, hemolytic uremic syndrome is currently divided into 2 categories, namely, typical and atypical hemolytic uremic syn- dromes. Atypical hemolytic uremic syndrome onsets rapidly,is dangerous disease, tends to recurring easily, and has high mortality in its acute phase. Nowadays, great interest is focused on the important role of the excessive activation of the alternative complement pathway in the pathogenesis of atypical hemolytic uremic syndrome. This article report the recent advances on the cause,diagnosis,and treatment of thrombotic microangiopathy.