摘要
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.
作者
刘小荣
Liu Xiaorong(Department of Nephrology,Beijing Children＇s Hospital Affiliated to Capital Medical University, Beijing Key Laboratory of Pediartic Chronic Kidney Disease and Blood Purification,Beijing 100045, China)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2016年第17期1292-1295,共4页
Chinese Journal of Applied Clinical Pediatrics
基金
北京市科学技术委员会首都临床特色应用研究（Z161100000516106）
首都卫生发展科研专项基金（2016-2-2094）
关键词
血栓性微血管病
溶血尿毒综合征
血栓性血小板减少性紫癜
诊断
治疗
儿童
Thrombotic microangiopathy
Hemolytic uremic syndrome
Thrombotic thrombocytopenic purpura
Diagnosis
Treatment
Child
