摘要
血栓性微血管病(TMA)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,包括溶血尿毒综合征(HUS)及血栓性血小板减少性紫癜(TTP)。HUS是危重肾小球疾病,根据发病机制的不同,目前分为经典型HUS和非典型HUS两大类。非典型HUS起病急骤,病情凶险,易反复,急性期病死率高。TTP是一种少见但致命的疾病,其病理生理基础为特异性血管性假性血友病因子(VWF)裂解蛋白酶(含有1型血栓反应蛋白重复序列的解整联蛋白和金属蛋白酶A成员13,ADAMTS-13)功能严重缺乏,这种缺乏可以是先天性或获得性,血浆ADAMTS-13活性<10%是唯一可确定TTP诊断的方法。现就儿童重症TMA的诊断及治疗研究进展介绍如下。
Thrombotic microangiopathy(TMA)is a group of clinical and pathological syndromes,characterized mainly by hemolytic anemia,thrombocytopenia,and multiple organ dysfunctions caused by a variety of factors.Classic types of TMA include hemolytic uremic syndrome(HUS)and thrombotic thrombocytopenic purpura(TTP).HUS is a critically ill glomerular disease.Depending on the pathogenesis,HUS is currently divided into 2 categories,namely typical and atypical HUS.Atypical HUS is at a rapid onset and is a type of dangerous disease,which tends to recur easily,and has high mortality in its acute phase.TTP is a rare but fatal entity of TMA.The pathophysiology of the disease is based on a severe functional deficiency of a disintegrin and metalloprotease with thrombospondin type 1 repeats,member 13(ADAMTS-13),the specific von Willebrand factor(VWF)-cleavage protease.This deficiency may be either acquired or congenital.The activity of plasma ADAMTS-13<10%is the only definitive method for diagnosing TTP.This article reports the recent advances on the diagnosis and treatment of severe TMA in children.
作者
李玖军
赵莹
Li Jiujun;Zhao Ying(Pediatric Intensive Care Unit,Shengjing Hospital of China Medical University,Shenyang 110004,China)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2019年第18期1372-1376,共5页
Chinese Journal of Applied Clinical Pediatrics
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
