非典型性溶血尿毒综合征发病机制与治疗的进展  

Advances in pathogenesis and treatment of atypical hemolytic uremic syndrome

作　　者：王兆钺[1] 

机构地区：[1]苏州大学附属第一医院,江苏省血液研究所,卫生部血栓与止血重点实验室

出　　处：《临床血液学杂志》2015年第5期812-815,共4页Journal of Clinical Hematology

基　　金：国家自然科学基金(No:30770917;81070395);江苏省科教兴卫工程-临床医学中心(No:ZX201102);江苏省临床医学科技专项(No:BL2012005)

摘　　要：非典型性溶血尿毒综合征(atypical hemolytic uremic syndrome,aHUS)是血栓性微血管病(thrombotic microangiopathy,TMA)中的一种疾病,临床上比较少见,在北美人群的发病率约为2/(百万人·年)〔1〕。长期以来aHUS未被人们了解与重视,将之归属为HUS,称为家族性HUS。HUS与aHUS的临床特征都表现为"三联征"Summary Atypical hemolytic uremic syndrome （aHUS） belongs to thrombotic microangiopathy, aHUS is characterized by thrombocytopenia,microangiopathic hemolytic anemia and renal dysfunction. In contrast to classical HUS,aHUS is caused by dysregulation of the alternate complement pathway resulting in the generation of C3 convertase complexes without prodromal Shiga toxin Escherichia Coli infection. Normal ADAMTS13 activity allow for its identification from thrombotic thrombocytopenic purpura. The most important advance in aHUS treatment is the initiation of eculizumab therapy which results in hematologic improvement, especially improvement in renal function and prognosis.

关 键 词：非典型性溶血尿毒综合征 补体旁路途径 诊断 治疗 ECULIZUMAB 

分 类 号：R558.9[医药卫生—血液循环系统疾病]