非典型溶血性尿毒综合征发病机制及治疗研究进展

Advances in pathogenesis and treatment of atypical hemolytic uremic syndrome

非典型溶血性尿毒综合征是一种主要累及肾脏的罕见血栓性微血管病变,主要由补体途径异常导致补体系统的过度激活和微血管血栓形成,其特征是微血管性溶血性贫血、血小板减少和肾功能损害,但也可导致多器官系统功能障碍。治疗手段目前有血浆置换、免疫抑制剂、补体抑制剂、肝肾移植等,但其疗效需长期多中心研究评价。现将其发病机制、临床特征及治疗作一综述。

Atypical hemolytic uremic syndrome( a HUS) is a rare thrombotic microangiopathy that mainly affects kidney. It is mainly caused by the imbalance of complements leading to microvascular thrombosis and characterized by microvascular hemolytic anemia,thrombocytopenia,renal dysfunction and multiple organ dysfunction.Common treatments include plasmapheresis, immunosuppression, complement inhibitors, liver and kidney transplantation,but the efficacy still needs to be evaluated by long-term multi-center studies. This paper summarized the pathogenesis,clinical features and therapeutic measures of a HUS.