非典型溶血尿毒综合征发病机制和治疗研究进展

摘要

Atypical haemolytic uraemic syndrome (aHUS) is an uncommon condition that is now widely accepted to be a disorder of complement over activation. Mutations have been identified in genes encoding complement regulators ( complement factor H and complement factor I) , membrane cofactor protein ( MCP) and complement activators (complement factor B and C3). It frequent recurs and carries a poorer prognosis with a 25% mortality rate and 50% developing end-stage renal failure. Therefore, the pathogenesis and treatment of aHUS has been a research focus. These two aspects of the research progress were reviewed.%非典型溶血尿毒综合征(atypical haemolytic uraemic syndrome,aHUS)是一种补体失调性疾病,补体调控蛋白H因子、I因子,以及膜辅助蛋白和血清补体固有成分(B因子、补体C3)的基因突变都可参与其发病,病情易反复,预后很差,25％的患者在急性期死亡,50％以上发展为终末期肾病.因此aHUS的发病机制及治疗一直是研究热点,文章就这两个方面的研究进展作了综述.