| 非典型溶血尿毒综合征发病机制和治疗研究进展 |
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| 引用本文： | 张婕,夏正坤,刘光陵.非典型溶血尿毒综合征发病机制和治疗研究进展[J].临床儿科杂志,2012,30(4):393-396. |
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| 作者姓名： | 张婕 夏正坤 刘光陵 |
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| 作者单位： | 南京军区南京总医院儿科南京大学医学院临床学院,江苏南京,210002 |
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| 摘 要： | 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。
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| 关 键 词： | 非典型溶血尿毒综合征 发病机制 治疗 |
Research progress in the pathogenesis and treatment of atypical hemolytic uremic syndrome |
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| ZHANG Jie
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XIA Zheng-kun
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LIU Guang-ling.Research progress in the pathogenesis and treatment of atypical hemolytic uremic syndrome[J].The Journal of Clinical Pediatrics,2012,30(4):393-396. |
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| Authors: | ZHANG Jie XIA Zheng-kun LIU Guang-ling |
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| Institution: | (Department of Pediatrics,Nanjing General Hospital of Nanjing Military Command,Nanjing University School of Clinical Medicine,Nanjing 210002,Jiangsu,China) |
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| Abstract: | 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. |
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| Keywords: | atypical haemolytic uraemic syndrome pathogenesis treatment |
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