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血栓性微血管病发病机制及分类新认识 被引量：8

Pathogenesis and classification of thrombotic microangiopathy:recent updates
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摘要 血栓性微血管病(TMA)分血栓性血小板减少性紫癜及溶血尿毒症综合征(HUS),HUS又分感染后HUS和非典型HUS,现认为HUS发病主要与补体旁路途径的过度激活相关。 Thrombocytopenic purpura （TTP） and hemolytic uremic syndrome （HUS） were distinguished from each other by whether with ADAMTS13 deficiency. Furthermore, hemolytic uremic syndrome （HUS） is sub- divided into postnfection HUS and atypical HUS. Currently,it is considered that the occurrence of HUS is related with excessive activation of the complement alternative pathway.
作者 林宛兵 陈楠
机构地区 上海交通大学医学院附属瑞金医院肾脏科
出处 《中国实用内科杂志》 CAS CSCD 北大核心 2011年第10期799-801,共3页 Chinese Journal of Practical Internal Medicine
关键词 血栓性微血管病 血栓性血小板减少性紫癜 溶血尿毒症综合征 thrombotic microangiopathy thrombotic thrombocytopenic purpura hemolytic uremic syndrome
分类号 R543 [医药卫生—心血管疾病]
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参考文献12

  • 1Manea M, Tati R, Karlsson J, et al. Biologically active ADAMTS13 is expressed in renal tubular epithelial ceils [ J ]. Pediatr Nephrol, 2010,25:87 - 96.
  • 2Terrell DR, Williams LA, Vesely SK, et al. The incidence of throm- botic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients ,and patients with severe ADAMTS-13 deficiency[ J]. J Thromb. Haemost ,2005,3:1432 - 1436.
  • 3Ferrari S, Mudde GC, Rieger M, et al. IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura [ J ]. J Thromb Haemost, 2009, 7 : 1703 - 1710.
  • 4Thurman JM, Marians R, Emlen W, et al. Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome [ J ]. Clin J Am Soc Nephrol,2009,4 : 1920 - 1924.
  • 5Orth D, Khan AB, Naim A, et al. Shiga toxin activates complement and binds factor H:evidence for an active role of complement in hemolytic uremic syndrome [ J ]. J Immunol, 2009,182 : 6394 - 6400.
  • 6Nilsson SC, Kalchishkova N, Trouw LA, et al. Mutations in comple- ment factor I as found in atypical hemolytic uremic syndrome lead to either altered secretion or altered function of factor I [ J ]. Eur J Immunol,2010,40 : 172 - 185.
  • 7Goicoechea de Jorge E, Harris CL, Esparza-Gordillo J, et al. Gain- of-function mutations in complement factor B are associated with a- typical hemolytic uremic syndrome [ J ]. Proc Natl Acad Sci USA, 2007,104:240 - 245.
  • 8Roumenina LT, Jablonski M, Hue C, et al. Hyperfunctional C3 convertase leads to complement deposition on endothelial cells and contributes to atypical hemolytic uremic syndrome [ J ]. Blood, 2009,114:2837 - 2845.
  • 9Fremeaux-Baechi V, Miller EC, Liszewski MK, et al. Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome [ J ]. Blood,2008,112:4948 - 4952.
  • 10DelvaeyeM, Noris M, De Vriese A, et al. Thrombomodulin muta- tions in atypical hemolytic-uremic syndrome [ J ]. N Engl J Med, 2009,361:345 - 357.

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中国实用内科杂志
2011年 第10期

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