Does hemolytic uremic syndrome differ from thrombotic thrombocytopenic purpura?

被引:44
作者
Fakhouri, Fadi
Fremeaux-Bacchi, Veronique
机构
[1] Hop Necker Enfants Malad, Serv Nephrol, F-75743 Paris 15, France
[2] Univ Paris 05, Hop Paris, Hop Necker, Dept Nephrol, Paris, France
[3] Hop Europeen Georges Pompidou, Dept Immunol, Paris, France
来源
NATURE CLINICAL PRACTICE NEPHROLOGY | 2007年 / 3卷 / 12期
关键词
ADAMTS13; complement; thrombotic microangiopathy;
D O I
10.1038/ncpneph0670
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Both hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are characterized by thrombotic microangiopathy (TMA), affecting mainly the kidney and brain, respectively. Diagnosis of HUS or TTP has been complicated by the fact that these disorders share several clinical characteristics, and by the dearth of knowledge regarding the pathogenesis of TMA. Advances in the identification of pathogenic features-deficiency of the metalloprotease ADAMTS13 in TTP and association of mutated complement proteins with atypical HUS-have gone some way towards improving clinicians' ability to distinguish between the two diseases. Here, we pose the following question: is it important to patient management that HUS be distinguished from TTP? By discussing what is known about the pathogenesis, clinical features and treatment of these two conditions we address this question, and propose a new nomenclature for TMA.
引用
收藏
页码:679 / 687
页数:9
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