Haemolytic uraemic syndrome

被引:359
|
作者
Fakhouri, Fadi [1 ,2 ]
Zuber, Julien [3 ]
Fremeaux-Bacchi, Veronique [4 ,5 ]
Loirat, Chantal [6 ]
机构
[1] CHU, Dept Nephrol, Nantes, France
[2] INSERM, UMR S1064, Nantes, France
[3] Univ Paris 05, Hop Necker, Dept Nephrol & Renal Transplantat, AP HP, Paris, France
[4] Hop Europaen Georges Pompidou, Dept Biol Immunol, AP HP, Paris, France
[5] INSERM, Complement & Malad, Ctr Rech Cordeliers, UMR S1138, Paris, France
[6] Univ Paris Diderot, Hop Robert Debre, Dept Pediat Nephrol, AP HP, Sorbonne Paris Cite, Paris, France
来源
LANCET | 2017年 / 390卷 / 10095期
关键词
STEM-CELL TRANSPLANTATION; ESCHERICHIA-COLI O104H4; COBALAMIN C DEFICIENCY; INDUCED THROMBOTIC MICROANGIOPATHY; GENETIC COMPLEMENT ABNORMALITIES; FACTOR-H ANTIBODIES; LONG-TERM OUTCOMES; ANTIPHOSPHOLIPID SYNDROME; ALTERNATIVE PATHWAY; NEUROLOGICAL INVOLVEMENT;
D O I
10.1016/S0140-6736(17)30062-4
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Haemolytic uraemic syndrome is a form of thrombotic microangiopathy affecting predominantly the kidney and characterised by a triad of thrombocytopenia, mechanical haemolytic anaemia, and acute kidney injury. The term encompasses several disorders: shiga toxin-induced and pneumococcus-induced haemolytic uraemic syndrome, haemolytic uraemic syndrome associated with complement dysregulation or mutation of diacylglycerol kinase epsilon, haemolytic uraemic syndrome related to cobalamin C defect, and haemolytic uraemic syndrome secondary to a heterogeneous group of causes ( infections, drugs, cancer, and systemic diseases). In the past two decades, experimental, genetic, and clinical studies have helped to decipher the pathophysiology of these various forms of haemolytic uraemic syndrome and undoubtedly improved diagnostic approaches. Moreover, a specific mechanism-based treatment has been made available for patients affected by atypical haemolytic uraemic syndrome due to complement dysregulation. Such treatment is, however, still absent for several other disease types, including shiga toxin-induced haemolytic uraemic syndrome.
引用
收藏
页码:681 / 696
页数:16
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