Clinical Features of Anti-Factor H Autoantibody–Associated Hemolytic Uremic Syndrome
نویسندگان
چکیده
منابع مشابه
Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome.
Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy that associates, in 70% of cases, with genetic or acquired disorders leading to dysregulation of the alternative pathway of complement. Autoantibody directed against Factor H causes at least 6% to 10% of aHUS cases, but only a few clinical reports are available. Here, we describe the clinical, biologic, genet...
متن کاملAnti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome.
Several studies have demonstrated genetic predisposition in non-shigatoxin-associated hemolytic uremic syndrome (HUS), involving regulatory proteins of the complement alternative pathway: Factor H (FH) and membrane co-factor protein (CD46). Regarding the observations of thrombotic thrombocytopenic purpura patients, in whom a von Willebrand factor protease (ADAMST-13) deficiency may be inherited...
متن کاملAnti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome.
The atypical form of the kidney disease hemolytic uremic syndrome (aHUS) is associated with defective complement regulation. In addition to mutations in complement regulators, factor H (FH)-specific autoantibodies have been reported for aHUS patients. The aim of the present study was to understand the role of these autoantibodies in aHUS. First, the binding sites of FH autoantibodies from 5 unr...
متن کاملComplement factor H and the hemolytic uremic syndrome
Immune recognition is coupled to powerful proinflammatory effector pathways that must be tightly regulated. The ancient alternative pathway of complement activation is one such proinflammatory pathway. Genetic susceptibility factors have been identified in both regulators and activating components of the alternative pathway that are associated with thrombotic microangiopathies, glomerulonephrit...
متن کاملAtypical hemolytic uremic syndrome due to factor H autoantibody.
Atypical hemolytic uremic syndrome (aHUS) is a disease caused by pathologies in the alternative complement system. The prevalence of aHUS is 10% of all aHUS cases. The subgroup of aHUS designated as DEAP (DEficiency of CFHR Proteins and CFH Autoantibody Positive)-HUS because of autoantibody to complement factor H (CFH) and CFH-related protein deficiency is seen very rarely, and the prevalence i...
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ژورنال
عنوان ژورنال: Journal of the American Society of Nephrology
سال: 2010
ISSN: 1046-6673,1533-3450
DOI: 10.1681/asn.2010030315