Atypical hemolytic uremic syndrome: telling the difference between H and Y

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Atypical Hemolytic Uremic Syndrome

Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described. Incomplete penetrance of mutations in all predisposing genes is r...

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Atypical hemolytic uremic syndrome

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Most cases of aHUS are caused by uncontrolled complement activation due to genetic mutations in the alternative pathway of complement. More recently, mutations in the gene of coagulation system have also been identified in...

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Atypical hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -...

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Concurrent Atypical Hemolytic Uremic Syndrome and Autoimmune Hemolytic Anemia: a case report

Background: Atypical hemolytic uremic syndrome (aHUS) is a life-threatening and scarce disorder characterized by acute renal failure and disease, non-immune microangiopathic hemolytic anemia and thrombocytopenia, leading to end-stage renal failure or death, and consequently maybe accompanying by extra renal manifestations. Case report: We reported aHUS accompanied by autoimmune hemolytic anemi...

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Atypical 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: Kidney International

سال: 2010

ISSN: 0085-2538

DOI: 10.1038/ki.2010.222