Atypical Hemolytic Uremic Syndrome
نویسندگان
چکیده
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 reported, suggesting that a precipitating event or trigger is required to unmask the complement regulatory deficiency. The underlying genetic defect predicts the prognosis both in native kidneys and after renal transplantation. The successful trials of the complement inhibitor eculizumab in the treatment of atypical HUS will revolutionize disease management.
منابع مشابه
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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