Abstract
Atypical hemolytic uremic syndrome (aHUS) is a rare disease in adults. It is a thrombotic microangiopathy (TMA) characterized by the presence of renal failure, microangiopathic hemolytic anemia and thrombocytopenia. It is the consequence of a defective complement regulation due to genetic mutations or autoantibodies of the activators or regulators of the complement alternative pathway. Renal involvement is the major prognostic factor. Evaluation of the complement pathway including estimation of blood levels of C3, factor H, I and anti-factor H antibodies is essential for the etiological diagnosis.
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