Article
Familial atypical hemolytic uremic syndrome: a review of its genetic and clinical aspects.
Clinical & developmental immunology - 1 Jan 2012
Bu Fengxiao, Borsa Nicolo, Gianluigi Ardissino, Smith Richard J H
Abstract excerpt
Atypical hemolytic uremic syndrome (aHUS) is a rare renal disease (two per one million in the USA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Both sporadic (80% of cases) and familial (20% of cases) forms are recognized. The study of familial aHUS has implicated genetic variation in multiple genes in the complement system in disease pathogenesis, helping to...
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