[HTML][HTML] Atypical hemolytic uremic syndrome

D Kavanagh, TH Goodship, A Richards - Seminars in nephrology, 2013 - Elsevier
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 …