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Atypical hemolytic uremic syndrome

David Kavanagh1, Tim H Goodship, Anna Richards

  • 1The Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.

Seminars in Nephrology
|October 29, 2013
PubMed

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.

Keywords:
Complementeculizumabfactor Hfactor Ihemolytic uremic syndromemembrane cofactor proteinthrombomodulintransplantation

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