Atypical Hemolytic Uremic Syndrome

Overview

Journal Semin Nephrol

Specialty Nephrology

Date 2013 Oct 29

PMID 24161037

Citations 150

Authors

David Kavanagh

Tim H Goodship

Anna Richards

Affiliations

Soon will be listed here.

Abstract

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.

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