Atypical Hemolytic Uremic Syndrome

David Kavanagh, Tim H. Goodship, Anna Richards

Research output: Contribution to journalArticlepeer-review

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
Original languageEnglish
Pages (from-to)508-530
Number of pages23
JournalSeminars in nephrology
Volume33
Issue number6
DOIs
Publication statusPublished - 1 Nov 2013

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