Mutations in proteins of the alternative pathway of complement and the pathogenesis of atypical hemolytic uremic syndrome.

Abstract excerpt

Atypical hemolytic uremic syndrome is associated with mutations in the complement proteins factor H, factor I, factor B, C3, or membrane cofactor protein in about 50% of patients. The evolution and prognosis of the disease in patients carrying mutations in factor H is particularly poor, and renal transplantation most often fails because of recurrence of the disease in the graft. The risk of rapid loss of renal...

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