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-  2017 

Discontinuation of Peritoneal Dialysis after Late Initiation of Eculizumab in a Case of Familial Atypical Hemolytic-Uremic Syndrome: A Case Report

DOI: 10.1159/000457950

Keywords: Atypical hemolytic-uremic syndrome, Eculizumab, Mutation, Complement inactivating agents

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Abstract:

Atypical hemolytic-uremic syndrome is caused by a thrombotic microangiopathy and manifests itself with hemolytic anemia, thrombocytopenia, and organ ischemia. Its etiology is a mutation affecting the genes encoding for proteins of the complement system. Early treatment with eculizumab (8.6 months from the moment of presentation), a humanized monoclonal antibody against complement, is shown to be effective in controlling symptoms and reversing organ damage. We present a patient with a mutation not previously described in the literature. Late treatment with eculizumab resulted in a good therapeutic response, eliminating the need for peritoneal dialysis

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