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Lookup NU author(s): Professor David KavanaghORCiD, Professor Tim Goodship
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Atypical haemolytic uraemic syndrome (aHUS) is a disease characterized by complement overactivation in which inherited defects in complement genes and acquired autoanti-bodies against complement regulatory proteins have been described. Identification of the underlying defect can both predict disease outcome and guide treatment. The ability to remove inhibitory autoantibodies and hyper-active complement components in addition to its ability to replace defective complement regulators means that plasma exchange is currently first-line therapy. In those with factor H and factor I mutations who do progress to end-stage renal failure, renal transplantation usually fails due to recurrent HUS. In this situation, combined liver-kidney transplantation has been suggested to correct the underlying genetic defect. Newer agents, such as the complement inhibitor eculizumab, may herald a new era in the treatment of aHUS. Copyright (C) 2010 S. Karger AG, Basel
Author(s): Kavanagh D, Goodship T
Publication type: Article
Publication status: Published
Journal: Nephron Clinical Practice
Year: 2011
Volume: 118
Issue: 1
Pages: C37-C42
Print publication date: 11/11/2010
ISSN (print): 1660-2110
ISSN (electronic): 1660-8151
Publisher: S. Karger AG
URL: http://dx.doi.org/10.1159/000320901
DOI: 10.1159/000320901
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