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Lookup NU author(s): Dr Edwin Wong,
Professor Tim Goodship,
Professor David KavanaghORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
Central to the pathogenesis of atypical haemolytic uraemic syndrome (aHUS) is over-activation of the alternative pathway of complement. Inherited defects in complement genes and autoantibodies against complement regulatory proteins have been described. The use of plasma exchange to replace non-functioning complement regulators and hyper-functional complement components in addition to the removal of CFH-autoantibodies made this the 'gold-standard' for management of aHUS. In the last 4 years the introduction of the complement inhibitor Eculizumab has revolutionised the management of aHUS. In this review we shall discuss the available literature on treatment strategies to date. (C) 2013 Elsevier Ltd. All rights reserved.
Author(s): Wong EKS, Goodship THJ, Kavanagh D
Publication type: Review
Publication status: Published
Journal: Molecular Immunology
Print publication date: 28/06/2013
Acceptance date: 10/05/2013
ISSN (print): 0161-5890
ISSN (electronic): 1872-9142
Publisher: PERGAMON-ELSEVIER SCIENCE LTD