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  • Title: Complement therapy in atypical haemolytic uraemic syndrome (aHUS).
    Author: Wong EK, Goodship TH, Kavanagh D.
    Journal: Mol Immunol; 2013 Dec 15; 56(3):199-212. PubMed ID: 23810412.
    Abstract:
    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.
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