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Lookup NU author(s): Dr Lisa Turnbull,
Professor Tim Goodship
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Kidney transplant in patients with atypical hemolytic uremic syndrome (aHUS) is associated with a poor outcome because of recurrent disease, especially in patients known to have a factor H mutation. Long-term prophylactic plasma exchange and combined liver-kidney transplant have prevented graft loss caused by recurrence. However, the mortality associated with liver transplant is not negligible, and prophylactic plasma exchange requires permanent vascular access and regular hospitalization and exposes the patient to potential allergic reactions to plasma. Eculizumab is a high-affinity humanized monoclonal antibody that binds to C5 and thus prevents generation of C5a and the membrane attack complex. We report the case of a 17-year-old girl with aHUS associated with a mutation in the gene for complement factor H (CFH; c.3572C > T, Ser1191Leu) who was highly dependent on plasma exchange. Because of severe allergic reactions to plasma after the third renal graft, eculizumab was introduced in place of plasma exchange without problems. This and other reports suggest that the promise of complement inhibitors in the management of aHUS is going to be fulfilled. Am J Kidney Dis 55:708-711. (C) 2010 by the National Kidney Foundation, Inc.
Author(s): Davin JC, Gracchi V, Bouts A, Groothoff J, Strain L, Goodship T
Publication type: Article
Publication status: Published
Journal: American Journal of Kidney Diseases
ISSN (print): 0272-6386
ISSN (electronic): 1523-6838
Publisher: WB Saunders Co.
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