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Am J Transplant. 2012 Jul;12(7):1938-44. doi: 10.1111/j.1600-6143.2012.04051.x. Epub 2012 Apr 11.

Renal transplantation under prophylactic eculizumab in atypical hemolytic uremic syndrome with CFH/CFHR1 hybrid protein.

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  • 1Pediatric Nephrology, Hôpital Necker-Enfants Malades, Paris, France.

Abstract

We report the first observation of successful kidney transplantation under pre-emptive eculizumab treatment in a 7-year-old boy with atypical hemolytic uremic syndrome (aHUS) and a known hybrid CFH/CFHR1 gene, who was dependent on plasma therapy during the 3-year dialysis period. The hybrid CFH/CFHR1 protein has an altered C3b/C3d binding, is incapable to protect cells from complement attack and is directly implicated in aHUS pathogenesis. There was no evidence of recurrence during the first 16-month follow-up period. We conclude that eculizumab alone, without plasma therapy (plasma infusion and/or plasma exchange), is sufficient to prevent recurrence of aHUS and to maintain long-term graft function.

© Copyright 2012 The American Society of Transplantation and the American Society of Transplant Surgeons.

PMID:
22494769
[PubMed - indexed for MEDLINE]
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