Article
Liver transplantation for aHUS: still needed in the eculizumab era?
Pediatric nephrology (Berlin, Germany) - 1 May 2016
Coppo Rosanna, Bonaudo Roberto, Peruzzi R Licia, Amore Alessandro, Brunati Andrea, Romagnoli Renato, Salizzoni Mauro, Galbusera Miriam, Gotti Eliana, Daina Erica, Noris Marina, Remuzzi Giuseppe
Abstract excerpt
BACKGROUND: The risk of disease recurrence after a kidney transplant is high in patients with atypical hemolytic uremic syndrome (aHUS) and mutations in the complement factor H (FH) gene (CFH). Since FH is mostly produced by the liver, a kidney transplant does not correct the genetic defect. The anti-C5 antibody eculizumab prevents post-transplant aHUS recurrence, but it does not cure the disease. Combined...
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