Article
Prophylactic eculizumab prior to kidney transplantation for atypical hemolytic uremic syndrome.
Pediatric nephrology (Berlin, Germany) - 1 Aug 2011
Weitz Marcus, Amon Oliver, Bassler Dirk, Koenigsrainer Alfred, Nadalin Silvio
Abstract excerpt
Atypical hemolytic uremic syndrome (aHUS) in childhood is a rare disease associated with high morbidity and mortality. Most cases progress to end-stage renal failure. In approximately 50% of affected patients, mutations in genes encoding complement proteins are causative of the impairment in the regulation of the complement alternative pathway. This leads to deficient host cell protection and inappropriate...
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