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Long-term successful liver-kidney transplantation in a child with atypical hemolytic uremic syndrome caused by homozygous factor H deficiency.

Gonzales, Emmanuel; Ulinski, Tim; Habes, Dalila; Deschênes, Georges; Frémeaux-Bacchi, Véronique; Bensman, Albert.
Pediatr Nephrol; 31(12): 2375-2378, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27744619
BACKGROUND: Rational options for the treatment of end-stage renal disease (ESRD) due to atypical hemolytic uremic syndrome (aHUS) in children are still open to discussion. In the case of human complement factor H (CFH) deficiency, the choice is either kidney transplantation in combination with eculizumab, a humanized anti-C5 monoclonal antibody, or a combined liver-kidney transplantation. CASE-DIAGNOSIS/TREATMENT: A child with a homozygous CFH deficiency underwent a successful liver-kidney transplantation. CFH levels normalized within days. After 6 years of follow-up, the graft function (Cockroft clearance 100 ml min-1 1.73 m-2) and the liver functions were normal. RESULTS AND CONCLUSIONS: The results of this long-term follow-up confirm that combined liver-kidney transplantation remains a reasonable option in patients with ESRD due to aHUS when an identified genetic abnormality of the C3 convertase regulator synthesized in the liver has been identified.
Selo DaSilva