Organ Transplantation in Hereditary Fibrinogen A α-Chain Amyloidosis: A Case Series of French Patients.
Fiche publication
Date publication
juillet 2020
Journal
American journal of kidney diseases : the official journal of the National Kidney Foundation
Auteurs
Membres identifiés du Cancéropôle Est :
Pr DUCLOUX Didier
Tous les auteurs :
Meyer L, Ulrich M, Ducloux D, Garrigue V, Vigneau C, Nochy D, Bobrie G, Ferlicot S, Colombat M, Boffa JJ, Clabault K, Mansour J, Mousson C, Azar R, Bacri JL, Dürrbach A, Duvic C, El Karoui K, Hoffmann M, Lionet A, Panescu V, Plaisier E, Ratsimbazafy A, Guerrot D, Vrigneaud L, Valleix S, François H
Lien Pubmed
Résumé
Fibrinogen A α-chain amyloidosis (AFib amyloidosis) is a form of amyloidosis resulting from mutations in the fibrinogen A α-chain gene (FGA), causing progressive kidney disease leading to kidney failure. Treatment may include kidney transplantation (KT) or liver-kidney transplantation (LKT), but it is not clear what factors should guide this decision. The aim of this study was to characterize the natural history and long-term outcomes of this disease, with and without organ transplantation, among patients with AFib amyloidosis and various FGA variants.
Mots clés
Amyloidosis, FGA, amyloid recurrence, case series, end-stage renal disease (ESRD), fibrinogen, genotype-phenotype correlation, hereditary disease, kidney biopsy, liver-kidney transplantation
Référence
Am. J. Kidney Dis.. 2020 Jul 10;: