Organ Transplantation in Hereditary Fibrinogen A α-Chain Amyloidosis: A Case Series of French Patients.

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

https://www.ncbi.nlm.nih.gov/pubmed/32660897

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;: