학술논문
Organ Transplantation in Hereditary Fibrinogen A α-Chain Amyloidosis: A Case Series of French Patients
Document Type
Article
Author
Meyer, Lara; Ulrich, Marc; Ducloux, Didier; Garrigue, Valérie; Vigneau, Cécile; Nochy, Dominique; Bobrie, Guillaume; Ferlicot, Sophie; Colombat, Magalie; Boffa, Jean-Jacques; Clabault, Karine; Mansour, Jeannette; Mousson, Christiane; Azar, Raymond; Bacri, Jean-Louis; Dürrbach, Antoine; Duvic, Christian; El Karoui, Khalil; Hoffmann, Maxime; Lionet, Arnaud; Panescu, Victor; Plaisier, Emmanuelle; Ratsimbazafy, Anderson; Guerrot, Dominique; Vrigneaud, Laurence; Valleix, Sophie; François, Hélène
Source
American Journal of Kidney Diseases; September 2020, Vol. 76 Issue: 3 p384-391, 8p
Subject
Language
ISSN
02726386; 15236838
Abstract
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 FGAvariants.