Long-term Outcomes of Kidney Transplantation in Fabry Disease.
Transplantation
; 102(11): 1924-1933, 2018 Nov.
Article
en En
| MEDLINE
| ID: mdl-29688992
ABSTRACT
BACKGROUND:
Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by mutations in the α-galactosidase A gene that obliterate or markedly reduce α-galactosidase A activity. This results in the systemic accumulation of its glycosphingolipid substrates in body fluids and organs, including the kidney. Fabry nephropathy can lead to end-stage renal disease requiring kidney transplantation. Little is known about its long-term outcomes and the overall patient survival after kidney transplantation.METHODS:
Here, we report 17 Fabry patients (15 male and 2 female subjects) who received kidney transplants and their long-term treatment and follow-up at 4 specialized Fabry centers.RESULTS:
The posttransplant follow-up ranged to 25 years, with a median of 11.5 (range, 0.8-25.5] years. Graft survival was similar, and death-censored graft survival was superior to matched controls. Fabry patients died with functioning kidneys, mostly from cardiac causes. In 2 male subjects 14 and 23 years posttransplant, the grafts had a few typical FD lamellar inclusions, presumably originating from invading host macrophages and vascular endothelial cells.CONCLUSIONS:
We conclude that kidney transplantation has an excellent long-term outcome in FD.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Trasplante de Riñón
/
Enfermedad de Fabry
/
Enfermedades Renales
Tipo de estudio:
Clinical_trials
/
Etiology_studies
Límite:
Adolescent
/
Adult
/
Female
/
Humans
/
Male
/
Middle aged
País/Región como asunto:
Europa
Idioma:
En
Revista:
Transplantation
Año:
2018
Tipo del documento:
Article
País de afiliación:
Suiza