Kidney transplantation improves survival and is indicated in Fabry's disease.

Details

Serval ID
serval:BIB_9CE3A1E15E18
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Kidney transplantation improves survival and is indicated in Fabry's disease.
Journal
Transplantation Proceedings
Author(s)
Inderbitzin D., Avital I., Largiadèr F., Vogt B., Candinas D.
ISSN
0041-1345 (Print)
ISSN-L
0041-1345
Publication state
Published
Issued date
2005
Volume
37
Number
10
Pages
4211-4214
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
BACKGROUND: Fabry's disease (FD) is an inborn error of glycosphingolipid catabolism with progressive systemic deposition of globotriaosylceramide thereby leading to renal and cardiac failure. Current therapy involves symptomatic medical management, dialysis, enzyme replacement therapy, kidney transplantation (KTx), and more recently gene therapy. Case fatalities occur in the fourth decade of life resulting from uremia unless dialysis or KTx is undertaken.
STUDY DESIGN: This is a retrospective study aimed at determining the effect of KTx on the long-term outcome of patients with FD.
RESULTS: Between 1964 and 1998, ten patients with FD received KTx at our institutions. Actuarial patient and graft survivals were 100% and 90% at 5 years; 76% and 66% at 10 years. One kidney graft was lost due to rejection. Patient survival data compared favorably at 5 years with survival of FD patients on hemodialysis alone (41%, P < .05). Five patients are alive at the time of this study, and five patients died with median survival time after KTx of 128 months (range: 74-160 months).
CONCLUSIONS: This study demonstrates an excellent outcome in patients with FD in the first decade after KTx. In the absence of a severe contraindication, we advocate KTx to improve the overall prognosis of patients with renal failure due to FD. Based on the data, enzyme replacement therapy after KTx seems indicated, as FD progresses posttransplant, leading to case fatalities in the second decade after KTx.
Keywords
Adolescent, Adult, Fabry Disease/mortality, Fabry Disease/surgery, Follow-Up Studies, Graft Survival, Humans, Kidney Transplantation/mortality, Kidney Transplantation/physiology, Middle Aged, Retrospective Studies, Survival Analysis, Survival Rate, Time Factors, Treatment Outcome
Pubmed
Web of science
Create date
25/01/2008 13:03
Last modification date
20/08/2019 15:03
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