Ersözlü, Sara; Desnick, Robert J; Huynh-Do, Uyen; Canaan-Kühl, Sima; Barbey, Frédéric; Genitsch, Vera; Mueller, Thomas F; Cheetham, Marcus; Flammer, Andreas J; Schaub, Stefan; Nowak, Albina (2018). Long-term Outcomes of Kidney Transplantation in Fabry Disease. Transplantation, 102(11), pp. 1924-1933. Wolters Kluwer Health 10.1097/TP.0000000000002252
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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.
Item Type: |
Journal Article (Original Article) |
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Division/Institute: |
04 Faculty of Medicine > Service Sector > Institute of Pathology 04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Nephrology and Hypertension 04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiology |
UniBE Contributor: |
Ersözlü, Sara, Huynh-Do, Uyen, Genitsch Gratwohl, Vera |
Subjects: |
500 Science > 570 Life sciences; biology 600 Technology > 610 Medicine & health |
ISSN: |
0041-1337 |
Publisher: |
Wolters Kluwer Health |
Language: |
English |
Submitter: |
Uyen Huynh-Do |
Date Deposited: |
17 Sep 2019 13:40 |
Last Modified: |
05 Dec 2022 15:29 |
Publisher DOI: |
10.1097/TP.0000000000002252 |
PubMed ID: |
29688992 |
BORIS DOI: |
10.7892/boris.131735 |
URI: |
https://boris.unibe.ch/id/eprint/131735 |
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