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Excellent outcome of renal transplantation in patients with Fabry's disease
A Ojo1, H U Meier-Kriesche, G Friedman
1University of Michigan Health System, Department of Internal Medicine, Ann Arbor 48109-0364, USA.
Introduction:
Fabry's disease is an X-linked error of glycosphingolipid metabolism. Clinical manifestations of the disease are secondary to accumulation of glycosphingolipids in various tissues. Renal failure and vascular complications are common. There are conflicting reports regarding the outcomes of patients with Fabry's disease after renal transplantation.
Methods:
We reviewed the United States Renal Data System Registry database from 1988 and 1998, and found 93 patients with Fabry's disease who had received a renal transplant. Case-matched patients were identified to serve as controls.
Results:
Patients with Fabry's disease demonstrated equivalent 5-year patient and graft survival, compared with controls (83% and 75%, respectively, for those with Fabry's disease vs. 82% and 67% for controls).
Conclusion:
Despite their high risk for cardiovascular complications, patients with Fabry's disease have excellent outcomes after renal transplantation.
Insights
Patients with Fabry's disease undergoing renal transplantation show comparable survival rates to control groups. This indicates successful outcomes despite the inherent cardiovascular risks associated with Fabry's disease.
Area of Science:
- Nephrology
- Genetics
- Transplantation Medicine
Background:
- Fabry disease is an X-linked genetic disorder of glycosphingolipid metabolism.
- Glycosphingolipid accumulation leads to renal failure and vascular complications.
- Outcomes after renal transplantation in Fabry disease patients are not well-established.
Purpose of the Study:
- To evaluate the outcomes of renal transplantation in patients with Fabry disease.
- To compare patient and graft survival rates in Fabry disease patients versus controls.
Main Methods:
- Retrospective review of the United States Renal Data System Registry (1988-1998).
- Inclusion of 93 patients with Fabry disease who received a renal transplant.
- Case-matched control group for comparison.
Main Results:
- Five-year patient survival was 83% for Fabry disease patients versus 82% for controls.
- Five-year graft survival was 75% for Fabry disease patients versus 67% for controls.
- No significant difference in survival rates between the groups.
Conclusions:
- Renal transplantation offers excellent outcomes for patients with Fabry disease.
- Despite cardiovascular risks, Fabry disease patients achieve comparable graft and patient survival post-transplant.