Excellent outcome of renal transplantation in patients with Fabry's disease

Transplantation. 2000 Jun 15;69(11):2337-9. doi: 10.1097/00007890-200006150-00020.


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.

MeSH terms

  • Adult
  • Fabry Disease / surgery*
  • Female
  • Graft Survival
  • Humans
  • Kidney Transplantation* / statistics & numerical data
  • Male
  • Middle Aged
  • Registries
  • Survival Analysis
  • Treatment Outcome