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

Introduction. Fabry's disease is an X-linked error of glycosphingolipid met abolism, Clinical manifestations of the disease are secondary to accumulati on of glycosphingolipids in various tissues. Renal failure and vascular com plications 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 rece ived a renal transplant. Case-matched patients were identified to serve as controls. Results, Patients with Fabry's disease demonstrated equivalent ri-year pati ent 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, patie nts with Fabry's disease have excellent outcomes after renal transplantatio n.