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.
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.
Authors: Matthias Lorenz; Anna-Christina Hauser; Margot Püspök-Schwarz; Peter Kotanko; Ingrid Arias; Herbert Zodl; Reinhard Kramar; Eduard Paschke; Till Voigtländer; Gere Sunder-Plassmann Journal: Wien Klin Wochenschr Date: 2003-04-30 Impact factor: 1.704
Authors: Greg Knoll; Sandra Cockfield; Tom Blydt-Hansen; Dana Baran; Bryce Kiberd; David Landsberg; David Rush; Edward Cole Journal: CMAJ Date: 2005-11-08 Impact factor: 8.262
Authors: A Sessa; M Meroni; G Battini; A Maglio; P L Brambilla; M Bertella; M Nebuloni; F Pallotti; F Giordano; B Bertagnolio; A Tosoni Journal: J Inherit Metab Dis Date: 2001 Impact factor: 4.982