EXCELLENT OUTCOME OF RENAL TRANSPLANTATION IN PATIENTS WITH FABRY???S DISEASE

Authors:
  • HMHerwig-Ulf Meier-Kriesche
    Herwig-Ulf Meier-Kriesche
  • GFGary Friedman
    Gary Friedman
  • JHJulie Hanson
    Julie Hanson
  • DCDiane Cibrik
    Diane Cibrik
  • ALAlan Leichtman
    Alan Leichtman
  • BKBruce Kaplan
    Bruce Kaplan
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Abstract
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. 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. 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). Despite their high risk for cardiovascular complications, patients with Fabry’s disease have excellent outcomes after renal transplantation.

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