RSS Feeds
Failure to Correct the Metabolic Defect by Renal Allotransplantation in Fabry's Disease
- MATTHEW W. SPENCE, M.D., Ph.D.;
- KAREN E. MacKINNON, B.SC.;
- JOHN K. BURGESS, B.SC.;
- DOREEN M. d'ENTREMONT, R.N., B.N.;
- PHILIP BELITSKY, M.D., C.M., F.R.C.S.(C);
- STAN G. LANNON, M.D., C.M., F.R.C.S.(C); and
- ALAN S. MacDONALD, M.D., C.M., F.R.C.S.(C)
Abstract
Plasma neutral glycolipid levels and plasma and leukocyte α-galactosidase activities were measured serially before and after renal allotransplantation in two men, aged 47 and 45 years, with renal failure due to Fabry's disease. The patients were followed posttransplantation for 92 and 64 weeks, respectively. No significant elevation of plasma or leukocyte α-galactosidase activities above levels in untreated men with Fabry's disease or decrease in the levels of trihexosyl ceramide was observed in either patient. The results do not support the use of renal allotransplantation for enzyme replacement in Fabry's disease.
Article and Author Information
-
▸From the Departments of Pediatrics, Biochemistry, Urology, and Surgery, Dalhousie University; and the Atlantic Research Centre for Mental Retardation; Halifax, Nova Scotia, Canada.
-
Grant support: by Health and Welfare Canada and the Medical Research Council of Canada. Doctor M. W. Spence is an Associate of the Medical Research Council of Canada.
-
▸Requests for reprints should be addressed to M. W. Spence, M.D., Ph.D., Department of Pediatrics, Sir Charles Tupper Medical Building, Dalhousie University, Halifax, Nova Scotia, Canada, B3H, 4H7.
-
- Received June 2, 1975.
- Accepted October 3, 1975.
