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); ALAN S. MacDONALD, M.D., C.M., F.R.C.S.(C)
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.
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SPENCE MW, MacKINNON KE, BURGESS JK, d'ENTREMONT DM, BELITSKY P, LANNON SG, et al. Failure to Correct the Metabolic Defect by Renal Allotransplantation in Fabry's Disease. Ann Intern Med. 1976;84:13–16. doi: 10.7326/0003-4819-84-1-13
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Published: Ann Intern Med. 1976;84(1):13-16.
Encephalopathy, Nephrology, Neurology.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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