Failure to correct the metabolic defect by renal allotransplantion in Fabry's disease.
Plasma neutral glycolipid levels and plasma and leukocyte alpha-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 alpha-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.
D000795:Fabry Disease / Q000378:metabolism / Q000601:surgery*; D005696:Galactosidases / Q000378:metabolism; D002517:Galactosylgalactosylglucosylceramidase / Q000172:deficiency; D006017:Glycolipids / Q000378:metabolism; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D014184:Transplantation, Homologous
D006017:Glycolipids; D005696:Galactosidases; D002517:Galactosylgalactosylglucosylceramidase