Literature DB >> 812404

Failure to correct the metabolic defect by renal allotransplantion in Fabry's disease.

M W Spence, K E MacKinnon, J K Burgess, D M d'Entremont, P Belitsky, S G Lannon, A S MacDonald.   

Abstract

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.

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Year:  1976        PMID： 812404     DOI： 10.7326/0003-4819-84-1-13

Source DB:  PubMed          Journal:  Ann Intern Med        ISSN： 0003-4819            Impact factor:   25.391


  5 in total

1.  Fabry disease in a large Nova Scotia kindred: carrier detection using leucocyte alpha-galactosidase activity and an NcoI polymorphism detected by an alpha-galactosidase cDNA clone.

Authors:  A J Kirkilionis; D C Riddell; M W Spence; R G Fenwick
Journal:  J Med Genet       Date:  1991-04       Impact factor: 6.318

2.  Angiokeratoma corporis diffusum (Anderson-Fabry disease) in a single large family in Nova Scotia.

Authors:  M W Spence; J T Clarke; D M D'Entremont; G A Sapp; E R Smith; A L Goldbloom; G Davar
Journal:  J Med Genet       Date:  1978-12       Impact factor: 6.318

3.  Proceedings of the Annual Meeting of the European Foundation for Bone Marrow Transplantation, Sils Maria (Engadine), Switzerland, April 13-16, 1980.

Authors: 
Journal:  Blut       Date:  1980-09

4.  Light- and electron-microscopic histochemistry of Fabry's disease.

Authors:  T Faraggiana; J Churg; E Grishman; L Strauss; A Prado; D F Bishop; E Schuchman; R J Desnick
Journal:  Am J Pathol       Date:  1981-05       Impact factor: 4.307

5.  Renal transplantation in Anderson-Fabry disease.

Authors:  M Clement; R J McGonigle; P M Monkhouse; A M Keogh; R H Marten; M Bewick; V Parsons
Journal:  J R Soc Med       Date:  1982-07       Impact factor: 18.000
  5 in total

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