Abstract | BACKGROUND: METHODS: We studied two patients with type IV glycogen storage disease 37 and 91 months after liver transplantation and a third patient with lysosomal glucocerebrosidase deficiency (type 1 Gaucher's disease), in whom tissue glucocerebroside deposition had decreased 26 months after liver replacement, to determine whether the migration of cells from the allograft (microchimerism) could explain the improved metabolism of enzyme-deficient tissues in the recipient. Samples of blood and biopsy specimens of the skin, lymph nodes, heart, bone marrow, or intestine were examined immunocytochemically with the use of donor-specific monoclonal anti-HLA antibodies and the polymerase chain reaction, with preliminary amplification specific to donor alleles of the gene for the beta chain of HLA-DR molecules, followed by hybridization with allele-specific oligonucleotide probes. RESULTS: CONCLUSIONS: Systemic microchimerism occurs after liver allotransplantation and can ameliorate pancellular enzyme deficiencies.
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Authors | T E Starzl, A J Demetris, M Trucco, C Ricordi, S Ildstad, P I Terasaki, N Murase, R S Kendall, M Kocova, W A Rudert |
Journal | The New England journal of medicine
(N Engl J Med)
Vol. 328
Issue 11
Pg. 745-9
(Mar 18 1993)
ISSN: 0028-4793 [Print] United States |
PMID | 8437594
(Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- Glucosylceramides
- HLA Antigens
- DNA
- Amylopectin
|
Topics |
- Adult
- Amylopectin
(metabolism)
- Cell Movement
- Chimera
- DNA
(analysis)
- Gaucher Disease
(metabolism, pathology, surgery)
- Glucosylceramides
(metabolism)
- Glycogen Storage Disease Type IV
(metabolism, pathology, surgery)
- HLA Antigens
(analysis)
- Humans
- Immunohistochemistry
- Infant
- Liver
(pathology)
- Liver Transplantation
- Lymph Nodes
(pathology)
- Male
- Myocardium
(pathology)
- Skin
(pathology)
- Transplantation, Homologous
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