| Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency. | |
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MedLine Citation:
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PMID: 14525785 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The syndrome of multiple intestinal atresia with immunodeficiency is a rare, invariably fatal congenital disorder. At 16 months of age, a child with this syndrome underwent liver-small bowel transplantation from a 1-of-6 HLA-matched donor. He acquired full enteral tolerance and normal liver function and has never shown evidence of allograft rejection. After mild graft-versus-host disease developed, studies revealed that more than 99% of his CD3(+) lymphocytes and 50% of his CD19(+) lymphocytes were of donor origin, whereas granulocytes and monocytes remained of recipient origin. He synthesizes polyclonal immunoglobulin G (IgG), IgA, and IgM and has developed antibodies to cytomegalovirus (CMV) and parainfluenza 3. His T lymphocytes are predominately CD3(+)CD4(-)CD8(-) with T-cell receptor gammadelta heterodimers and CD3(+)CD4(-)CD8(+) with CD8alphaalpha homodimers, populations consistent with an intraepithelial lymphocyte phenotypic profile. We postulate that he has engrafted a donor intestine-derived immune system and is incapable of rejecting his engrafted organs. |
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Authors:
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Richard K Gilroy; Peter F Coccia; James E Talmadge; Lori I Hatcher; Samuel J Pirruccello; Byers W Shaw; Ronald J Rubocki; Debra L Sudan; Alan N Langnas; Simon P Horslen |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2003-10-02 |
Journal Detail:
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Title: Blood Volume: 103 ISSN: 0006-4971 ISO Abbreviation: Blood Publication Date: 2004 Feb |
Date Detail:
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Created Date: 2004-01-19 Completed Date: 2004-03-10 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: United States |
Other Details:
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Languages: eng Pagination: 1171-4 Citation Subset: AIM; IM |
Affiliation:
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Department of Pediatric Hematology/Oncology, University of Nebraska Medical Center, 982168 Nebraska Medical Center, Omaha, NE 68198-2168, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cytomegalovirus Infections
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etiology Graft Survival Graft vs Host Disease / etiology Humans Immunoglobulins / biosynthesis Immunologic Deficiency Syndromes / immunology, surgery* Infant Intestinal Atresia / immunology, surgery* Intestine, Small / transplantation* Liver Transplantation* Lymphocyte Subsets / immunology Male Tissue Donors |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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