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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
10
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pubmed:dateCreated |
2005-9-26
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pubmed:abstractText |
Leukocyte adhesion deficiency (LAD)-1, a primary immunodeficiency disease caused by molecular defects in the leukocyte integrin CD18 molecule, is characterized by recurrent, life-threatening bacterial infections. Myeloablative hematopoietic stem cell transplantation is the only curative treatment for LAD-1. Recently, canine LAD (CLAD) has been shown to be a valuable animal model for the preclinical testing of nonmyeloablative transplantation regimens for the treatment of children with LAD-1. To develop new allogeneic transplantation approaches for LAD-1, we assessed a nonmyeloablative conditioning regimen consisting of busulfan as a single agent before matched littermate allogeneic bone marrow transplantation in CLAD. Three CLAD dogs received busulfan 10 mg/kg intravenously before infusion of matched littermate bone marrow, and all dogs received posttransplantation immunosuppression with cyclosporin A and mycophenolate mofetil. Initially, all 3 dogs became mixed chimeras, and levels of donor chimerism sufficient to reverse the CLAD phenotype persisted in 2 animals. The third dog maintained donor microchimerism with an attenuated CLAD phenotype. These 3 dogs have all been followed up for at least 1 year after transplantation. These results indicate that a nonmyeloablative conditioning regimen with chemotherapy alone is capable of generating stable mixed chimerism and reversal of the disease phenotype in CLAD.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-10459536,
http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-10465099,
http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-10512685,
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http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-11233907,
http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-11939604,
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http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-14711903,
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http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-8824495,
http://linkedlifedata.com/resource/pubmed/commentcorrection/16182176-8899193
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
1083-8791
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
11
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
755-63
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:16182176-Animals,
pubmed-meshheading:16182176-Bone Marrow Transplantation,
pubmed-meshheading:16182176-Busulfan,
pubmed-meshheading:16182176-Cyclosporine,
pubmed-meshheading:16182176-Dog Diseases,
pubmed-meshheading:16182176-Dogs,
pubmed-meshheading:16182176-Follow-Up Studies,
pubmed-meshheading:16182176-Immunosuppressive Agents,
pubmed-meshheading:16182176-Leukocyte-Adhesion Deficiency Syndrome,
pubmed-meshheading:16182176-Mycophenolic Acid,
pubmed-meshheading:16182176-Phenotype,
pubmed-meshheading:16182176-Transplantation Chimera,
pubmed-meshheading:16182176-Transplantation Conditioning,
pubmed-meshheading:16182176-Treatment Outcome
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pubmed:year |
2005
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pubmed:articleTitle |
Nonmyeloablative conditioning with busulfan before matched littermate bone marrow transplantation results in reversal of the disease phenotype in canine leukocyte adhesion deficiency.
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pubmed:affiliation |
Experimental Transplantation and Immunology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA. sokolicr@mail.nih.gov
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pubmed:publicationType |
Journal Article,
Research Support, N.I.H., Intramural
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