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231223s2008 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2008.03.524
|2 doi
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|a pubmed25n0600.xml
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|e rakwb
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|a eng
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| 100 |
1 |
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|a Daguindau, Nicolas
|e verfasserin
|4 aut
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| 245 |
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|a Immune constitution monitoring after PBMC transplantation in complete DiGeorge syndrome
|b an eight-year follow-up
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| 264 |
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|c 2008
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| 336 |
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
|b c
|2 rdamedia
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| 338 |
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|a ƒa Online-Ressource
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|2 rdacarrier
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| 500 |
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|a Date Completed 03.09.2008
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|a Date Revised 15.07.2008
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a A young boy with a confirmed complete DiGeorge Syndrome (cDGS) underwent a peripheral blood mononuclear cell transplantation (PBMCT) from his HLA-identical sister at 4.5 years of age, without a conditioning regimen. Eight years later, he is healthy with good immunological functions in the presence of a stable mixed T-cell chimerism. Absence of recent thymic emigrants is confirmed. We observe an inverted CD4+/CD8+ ratio, related to the CD8 subset expansion, a skewing of the TCR repertoire, especially on the CD8+ subset and a telomere loss on the CD8+ cells compared to the donor. However, these anomalies do not seem to have an impact on functional immunity. PBMCT in cDGS using an HLA-matched sibling donor provides good long-lasting immunity and is an easy alternative to bone marrow transplantation and to thymic transplantation
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| 650 |
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|a Case Reports
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|a Journal Article
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|2 NLM
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|a DNA
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|a 9007-49-2
|2 NLM
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| 700 |
1 |
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|a Decot, Véronique
|e verfasserin
|4 aut
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| 700 |
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|a Nzietchueng, Rosine
|e verfasserin
|4 aut
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| 700 |
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|a Ferrand, Christophe
|e verfasserin
|4 aut
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| 700 |
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|a Picard, Capucine
|e verfasserin
|4 aut
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| 700 |
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|a Latger-Cannard, Véronique
|e verfasserin
|4 aut
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| 700 |
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|a Gregoire, Marie José
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Beri, Mylène
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Salmon, Alexandra
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Stoltz, Jean François
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Bordigoni, Pierre
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Bensoussan, Danièle
|e verfasserin
|4 aut
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| 773 |
0 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 128(2008), 2 vom: 15. Aug., Seite 164-71
|w (DE-627)NLM098196855
|x 1521-7035
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|g volume:128
|g year:2008
|g number:2
|g day:15
|g month:08
|g pages:164-71
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|u http://dx.doi.org/10.1016/j.clim.2008.03.524
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