|
|
|
|
| LEADER |
01000naa a22002652 4500 |
| 001 |
NLM185003524 |
| 003 |
DE-627 |
| 005 |
20231223171636.0 |
| 007 |
cr uuu---uuuuu |
| 008 |
231223s2009 xx |||||o 00| ||eng c |
| 024 |
7 |
|
|a 10.1016/j.clim.2008.09.018
|2 doi
|
| 028 |
5 |
2 |
|a pubmed24n0617.xml
|
| 035 |
|
|
|a (DE-627)NLM185003524
|
| 035 |
|
|
|a (NLM)19064334
|
| 040 |
|
|
|a DE-627
|b ger
|c DE-627
|e rakwb
|
| 041 |
|
|
|a eng
|
| 100 |
1 |
|
|a Schönberger, Stefan
|e verfasserin
|4 aut
|
| 245 |
1 |
0 |
|a Saving the red baby
|b successful allogeneic cord blood transplantation in Omenn syndrome
|
| 264 |
|
1 |
|c 2009
|
| 336 |
|
|
|a Text
|b txt
|2 rdacontent
|
| 337 |
|
|
|a ƒaComputermedien
|b c
|2 rdamedia
|
| 338 |
|
|
|a ƒa Online-Ressource
|b cr
|2 rdacarrier
|
| 500 |
|
|
|a Date Completed 03.03.2009
|
| 500 |
|
|
|a Date Revised 09.02.2009
|
| 500 |
|
|
|a published: Print-Electronic
|
| 500 |
|
|
|a Citation Status MEDLINE
|
| 520 |
|
|
|a Haematopoietic stem cell transplantation is the treatment of choice for severe primary immunodeficiencies, but only has moderate prognosis in Omenn syndrome as it is complicated by highly activated Omenn T-cells resulting in delayed T-cell engraftment and a high rate of graft failure. A 6 1/2 months old patient with a previously unknown compound heterozygous defect within the RAG1 gene (R474C; R975W) underwent 8/10 HLA-matched cord blood transplantation after myeloablative conditioning. Immune reconstitution was impressive with T-, B- and NK-cells reaching the median of age-dependent reference values within twelve, four and two months respectively. With a continuous decrease of activated Omenn T-cells there was a steady increase of naive, probably thymus-derived T-cells. Polyclonal B-cell activation and hypergammaglobulinaemia disappeared with B-cell engraftment. This case emphasizes that, despite their naive status and HLA-barriers, cord blood T-cells were apparently able to achieve T-effector function resulting in the elimination of all activated Omenn T-cells
|
| 650 |
|
4 |
|a Case Reports
|
| 650 |
|
4 |
|a Journal Article
|
| 700 |
1 |
|
|a Ott, Hagen
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Gudowius, Sonja
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Wüller, Stefan
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Baron, Jens Malte
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Merk, Hans-Friedrich
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Lassay, Lisa
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Megahed, Mosaad
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Feyen, Oliver
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Laws, Hans-Jürgen
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Dilloo, Dagmar
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Borkhardt, Arndt
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Niehues, Tim
|e verfasserin
|4 aut
|
| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 130(2009), 3 vom: 15. März, Seite 259-63
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
|
| 773 |
1 |
8 |
|g volume:130
|g year:2009
|g number:3
|g day:15
|g month:03
|g pages:259-63
|
| 856 |
4 |
0 |
|u http://dx.doi.org/10.1016/j.clim.2008.09.018
|3 Volltext
|
| 912 |
|
|
|a GBV_USEFLAG_A
|
| 912 |
|
|
|a SYSFLAG_A
|
| 912 |
|
|
|a GBV_NLM
|
| 912 |
|
|
|a GBV_ILN_11
|
| 912 |
|
|
|a GBV_ILN_24
|
| 912 |
|
|
|a GBV_ILN_350
|
| 951 |
|
|
|a AR
|
| 952 |
|
|
|d 130
|j 2009
|e 3
|b 15
|c 03
|h 259-63
|