Source:http://linkedlifedata.com/resource/pubmed/id/14525761
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
3
|
| pubmed:dateCreated |
2004-1-19
|
| pubmed:abstractText |
CD40 ligand (CD40L) deficiency causes recurrent sinopulmonary infection, Pneumocystis carinii pneumonia, and Cryptosporidium parvum infection. Approximately 40% to 50% of patients survive to the third decade: long-term survival is unclear. Hematopoietic stem cell transplantation (HSCT) is curative. We present a retrospective analysis of 38 European patients undergoing HSCT for CD40L deficiency in 8 European countries between 1993 and 2002. Donor stem cell source included 14 HLA-identical siblings, 22 unrelated donors, and 2 phenotypically matched parental stem cells (12 T-cell depleted). Of the patients, 34 engrafted and 26 (68%) survived; 3 had autologous reconstitution, 22 (58%) were cured, and 1 engrafted but has poor T-cell immune reconstitution. There were 18 evaluated patients who responded to vaccination. Of the patients, 12 (32%) died from infection-related complications, with severe cryptosporidiosis in 6. Grades 2 to 4 graft-versus-host disease (GvHD) associated with infection occurred in 6 of 12 fatal cases. HSCT cured 58% of patients, 72% of those without hepatic disease. Early T-cell function following whole marrow HSCT may limit cryptosporidial disease, but survival was similar after T-cell-depleted HSCT. Preexisting lung damage was the most important adverse risk factor. Further studies will determine optimal timing and type of HSCT.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
AIM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Feb
|
| pubmed:issn |
0006-4971
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| pubmed:author |
pubmed-author:AndolinaMarinoM,
pubmed-author:BensoussanDanielleD,
pubmed-author:BordigoniPierreP,
pubmed-author:BrediusRobbert G MRG,
pubmed-author:CantAndrew JAJ,
pubmed-author:Cavazzana-CalvoMarinaM,
pubmed-author:DaviesE GrahamEG,
pubmed-author:FasthAndersA,
pubmed-author:FischerAlainA,
pubmed-author:FriedrichWilhelmW,
pubmed-author:GenneryAndrew RAR,
pubmed-author:KhawajaKhuloodK,
pubmed-author:LandaisPaulP,
pubmed-author:LangeAndrzejA,
pubmed-author:Matthes-MartinSusanneS,
pubmed-author:MazzolariEvelinaE,
pubmed-author:NotarangeloLuigi DLD,
pubmed-author:PagliucaAntonioA,
pubmed-author:VeysPaulP,
pubmed-author:WulffraatNico MNM
|
| pubmed:issnType |
Print
|
| pubmed:day |
1
|
| pubmed:volume |
103
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
1152-7
|
| pubmed:dateRevised |
2004-11-17
|
| pubmed:meshHeading |
pubmed-meshheading:14525761-Adolescent,
pubmed-meshheading:14525761-Adult,
pubmed-meshheading:14525761-CD40 Ligand,
pubmed-meshheading:14525761-Child,
pubmed-meshheading:14525761-Child, Preschool,
pubmed-meshheading:14525761-Data Collection,
pubmed-meshheading:14525761-Europe,
pubmed-meshheading:14525761-Genetic Diseases, X-Linked,
pubmed-meshheading:14525761-Graft Survival,
pubmed-meshheading:14525761-Graft vs Host Disease,
pubmed-meshheading:14525761-Hematopoietic Stem Cell Transplantation,
pubmed-meshheading:14525761-Humans,
pubmed-meshheading:14525761-Hypergammaglobulinemia,
pubmed-meshheading:14525761-Immunoglobulin M,
pubmed-meshheading:14525761-Infant,
pubmed-meshheading:14525761-Opportunistic Infections,
pubmed-meshheading:14525761-Retrospective Studies
|
| pubmed:year |
2004
|
| pubmed:articleTitle |
Treatment of CD40 ligand deficiency by hematopoietic stem cell transplantation: a survey of the European experience, 1993-2002.
|
| pubmed:affiliation |
Newcastle General Hospital, Westgate Rd, Newcastle upon Tyne, NE4 6BE United Kingdom. a.r.gennery@ncl.ac.uk
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| pubmed:publicationType |
Journal Article
|