| pubmed-article:14525761 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:14525761 | lifeskim:mentions | umls-concept:C0239307 | lld:lifeskim |
| pubmed-article:14525761 | lifeskim:mentions | umls-concept:C0087111 | lld:lifeskim |
| pubmed-article:14525761 | lifeskim:mentions | umls-concept:C0472699 | lld:lifeskim |
| pubmed-article:14525761 | lifeskim:mentions | umls-concept:C2348037 | lld:lifeskim |
| pubmed-article:14525761 | lifeskim:mentions | umls-concept:C0596545 | lld:lifeskim |
| pubmed-article:14525761 | lifeskim:mentions | umls-concept:C0038951 | lld:lifeskim |
| pubmed-article:14525761 | pubmed:issue | 3 | lld:pubmed |
| pubmed-article:14525761 | pubmed:dateCreated | 2004-1-19 | lld:pubmed |
| pubmed-article:14525761 | 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. | lld:pubmed |
| pubmed-article:14525761 | pubmed:language | eng | lld:pubmed |
| pubmed-article:14525761 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:14525761 | pubmed:citationSubset | AIM | lld:pubmed |
| pubmed-article:14525761 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:14525761 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:14525761 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:14525761 | pubmed:month | Feb | lld:pubmed |
| pubmed-article:14525761 | pubmed:issn | 0006-4971 | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:FischerAlainA | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:FasthAndersA | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:NotarangeloLu... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:LandaisPaulP | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:Cavazzana-Cal... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:BensoussanDan... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:CantAndrew... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:PagliucaAnton... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:BordigoniPier... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:DaviesE... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:FriedrichWilh... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:VeysPaulP | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:LangeAndrzejA | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:MazzolariEvel... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:BrediusRobber... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:Matthes-Marti... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:WulffraatNico... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:AndolinaMarin... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:GenneryAndrew... | lld:pubmed |
| pubmed-article:14525761 | pubmed:author | pubmed-author:KhawajaKhuloo... | lld:pubmed |
| pubmed-article:14525761 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:14525761 | pubmed:day | 1 | lld:pubmed |
| pubmed-article:14525761 | pubmed:volume | 103 | lld:pubmed |
| pubmed-article:14525761 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:14525761 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:14525761 | pubmed:pagination | 1152-7 | lld:pubmed |
| pubmed-article:14525761 | pubmed:dateRevised | 2004-11-17 | lld:pubmed |
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| pubmed-article:14525761 | pubmed:meshHeading | pubmed-meshheading:14525761... | lld:pubmed |
| pubmed-article:14525761 | pubmed:meshHeading | pubmed-meshheading:14525761... | lld:pubmed |
| pubmed-article:14525761 | pubmed:meshHeading | pubmed-meshheading:14525761... | lld:pubmed |
| pubmed-article:14525761 | pubmed:year | 2004 | lld:pubmed |
| pubmed-article:14525761 | pubmed:articleTitle | Treatment of CD40 ligand deficiency by hematopoietic stem cell transplantation: a survey of the European experience, 1993-2002. | lld:pubmed |
| pubmed-article:14525761 | pubmed:affiliation | Newcastle General Hospital, Westgate Rd, Newcastle upon Tyne, NE4 6BE United Kingdom. a.r.gennery@ncl.ac.uk | lld:pubmed |
| pubmed-article:14525761 | pubmed:publicationType | Journal Article | lld:pubmed |
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| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:14525761 | lld:pubmed |
