| pubmed-article:20978268 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C0087111 | lld:lifeskim |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C0085110 | lld:lifeskim |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C0684224 | lld:lifeskim |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C0194035 | lld:lifeskim |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C0011155 | lld:lifeskim |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C1421504 | lld:lifeskim |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C0868928 | lld:lifeskim |
| pubmed-article:20978268 | lifeskim:mentions | umls-concept:C1524063 | lld:lifeskim |
| pubmed-article:20978268 | pubmed:issue | 2 | lld:pubmed |
| pubmed-article:20978268 | pubmed:dateCreated | 2011-1-14 | lld:pubmed |
| pubmed-article:20978268 | pubmed:abstractText | FOXN1 deficiency is a primary immunodeficiency characterized by athymia, alopecia totalis, and nail dystrophy. Two infants with FOXN1 deficiency were transplanted with cultured postnatal thymus tissue. Subject 1 presented with disseminated Bacillus Calmette-Guérin infection and oligoclonal T cells with no naive markers. Subject 2 had respiratory failure, human herpes virus 6 infection, cytopenias, and no circulating T cells. The subjects were given thymus transplants at 14 and 9 months of life, respectively. Subject 1 received immunosuppression before and for 10 months after transplantation. With follow up of 4.9 and 2.9 years, subjects 1 and 2 are well without infectious complications. The pretransplantation mycobacterial disease in subject 1 and cytopenias in subject 2 resolved. Subject 2 developed autoimmune thyroid disease 1.6 years after transplantation. Both subjects developed functional immunity. Subjects 1 and 2 have 1053/mm(3) and 1232/mm(3) CD3(+) cells, 647/mm(3) and 868/mm(3) CD4(+) T cells, 213/mm(3) and 425/mm(3) naive CD4(+) T cells, and 10 200 and 5700 T-cell receptor rearrangement excision circles per 100 000 CD3(+) cells, respectively. They have normal CD4 T-cell receptor ? variable repertoires. Both subjects developed antigen-specific proliferative responses and have discontinued immunoglobulin replacement. In summary, thymus transplantation led to T-cell reconstitution and function in these FOXN1 deficient infants. | lld:pubmed |
| pubmed-article:20978268 | pubmed:grant | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20978268 | pubmed:grant | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20978268 | pubmed:grant | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20978268 | pubmed:language | eng | lld:pubmed |
| pubmed-article:20978268 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20978268 | pubmed:citationSubset | AIM | lld:pubmed |
| pubmed-article:20978268 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20978268 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20978268 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:20978268 | pubmed:month | Jan | lld:pubmed |
| pubmed-article:20978268 | pubmed:issn | 1528-0020 | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:FischerAlainA | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:PicardCapucin... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:SousaAna EAE | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:PignataClaudi... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:NevenBénédict... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:de Saint... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:MarkertM... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:McCarthyEliza... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:DevlinBlythe... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:MahlaouiNizar... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:DebreMarianne... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:ChinnIvan KIK | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:AlbuquerqueAd... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:MarquesJosé... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:SilvaSusana... | lld:pubmed |
| pubmed-article:20978268 | pubmed:author | pubmed-author:VictorinoRui... | lld:pubmed |
| pubmed-article:20978268 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:20978268 | pubmed:day | 13 | lld:pubmed |
| pubmed-article:20978268 | pubmed:volume | 117 | lld:pubmed |
| pubmed-article:20978268 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:20978268 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:20978268 | pubmed:pagination | 688-96 | lld:pubmed |
| pubmed-article:20978268 | pubmed:dateRevised | 2011-3-4 | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:meshHeading | pubmed-meshheading:20978268... | lld:pubmed |
| pubmed-article:20978268 | pubmed:year | 2011 | lld:pubmed |
| pubmed-article:20978268 | pubmed:articleTitle | First use of thymus transplantation therapy for FOXN1 deficiency (nude/SCID): a report of 2 cases. | lld:pubmed |
| pubmed-article:20978268 | pubmed:affiliation | Department of Pediatrics, Duke University Medical Center, Durham, NC, USA. marke001@mc.duke.edu | lld:pubmed |
| pubmed-article:20978268 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:20978268 | pubmed:publicationType | Clinical Trial | lld:pubmed |
| pubmed-article:20978268 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| pubmed-article:20978268 | pubmed:publicationType | Research Support, N.I.H., Extramural | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:20978268 | lld:pubmed |
