pubmed-article:16904870 | rdf:type | pubmed:Citation | lld:pubmed |
pubmed-article:16904870 | lifeskim:mentions | umls-concept:C0008312 | lld:lifeskim |
pubmed-article:16904870 | lifeskim:mentions | umls-concept:C0034819 | lld:lifeskim |
pubmed-article:16904870 | lifeskim:mentions | umls-concept:C0011155 | lld:lifeskim |
pubmed-article:16904870 | lifeskim:mentions | umls-concept:C2003941 | lld:lifeskim |
pubmed-article:16904870 | lifeskim:mentions | umls-concept:C2348519 | lld:lifeskim |
pubmed-article:16904870 | pubmed:issue | 1 | lld:pubmed |
pubmed-article:16904870 | pubmed:dateCreated | 2006-8-28 | lld:pubmed |
pubmed-article:16904870 | pubmed:abstractText | Congenital immune deficiency states have often been valuable experimental models of nature that have significantly enhanced our understanding of the immune response. The relationship between CD4+, CD25+ and Treg cells in the induction of autoimmunity has attracted significant attention. We report herein a male child of consanguineous parents who developed at six months recurrent infections, and at age 5 years, liver dysfunction with serological expression of primary biliary cirrhosis (PBC), an autoimmune liver disease that usually affects middle-aged women. Histologically, there was intense mononuclear cell lymphoid infiltration of the intrahepatic portal tracts, CD3+CD4+T cell lymphopenia in blood and serum antibody to PDC-E2. Peripheral blood lymphocytes were completely deficient of the alpha subunit of the IL-2 receptor (IL-2Ralpha, CD 25), a marker for regulatory T cells (Tregs). Allogenic stem cell transplantation led to full recovery. This case illustrates the role of deficiency of CD4+CD25+ Treg cells in causing autoimmunity, and speaks to the potential use of allogenic stem cell transplantation for immunoreconstitution in adult PBC. | lld:pubmed |
pubmed-article:16904870 | pubmed:language | eng | lld:pubmed |
pubmed-article:16904870 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:16904870 | pubmed:citationSubset | IM | lld:pubmed |
pubmed-article:16904870 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:16904870 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:16904870 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:16904870 | pubmed:status | MEDLINE | lld:pubmed |
pubmed-article:16904870 | pubmed:month | Aug | lld:pubmed |
pubmed-article:16904870 | pubmed:issn | 0896-8411 | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:RoifmanChaim... | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:GershwinM... | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:LianZhe-Xiong... | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:ShoenfeldYehu... | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:MackayIan RIR | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:BowlusChristo... | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:NormanGary... | lld:pubmed |
pubmed-article:16904870 | pubmed:author | pubmed-author:AokiChristoph... | lld:pubmed |
pubmed-article:16904870 | pubmed:issnType | Print | lld:pubmed |
pubmed-article:16904870 | pubmed:volume | 27 | lld:pubmed |
pubmed-article:16904870 | pubmed:owner | NLM | lld:pubmed |
pubmed-article:16904870 | pubmed:authorsComplete | Y | lld:pubmed |
pubmed-article:16904870 | pubmed:pagination | 50-3 | lld:pubmed |
pubmed-article:16904870 | pubmed:dateRevised | 2007-1-8 | lld:pubmed |
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pubmed-article:16904870 | pubmed:meshHeading | pubmed-meshheading:16904870... | lld:pubmed |
pubmed-article:16904870 | pubmed:year | 2006 | lld:pubmed |
pubmed-article:16904870 | pubmed:articleTitle | IL-2 receptor alpha deficiency and features of primary biliary cirrhosis. | lld:pubmed |
pubmed-article:16904870 | pubmed:affiliation | Division of Gastroenterology, University of California at Davis, 95616, USA. | lld:pubmed |
pubmed-article:16904870 | pubmed:publicationType | Journal Article | lld:pubmed |
pubmed-article:16904870 | pubmed:publicationType | Case Reports | lld:pubmed |
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