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dc.contributor.authorNaess, Sigrid
dc.contributor.authorBjörnsson, Einar
dc.contributor.authorAnmarkrud, Jarl A
dc.contributor.authorAl Mamari, Said
dc.contributor.authorJuran, Brian D
dc.contributor.authorLazaridis, Konstantinos N
dc.contributor.authorChapman, Roger
dc.contributor.authorBergquist, Annika
dc.contributor.authorMelum, Espen
dc.contributor.authorMarsh, Steven G E
dc.contributor.authorSchrumpf, Erik
dc.contributor.authorLie, Benedicte A
dc.contributor.authorBoberg, Kirsten M
dc.contributor.authorKarlsen, Tom H
dc.contributor.authorHov, Johannes R
dc.date.accessioned2015-05-07T13:15:43Zen
dc.date.available2015-05-07T13:15:43Zen
dc.date.issued2014-11en
dc.date.submitted2015en
dc.identifier.citationLiver Int. 2014, 34 (10):1488-95en
dc.identifier.issn1478-3231en
dc.identifier.pmid24517468en
dc.identifier.doi10.1111/liv.12492en
dc.identifier.urihttp://hdl.handle.net/2336/552436en
dc.descriptionTo access publisher's full text version of this article click on the hyperlink at the bottom of the pageen
dc.description.abstractSmall duct primary sclerosing cholangitis (PSC) is phenotypically a mild version of large duct PSC, but it is unknown whether these phenotypes share aetiology. We aimed to characterize their relationship by investigating genetic associations in the human leucocyte antigen (HLA) complex, which represent the strongest genetic risk factors in large duct PSC.
dc.description.abstractFour classical HLA loci (HLA-A, HLA-B, HLA-C and HLA-DRB1) were genotyped in 87 small duct PSC patients, 485 large duct PSC patients and 1117 controls across three geographical regions.
dc.description.abstractHLA-DRB1*13:01 (OR = 2.0, 95% CI 1.2-3.4, P = 0.01) and HLA-B*08 (OR = 1.6, 95% CI 1.1-2.4, P = 0.02) were significantly associated with small duct PSC compared with healthy controls. Based on the observed frequency of HLA-B*08 in small duct PSC, the strongest risk factor in large duct PSC, an estimated 32% (95% CI 4-65%) of this population can be hypothesized to represent early stages or mild variants of large duct PSC. This subgroup may be constituted by small duct PSC patients with inflammatory bowel disease (IBD), which greatly resembled large duct PSC in its HLA association. In contrast, small duct PSC without IBD was only associated with HLA-DRB1*13:01(P = 0.03) and was otherwise distinctly dissimilar from large duct PSC.
dc.description.abstractSmall duct PSC with IBD resembles large duct PSC in its HLA association and may represent early stages or mild variants of large duct disease. Different HLA associations in small duct PSC without IBD could indicate that this subgroup is a different entity. HLA-DRB1*13:01 may represent a specific risk factor for inflammatory bile duct disease.
dc.description.sponsorshipNational Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institute of Allergy and Infectious Diseases (NIAID) National Human Genome Research Institute (NHGRI) National Institute of Child Health and Human Development (NICHD) Juvenile Diabetes Research Foundation International (JDRF) NIH DK084960 Norwegian PSC Research Center / U01 DK062418en
dc.language.isoenen
dc.publisherWiley-Blackwellen
dc.relation.urlhttp://dx.doi.org/ 10.1111/liv.12492en
dc.relation.urlhttp://onlinelibrary.wiley.com/doi/10.1111/liv.12492/epdfen
dc.rightsArchived with thanks to Liver international : official journal of the International Association for the Study of the Liveren
dc.subject.meshCholangitis, Sclerosingen
dc.subject.meshInflammatory Bowel Diseasesen
dc.subject.meshLeukocytesen
dc.titleSmall duct primary sclerosing cholangitis without inflammatory bowel disease is genetically different from large duct disease.en
dc.typeArticleen
dc.contributor.department[ 1 ] Oslo Univ Hosp, Rikshosp, Div Canc Med Surg & Transplantat, Norwegian PSC Res Ctr,Dept Transplantat Med, N-0424 Oslo, Norway [ 2 ] Univ Oslo, Inst Clin Med, Oslo, Norway [ 3 ] Oslo Univ Hosp, Rikshosp, Internal Med Res Inst, N-0424 Oslo, Norway [ 4 ] Landspitali Univ Hosp, Div Gastroenterol & Hepatol, Dept Internal Med, Reykjavik, Iceland [ 5 ] Oxford Univ Hosp, Transit Gastroenterol Unit, Oxford, England [ 6 ] Sultan Qaboos Hosp, Liver Unit, Salalah, Oman [ 7 ] Mayo Clin, Coll Med, Ctr Basic Res Digest Dis, Div Gastroenterol & Hepatol, Rochester, MN USA [ 8 ] John Radcliffe Univ Hosp NHS Trust, Dept Hepatol, Oxford, England [ 9 ] Karolinska Inst, Karolinska Univ Hosp, Dept Gastroenterol & Hepatol, Stockholm, Sweden [ 10 ] Royal Free Hosp, Anthony Nolan Res Inst, London NW3 2QG, England [ 11 ] Royal Free Hosp, UCL Canc Inst, London NW3 2QG, England [ 12 ] Oslo Univ Hosp, Rikshosp, Dept Immunol, N-0424 Oslo, Norway [ 13 ] Univ Oslo, Dept Med Genet, Oslo, Norway [ 14 ] Oslo Univ Hosp, N-0424 Oslo, Norway [ 15 ] Univ Oslo, Inst Clin Med, KG Jebsen Inflammat Res Ctr, Oslo, Norway [ 16 ] Oslo Univ Hosp, Rikshosp, Dept Transplantat Med, Sect Gastroenterol,Div Canc Med Surg & Transplant, N-0424 Oslo, Norway [ 17 ] Univ Bergen, Dept Clin Med, Div Gastroenterol, Bergen, Norwayen
dc.identifier.journalLiver international : official journal of the International Association for the Study of the Liveren
dc.rights.accessNational Consortium - Landsaðganguren
html.description.abstractSmall duct primary sclerosing cholangitis (PSC) is phenotypically a mild version of large duct PSC, but it is unknown whether these phenotypes share aetiology. We aimed to characterize their relationship by investigating genetic associations in the human leucocyte antigen (HLA) complex, which represent the strongest genetic risk factors in large duct PSC.
html.description.abstractFour classical HLA loci (HLA-A, HLA-B, HLA-C and HLA-DRB1) were genotyped in 87 small duct PSC patients, 485 large duct PSC patients and 1117 controls across three geographical regions.
html.description.abstractHLA-DRB1*13:01 (OR = 2.0, 95% CI 1.2-3.4, P = 0.01) and HLA-B*08 (OR = 1.6, 95% CI 1.1-2.4, P = 0.02) were significantly associated with small duct PSC compared with healthy controls. Based on the observed frequency of HLA-B*08 in small duct PSC, the strongest risk factor in large duct PSC, an estimated 32% (95% CI 4-65%) of this population can be hypothesized to represent early stages or mild variants of large duct PSC. This subgroup may be constituted by small duct PSC patients with inflammatory bowel disease (IBD), which greatly resembled large duct PSC in its HLA association. In contrast, small duct PSC without IBD was only associated with HLA-DRB1*13:01(P = 0.03) and was otherwise distinctly dissimilar from large duct PSC.
html.description.abstractSmall duct PSC with IBD resembles large duct PSC in its HLA association and may represent early stages or mild variants of large duct disease. Different HLA associations in small duct PSC without IBD could indicate that this subgroup is a different entity. HLA-DRB1*13:01 may represent a specific risk factor for inflammatory bile duct disease.


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