{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,29]],"date-time":"2025-05-29T16:07:59Z","timestamp":1748534879647,"version":"3.40.5"},"reference-count":35,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,5,11]],"date-time":"2024-05-11T00:00:00Z","timestamp":1715385600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100015738","name":"Mary och Georg C. Ehrnrooths Stiftelse","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100015738","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["UEG Journal"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Objectives<\/jats:title><jats:p>The impact of dominant stricture (DS) on the outcomes of paediatric\u2010onset primary sclerosing cholangitis (PSC) is unknown. This study was aimed at investigating the impact of DS on the clinical course and prognosis of patients with paediatric\u2010onset PSC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with paediatric\u2010onset PSC diagnosed between January 1993 and May 2017 were identified from hospital records or our PSC registry. Data including clinical, laboratory, cholangiography, and cytology at diagnosis and during follow\u2010up (until July 2023) were reviewed. We graphed the Kaplan\u2010Meier failure function and fitted crude and multivariable Cox model to calculate hazard ratios (HR) and 95% confidence intervals (CI) for selected variables. In these analyses, DS was treated as a time\u2010varying variable.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 68 patients (42 males) with paediatric\u2010onset PSC (median age at diagnosis 15\u00a0years). The median follow\u2010up was 13\u00a0years and the median age at the last follow\u2010up was 27\u00a0years. In total, 35 (51%) had concomitant autoimmune hepatitis. DS was diagnosed in 33 patients (48%): in eight at the time of PSC diagnosis (12%) and in 25 (37%) by the end of follow\u2010up. In patients with DS, two developed cirrhosis, seven were transplanted and one patient was operated for a biliary mass with low\u2010grade dysplasia. In patients without a DS, two developed cirrhosis, and four were transplanted; one female was excluded from survival analysis because she already had cirrhosis at the time of PSC diagnosis. Cirrhosis or biliary dysplasia or needing liver transplantation for these indications were more frequent after the development of DS (10\/33, adjusted HR 4.26, 95%CI: 1.26\u201314.4). No cholangiocarcinomas or deaths occurred during the follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>DS was present at diagnosis or developed during follow\u2010up in about half of the patients with paediatric\u2010onset PSC and was associated with impaired outcome.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ueg2.12583","type":"journal-article","created":{"date-parts":[[2024,5,11]],"date-time":"2024-05-11T17:59:34Z","timestamp":1715450374000},"page":"717-725","source":"Crossref","is-referenced-by-count":3,"title":["Dominant stricture in paediatric\u2010onset primary sclerosing cholangitis is associated with impaired prognosis in a long\u2010term follow\u2010up"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9064-0612","authenticated-orcid":false,"given":"Andrea","family":"Tenca","sequence":"first","affiliation":[{"name":"Abdominal Center Helsinki University and Helsinki University Hospital HUS  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaija\u2010Leena","family":"Kolho","sequence":"additional","affiliation":[{"name":"Department of Paediatric Gastroenterology Helsinki University and Children's Hospital Helsinki University Hospital HUS  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dario","family":"Consonni","sequence":"additional","affiliation":[{"name":"Epidemiology Unit Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico  Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kalle","family":"Jokelainen","sequence":"additional","affiliation":[{"name":"Abdominal Center Helsinki University and Helsinki University Hospital HUS  Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0250-8559","authenticated-orcid":false,"given":"Martti","family":"F\u00e4rkkil\u00e4","sequence":"additional","affiliation":[{"name":"Abdominal Center Helsinki University and Helsinki University Hospital HUS  Helsinki 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