{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,29]],"date-time":"2025-05-29T16:07:28Z","timestamp":1748534848070,"version":"3.40.5"},"reference-count":26,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T00:00:00Z","timestamp":1649030400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic disorder associated with ulcerative colitis (UC). Although the inflammatory bowel disease phenotype has been characterised in patients with PSC, the impact of UC on the course and progression of PSC\u2010UC is less clear. We aimed to evaluate the effects of UC on liver\u2010related outcomes in children with PSC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective analysis of children aged \u226418\u2009years diagnosed with PSC with\/without UC at a single tertiary paediatric liver unit between January 1998 and May 2016. Patients were followed up until transition to an adult service. Outcomes studied included biliary complications, clinically significant portal hypertension, need for liver transplantation and post\u2010transplantation recurrence.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifty\u2010one children (31 female) were diagnosed with PSC (median age \u2013 11.3\u2009years (interquartile range 7)), follow\u2010up median duration 54\u2009months (interquartile range 56). Thirty\u2010seven (73%) patients had concurrent UC, of which 26 had their diagnosis confirmed prior to or within 6\u00a0months of PSC diagnosis (early\u2010onset). PSC complications were more common in children with PSC\u2010UC compared with PSC alone (24\/37 (65%) vs. 2\/14 (14%); <jats:italic>P<\/jats:italic>\u00a0=\u00a00.001). Furthermore, children with endoscopically mild or moderate UC at diagnosis showed a greater propensity for liver\u2010related complications compared with children with severe UC (24\/32 vs. 0\/5; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.003). Children with late\u2010onset UC had higher rates of clinically significant portal hypertension (5\/11 (45%) vs. 3\/26 (12%); <jats:italic>P<\/jats:italic>\u00a0=\u00a00.007) and liver transplantation (5\/11(45%) vs. 2\/26 (8%); <jats:italic>P<\/jats:italic>\u00a0=\u00a00.02). Children with PSC\u2010UC had significantly higher rates of pancolitis, rectal sparing and milder colitis than those with UC alone.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The presence and a later\u2010onset of UC are associated with more significant progression to end\u2010stage liver disease. There is an inverse trend between UC severity and PSC severity in children with concurrent PSC\u2010UC.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jpc.15954","type":"journal-article","created":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T12:25:24Z","timestamp":1649075124000},"page":"1221-1227","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Impact of ulcerative colitis on liver\u2010related outcomes of children with primary sclerosing cholangitis"],"prefix":"10.1111","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3620-0413","authenticated-orcid":false,"given":"Mohana","family":"Sathiaseelan","sequence":"first","affiliation":[{"name":"Department of Gastroenterology, Hepatology and Clinical Nutrition The Royal Children's Hospital Melbourne  Melbourne Victoria 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