{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:22:18Z","timestamp":1787008938002,"version":"build-2736575974"},"reference-count":35,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2023,9,28]],"date-time":"2023-09-28T00:00:00Z","timestamp":1695859200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Whether primary sclerosing cholangitis related to inflammatory bowel disease (PSC-IBD) diagnosed before 6 years (ie, VEO-IBD) has a distinct phenotype and disease course is uninvestigated. We aimed to analyze the characteristics and natural history of VEO-PSC-IBD, compared with early and adolescent-onset PSC-IBD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This is a multicenter, retrospective, case-control study from 15 centers affiliated with the Porto and Interest IBD group of ESPGHAN. Demographic, clinical, laboratory, endoscopic, and imaging data were collected at baseline and every 6 months. Inflammatory bowel disease\u2013related (clinical remission, need for systemic steroids and biologics, and surgery) and PSC-related (biliary and portal hypertensive complications, need for treatment escalation and liver transplantation, cholangiocarcinoma, or death) outcomes were compared between the 2 groups.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Sixty-nine children were included, with a median follow-up of 3.63 years (interquartile range, 1-11): 28 with VEO-PSC-IBD (23 UC [82%], 2 IBD-U [7%] and 3 [11%] CD), and 41 with PSC-IBD (37 UC [90%], 3 IBDU [7.5%] and 1 [2.5%] CD). Most patients with UC presented with pancolitis (92% in VEO-PSC-UC vs 85% in PSC-UC, P = .2). A higher number of patients with VEO-PSC-IBD were diagnosed with PSC\/autoimmune hepatitis overlap syndrome than older children (24 [92%] vs 27 [67.5%] PSC-IBD, P = .03), whereas no other differences were found for PSC-related variables. Time to biliary strictures and infective cholangitis was lower in the VEO-PSC-IBD group (P = .01 and P = .04, respectively), while no difference was found for other outcomes. No cases of cholangiocarcinoma were reported.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Primary sclerosing cholangitis related to inflammatory bowel disease has similar baseline characteristics whether diagnosed as VEO-IBD or thereafter. A milder disease course in terms of biliary complications characterizes VEO-PSC-IBD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ibd\/izad218","type":"journal-article","created":{"date-parts":[[2023,9,12]],"date-time":"2023-09-12T10:21:30Z","timestamp":1694514090000},"page":"1662-1669","source":"Crossref","is-referenced-by-count":8,"title":["Outcome of Very Early Onset Inflammatory Bowel Disease Associated With Primary Sclerosing Cholangitis: A Multicenter Study From the Pediatric IBD Porto Group of ESPGHAN"],"prefix":"10.1093","volume":"30","author":[{"given":"Giulia","family":"Catassi","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology and Liver Unit, Sapienza University of Rome, Umberto I Hospital , Rome ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"D\u2019Arcangelo","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Liver Unit, Sapienza University of Rome, Umberto I Hospital , Rome ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3322-2921","authenticated-orcid":false,"given":"Lorenzo","family":"Norsa","sequence":"additional","affiliation":[{"name":"Pediatric Hepatology Gastroenterology and Transplantation, Papa Giovanni XXIII Hospital , Bergamo ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5249-8248","authenticated-orcid":false,"given":"Matteo","family":"Bramuzzo","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health, IRCCS \u201cBurlo Garofolo,\u201d Trieste ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iva","family":"Hojsak","sequence":"additional","affiliation":[{"name":"University Children\u2019s Hospital Zagreb, University of Zagreb Medical School , Zagreb ,","place":["Croatia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9170-8415","authenticated-orcid":false,"given":"Kaija-Leena","family":"Kolho","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital, Helsinki University Hospital and University of Helsinki , Helsinki ,","place":["Finland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Romano","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Cystic Fibrosis Unit, University of Messina , Messina ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Gasparetto","sequence":"additional","affiliation":[{"name":"Department of Paediatric Gastroenterology, Barts Health Trust, The Royal London Children\u2019s Hospital, London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelo","family":"Di Giorgio","sequence":"additional","affiliation":[{"name":"Pediatric Hepatology Gastroenterology and Transplantation, Papa Giovanni XXIII Hospital , Bergamo ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9362-0465","authenticated-orcid":false,"given":"Seamus","family":"Hussey","sequence":"additional","affiliation":[{"name":"National Children\u2019s Research Centre, Royal College of Surgeons of Ireland and University College Dublin , Dublin ,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anat","family":"Yerushalmy-Feler","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Institute \u201cDana-Dwek\u201d Children\u2019s Hospital, Tel Aviv University , Tel Aviv ,","place":["Israel"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan","family":"Turner","sequence":"additional","affiliation":[{"name":"Shaare Zedek Medical Center, the Hebrew 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