{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T04:37:31Z","timestamp":1740112651177,"version":"3.37.3"},"reference-count":45,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2018,2,19]],"date-time":"2018-02-19T00:00:00Z","timestamp":1518998400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Sclerosing cholangitis (SC) is a chronic cholestatic liver disease that is being increasingly diagnosed in childhood. The long\u2010term course and prognosis of pediatric SC are poorly described.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We reviewed data of pediatric SC patients, followed in two referral centers, during a period of up to 20\u00a0years. We aimed to evaluate long\u2010term outcomes according to SC phenotype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among 45 patients (median age, 10.4\u00a0years; male patients, 73.4%) 29 (64.4%) were asymptomatic at presentation. Twenty patients (44%) had a concomitant inflammatory bowel disease (SC\/IBD). Autoimmune features were found in 20 patients (44%). Liver biopsy showed severe fibrosis or cirrhosis in 32% of cases. Patients with SC alone had a higher rate of interface hepatitis at liver biopsy than SC\/IBD patients. All children received ursodeoxycholic acid at diagnosis, and 17 received steroids and\/or azathioprine. After a mean follow\u2010up of 8.7\u2009\u00b1\u20095.6\u00a0years, all patients were alive and seven developed at least one liver\u2010related complication. At the end of follow\u2010up, 10 patients stopped immunosuppressants and two had no therapy. Only two patients underwent liver transplantation. Complication\u2010free survival did not differ between SC\/IBD and SC patients, but survival was longer in patients without autoimmune features.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In our early diagnosed cohort, the 9\u2010year survival with native liver was better than that reported in other studies. Approximately 15% of patients developed liver\u2010related disease complications, less than previously reported. The long\u2010term course of SC was negatively influenced by the presence of autoimmune features, but not by concomitant IBD.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hepr.13059","type":"journal-article","created":{"date-parts":[[2018,1,10]],"date-time":"2018-01-10T13:41:56Z","timestamp":1515591716000},"page":"556-565","source":"Crossref","is-referenced-by-count":1,"title":["A promising medium\u2010term follow\u2010up of pediatric sclerosing cholangitis: Mild phenotype or early diagnosis?"],"prefix":"10.1111","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7250-3799","authenticated-orcid":false,"given":"Federica","family":"Ferrari","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology and Liver Unit, Department of Pediatrics Sapienza University of Rome  Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giusy","family":"Ranucci","sequence":"additional","affiliation":[{"name":"Department of Translational Medical Science, Section of 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