{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T16:00:11Z","timestamp":1791648011747,"version":"4.3.4"},"reference-count":25,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":4445,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2003,7]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Primary sclerosing cholangitis (PSC) is increasingly diagnosed in children and adolescents, but its long\u2013term prognosis remains uncertain. The aim of this longitudinal, cohort study was to determine the long\u2013term outcome of children with PSC. Fifty\u2013two children with cholangiography\u2013proven PSC (34 boys and 18 girls; mean age 13.8 \u00b1 4.2 years; range, 1.5\u201319.6 years) who were seen at our institution over a 20\u2013year period were followed\u2013up for up to 16.7 years. Two thirds presented with symptoms and\/or signs of PSC and 81% had concomitant inflammatory bowel disease (IBD). Twenty\u2013five percent had total alkaline phosphatase activity within the normal range for the age group, but all of them had elevated \u03b3\u2013glutamyl transpeptidase levels. Autoimmune hepatitis overlapping with PSC was present in 35% of children. A positive but transient clinical and\/or biochemical response occurred under therapy with ursodeoxycholic acid, alone or in combination with immunosuppressive medications. During follow\u2013up, 11 children underwent liver transplantation for end\u2013stage PSC and 1 child died. The median (50%) survival free of liver transplantation was 12.7 years. Compared with an age\u2013 and gender\u2013matched U.S. population, survival was significantly shorter in children with PSC (<jats:italic toggle=\"yes\">P<\/jats:italic> &lt; .001). In a Cox regression model, lower platelet count, splenomegaly, and older age were associated with shorter survival. Presence of autoimmune hepatitis overlapping with PSC (<jats:italic toggle=\"yes\">P<\/jats:italic> = .2) or medical therapy (<jats:italic toggle=\"yes\">P<\/jats:italic> = .2) did not affect survival. In conclusion, PSC significantly decreases survival in this child population. Although pharmacologic therapy may improve symptoms and liver test results initially, it does not seem to impact the long\u2013term outcome.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1053\/jhep.2003.50289","type":"journal-article","created":{"date-parts":[[2003,6,26]],"date-time":"2003-06-26T16:37:21Z","timestamp":1056645441000},"page":"210-217","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":219,"title":["Primary Sclerosing Cholangitis in Children: A Long\u2013Term Follow\u2013Up Study"],"prefix":"10.1097","volume":"38","author":[{"given":"Ariel E.","family":"Feldstein","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean","family":"Perrault","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mounif","family":"El-Youssif","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keith D.","family":"Lindor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah K.","family":"Freese","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Angulo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"10.1053\/jhep.2003.50289-BIB1","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1002\/hep.510300101","volume":"30","author":"Angulo","year":"1999","journal-title":"Hepatology"},{"key":"10.1053\/jhep.2003.50289-BIB2","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1002\/hep.510320429","volume":"32","author":"Angulo","year":"2000","journal-title":"Hepatology"},{"key":"10.1053\/jhep.2003.50289-BIB3","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1002\/hep.510300501","volume":"30","author":"Graziadei","year":"1999","journal-title":"Hepatology"},{"key":"10.1053\/jhep.2003.50289-BIB4","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1016\/S0016-5085(87)91082-1","volume":"92","author":"El-Shabrawi","year":"1987","journal-title":"Gastroenterology"},{"key":"10.1053\/jhep.2003.50289-BIB5","first-page":"1415","volume":"22","author":"Wilschanski","year":"1995","journal-title":"Hepatology"},{"key":"10.1053\/jhep.2003.50289-BIB6","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1111\/j.1478-3231.1999.tb00040.x","volume":"19","author":"Floreani","year":"1999","journal-title":"Liver"},{"key":"10.1053\/jhep.2003.50289-BIB7","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/S0022-3476(94)70253-5","volume":"124","author":"Debray","year":"1994","journal-title":"J Pediatr"},{"key":"10.1053\/jhep.2003.50289-BIB8","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1148\/radiology.149.1.6412283","volume":"149","author":"MacCarty","year":"1983","journal-title":"Radiology"},{"key":"10.1053\/jhep.2003.50289-BIB9","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1016\/S0168-8278(99)80297-9","volume":"31","author":"Alvarez","year":"1999","journal-title":"J Hepatol"},{"key":"10.1053\/jhep.2003.50289-BIB10","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1002\/hep.1840010612","volume":"1","author":"Ludwig","year":"1981","journal-title":"Hepatology"},{"key":"10.1053\/jhep.2003.50289-BIB11","doi-asserted-by":"crossref","first-page":"581","DOI":"10.7326\/0003-4819-102-5-581","volume":"102","author":"Wee","year":"1985","journal-title":"Ann Intern Med"},{"key":"10.1053\/jhep.2003.50289-BIB12","first-page":"193","volume-title":"Contemporary Issues in Surgical Pathology: Liver Pathology","author":"Ludwig","year":"1986","unstructured":", . Primary sclerosing cholangitis. In: , eds. Contemporary Issues in Surgical Pathology: Liver Pathology. New York: Churchill Livingstone, 1986;193-213."},{"key":"10.1053\/jhep.2003.50289-BIB13","doi-asserted-by":"crossref","DOI":"10.1007\/978-1-4757-3294-8","volume-title":"Modeling survival data: extending the Cox model","author":"Therneau","year":"2000","unstructured":". Modeling survival data: extending the Cox model. New York: Springer-Verlag, 2000."},{"key":"10.1053\/jhep.2003.50289-BIB14","series-title":"Technical Rep","author":"Therneau","year":"1999","unstructured":". Expected Survival based on Hazard Rates (Update). Technical Report Series No. 63. 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