{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T10:58:59Z","timestamp":1789556339203,"version":"build-2803163510"},"reference-count":39,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2020,12,19]],"date-time":"2020-12-19T00:00:00Z","timestamp":1608336000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2020,12,19]],"date-time":"2020-12-19T00:00:00Z","timestamp":1608336000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Hepatology"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk\u2010stratification tools exist for adult\u2010onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Approach and Results<\/jats:title>\n                    <jats:p>\n                      We used retrospective data from the Pediatric PSC Consortium. The training cohort contained 1,012 patients from 40 centers. We generated a multivariate risk index (Sclerosing Cholangitis Outcomes in Pediatrics [SCOPE] index) that contained total bilirubin, albumin, platelet count, gamma glutamyltransferase, and cholangiography to predict a primary outcome of liver transplantation or death (TD) and a broader secondary outcome that included portal hypertensive, biliary, and cancer complications termed hepatobiliary complications (HBCs). The model stratified patients as low, medium, or high risk based on progression to TD at rates of &lt;1%, 3%, and 9% annually and to HBCs at rates of 2%, 6%, and 13% annually, respectively (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0&lt;\u00a00.001). C\u2010statistics to discriminate outcomes at 1 and 5\u00a0years were 0.95 and 0.82 for TD and 0.80 and 0.76 for HBCs, respectively. Baseline hepatic fibrosis stage was worse with increasing risk score, with extensive fibrosis in 8% of the lowest versus 100% with the highest risk index (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0&lt;\u00a00.001). The model was validated in 240 children from 11 additional centers and performed well.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The SCOPE index is a pediatric\u2010specific prognostic tool for PSC. It uses routinely obtained, objective data to predict a complicated clinical course. It correlates strongly with biopsy\u2010proven liver fibrosis. SCOPE can be used with families for shared decision making on clinical care based on a patient\u2019s individual risk, and to account for variable disease progression when designing future clinical trials.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hep.31393","type":"journal-article","created":{"date-parts":[[2020,5,28]],"date-time":"2020-05-28T18:25:21Z","timestamp":1590690321000},"page":"1074-1087","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":43,"title":["The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children"],"prefix":"10.1097","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0459-9404","authenticated-orcid":false,"given":"Mark R.","family":"Deneau","sequence":"first","affiliation":[{"name":"University of Utah and Intermountain Primary Children\u2019s Hospital,Salt Lake City,UT"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cara","family":"Mack","sequence":"additional","affiliation":[{"name":"University of Colorado School of Medicine,Aurora,CO"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily R.","family":"Perito","sequence":"additional","affiliation":[{"name":"University of California, San Francisco,San Francisco,CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9538-3005","authenticated-orcid":false,"given":"Amanda","family":"Ricciuto","sequence":"additional","affiliation":[{"name":"University of Toronto,Toronto,Ontario,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8227-2004","authenticated-orcid":false,"given":"Pamela L.","family":"Valentino","sequence":"additional","affiliation":[{"name":"Yale University School of Medicine,New Haven,CT"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mansi","family":"Amin","sequence":"additional","affiliation":[{"name":"Phoenix Children\u2019s Hospital,Phoenix,AZ"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Achiya Z.","family":"Amir","sequence":"additional","affiliation":[{"name":"The Dana\u2010Dwek Children\u2019s Hospital,The Tel\u2010Aviv Medical Center,Tel\u2010Aviv University,Tel Aviv,Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madeleine","family":"Aumar","sequence":"additional","affiliation":[{"name":"University of Lille,CHU Lille,Lille,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcus","family":"Auth","sequence":"additional","affiliation":[{"name":"Alder Hey Children\u2019s Hospital,Liverpool,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annemarie","family":"Broderick","sequence":"additional","affiliation":[{"name":"Children\u2019s Health 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Manitoba,Winnipeg,Manitoba,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Ferrari","sequence":"additional","affiliation":[{"name":"Sapienza University of Rome,Rome,Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katryn N.","family":"Furuya","sequence":"additional","affiliation":[{"name":"University of Wisconsin\u2010Madison School of Medicine and Public Health,Madison,WI"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6171-4731","authenticated-orcid":false,"given":"Nitika","family":"Gupta","sequence":"additional","affiliation":[{"name":"Emory University School of Medicine,Atlanta,GA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica T.","family":"Hochberg","sequence":"additional","affiliation":[{"name":"University of 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