The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration.
Mark R DeneauWael El-MataryPamela L ValentinoReham AbdouKhaled AlqoaerMansi AminAchiya Z AmirMarcus AuthFateh BazerbachiAnnemarie BroderickAlbert ChanJillian CotterSylvia DoanMounif El-YoussefFederica FerrariKatryn N FuruyaMadeleine GottrandFrederic GottrandNitika GuptaMatjaz HomanBinita M KamathKyung Mo KimKaija-Leena KolhoAnastasia KonidariBart KootRaffaele IorioOren LedderCara MackMercedes MartinezTamir MilohParvathi MohanNiamh O'CathainAlexandra PapadopoulouAmanda RicciutoLawrence SaubermannPushpa SathyaEyal ShteyerVratislav SmolkaAtushi TanakaRaghu VarierVeena VenkatBernadette VitolaMiriam B VosMarek WoynarowskiJason YapM Kyle JensenPublished in: Hepatology (Baltimore, Md.) (2017)
PSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC-inflammatory bowel disease are more favorable disease phenotypes. (Hepatology 2017;66:518-527).