The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration

• Published in: Hepatology (Baltimore, Md.) Vol. 66; no. 2; pp. 518 - 527
• Main Authors: Deneau, Mark R., El‐Matary, Wael, Valentino, Pamela L., Abdou, Reham, Alqoaer, Khaled, Amin, Mansi, Amir, Achiya Z., Auth, Marcus, Bazerbachi, Fateh, Broderick, Annemarie, Chan, Albert, Cotter, Jillian, Doan, Sylvia, El‐Youssef, Mounif, Ferrari, Federica, Furuya, Katryn N., Gottrand, Madeleine, Gottrand, Frederic, Gupta, Nitika, Homan, Matjaz, Kamath, Binita M., Kim, Kyung Mo, Kolho, Kaija‐Leena, Konidari, Anastasia, Koot, Bart, Iorio, Raffaele, Ledder, Oren, Mack, Cara, Martinez, Mercedes, Miloh, Tamir, Mohan, Parvathi, O'Cathain, Niamh, Papadopoulou, Alexandra, Ricciuto, Amanda, Saubermann, Lawrence, Sathya, Pushpa, Shteyer, Eyal, Smolka, Vratislav, Tanaka, Atushi, Varier, Raghu, Venkat, Veena, Vitola, Bernadette, Vos, Miriam B., Woynarowski, Marek, Yap, Jason, Jensen, M. Kyle
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.08.2017
• Subjects: Analysis of Variance; Aspartate aminotransferase; Bilirubin; Biopsy, Needle; Child; Children; Cholangiocarcinoma; Cholangitis; Cholangitis, Sclerosing - mortality; Cholangitis, Sclerosing - pathology; Cholangitis, Sclerosing - surgery; Cohort Studies; Confidence intervals; Disease Progression; Disease-Free Survival; Female; Follow-Up Studies; Hepatitis; Hepatology; Humans; Hypertension; Immunohistochemistry; Inflammatory bowel disease; Inflammatory bowel diseases; Internationality; Intestine; Japan; Liver; Liver Function Tests; Liver transplantation; Liver Transplantation - methods; Liver Transplantation - mortality; Male; Medical prognosis; Multivariate Analysis; Pediatrics; Predictive Value of Tests; Proportional Hazards Models; Retrospective Studies; Risk Assessment; Severity of Illness Index; Survival; Survival Analysis; Transplantation; γ-Glutamyltransferase

There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long‐term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a surviva...