Autor: |
Deneau, M.R. Mack, C. Perito, E.R. Ricciuto, A. Valentino, P.L. Amin, M. Amir, A.Z. Aumar, M. Auth, M. Broderick, A. DiGuglielmo, M. Draijer, L.G. Tavares Fagundes, E.D. El-Matary, W. Ferrari, F. Furuya, K.N. Gupta, N. Hochberg, J.T. Homan, M. Horslen, S. Iorio, R. Jensen, M.K. Jonas, M.M. Kamath, B.M. Kerkar, N. Kim, K.M. Kolho, K.-L. Koot, B.G.P. Laborda, T.J. Lee, C.K. Loomes, K.M. Martinez, M. Miethke, A. Miloh, T. Mogul, D. Mohammad, S. Mohan, P. Moroz, S. Ovchinsky, N. Palle, S. Papadopoulou, A. Rao, G. Rodrigues Ferreira, A. Sathya, P. Schwarz, K.B. Shah, U. Shteyer, E. Singh, R. Smolka, V. Soufi, N. Tanaka, A. Varier, R. Vitola, B. Woynarowski, M. Zerofsky, M. Zizzo, A. Guthery, S.L. |
Jazyk: |
angličtina |
Rok vydání: |
2021 |
Popis: |
Background and Aims: Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset 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. Approach and Results: 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 |
Databáze: |
OpenAIRE |
Externí odkaz: |
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