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Multicenter cohort study reveals progressive liver disease and predictive biomarkers in pediatric primary sclerosing cholangitis, highlighting phenotypes and risk factors guiding early care.
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).
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Deneau et al. (2017) studied this question.
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