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  • Title: Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study.
    Author: Zhao D, Zhou K, Chen Y, Xie W, Zhang Y.
    Journal: BMC Pediatr; 2020 May 27; 20(1):255. PubMed ID: 32460787.
    Abstract:
    BACKGROUND: Early distinguishing biliary atresia from other causes of infantile cholestasis remains a major challenge. We aimed to develop and validate a scoring system based on bile acid for identification of biliary atresia. METHODS: In a prospective study, a total of 141 infants with cholestasis were enrolled in two sets (derivation cohort, n = 66; validation cohort, n = 75) from 2014 to 2018. Variables with significant difference between biliary atresia and non-biliary atresia infants were selected in the derivation cohort. Then, a scoring system including those variables was designed and validated. RESULTS: Among 66 patients in the derivation cohort, 34 (51.5%) had biliary atresia. A scoring system was proposed with the following variables: glycochenodeoxycholic acid/chenodeoxycholic acid, clay stool, and gamma-glutamyl transferase. The total score ranged from 0 to 41, and a cutoff value of 15 identified biliary atresia with an area under receiver operating characteristic curve of 0.87 (95% confidence interval, 0.77-0.94), sensitivity of 85.3%, and specificity of 81.3% in the derivation cohort; these values were also confirmed in a validation cohort with a sensitivity of 90.0% and specificity of 80.0%. CONCLUSIONS: The proposed simple scoring system had good diagnostic accuracy for estimating the risk of biliary atresia in infants with cholestasis.
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