Published in:
Open Access 01-12-2020 | Research article
Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study
Published in: BMC Pediatrics | Issue 1/2020
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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.