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Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study.

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.

SUBMITTER: Zhao D

PROVIDER: S-EPMC7251733 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature

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Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study.

Zhao Dongying D Zhou Kejun K Chen Yan Y Xie Wei W Zhang Yongjun Y

BMC pediatrics 20200527 1

<h4>Background</h4>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.<h4>Methods</h4>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 i ...[more]

PMID: 32460787

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Project description:Background and Aims: Biliary atresia (BA) is the most common cholestatic liver disease in neonates. Although the Kasai procedure can improve temporary biliary drainage in some cases, complications and liver fibrosis still develop. Liver transplantation is the ultimate treatment. The current study aimed to investigate the effect of previous Kasai surgery on gut microbiota and bile acid in BA with end-stage liver disease. Methods: Patients with BA with end-stage liver disease were divided into two groups according to whether they had previously undergone Kasai surgery (non-Kasai: n = 8, post-Kasai: n = 8). Metagenomic sequencing and ultraperformance liquid chromatography/tandem mass spectrometry were performed to identify the gut microbiota and bile acid. Results: Previous Kasai surgery had some effects on gut microbiota and bile acid in BA with end-stage liver disease. In the gut microbiome, the differential species were mainly distributed at the species level. Veillonella atypica had a significant increase in the non-Kasai group (P < 0.05). Bacteroides spp., Prevotella spp., Barnesiella spp., Parabacteroides spp., Heliobacterium spp., Erysipelatoclostridium spp. and Diaporthe spp. were increased in the post-Kasai group (P < 0.05). Concerning functional profiles, methionine biosynthesis was enriched in the non-Kasai group, while pyridoxal biosynthesis and riboflavin biosynthesis were enriched in the post-Kasai group (linear discriminant analysis > 2, P < 0.05). In stools, 17 bile acids were distinctly elevated in the post-Kasai group, such as cholic acid, chenodeoxycholic acid, β-muricholic acid and tauro α-muricholate (P < 0.05). Spearman correlation test showed that V. atypica had an enormously positive correlation with liver enzymes. Faecalibacterium prausnitzii and Escherichia coli were associated with derivatives of the alternative pathway of bile acid metabolism. Conclusion: Previous Kasai surgery can improve the gut microbiota and bile acid in patients with BA with end-stage liver disease. This improvement contributes to maintaining the intestinal barrier.

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Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study.

Publications

Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study.

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