Design and validation of a diagnostic score for biliary atresia

J Hepatol. 2014 Jul;61(1):116-23. doi: 10.1016/j.jhep.2014.03.016. Epub 2014 Mar 18.


Background & aims: The dilemma of early diagnosis of biliary Atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis is challenging. The aim was to design and validate a scoring system for early discrimination of BA from other causes of neonatal cholestasis.

Methods: A twelve-point scoring system was proposed according to clinical, laboratory, ultrasonographic, and histopathological parameters. A total of 135 patients with neonatal cholestasis in two sets were recruited to design (n=60) and validate (n=75) a scoring system. Parameters with significant statistical difference between BA (n=30) and non-BA (n=30) patients in the design set were analyzed by logistic regression to predict the presence or absence of BA then a scoring system was designed and validated.

Results: The total score ranged from 0 to 37.18 and a cut-off value of >23.927 could discriminate BA from other causes of neonatal cholestasis with sensitivity and specificity of 100% each. By applying this score in the validation set, the accuracy was 98.83% in predicting BA. The diagnosis of BA was proposed correctly in 100% and the diagnosis of non-BA was proposed correctly in 97.67% of patients. By applying this model, unnecessary intraoperative cholangiography would be avoided in non-BA patients.

Conclusions: This scoring system accurately separates infants with BA and those with non-BA, rendering intraoperative cholangiography for confirming or excluding BA unnecessary in a substantial proportion of patients.

Keywords: Clay stool; Doppler ultrasonography; Hepatic subcapsular flow; Liver biopsy; Neonatal cholestasis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Validation Study

MeSH terms

  • Biliary Atresia / complications
  • Biliary Atresia / diagnosis*
  • Biliary Atresia / diagnostic imaging
  • Cholangiography
  • Cholestasis / etiology
  • Cohort Studies
  • Depsipeptides
  • Diagnosis, Differential
  • Early Diagnosis
  • Female
  • Fusarium
  • Humans
  • Infant
  • Infant, Newborn
  • Liver / diagnostic imaging
  • Liver / pathology
  • Logistic Models
  • Male
  • Predictive Value of Tests
  • Prospective Studies
  • Sensitivity and Specificity
  • Ultrasonography


  • Depsipeptides
  • fusafungin