Triaging Infantile Cholestasis: Validation of Non-Invasive Diagnostic Modalities and a Composite Scoring System for Early Detection of Biliary Atresia
In brief
Simple Biliary Atresia Score catches 98% of infants with BA using bedside measurements
In a UK cohort of 143 infants with early cholestasis, the SBAS (score at least 3) identified biliary atresia with 97.7% sensitivity and 98.4% negative predictive value, outperforming individual ultrasound and lab tests. Though specificity was modest at 68%, the tool offers a low-cost, rapid triage to flag babies needing urgent surgery, with false positives often due to Alagille syndrome.
- Journal
- Journal of pediatric surgery (Q1)
- Published
- 2 September 2026
- Study design
- Retrospective cohort
- Evidence level
- Level 3, Low (CEBM 3b)
- Authors
- Yang Yang Lee, Siddhant Chavan, Alba Bueno, Evelyn G P Ong, Khalid Sharif, Girish Gupte
- PMID
- 42685829
- DOI
- 10.1016/j.jpedsurg.2026.163437
Why clinicians should know about it
- Picked for Pediatric Surgery (paper of the day, 5 September 2026): SBAS composite score for early biliary atresia detection
Abstract
BACKGROUND: Biliary atresia (BA) is the most common cause of infantile obstructive jaundice, with outcomes critically dependent on timely Kasai portoenterostomy. Differentiating BA from other causes of infantile cholestasis remains challenging. We aimed to evaluate the diagnostic performance of individual investigations and the composite predictive index (the Simple Biliary Atresia Score, SBAS) within a UK national tertiary paediatric liver unit. METHODS: This retrospective observational cohort study included all infants referred with conjugated hyperbilirubinaemia at less than 100 days of life to the Liver Unit at Birmingham Children's Hospital between March 2022 and March 2025. . The primary endpoint was the diagnosis or exclusion of BA, confirmed by intra-operative cholangiography and histopathology. SBAS was calculated from gallbladder length, common bile duct diameter, periportal echogenicity, direct-to-total bilirubin ratio, and GGT, yielding a 0-7 composite score; a cutoff ≥3 defined high risk. Diagnostic performance was assessed using sensitivity, specificity, predictive values, and AUROC. RESULTS: Of 143 included infants, 46 (32.2%) were diagnosed with biliary atresia. Direct bilirubin (median 102 vs 85 μmol/L, p=0.015) and GGT (333 vs 135 U/L, p<0.001) were higher in biliary atresia, and all sonographic markers differed significantly between groups. At SBAS ≥3 the score achieved sensitivity 97.7% (95% CI 87.9-99.6), specificity 68.5% (58.3-77.2), positive predictive value 60.0% (48.3-70.7) and negative predictive value 98.4% (91.4-99.7); the AUROC of the score as a continuous variable was 0.874 (0.817-0.930), exceeding that of every individual investigation. The single false negative was a cystic biliary atresia misinterpreted on ultrasound. Alagille syndrome was over-represented among false positives (17.9% vs 1.6% of true negatives). Liver biopsy was performed in 55 of 142 infants (38.7%), in 50 cases concurrently with operative cholangiography. CONCLUSION: SBAS is a robust, low-cost, bedside triage tool that retains excellent sensitivity and discriminative performance in a UK tertiary referral cohort, supporting its use as secondary triage to identify infants requiring urgent escalation to definitive management.
Abstract as published, via PubMed.
For healthcare professionals. The summary is generated by AI from the published abstract, and the evidence level is assigned automatically from the study design on the Oxford CEBM hierarchy. Neither is medical advice. Read the full paper before changing practice.