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Population-based screening methods in biliary atresia: a systematic review and meta-analysis
  1. Adam Arshad1,
  2. Julian Gardiner1,
  3. Carmen Ho1,
  4. Philippa Rees1,
  5. Karan Chadda1,
  6. Alastair Baker2,
  7. Alastair G Sutcliffe3
  1. 1Population, Policy & Practice Department, UCL GOS Institute of Child Health, London, UK
  2. 2Department of Paediatric Hepatology, Kings College Hospital, London, UK
  3. 3Population, Policy & Practice Department, UCL GOS Institute of Child Health, Institute of Child Health, London, UK
  1. Correspondence to Dr Adam Arshad, University College London Hospitals NHS Foundation Trust, London, UK; adam.arshad95{at}


Objective The aim of this study was to investigate tested methods of population-based biliary atresia (BA) screening.

Design We searched 11 databases between 1 January 1975 and 12 September 2022. Data extraction was independently done by two investigators.

Main outcome measures Our primary outcomes were: sensitivity and specificity of screening method in BA detection, age at Kasai, BA associated morbidity and mortality, cost-effectiveness of screening.

Results Six methods of BA screening were evaluated: stool colour charts (SCCs), conjugated bilirubin measurements, stool colour saturations (SCSs), measurements of urinary sulfated bile acids (USBAs), assessments of blood spot bile acids and blood carnitine measurements.

In a meta-analysis, USBA was the most sensitive and specific, with a pooled sensitivity and specificity of 100.0% (95% CI 2.5% to 100.0%) and 99.5% (95% CI 98.9% to 99.8%) (based on one study). This was followed by conjugated bilirubin measurements: 100.0% (95% CI 0.0% to 100.0%) and 99.3% (95% CI 91.9% to 99.9%), SCS: 100.0% (95% CI 0.00% to 100.0%) and 92.4% (95% CI 83.4% to 96.7%), and SCC: 87.9% (95% CI 80.4% to 92.8%) and 99.9% (95% CI 99.9% to 99.9%).

SCC reduced the age of Kasai to ~60 days, compared with 36 days for conjugated bilirubin. Both SCC and conjugated bilirubin improved overall and transplant-free survival. The use of SCC was considerably more cost-effective than conjugated bilirubin measurements.

Conclusion Conjugated bilirubin measurements and SCC are the most researched and demonstrate improved sensitivity and specificity in detecting BA. However, their use is expensive. Further research into conjugated bilirubin measurements, as well as alternative methods of population-based BA screening, is required.

PROSPERO registration number CRD42021235133.

  • gastroenterology
  • global health
  • jaundice
  • neonatology

Data availability statement

Data are available upon reasonable request.

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Data availability statement

Data are available upon reasonable request.

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  • Contributors AA designed the study; conducted data extraction, data analysis and data interpretation; and further wrote the original draft and reviewed the final manuscript. JG conducted statistical analysis for the meta-analysis. CH contributed to the data analysis. PR contributed to study design and data interpretation. KC aided the data extraction of the required studies from databases for this study. AB designed the study and aided the data interpretation. AA acts as guarantor.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.