Population-based screening methods in biliary atresia: a systematic review and meta-analysis

Area of Science:

• Hepatology and Pediatric Gastroenterology
• Diagnostic Test Evaluation
• Public Health Screening Programs

Background:

• Biliary atresia (BA) is a critical neonatal liver disease requiring early diagnosis for effective treatment.
• Current screening methods for BA vary in sensitivity, specificity, and cost-effectiveness.
• Population-based screening aims to improve early detection rates and patient outcomes.

Approach:

• A systematic literature search was conducted across 11 databases from 1975 to 2022.
• Six distinct BA screening methods were evaluated: stool colour charts (SCCs), conjugated bilirubin measurements, stool colour saturations (SCSs), urinary sulfated bile acids (USBAs), blood spot bile acids, and blood carnitine.
• Meta-analysis was employed to determine the sensitivity, specificity, impact on Kasai procedure timing, morbidity, mortality, and cost-effectiveness of each method.

Key Points:

• Urinary sulfated bile acids (USBAs) demonstrated the highest pooled sensitivity (100.0%) and specificity (99.5%), though based on limited data.
• Stool colour charts (SCCs) showed high specificity (99.9%) and sensitivity (87.9%), significantly reducing the age at Kasai procedure (~60 days) compared to conjugated bilirubin (~36 days).
• Both SCC and conjugated bilirubin improved overall and transplant-free survival, with SCC being more cost-effective.

Conclusions:

• Conjugated bilirubin measurements and SCC are the most researched methods, offering improved sensitivity and specificity for BA detection.
• Despite their effectiveness, the high cost associated with conjugated bilirubin measurements and SCC necessitates further investigation.
• Continued research into optimizing conjugated bilirubin measurements and exploring alternative, cost-effective population-based BA screening strategies is crucial for widespread implementation.