Newborn screening for biliary atresia using direct bilirubin: An implementation science study.

Area of Science:

• Neonatal Medicine
• Pediatric Gastroenterology
• Public Health Screening

Background:

• Biliary atresia (BA) is a severe infant liver disease requiring early intervention.
• Optimal outcomes for BA depend on Kasai portoenterostomy before 45 days of life (DoL).
• Late presentation in our system averaged 60 DoL, necessitating improved screening.

Purpose of the Study:

• To implement and evaluate a two-stage direct bilirubin (DB) screening strategy for early BA detection.
• To reduce the average age at diagnosis and treatment for infants with BA.

Main Methods:

• Newborns screened for DB at 24-48 hours of life.
• Infants with DB ≥0.5 mg/dL underwent repeat testing at 2 weeks.
• DB ≥1.0 mg/dL prompted gastroenterology evaluation.

Main Results:

• 99.5% of 3880 infants were screened; 1.3% initially had DB ≥0.5 mg/dL.
• 12 infants (0.3% false positive rate) had repeat DB ≥1.0 mg/dL, prompting evaluation.
• Average time to gastroenterology evaluation was 18.3 DoL, with BA excluded by 28 DoL.

Conclusions:

• The two-stage DB screening protocol effectively identifies infants needing further evaluation for BA.
• This strategy significantly shortens the diagnostic and treatment timeline compared to previous practices.
• The protocol offers a replicable model for other institutions to improve BA detection rates.