A new clinical report from the American Academy of Pediatrics provides pediatric primary care providers with a systematic approach to identify biliary atresia between 2 and 4 weeks of life, potentially improving outcomes for the rare but serious condition accounting for 60% of liver transplants in infants under 1 year.
In the report, published in the March 2025 issue of Pediatrics, researchers present a three-step screening strategy to be implemented during the "by 1 month" well-child visit, focusing on eye color, stool color, and laboratory results to determine if direct or conjugated bilirubin testing is warranted.
"Ideally, infants are treated before 30 to 45 days of life to achieve the best outcomes," the study authors wrote. "In practice, however, infants are treated on average after 60 days of life in the [United States]," they added.
The strategy requires physicians to assess:
- Presence of jaundice in eyes or skin beyond 2 weeks of life
- Pale, gray, or white stools through direct inspection or caregiver report
- Previous direct or conjugated bilirubin levels and whether initial values exceeded laboratory reference ranges.
The report specifies that a direct or conjugated bilirubin level ≥ 1 mg/dL warrants urgent consultation with a pediatric gastroenterologist. However, the researchers note that blood draws may be avoided in certain cases, such as when prior normal results exist or when abnormal levels show a decreasing trend below 1 mg/dL.
"Biliary atresia (BA) can be asymptomatic initially," the study authors explained. "When symptoms do develop, the 1 in 8,000 to 18,000 affected newborn infants each year are difficult to distinguish from the 65% of healthy newborn infants with benign causes of jaundice," they indicated.
The report also addresses racial disparities in BA diagnosis, noting that "Hispanic and non-Hispanic Black infants with BA are referred later than non-Hispanic white infants." The researchers suggest this may stem from missed well-child visits as a result of social determinants of health or implicit bias affecting jaundice assessment in infants with darker skin tones.
Looking ahead, the report discusses two promising universal screening approaches: stool color card programs and newborn direct or conjugated bilirubin screening prior to hospital discharge. Both methods have shown success in various global locations, with implementation studies ongoing to evaluate integration into existing newborn protocols.
The full clinical report includes detailed guidance on billing considerations, scheduling recommendations, and specific activities for the first three American Academy of Pediatrics periodicity schedule patient encounters to optimize BA identification.
The authors declared having no competing interests.