Advancing Prognostic Tools in Biliary Atresia Care, With Ahmad Anouti, MD

Early recognition remains the single most important determinant of outcomes in biliary atresia, a rare but life-threatening cholestatic liver disease of infancy.

Hepatoportoenterostomy, also known as a Kasai procedure, can effectively treat biliary atresia and delay, or in some cases prevent, the need for a liver transplant, especially when performed early. While several serum markers have been suggested to better predict post-hepatoportoenterostomy outcomes, emerging research is shedding light on the potential utility of serum predictors of native liver survival in these patients.

In a recent interview with HCPLive, Ahmad Anouti, MD, a pediatric resident at UT Southwestern, underscored how easily biliary atresia can be overlooked and how costly delays can be.

Biliary atresia is characterized by absent, collapsed, or nonfunctional bile ducts, preventing bile from draining from the liver into the intestine. As bile accumulates, progressive inflammation and fibrosis lead to cirrhosis. Clinically, infants present with jaundice driven by rising direct bilirubin levels.

“The most common causes of jaundice are physiologic: breastfeeding, breast milk, just natural jaundice, and so and the majority of babies are jaundice, more than 60%,” Anouti explained. “Most of the time, it is not biliary atresia, but if it is biliary atresia, and we're saying ‘Oh, this is normal jaundice, this is physiological, we're not fractionating,’ and we're missing the direct bilirubin, then the kids are going to get worse.”

Timing is critical for the primary surgical intervention, hepatoportoenterostomy, which connects the intestine directly to the liver in an attempt to restore bile flow. Outcomes decline significantly after 60 days of life, with each day of delay associated with increased mortality risk. If the Kasai fails, liver transplantation becomes the only definitive option.

Against this clinical backdrop, Anouti and colleagues conducted a meta-analysis, published in the Journal of Pediatric Gastroenterology and Nutrition, examining serum biomarkers as potential prognostic tools in biliary atresia. While direct bilirubin and other markers help establish diagnosis, reliable predictors of long-term outcomes remain elusive. The team systematically reviewed existing literature to identify serum values that may correlate with prognosis.

Their findings suggest several potential thresholds. Elevated direct bilirubin > 1 mg/dL within the first 48 to 72 hours of life may signal biliary atresia. Beyond diagnosis, persistent direct bilirubin levels > 1 mg/dL at 12 months may indicate a poorer prognosis. Total bile acids also show promise as longer-term prognostic indicators.

However, as Anouti noted, the analysis is limited by the quality and heterogeneity of the underlying studies. Additionally, he pointed out much of the existing literature focuses on diagnosis rather than risk stratification.

Ultimately, the study highlights both progress and gaps. While early detection remains paramount, advancing prognostic tools could help clinicians better counsel families, tailor surveillance, and identify patients most likely to require transplant, moving the field beyond diagnosis toward precision management.

Editors’ Note: Anouti reports no relevant disclosures.

References

1. Anouti A, Dahshi H, Cotter TG, Rabbani TA, Corbitt N, Hassan S. Serum predictors of native liver survival post-Kasai: Systematic review and meta-analysis. J Pediatr Gastroenterol Nutr. Published online January 29, 2026. doi:10.1002/jpn3.70355

2. NIDDK. Treatment for Biliary Atresia. September 2017. Accessed February 18, 2026. https://www.niddk.nih.gov/health-information/liver-disease/biliary-atresia/treatment