Factors Influencing Time-to-diagnosis of Biliary Atresia

Area of Science:

• Pediatric Gastroenterology
• Hepatology
• Neonatal Surgery

Background:

• Early diagnosis of biliary atresia (BA) is critical for timely treatment and improved outcomes, potentially reducing the need for liver transplantation.
• Current diagnosis timelines for BA are often delayed, with infants typically treated after 60 days of life, highlighting a need for accelerated diagnostic strategies.

Purpose of the Study:

• To investigate factors influencing the diagnostic timeline for biliary atresia (BA) in infants.
• To identify opportunities for accelerating the diagnosis and treatment of BA.

Main Methods:

• Retrospective analysis of 65 infants diagnosed with BA between 2007 and 2014.
• Examination of two diagnostic periods: birth to specialist referral (P1) and specialist referral to treatment (P2).
• Analysis of sociodemographic factors, laboratory results, and early tissue histology in relation to diagnostic timelines using Kaplan-Meier curves and Cox proportional hazard models.

Main Results:

• Race/ethnicity significantly impacted P1, with non-Hispanic white infants experiencing shorter referral times compared to non-Hispanic black and Hispanic infants.
• Referral age influenced P2, with shorter treatment times observed in infants referred after 30, 45, or 60 days of life.
• Normal or near-normal aminotransferase levels were noted in some infants with delayed P2, yet early tissue histology consistently revealed key features of BA.

Conclusions:

• Opportunities exist to accelerate BA diagnosis through universal screening strategies to ensure prompt referrals across all racial/ethnic groups.
• Development of algorithms to detect early BA features is recommended to ensure efficient evaluations, irrespective of infant age or initial laboratory values.