G Boroni et al. J Pediatr Gastroenterol Nutr. 2026;83:491–499. The Italian biliary atresia registry: Insights and lessons from the retrospective analysis of a 10-year period cohort
This retrospective study (2012-2012) examined the outcomes of 309 children with biliary atresia (BA) from 13 centers. Clearance of jaundice (CoJ) was defined as total bilirubin <1.2 mg/dL within 6 months.
Key findings:
- The calculated incidence of BA in Italy during the study period was 1/15,142 live births
- Kasai portoenterostomy (KPE) was performed in 272 infants (88%) at a median age of 68.5 days.
- CoJ was achieved in 39% of cases.
- CoJ was significantly associated with younger age and lower total bilirubin at referral (p = 0.0446 and p = 0.0144, respectively), younger age at KPE (p = 0.0345), and postoperative corticosteroid use (p = 0.0036).
- Liver transplantation was performed in 212/309 patients, and overall 5-year actuarial patient survival was 97.4%.
- Overall patient survival was excellent: 97% at 5 years and 95% at 10 years.
In those with a failed Kasai, steroid use did not improve outcomes.
Discussion points:
- “This analysis contributes further to the controversy surrounding the effect of steroids.”
- “The success of KPE decreases progressively with age at operation, with each additional day reducing the odds of CoJ by approximately 1.3%.”
- “Despite decades of calls for earlier diagnosis and earlier KPE, little progress has been made in achieving earlier access to surgery…The evidence suggests that fibrosis is already advanced when KPE is performed at around 60 days of life and is likely to persist for life even when CoJ is achieved. Cirrhosis and portal hypertension remain common in long‐term survivors, who may face severe comorbidities.”
- “Standards should therefore aim for KPE closer to, or before, 30 days of age, rather than accepting the current usual timing of 50–70 days achieved worldwide.”
My take: In this cohort, ~70% needed a liver transplant. Even with optimal timing and management, more than 50% are likely to need a liver transplant for long-term survival.
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