The Italian biliary atresia registry: Insights and lessons from the retrospective analysis of a 10‐year period cohort

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OBJECTIVES: To describe national patterns of management, outcomes, and prognostic factors in infants with biliary atresia (BA) in Italy. METHODS: This retrospective study used data from the Italian BA Registry from January 2012 to December 2021. Overall, 309 infants with BA were identified. Clearance of jaundice (CoJ) was defined as total bilirubin <1.2 mg/dL within 6 months. Univariate and multivariate analyses were used to identify prognostic factors for native liver and patient survival. RESULTS: Kasai portoenterostomy (KPE) was performed in 272 infants (88%) at a median age of 68.5 days (interquartile range 52-83). The CoJ rate after KPE was 39%. Multivariate analysis confirmed that 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). Multiple logistic regression confirmed the major effect of steroid use: the odds ratio for steroid use was 2.51 (95% confidence interval 1.38-4.58; p = 0.0027), whereas the odds ratio for age at KPE was 0.987 (95% CI: 0.975-0.999; p = 0.0371). Liver transplantation was performed in 212/309 patients, and overall 5-year actuarial patient survival was 97.4%. CONCLUSIONS: This retrospective analysis confirms that age at KPE and postoperative corticosteroid use are key favorable factors for successful CoJ, with steroid use showing the strongest association. The excellent overall patient survival highlights the critical role of management in expert centers, even within a decentralized model.

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