Multicenter Study on Early Predictors of Biliary Atresia Outcomes

Area of Science:

• Pediatric Hepatology
• Surgical Gastroenterology
• Neonatal Surgery

Background:

• Biliary atresia (BA) is a severe neonatal liver disease requiring prompt surgical intervention.
• Predicting outcomes after portoenterostomy is crucial for patient management and prognosis.
• Liver biochemistry, APRI, and patient characteristics are potential prognostic indicators.

Purpose of the Study:

• To compare the predictive value of liver biochemistry, APRI, and patient characteristics for BA outcomes at the time of portoenterostomy.
• To analyze predictors of jaundice clearance and native liver survival.

Main Methods:

• Retrospective observational study involving seven tertiary centers.
• Analysis of predictors for clearance of jaundice (COJ) and native liver survival (NLS) using logistic and Cox regression.
• Definition of CMV-associated BA based on cytomegalovirus detection.

Main Results:

• 58% of 630 patients achieved COJ.
• Absence of cirrhosis, CMV infection, age <60 days at portoenterostomy, and lower AST/APRI predicted COJ.
• COJ, biliary atresia splenic malformation (BASM), and portoenterostomy age >60 days predicted transplantation/death.
• Among COJ patients, BASM predicted transplantation/death.

Conclusions:

• APRI is not superior to AST in predicting BA outcomes.
• In patients achieving COJ, AST and APRI are unrelated to NLS.
• BASM is a strong predictor of shorter NLS, despite younger portoenterostomy age and similar COJ rates.
• COJ is achievable in CMV-positive patients with similar NLS post-COJ.