Clinical characteristics and prognostic factors of mortality in pediatric patients with biliary atresia awaiting liver transplantation

BackgroundDespite advancements in Kasai portoenterostomy (KP) for biliary atresia (BA), 70-80% of patients require liver transplants with long waiting periods time. This study aims to investigate factors associated with mortality in BA patients awaiting liver transplantation. MethodsThis cross-sectional study included BA patients indicated for liver transplantation from May 1, 2023 to August 30, 2024. Factors associated with the survival outcome were explored using univariable and multivariable logistic regression. The effect of the KP on overall survival was assessed through Kaplan-Meier survival analysis and multivariable Cox regression. ResultsThis study included 97 patients with a mortality rate of 16.5%. Multivariable logistic regression showed that KP (adjusted OR (aOR): 0.119; 95% CI: 0.021-0.678; P-value: 0.016), low pediatric end-stage liver disease (PELD) scores (aOR: 1.201; 95% CI: 1.074-1.343; P-value: 0.001), and high weight-for-age (WA) percentiles (aOR: 0.940; 95% CI: 0.884-0.999; P-value: 0.047) were associated with lower mortality. Sub-group analyses in patients with KP also showed low PELD scores (aOR: 1.155; 95% CI: 1.020-1.309; P-value: 0.023) related to lower mortality, whereas high GGT levels showed a potential association with survival (OR: 0.990; 95%CI: 0.981-1.000; P-value: 0.039). Further survival analysis demonstrated the prognostic value of KP (adjusted hazard ratio: 0.261; 95% CI: 0.088-0.770; P-value: 0.015). ConclusionKP status and PELD scores are significantly associated with mortality risk in BA patients waiting for liver transplants, suggesting their potential role as early indicators for liver transplant prioritization. These findings are significant for informing follow-up care and early intervention strategies, though additional validation is necessary.