JOURNAL ARTICLE

Mortality Determinants in Children with Biliary Atresia Awaiting Liver Transplantation

Ioannis A Ziogas, Fei Ye, Zhiguo Zhao, Shu Cao, Muhammad A Rauf, Manhal Izzy, Lea K Matsuoka, Lynette A Gillis, Sophoclis P Alexopoulos
Journal of Pediatrics 2020 September 17
32950533

OBJECTIVE: To determine risk factors for waitlist mortality in children with biliary atresia listed for liver transplantation.

STUDY DESIGN: 2,704 children with biliary atresia (<12 years) listed for first LT (2002-2018) in the United Network for Organ Sharing database were identified. Fine-Gray regression models for competing risks analysis (main risk=waitlist mortality/delisting due to too sick, competing risk=LT) were implemented to identify risk factors for waitlist mortality.

RESULTS: The median waitlist time was 83 days (IQR: 34-191). The cumulative incidence of waitlist mortality was 5.2%. In multivariable analysis (n=2,253), increasing bilirubin level (p<.001), portal vein thrombosis (p=.03), and ventilator dependence (p<.001) at listing were associated with a higher risk, while weight ≥10 kg at listing (p=0.009) with a lower risk of waitlist mortality. When ascites at listing was included in multivariable analysis (n=1,376), it was associated with a higher risk for the composite outcome (p=.03). Encephalopathy at listing was not associated with waitlist mortality (n=1,376; P = .15).

CONCLUSIONS: These parameters can be utilized to more objectively prioritize children with BA awaiting LT and identify children with BA-related end-stage liver disease at high-risk of mortality.

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