Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia

J Pediatr. 2016 Mar;170:211-7.e1-2. doi: 10.1016/j.jpeds.2015.11.058. Epub 2015 Dec 24.

Abstract

Objectives: To prospectively assess the value of serum total bilirubin (TB) within 3 months of hepatoportoenterostomy (HPE) in infants with biliary atresia as a biomarker predictive of clinical sequelae of liver disease in the first 2 years of life.

Study design: Infants with biliary atresia undergoing HPE between June 2004 and January 2011 were enrolled in a prospective, multicenter study. Complications were monitored until 2 years of age or the earliest of liver transplantation (LT), death, or study withdrawal. TB below 2 mg/dL (34.2 μM) at any time in the first 3 months (TB <2.0, all others TB ≥ 2) after HPE was examined as a biomarker, using Kaplan-Meier survival and logistic regression.

Results: Fifty percent (68/137) of infants had TB < 2.0 in the first 3 months after HPE. Transplant-free survival at 2 years was significantly higher in the TB < 2.0 group vs TB ≥ 2 (86% vs 20%, P < .0001). Infants with TB ≥ 2 had diminished weight gain (P < .0001), greater probability of developing ascites (OR 6.4, 95% CI 2.9-14.1, P < .0001), hypoalbuminemia (OR 7.6, 95% CI 3.2-17.7, P < .0001), coagulopathy (OR 10.8, 95% CI 3.1-38.2, P = .0002), LT (OR 12.4, 95% CI 5.3-28.7, P < .0001), or LT or death (OR 16.8, 95% CI 7.2-39.2, P < .0001).

Conclusions: Infants whose TB does not fall below 2.0 mg/dL within 3 months of HPE were at high risk for early disease progression, suggesting they should be considered for LT in a timely fashion. Interventions increasing the likelihood of achieving TB <2.0 mg/dL within 3 months of HPE may enhance early outcomes.

Trial registration: ClinicalTrials.gov: NCT00061828 and NCT00294684.

Publication types

  • Multicenter Study
  • Observational Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Ascites / epidemiology
  • Biliary Atresia / epidemiology
  • Biliary Atresia / surgery*
  • Bilirubin / blood*
  • Biomarkers / blood
  • Canada / epidemiology
  • Child, Preschool
  • Databases, Factual
  • Disease Progression*
  • Disseminated Intravascular Coagulation / epidemiology
  • Follow-Up Studies
  • Growth Disorders / epidemiology
  • Humans
  • Hypoalbuminemia / epidemiology
  • Infant
  • Infant, Newborn
  • Liver Transplantation / statistics & numerical data
  • Logistic Models
  • Portoenterostomy, Hepatic*
  • Prognosis
  • Prospective Studies
  • United States / epidemiology

Substances

  • Biomarkers
  • Bilirubin

Associated data

  • ClinicalTrials.gov/NCT00061828
  • ClinicalTrials.gov/NCT00294684

Grant support