Biliary Atresia Research Consortium (BARC)

General Description

The BARC study was a retrospective study that analyzed the results of a large cohort of patients with biliary atresia from US institutions with expertise in management of children with liver disease. The study consisted of a comprehensive retrospective chart review of clinical data and outcomes for all patients with biliary atresia undergoing HPE between January 1, 1997 and December 31, 2000 at 9 clinical centers. All children included in the study were followed for 2 years or until loss of the native liver as a result of transplantation or death. The data showed that there was no standard approach to the diagnosis of biliary atresia and that postsurgical management varied considerably among the BARC centers; nevertheless, the outcomes at age 2 years for the 9 centers were comparable to the best outcome data published from other countries and in large single-center reports. The study also showed that total bilirubin in early follow-up after HPE was highly predictive of outcome.

Data from the P001 and P002 protocols are now available.

Objectives

The primary aim of the BARC study was to collect and correlate data about the presentation, diagnostic interventions, and medical and surgical management of infants with biliary atresia in order to identify risk factors that affect outcomes at two years of age.

Outcome Measure

Survival, transplant status, and serum bilirubin levels were used to measure outcome. Good outcome was defined as alive with native liver and total serum bilirubin level < 6.0 mg/dL at age 24 months; poor outcome was defined as either death or liver transplantation before age 24 months; and indeterminate outcome was defined as alive with native liver but with serum total bilirubin level > 6.0 mg/dL.

Criteria

The study analyzed the case records of children born between January 1, 1997 and December 31, 2000 who were diagnosed with biliary atresia and had hepatopotoenterostomy (HPE) performed as surgical therapy. Children with a diagnosis of choledochal cyst or of an additional disease manifesting as cholestatic liver disease were excluded from the study.

Outcome

The study found that contemporary management of biliary atresia in the 9 US centers resulted in outcomes equivalent to those reported from Western Europe and Japan despite significant variability in the approaches to diagnosis and management among the BARC centers and differences in approach with respect to other countries. Total serum bilirubin level measured at 3 months post-HPE appeared to be a biomarker that was predictive of subsequent outcome. Additionally, the data showed that the coexistence of splenic malformations with biliary atresia (BASM syndrome) had negative implications with regard to outcome.