INTRODUCTION: Biliary atresia is the leading cause of liver transplantation in children. It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four children are born every year with biliary atresia. Early correction of biliary obstruction is essential to prevent fatal biliary cirrhosis. The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of children with elevated level of serum bilirubin after two weeks of age. Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 μmol/l, and/or more than 20% of total bilirubin. This percentage value has caused diagnostic trouble over the years. The objective of the present study was to investigate the possibility of changing the recommendations.

METHODS: This was a retrospective analysis of the medical records of children operated for biliary atresia in the 1993-2012 period.

RESULTS: During the period, 73 patients where operated with a portoenterostomy ad modum Kasai. Patients older than 84 days at the time of operation were excluded, 54 patients were available for analysis. Conjugated bilirubin in μmol/l and the percentage value were significantly above the DHMA threshold limit: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively.

CONCLUSION: The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion.

FUNDING: none.

TRIAL REGISTRATION: not relevant.