INTRODUCTION: @entity2876 is the leading cause of liver transplantation in @entity1 . It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four @entity1 are born every year with @entity2876 . Early correction of @entity253 is essential to prevent @entity2835 . The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of @entity1 with elevated level of serum @entity648 after two weeks of age. @entity2876 has to be excluded if conjugated @entity648 level is above than 20  mol/l, and/or more than 20% of total @entity648 . 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 @entity1 operated for @entity2876 in the 1993-2012 period. RESULTS: During the period, 73 @entity1 where operated with a portoenterostomy ad modum Kasai. @entity1 older than 84 days at the time of operation were excluded, 54 @entity1 were available for analysis. Conjugated @entity648 in  mol/l and the percentage value were significantly above the DHMA threshold limit: mean 129.7  mol/l (42-334  mol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated @entity648 above 20  mol/l is sufficient to require further evaluation for @entity2876 . The percentage value is unnecessary and may cause @entity911 . FUNDING: none. TRIAL REGISTRATION: not relevant.

Increased conjugated XXXX is sufficient to initiate screening for @entity2876 .

@entity1 :: ('9606', 'Species') :: ['children', 'Patients', 'patients'] @entity2835 :: ('MESH:D008105', 'Disease') :: ['fatal biliary cirrhosis'] @entity648 :: ('MESH:D001663', 'Chemical') :: ['bilirubin'] @entity911 :: ('MESH:D003221', 'Disease') :: ['confusion'] @entity2876 :: ('MESH:D001656', 'Disease') :: ['Biliary atresia', 'biliary atresia'] @entity253 :: ('MESH:D001660', 'Disease') :: ['biliary obstruction']
@entity648 :: (MESH:D001663,Chemical) :: ['bilirubin']
