Presentation and treatment of biliary atresia

Biliary atresia (BA) is a rare disease of infancy in which obliteration of the biliary tract results in cholestasis and if left untreated in progressive liver failure and death at very young age. Kasai surgery is the only treatment that can postpone or prevent liver failure. Timely surgical correction is an important prognostic factor. However, two-thirds of patients still need a liver transplantation at child age. The aim of this thesis was to identify targets for improvement in diagnosis and treatment of BA. Therefore, we used our nationwide registry with patient data of all BA patients since 1987. Insufficient recognition of early symptoms of BA, such as acholic stools, might be an important underlying contributor to the delay in diagnosis and therefore to late surgery. Implementation of the Infant Stool Colour Card might lead to earlier diagnosis and treatment of BA, and thereby to significantly improved outcome.Our data show that the Dutch regimen of vitamin K prophylaxis in form of oral vitamin K drops fails to prevent Vitamin K Deficiency Bleeding in breastfed infants with BA. Based on our results, in 2017 the Health Council of the Netherlands recommended adaption of the Dutch guideline towards a regimen of a single injection of vitamin K at birth. Until now, the minister has not yet implemented this advice.Reaching native liver survival at the age of 2 years is an important prognostic milestone for BA patients. Almost 90% of these patients reach adulthood, and the majority even without liver transplantation