Affected neuropsychological development, malnutrition and unknown etiology in children with biliary atresia

Biliary atresia children have affected neuropsychological development, malnutrition and unknown etiology. The hypothesis is that affected neuropsychological development can be identified in early childhood and that malnutrition and immunological status are risk factors. We aim to improve the prognosis by mapping the etiology in a prospective study.

A research project by PhD-student Thora Wesenberg Kjær

Biliary atresia is the most severe congenital liver disease. It is a condition where bile flow from the liver to the intestine is restricted resulting in increased plasma levels of bile acids and bilirubin. The disease is associated with high morbidity and mortality, affecting approximately 5 children each year in Denmark, of which 50% require liver transplantation to survive. Among the pathological signs are pruritus, hepatic injury, failure to thrive, and neurodevelopmental delay. There are currently no treatment to alleviate the clinical symptoms of elevated bile acids and their effects on the brain.

The aim of the study is to improve the prognosis for children with biliary atresia by mapping the etiology through comprehensive diagnostic and risk stratification with focus on 
  • Neuro cognitive function
  • Nutritional status
  • Infections and immunological status
  • Motoric development
  • Genetic profile
  • Microbiom
The hypothesis is that affected neuropsychological development can be identified in early childhood and that nutritional status, liver function, immunological status, drug use, hospitalization and late diagnosis are risk factors for poor neuropsychological development. The children with biliary atresia will be followed from diagnosis to 18 years and will be compared with gender and age-matched children with tetra fallot and healthy controls. The study includes neurodevelopmental tests, liver function tests, and investigation of nutritional and immunological status.


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