Neurodevelopmental outcome and quality of life in infants with (congenital) liver disease

Background

Congenital liver disorders often present shortly after birth, and in a large number of infants

this leads to a liver transplantation before the age of 18 years. Previous studies showed that

children who underwent a liver transplantation often have neuropsychological developmental

problems. Recently we performed a pilot study in which the neuropsychological development

at school age (6-12 years) in children with biliary atresia was examined. This analysis

showed that these children achieve poorer results in motor, cognitive and behavioral tests

compared to their healthy peers. The reason for these developmental problems is still

unclear.



Methods

All children born in the Netherlands and Denmark with congenital

liver disease will be asked to undergo validated neuropsychological tests, at different ages (3

months post-term, 18 months and between 6 and 12 years old). The results of these tests

are correlated with different clinical parameters and risk factors to identify those children at

high-risk for developmental problems.

Expected results

We expect to give an overview of the neuropsychological development of children born with

congenital liver disease at school age. And furthermore to identify risk factors, in this

population, for impaired neuropsychological development. Early identification of those infants

at high risk for developmental problems may give us the ability to provide extra guidance or

treatment in order to promote their quality of life and social participation.

Infants with congenital liver disease and/or who underwent a liver transplantation < 5 year will have more neurodevelopmental problems and an impaired quality of life at school age when compared to healthy peers.

School age (6-12 years of age)

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