Long-term neurodevelopmental outcome in patients with biliary atresia.

Rodijk, L. (Lyan) (2015) Long-term neurodevelopmental outcome in patients with biliary atresia. thesis, Medicine.

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Abstract

Background information: Biliary atresia (BA) is a rare liver disease characterized by obliteration and fibrosis of the bile ducts, affecting neonates within the first months after birth. It is, if left untreated, a fatal disease with a median survival of only 8 months. The only cure is resection of the fibrotic tissue and re-establishing bile-flow via a porto-enterostomy (Kasai procedure). Nevertheless, BA is the most frequent indication for liver transplantation in pediatrics. Little is known about the long-term neurodevelopmental outcome in survivors of BA. Study objective: The primary objective is to obtain a better understanding of the neurodevelopmental outcome in school-aged survivors of BA. Secondary objectives are the identification of surgical, medical and/or social factors associated with neurodevelopmental outcome in BA survivors. Methods: This pilot study was an observational cohort study including all surviving patients from the University Medical Centre Groningen who have been diagnosed with BA in the years 2002-2009. To assess neurodevelopmental outcome (motor, cognitive and behavioral), a test battery for the patient and parental questionnaires, to be filled in by the caregivers, were used. All children were school-aged (6-12 years old). The outcomes were correlated with the age-specific Dutch norm. Results: A total of 10 children was included, with only 1 child having a normal total motor score. In the other children, all motor domains were affected; fine motor skills, ball skills and balance, (p=<.001, p=<.001 and p=0.01, respectively). In cognitive outcome, the children showed a lower performance intelligence, selective attention and perceptual ability (p=0.03, p=<.001 and p=0.01, respectively). At school, 56% of the children had learning problems. Furthermore, the parents reported peer problems and behavioral problems of their children, of which especially internalizing problems, attentional problems, hyperactivity and problems in metacognition. Any prognostic factors associated with impaired outcome could not be detected. Conclusions: Our study shows impaired motor and cognitive outcome in school-aged survivors of BA, as well as more behavioral problems at school-age. Further research is needed to confirm these results in a larger cohort and investigate possible factors associated with impaired outcomes.

Item Type:	Thesis (Thesis)
Supervisor name:	Hulscher, dr. J.B.F. and Bruggink, dr. J.L.M.
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:57
Last Modified:	25 Jun 2020 10:57
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/1792

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