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Neurocognitive and Motor Functions in Biliary Atresia Patients: A Cross-sectional, Prospective National Cohort Study
Satu Ruuska1,2,3, Mikko Lähteenmäki4, Taru Häyrinen5
1Department of Pediatric Gastroenterology.
Insights
Biliary atresia (BA) patients show impaired intelligence quotient (IQ) and motor development compared to norms. Early jaundice clearance is linked to better IQ, highlighting the need for early assessments in BA patients.
Area of Science:
- Pediatric Gastroenterology
- Neurodevelopmental Pediatrics
- Pediatric Surgery
Background:
- Biliary atresia (BA) is a severe neonatal liver disease.
- Long-term neurodevelopmental and motor outcomes in BA patients require further investigation.
- Identifying risk factors is crucial for improving patient prognosis.
Purpose of the Study:
- To assess the neurocognitive and motor development of children and adolescents with BA.
- To identify risk factors associated with impaired neurodevelopmental outcomes in BA patients.
Main Methods:
- A cohort of BA patients aged 1-20 years was recruited from Helsinki University Children's Hospital.
- Neurocognitive development was evaluated using age-appropriate validated tests.
- Motor development was assessed using the Movement Assessment Battery for Children (MABC-2), and parental questionnaires (5-15R) were utilized.
Main Results:
- BA patients exhibited a mean total IQ of 91 ± 15, significantly lower than test norms (P < 0.01).
- Earlier clearance of jaundice (COJ < 3 months) correlated with higher mean total IQ (96 ± 13 vs. 84 ± 13, P < 0.05).
- 43% of participants showed impaired motor development, with reported functional difficulties in daily life.
Conclusions:
- Biliary atresia patients demonstrate moderately impaired IQ and markedly impaired motor skills compared to normative data.
- Standardized cognitive and motor assessments are recommended before school age for early identification of BA patients needing support.
Objectives:
The aim of the study was to evaluate the neurocognitive and motor development of biliary atresia (BA) patients in childhood and adolescence and to identify risk factors for impaired outcome.
Methods:
We invited all BA patients between ages 1 and 20 years followed up at Helsinki University Children's Hospital in Finland between 1 January 2019 to 31 January 2020 to participate. All participants underwent age-appropriate validated neurocognitive tests. Participants between 3.0 and 16.9 years of age were assessed with the Movement Assessment Battery for children, version 2. Guardians of participants between ages 5 and 17 years filled the Five-to-Fifteen-Revised (5-15R) parental questionnaire.
Results:
The mean (±standard deviation [SD]) total intelligence quotient (IQ) of the 39 participants was 91 ± 15, lower compared with test norms (mean IQ 100 ± 15, P < 0.01). Earlier clearance of jaundice (COJ) had a positive effect on mean (±SD) total IQ (COJ <3 months 96 ± 13 vs COJ ≥3 months post-portoenterostomy 84 ± 13, P < 0.05). Out of 30 participants assessed, 13 (43%) were either at risk or fulfilled the criteria for impaired motor development. Guardians reported elevated rates of functional difficulties affecting everyday life. There were no significant differences between native liver and liver transplanted (16/41%) groups.
Conclusions:
IQ is moderately, and motor scores markedly impaired in BA patients compared with normative data. Standardised cognitive and motor assessment before school-age for all BA patients is advisable to identify individuals in need of additional support.
