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Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study
Vicky L Ng1, Lisa G Sorensen2, Estella M Alonso3
1Division of Pediatric Gastroenterology, Hepatology and Nutrition, Transplant and Regenerative Medicine Center, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Insights
Children with biliary atresia surviving with their native liver face higher risks of neurodevelopmental delays. Unsuccessful surgery significantly increases this risk, highlighting the need for early intervention.
Area of Science:
- Pediatric Gastroenterology
- Neurodevelopmental Pediatrics
- Hepatology
Background:
- Biliary atresia is a severe neonatal liver disease requiring surgical intervention.
- Long-term neurodevelopmental outcomes in children with biliary atresia surviving with native livers are not fully understood.
- Early identification of risk factors for neurodevelopmental impairment is crucial for timely intervention.
Purpose of the Study:
- To assess neurodevelopmental outcomes in children with biliary atresia at 12 and 24 months of age.
- To identify predictors of neurodevelopmental impairment in this population.
- To evaluate the impact of surgical success on neurodevelopmental trajectories.
Main Methods:
- Prospective, longitudinal, multicenter study involving 148 children with biliary atresia.
- Neurodevelopmental testing using Bayley Scales of Infant and Toddler Development (2nd or 3rd edition).
- Logistic regression analysis to identify risk factors for neurodevelopmental impairment.
Main Results:
- Neurodevelopmental scores were significantly lower than normative values at 12 and 24 months.
- Ascites and low length z-scores predicted motor impairment; low weight z-score and ascites predicted cognitive/language impairment at 1 year.
- Unsuccessful hepatoportoenterostomy predicted both motor and cognitive/language impairment at 2 years.
Conclusions:
- Children with biliary atresia surviving with native livers are at increased risk for neurodevelopmental delays.
- Unsuccessful hepatoportoenterostomy is a significant predictor of neurodevelopmental impairment.
- Growth delays and signs of advanced liver disease warrant close monitoring for neurodevelopmental issues.
Objectives:
To assess neurodevelopmental outcomes among participants with biliary atresia with their native liver at ages 12 months (group 1) and 24 months (group 2), and to evaluate variables predictive of neurodevelopmental impairment.
Study Design:
Participants enrolled in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with either the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition. Scores (normative mean = 100 ± 15) were categorized as ≥100, 85-99, and <85 for χ2 analysis. Risk for neurodevelopmental impairment (defined as ≥1 score of <85 on the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition, scales) was analyzed using logistic regression.
Results:
There were 148 children who completed 217 Bayley Scales of Infant and Toddler Development, 3rd edition, examinations (group 1, n = 132; group 2, n = 85). Neurodevelopmental score distributions significantly shifted downward compared with test norms at 1 and 2 years of age. Multivariate analysis identified ascites (OR, 3.17; P = .01) and low length z-scores at time of testing (OR, 0.70; P < .04) as risk factors for physical/motor impairment; low weight z-score (OR, 0.57; P = .001) and ascites (OR, 2.89; P = .01) for mental/cognitive/language impairment at 1 year of age. An unsuccessful hepatoportoenterostomy was predictive of both physical/motor (OR, 4.88; P < .02) and mental/cognitive/language impairment (OR, 4.76; P = .02) at 2 years of age.
Conclusion:
Participants with biliary atresia surviving with native livers after hepatoportoenterostomy are at increased risk for neurodevelopmental delays at 12 and 24 months of age. Those with unsuccessful hepatoportoenterostomy are >4 times more likely to have neurodevelopmental impairment compared with those with successful hepatoportoenterostomy. Growth delays and/or complications indicating advanced liver disease should alert clinicians to the risk for neurodevelopmental delays, and expedite appropriate interventions.
Trial Registration:
Clinicaltrials.gov: NCT00061828 and NCT00294684.
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