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Neurodevelopmental Outcomes in Preschool and School Aged Children With Biliary Atresia and Their Native Liver
James E Squires1, Vicky Lee Ng2, Kieran Hawthorne3
1Division of Pediatric Gastroenterology and Hepatology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA.
Insights
Children with biliary atresia (BA) surviving with their native liver show normal neurodevelopment. Advanced liver disease markers correlate with lower IQ, identifying a subset needing intervention.
Area of Science:
- Pediatric Hepatology
- Neurodevelopmental Pediatrics
- Longitudinal Cohort Studies
Background:
- Biliary atresia (BA) is a severe neonatal liver disease.
- Long-term neurodevelopmental outcomes for BA survivors with native livers are not well-established.
- Understanding neurodevelopmental trajectories is crucial for post-transplant care.
Purpose of the Study:
- To assess neurodevelopmental outcomes in children with BA aged 3-12 years surviving with their native liver.
- To identify factors associated with neurodevelopmental performance in this cohort.
Main Methods:
- Prospective, longitudinal, multicenter study of 93 children (ages 3-12).
- Neurodevelopmental testing using Wechsler Preschool and Primary Scale of Intelligence (WPPSI-III) and Wechsler Intelligence Scale for Children (WISC-IV).
- Statistical analysis included Kolmogorov-Smirnov tests and linear regression for covariate effects on Full-Scale Intelligence Quotient (FSIQ).
Main Results:
- No increased prevalence of neurodevelopmental delays observed in BA survivors.
- WPPSI-III and WISC-IV scores were generally within or above normal ranges.
- Parental education predicted higher FSIQ; male sex, elevated bilirubin/GGT (age ≤5), and portal hypertension (age ≥6) predicted lower FSIQ.
Conclusions:
- Children with BA surviving with native livers do not exhibit a higher prevalence of neurodevelopmental delays.
- Markers of advanced liver disease correlate with lower FSIQ, indicating a vulnerable subgroup.
- Early identification of these markers may guide targeted interventions for improved neurodevelopmental outcomes.
Objectives:
The aim of the study was to assess neurodevelopmental outcomes among children with biliary atresia (BA) surviving with their native liver at ages 3 to 12 years and evaluate variables that associate with neurodevelopment.
Methods:
Participants (ages 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, ages 3-5 years) and Weschler Intelligence Scale for Children, 4th edition (WISC-IV, ages 6-12 years). Continuous scores were analyzed using Kolmogorov-Smironov tests compared with a normal distribution (mean = 100 ± 15). Effect of covariates on Full-Scale Intelligence Quotient (FSIQ) was analyzed using linear regression.
Results:
Ninety-three participants completed 164 WPPSI-III (mean age 3.9) and 51 WISC-IV (mean age 6.9) tests. WPPSI-III FSIQ (104 ± 14, P < 0.02), Verbal IQ (106 ± 14, P < 0.001), and General Language Composite (107 ± 16, P < 0.001) distributions were shifted higher compared with test norms. WISC-IV FSIQ (105 ± 12, P < 0.01), Perceptual Reasoning Index (107 ± 12, P < 0.01), and Processing Speed Index (105 ± 10, P < 0.02) also shifted upwards. In univariate and multivariable analysis, parent education (P < 0.01) was a significant predictor of FSIQ on WPPSI-III and positively associated with WISC-IV FSIQ. Male sex and higher total bilirubin and gamma glutamyl transferase (GGT) predicted lower WPPSI-III FSIQ. Portal hypertension was predictive of lower WISC-IV FSIQ.
Conclusions:
This cohort of children with BA and native liver did not demonstrate higher prevalence of neurodevelopmental delays. Markers of advanced liver disease (higher total bilirubin and GGT for age ≤5 years; portal hypertension for age ≥6) correlate with lower FSIQ and may identify a vulnerable subset of patients who would benefit from intervention.
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