Neurodevelopmental Outcomes in Preschool and School Aged Children with Biliary Atresia and Their Native Liver

James E. Squires, Vicky Lee Ng, Kieran Hawthorne, Lisa L. Henn, Lisa G. Sorensen, Emily M. Fredericks, Estella M. Alonso, Karen F. Murray, Kathleen M. Loomes, Saul J. Karpen, Laurel A. Cavallo, Jean P. Molleston, Jorge A. Bezerra, Philip Rosenthal, Robert H. Squires, Kasper S. Wang, Kathleen B. Schwarz, Ronen Arnon, John C. Magee, Ronald J. Sokol

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)79-86
Number of pages8
JournalJournal of pediatric gastroenterology and nutrition
Issue number1
StatePublished - Jan 1 2020


  • chronic liver disease
  • cognitive
  • pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Gastroenterology


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