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J Pediatr Gastroenterol Nutr. 2019 Sep 6. doi: 10.1097/MPG.0000000000002489. [Epub ahead of print]

Neurodevelopmental Outcomes in Pre-School and School Aged Children with Biliary Atresia and their Native Liver.

Author information

Division of Pediatric Gastroenterology and Hepatology, Department of Pediatrics, University of Pittsburgh School of Medicine.
Division of GI, Hepatology and Nutrition, Hospital for Sick Children and University of Toronto, Toronto, ON, Canada.
Arbor Research Collaborative for Health, Ann Arbor, MI.
Department of Pediatrics, Michigan Medicine, Ann Arbor, Michigan.
Ann and Robert Lurie Children's Hospital, Chicago, IL.
Division of GI and Hepatology, University of Washington School of Medicine and Seattle Children's Hospital, Seattle, WA.
Division of GI, Hepatology and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, PA.
Division of GI, Hepatology and Nutrition, Emory University School of Medicine, Atlanta, GA.
Division of GI, Hepatology and Nutrition, Baylor College of Medicine, Houston, TX.
Division of GI, Hepatology and Nutrition, Indiana University, Riley Hospital for Children, Indianapolis, IN.
Division of GI, Hepatology and Nutrition, Cincinnati Children's Medical Center, Cincinnati, OH.
Division of GI, Hepatology and Nutrition, UCSF, San Francisco, CA.
Department of Pediatric Surgery, Children's Hospital Los Angeles,CA.
Johns Hopkins School of Medicine, Baltimore, MD.
Division of GI, Hepatology and Nutrition, Mt. Sinai Medical Center, New York, NY.
Department of Surgery, University of Michigan School of Medicine, Ann Arbor, MI.
Section of Pediatric GI, Hepatology and Nutrition, Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, CO.



To assess neurodevelopmental outcomes among children with biliary atresia (BA) surviving with their native liver at age 3-12 years and evaluate variables that associate with neurodevelopment.


Participants (age 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3 edition (WPPSI-III, age 3-5 yrs.) and Weschler Intelligence Scale for Children, 4 edition (WISC-IV, age 6-12 yrs.). Continuous scores were analyzed using Kolmogorov-Smironov tests compared to a normal distribution (mean = 100 ± 15). Effect of covariates on Full-Scale Intelligence Quotient (FSIQ) was analyzed using linear regression.


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 to 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.


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 yrs; 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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