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

James E. Squires, Vicky Lee Ng, Kieran Hawthorne, Lisa Henn, Lisa G. Sorensen, Emily M. Fredericks, Estella M. Alonso, Karen F. Murray, Kathleen M. Loomes, Saul J. Karpen, Laurel A. Cavallo, Jean 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 journalArticle

Abstract

Objectives: 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. Methods: Participants (age 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, age 3-5 yrs.) and Weschler Intelligence Scale for Children, 4th 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. 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 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. Conclusion: 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.

Original languageEnglish (US)
JournalJournal of pediatric gastroenterology and nutrition
DOIs
StateAccepted/In press - Jan 1 2019

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Biliary Atresia
Intelligence
Liver
Portal Hypertension
Transferases
Bilirubin
Normal Distribution
Multicenter Studies
Longitudinal Studies
Liver Diseases
Linear Models
Language
Education

Keywords

  • chronic liver disease
  • cognitive
  • pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Gastroenterology

Cite this

Neurodevelopmental Outcomes in Pre-School and School Aged Children with Biliary Atresia and their Native Liver. / Squires, James E.; Ng, Vicky Lee; Hawthorne, Kieran; Henn, Lisa; Sorensen, Lisa G.; Fredericks, Emily M.; Alonso, Estella M.; Murray, Karen F.; Loomes, Kathleen M.; Karpen, Saul J.; Cavallo, Laurel A.; Molleston, Jean; Bezerra, Jorge A.; Rosenthal, Philip; Squires, Robert H.; Wang, Kasper S.; Schwarz, Kathleen B.; Arnon, Ronen; Magee, John C.; Sokol, Ronald J.

In: Journal of pediatric gastroenterology and nutrition, 01.01.2019.

Research output: Contribution to journalArticle

Squires, JE, Ng, VL, Hawthorne, K, Henn, L, Sorensen, LG, Fredericks, EM, Alonso, EM, Murray, KF, Loomes, KM, Karpen, SJ, Cavallo, LA, Molleston, J, Bezerra, JA, Rosenthal, P, Squires, RH, Wang, KS, Schwarz, KB, Arnon, R, Magee, JC & Sokol, RJ 2019, 'Neurodevelopmental Outcomes in Pre-School and School Aged Children with Biliary Atresia and their Native Liver', Journal of pediatric gastroenterology and nutrition. https://doi.org/10.1097/MPG.0000000000002489
Squires, James E. ; Ng, Vicky Lee ; Hawthorne, Kieran ; Henn, Lisa ; Sorensen, Lisa G. ; Fredericks, Emily M. ; Alonso, Estella M. ; Murray, Karen F. ; Loomes, Kathleen M. ; Karpen, Saul J. ; Cavallo, Laurel A. ; Molleston, Jean ; Bezerra, Jorge A. ; Rosenthal, Philip ; Squires, Robert H. ; Wang, Kasper S. ; Schwarz, Kathleen B. ; Arnon, Ronen ; Magee, John C. ; Sokol, Ronald J. / Neurodevelopmental Outcomes in Pre-School and School Aged Children with Biliary Atresia and their Native Liver. In: Journal of pediatric gastroenterology and nutrition. 2019.
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abstract = "Objectives: 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. Methods: Participants (age 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, age 3-5 yrs.) and Weschler Intelligence Scale for Children, 4th 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. 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 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. Conclusion: 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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T1 - Neurodevelopmental Outcomes in Pre-School and School Aged Children with Biliary Atresia and their Native Liver

AU - Squires, James E.

AU - Ng, Vicky Lee

AU - Hawthorne, Kieran

AU - Henn, Lisa

AU - Sorensen, Lisa G.

AU - Fredericks, Emily M.

AU - Alonso, Estella M.

AU - Murray, Karen F.

AU - Loomes, Kathleen M.

AU - Karpen, Saul J.

AU - Cavallo, Laurel A.

AU - Molleston, Jean

AU - Bezerra, Jorge A.

AU - Rosenthal, Philip

AU - Squires, Robert H.

AU - Wang, Kasper S.

AU - Schwarz, Kathleen B.

AU - Arnon, Ronen

AU - Magee, John C.

AU - Sokol, Ronald J.

PY - 2019/1/1

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N2 - Objectives: 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. Methods: Participants (age 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, age 3-5 yrs.) and Weschler Intelligence Scale for Children, 4th 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. 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 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. Conclusion: 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.

AB - Objectives: 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. Methods: Participants (age 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, age 3-5 yrs.) and Weschler Intelligence Scale for Children, 4th 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. 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 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. Conclusion: 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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KW - cognitive

KW - pediatric

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