Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study

Childhood Liver Disease Research Network (ChiLDReN)

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)139-147.e3
JournalJournal of Pediatrics
Volume196
DOIs
StatePublished - May 1 2018

Fingerprint

Biliary Atresia
Child Development
Liver
Multicenter Studies
Longitudinal Studies
Liver Diseases
Growth

Keywords

  • chronic liver disease
  • cognitive
  • Kasai
  • motor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver : Results from the ChiLDReN Study. / Childhood Liver Disease Research Network (ChiLDReN).

In: Journal of Pediatrics, Vol. 196, 01.05.2018, p. 139-147.e3.

Research output: Contribution to journalArticle

Childhood Liver Disease Research Network (ChiLDReN). / Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver : Results from the ChiLDReN Study. In: Journal of Pediatrics. 2018 ; Vol. 196. pp. 139-147.e3.
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title = "Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study",
abstract = "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.",
keywords = "chronic liver disease, cognitive, Kasai, motor",
author = "{Childhood Liver Disease Research Network (ChiLDReN)} and Ng, {Vicky L.} and Sorensen, {Lisa G.} and Alonso, {Estella M.} and Fredericks, {Emily M.} and Wen Ye and Jeff Moore and Karpen, {Saul J.} and Shneider, {Benjamin L.} and Jean Molleston and Bezerra, {Jorge A.} and Murray, {Karen F.} and Loomes, {Kathleen M.} and Philip Rosenthal and Squires, {Robert H.} and Kasper Wang and Ronen Arnon and Schwarz, {Kathleen B.} and Turmelle, {Yumirle P.} and Haber, {Barbara H.} and Sherker, {Averell H.} and Magee, {John C.} and Sokol, {Ronald J.} and Hertel, {Paula M.} and Sanjiv Harpavat and Brandt, {Mary L.} and Leung, {Daniel H.} and Wikrom Karnsakul and Rebecca Torrance and Sherry Hall and Edward Doo and Hoofnagle, {Jay H.} and Peter Whitington and Lee Bass and Miethke, {Alexander G.} and Heubi, {James E.} and Kenneth Setchell and Bove, {Kevin E.} and Greg Tiao and Mack, {Cara L.} and Narkewicz, {Michael R.} and Feldman, {Amy G.} and Sundaram, {Shikha S.} and Suchy, {Frederick J.} and Karrer, {Frederick M.} and Mark Lovell and {Van Hove}, {Johan L.} and Rand, {Elizabeth B.} and Squires, {James E.} and Venkat, {Veena L.} and Girish Subbarao",
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TY - JOUR

T1 - Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver

T2 - Results from the ChiLDReN Study

AU - Childhood Liver Disease Research Network (ChiLDReN)

AU - Ng, Vicky L.

AU - Sorensen, Lisa G.

AU - Alonso, Estella M.

AU - Fredericks, Emily M.

AU - Ye, Wen

AU - Moore, Jeff

AU - Karpen, Saul J.

AU - Shneider, Benjamin L.

AU - Molleston, Jean

AU - Bezerra, Jorge A.

AU - Murray, Karen F.

AU - Loomes, Kathleen M.

AU - Rosenthal, Philip

AU - Squires, Robert H.

AU - Wang, Kasper

AU - Arnon, Ronen

AU - Schwarz, Kathleen B.

AU - Turmelle, Yumirle P.

AU - Haber, Barbara H.

AU - Sherker, Averell H.

AU - Magee, John C.

AU - Sokol, Ronald J.

AU - Hertel, Paula M.

AU - Harpavat, Sanjiv

AU - Brandt, Mary L.

AU - Leung, Daniel H.

AU - Karnsakul, Wikrom

AU - Torrance, Rebecca

AU - Hall, Sherry

AU - Doo, Edward

AU - Hoofnagle, Jay H.

AU - Whitington, Peter

AU - Bass, Lee

AU - Miethke, Alexander G.

AU - Heubi, James E.

AU - Setchell, Kenneth

AU - Bove, Kevin E.

AU - Tiao, Greg

AU - Mack, Cara L.

AU - Narkewicz, Michael R.

AU - Feldman, Amy G.

AU - Sundaram, Shikha S.

AU - Suchy, Frederick J.

AU - Karrer, Frederick M.

AU - Lovell, Mark

AU - Van Hove, Johan L.

AU - Rand, Elizabeth B.

AU - Squires, James E.

AU - Venkat, Veena L.

AU - Subbarao, Girish

PY - 2018/5/1

Y1 - 2018/5/1

N2 - 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.

AB - 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.

KW - chronic liver disease

KW - cognitive

KW - Kasai

KW - motor

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