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 P.
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 - Sindhi, Rakesh
N1 - Publisher Copyright:
© 2018 Elsevier Inc.
PY - 2018/5
Y1 - 2018/5
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 χ 2 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 χ 2 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 - Kasai
KW - chronic liver disease
KW - cognitive
KW - motor
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U2 - 10.1016/j.jpeds.2017.12.048
DO - 10.1016/j.jpeds.2017.12.048
M3 - Article
C2 - 29519540
AN - SCOPUS:85043331672
SN - 0022-3476
VL - 196
SP - 139-147.e3
JO - Journal of Pediatrics
JF - Journal of Pediatrics
ER -