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

• Type of Material: Article
• Authors: Vicky L. Ng, University of Toronto; Lisa G. Sorensen, Northwestern University; Estella M. Alonso, Northwestern University; Emily M. Fredericks, University of Michigan; Wen Ye, University of Michigan; Jeff Moore, University of Michigan; Saul Karpen, Emory University; Benjamin L. Shneider, Baylor College of Medicine; Jean P. Molleston, Indiana University School of Medicine; Jorge A. Bezerra, Cincinnati Children's Hospital Medical Center; Karen F. Murray, University of Washington & Seattle Children's; Kathleen M. Loomes, Children's Hospital of Philadelphia; Philip Rosenthal, University of California, San Francisco; Robert H. Squires, Children's Hospital of Pittsburgh; Kasper Wang, University of Southern California; Ronen Arnon, Mount Sinai Hospital; Kathleen B. Schwarz, Johns Hopkins School of Medicine; Matthew Clifton, Emory University; Nitika Gupta, Emory University; Rene Romero, Emory University; Shelley Caltharp, Emory University
• Language: English
• Date: 2018-05-01
• Publisher: Elsevier: 12 months
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2018 Elsevier Inc. All rights reserved.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.jpeds.2017.12.048
• Title of Journal or Parent Work: Journal of Pediatrics
• ISSN: 0022-3476
• Volume: 196
• Start Page: 139
• End Page: +
• Grant/Funding Information: For complete funding information, please see the full article.
• Keywords: TRANSPLANTATION; Pediatrics; AGE; TOTAL SERUM BILIRUBIN; GENERAL-ANESTHESIA; CORTICOSTEROIDS; BAYLEY-III; PORTAL-VEIN OBSTRUCTION; DISEASE; MOTOR DEVELOPMENT; Science & Technology; INFANTS; Life Sciences & Biomedicine
• Research Categories: Biology, Neuroscience; Health Sciences, Medicine and Surgery

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 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.