Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study
Vicky L Ng, Lisa G Sorensen, Estella M Alonso, Emily M Fredericks, Wen Ye, Jeff Moore, Saul J Karpen, Benjamin L Shneider, Jean P Molleston, Jorge A Bezerra, Karen F Murray, Kathleen M Loomes, Philip Rosenthal, Robert H Squires, Kasper Wang, Ronen Arnon, Kathleen B Schwarz, Yumirle P Turmelle, Barbara H Haber, Averell H Sherker, John C Magee, Ronald J Sokol, Childhood Liver Disease Research Network (ChiLDReN), Paula M Hertel, Sanjiv Harpavat, Mary L Brandt, Daniel H Leung, Wikrom Karnsakul, Rebecca Torrance, Sherry Hall, Edward Doo, Jay H Hoofnagle, Peter Whitington, Lee Bass, Alexander G Miethke, James E Heubi, Kenneth Setchell, Kevin E Bove, Greg Tiao, Cara L Mack, Michael R Narkewicz, Amy G Feldman, Shikha S Sundaram, Frederick J Suchy, Frederick M Karrer, Mark Lovell, Johan L Van Hove, Elizabeth B Rand, James E Squires, Veena L Venkat, Rakesh Sindhi, Sarangarajan Ranganathan, Laura Bull, Jeffrey Teckman, Molly Bozic, Girish Subbarao, Simon Horslen, Evelyn Hsu, Laura Finn, Patrick Healey, Rohit Kohli, Danny Thomas, Nisreen Soufi, Sonia Michail, Matt Clifton, Nitika Gupta, Rene Romero, Miriam Vos, Shelley Caltharp, Binita M Kamath, Simon C Ling, Anna Gold, Annie Fecteau, Stephen L Guthery, Kyle Jensen, Rebecka Meyers, Amy Lowichik, Linda Book, Robert M Merion, Cathie Spino, Karen Jones, Vicky L Ng, Lisa G Sorensen, Estella M Alonso, Emily M Fredericks, Wen Ye, Jeff Moore, Saul J Karpen, Benjamin L Shneider, Jean P Molleston, Jorge A Bezerra, Karen F Murray, Kathleen M Loomes, Philip Rosenthal, Robert H Squires, Kasper Wang, Ronen Arnon, Kathleen B Schwarz, Yumirle P Turmelle, Barbara H Haber, Averell H Sherker, John C Magee, Ronald J Sokol, Childhood Liver Disease Research Network (ChiLDReN), Paula M Hertel, Sanjiv Harpavat, Mary L Brandt, Daniel H Leung, Wikrom Karnsakul, Rebecca Torrance, Sherry Hall, Edward Doo, Jay H Hoofnagle, Peter Whitington, Lee Bass, Alexander G Miethke, James E Heubi, Kenneth Setchell, Kevin E Bove, Greg Tiao, Cara L Mack, Michael R Narkewicz, Amy G Feldman, Shikha S Sundaram, Frederick J Suchy, Frederick M Karrer, Mark Lovell, Johan L Van Hove, Elizabeth B Rand, James E Squires, Veena L Venkat, Rakesh Sindhi, Sarangarajan Ranganathan, Laura Bull, Jeffrey Teckman, Molly Bozic, Girish Subbarao, Simon Horslen, Evelyn Hsu, Laura Finn, Patrick Healey, Rohit Kohli, Danny Thomas, Nisreen Soufi, Sonia Michail, Matt Clifton, Nitika Gupta, Rene Romero, Miriam Vos, Shelley Caltharp, Binita M Kamath, Simon C Ling, Anna Gold, Annie Fecteau, Stephen L Guthery, Kyle Jensen, Rebecka Meyers, Amy Lowichik, Linda Book, Robert M Merion, Cathie Spino, Karen Jones
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: Kasai; chronic liver disease; cognitive; motor.
Conflict of interest statement
The other authors declare no conflicts of interest.
Copyright © 2018 Elsevier Inc. All rights reserved.
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Source: PubMed