Vicky L Ng1, Lisa G Sorensen2, Estella M Alonso3, Emily M Fredericks4, Wen Ye5, Jeff Moore5, Saul J Karpen6, Benjamin L Shneider7, Jean P Molleston8, Jorge A Bezerra9, Karen F Murray10, Kathleen M Loomes11, Philip Rosenthal12, Robert H Squires13, Kasper Wang14, Ronen Arnon15, Kathleen B Schwarz16, Yumirle P Turmelle17, Barbara H Haber11, Averell H Sherker18, John C Magee19, Ronald J Sokol20. 1. Division of Pediatric Gastroenterology, Hepatology and Nutrition, Transplant and Regenerative Medicine Center, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: vicky.ng@sickkids.ca. 2. Department of Child & Adolescent Psychiatry, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL. 3. Division of Gastroenterology, Hepatology, and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL. 4. C.S. Mott Children's Hospital, University of Michigan Medical School, Ann Arbor, MI. 5. Department of Biostatistics, University of Michigan, Ann Arbor, MI. 6. Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Emory University School of Medicine/Children's Healthcare of Atlanta, Atlanta, GA. 7. Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Baylor College of Medicine, Houston, TX. 8. Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Indiana University School of Medicine, Rylie Hospital for Children, Indianapolis, IN. 9. Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. 10. Division of Gastroenterology and Hepatology, Department of Pediatrics, University of Washington and Seattle Children's, Seattle, WA. 11. Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA. 12. Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of California, San Francisco Benioff Children's Hospital, San Francisco, CA. 13. Children's Hospital of Pittsburgh, Pittsburgh, PA. 14. Division of Pediatric Surgery, Children's Hospital Los Angeles, University of Southern California, Los Angeles, CA. 15. Division of Gastroenterology, Hepatology and Nutrition, Mount Sinai Hospital, New York, NY. 16. Johns Hopkins School of Medicine, Baltimore, MD. 17. Washington University School of Medicine, St. Louis, MO. 18. Liver Diseases Research Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD. 19. Department of Surgery, University of Michigan Medical School, Ann Arbor, MI. 20. Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO.
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.
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.
Authors: Noelle Younge; Ricki F Goldstein; Carla M Bann; Susan R Hintz; Ravi M Patel; P Brian Smith; Edward F Bell; Matthew A Rysavy; Andrea F Duncan; Betty R Vohr; Abhik Das; Ronald N Goldberg; Rosemary D Higgins; C Michael Cotten Journal: N Engl J Med Date: 2017-02-16 Impact factor: 91.245
Authors: Nicole Lind Henriksen; Svend Høime Hansen; Matthew Domenic Lycas; Xiaoyu Pan; Thomas Eriksen; Lars Søndergaard Johansen; Richard R Sprenger; Christer Stenby Ejsing; Douglas G Burrin; Kerstin Skovgaard; Vibeke Brix Christensen; Thomas Thymann; Stanislava Pankratova Journal: Physiol Rep Date: 2022-07
Authors: Daniel H Leung; Lisa G Sorensen; Wen Ye; Kieran Hawthorne; Vicky L Ng; Kathleen M Loomes; Emily M Fredericks; Estella M Alonso; James E Heubi; Simon P Horslen; Saul J Karpen; Jean P Molleston; Philip Rosenthal; Ronald J Sokol; Robert H Squires; Kasper S Wang; Binita M Kamath; John C Magee Journal: J Pediatr Gastroenterol Nutr Date: 2022-01-01 Impact factor: 3.288