studies

HEPATOLOGY, VOLUME 62, NUMBER 1 (SUPPL) AASLD ABSTRACTS 277A
133
Neurodevelopmental Outcomes in Patients with Biliary
Atresia and Native Liver at Ages 1 and 2 Years: Results
from ChiLDReN
Vicky L. Ng 1 , Lisa G. Sorensen 16 , Estella M. Alonso 2 , Emily M.
Fredericks 3 , Wen Ye 21 , Saul J. Karpen 17 , Benjamin Shneider 18 ,
Jorge A. Bezerra 4 , Jean P. Molleston 5 , Karen F. Murray 6 , Philip
Rosenthal 7 , Kasper S. Wang 19 , Kathleen M. Loomes 8 , Paula M.
Hertel 9 , Nanda Kerkar 20,22 , Kathleen B. Schwarz 10 , Yumirle P.
Turmelle 11 , Barbara A. Haber 12 , Averell H. Sherker 15 , John C.
Magee 13 , Ronald J. Sokol 14 ; 1 Division of Pediatric Gastroenterology,
Hepatology and Nutrition, The Hospital for Sick Children,
University of Toronto, Toronto, ON, Canada; 2 Division of Pediatric
Gastroenterology, Hepatology and Nutrition, Ann & Robert H.
Lurie Children’s Hospital, Chicago, IL; 3 Division of Child Behavioral
Health, University of Michigan and C.S. Mott Children’s
Hospital, Ann Arbor, MI; 4 Division of Pediatric Gastroenterology,
Hepatology and Nutrition, Cincinnat Children’s Hospital Medical,
Cincinnati, OH; 5 Pediatric Gastroenterology, Hepatology
and Nutrition, Indiana Universtiy School of Medicine/Riley Hospital
for Children, Indianapolis, IN; 6 Division of Gastroenterology
and Hepatology, University of Washington Medical Center,
Seattle Children’s, Seattle, WA; 7 Division of Gastroenterology,
Hepatology and Nutrition, Department of Pediatrics, University
of California, San Francisco, San Francisco, CA; 8 Pediatric Gastroenterology,
Hepatology and Nutrition, Children’s Hospital of
Philadelphia, Philadelphia, PA; 9 Baylor College of Medicine,
Houston, TX; 10 Johns Hopkins School of Medicine, Baltimore, MD;
11 Pediatric Gastroenterology, Hepatology and Nutrition, Washington
University School of Medicine, St. Louis, MO; 12 Infectious
Disease, Clinical Research, Merck, North Wales, PA; 13 University
of Michigan Medical School, Ann Arbor, MI; 14 Section of Pediatric
Gastroenterology, Hepatology and Nutrition, Department of Pediatrics,
University of Colorado School of Medicine, Denver, CO;
15 Liver Diseases Research Branch, National Institute of Diabetes
and Digestive and Kidney Diseases, National Institutes of Health,
Bethesda, MD; 16 Ann & Robert H. Lurie Children’s Hospital of
Chicago, Northwestern University Feinberg School of Medicine,
Chicago, IL; 17 Pediatric Gastroenterology, Hepatology and Nutrition,
Emory University School of Medicine/Children’s Healthcare
of Atlanta, Atlanta, GA; 18 Pediatric Gastroenterology, Hepatology
and Nutrition, Baylor College of Medicine, Houston, TX; 19 Division
of Pediatric Surgery, Children’s Hospital Los Angeles, Los Angeles,
CA; 20 Children’s Hospital of Los Angeles, Los Angeles, CA;
21 Department of Biostatistics, University of Michigan, Ann Arbor,
MI; 22 Mount Sinai, New York, NY
Background: One of the most serious consequences of liver
disease and malnutrition early in life is neurodevelopmental
(ND) delay for which prompt identification and intervention
may improve long-term outcomes. We hypothesized i) biliary
atresia (BA) patients with their native liver at ages 1 and 2
yrs will have significant ND delays and ii) that specific demographic
and clinical variables will predict worse ND outcomes.
Methods: Infants with BA, birth weight >2 kg, who received
hepatoportoenterostomy (HPE) and were enrolled between
2004-2012 in the multi-center, prospective NIDDK-funded
ChiLDReN study (PROBE-NCT00061828) underwent ND testing
at ages 12±2 mos (T1) and 24±2mos (T2) using either
Bayley Scales of Infant Development-2(B2) or Bayley-3(B3),
based on era. A subset of these infants was also enrolled in a
blinded randomized placebo-controlled study of corticosteroids
as adjunctive therapy to HPE (START–NCT00294684). Scores
(normative mean=100±15) were categorized as ≥100, 85-99,
70-84,