title: Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia.
abstract: OBJECTIVE
  To prospectively assess the value of serum total bilirubin (TB) within 3 months of hepatoportoenterostomy (HPE) in infants with biliary atresia as a biomarker predictive of clinical sequelae of liver disease in the first 2 years of life.
  
  
  METHODS
  Infants with biliary atresia undergoing HPE between June 2004 and January 2011 were enrolled in a prospective, multicenter study. Complications were monitored until 2 years of age or the earliest of liver transplantation (LT), death, or study withdrawal. TB below 2 mg/dL (34.2 μM) at any time in the first 3 months (TB <2.0, all others TB ≥ 2) after HPE was examined as a biomarker, using Kaplan-Meier survival and logistic regression.
  
  
  RESULTS
  Fifty percent (68/137) of infants had TB < 2.0 in the first 3 months after HPE. Transplant-free survival at 2 years was significantly higher in the TB < 2.0 group vs TB ≥ 2 (86% vs 20%, P < .0001). Infants with TB ≥ 2 had diminished weight gain (P < .0001), greater probability of developing ascites (OR 6.4, 95% CI 2.9-14.1, P < .0001), hypoalbuminemia (OR 7.6, 95% CI 3.2-17.7, P < .0001), coagulopathy (OR 10.8, 95% CI 3.1-38.2, P = .0002), LT (OR 12.4, 95% CI 5.3-28.7, P < .0001), or LT or death (OR 16.8, 95% CI 7.2-39.2, P < .0001).
  
  
  CONCLUSIONS
  Infants whose TB does not fall below 2.0 mg/dL within 3 months of HPE were at high risk for early disease progression, suggesting they should be considered for LT in a timely fashion. Interventions increasing the likelihood of achieving TB <2.0 mg/dL within 3 months of HPE may enhance early outcomes.
  
  
  BACKGROUND
  ClinicalTrials.gov: NCT00061828 and NCT00294684.
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Baylor College of Medicine, Houston, TX. Electronic address: Benjamin.Shneider@bcm.edu.
authors.forename: Benjamin L
authors.initials: BL
authors.lastname: Shneider
authors.affiliation: University of Michigan Medical School, Ann Arbor, MI.
authors.forename: John C
authors.initials: JC
authors.lastname: Magee
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Emory University School of Medicine/Children's Healthcare of Atlanta, Atlanta, GA.
authors.forename: Saul J
authors.initials: SJ
authors.lastname: Karpen
authors.affiliation: Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA.
authors.forename: Elizabeth B
authors.initials: EB
authors.lastname: Rand
authors.affiliation: Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Narkewicz
authors.affiliation: Pediatrics Division of Gastroenterology, Hepatology, and Nutrition, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL.
authors.forename: Lee M
authors.initials: LM
authors.lastname: Bass
authors.affiliation: Johns Hopkins School of Medicine, Baltimore, MD.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Schwarz
authors.affiliation: Pediatrics Division of Gastroenterology, Hepatology, and Nutrition, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL.
authors.forename: Peter F
authors.initials: PF
authors.lastname: Whitington
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Jorge A
authors.initials: JA
authors.lastname: Bezerra
authors.affiliation: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Los Angeles, University of Southern California, Los Angeles, CA.
authors.forename: Nanda
authors.initials: N
authors.lastname: Kerkar
authors.affiliation: Merck Sharp and Dohme Corp, Merck Research Laboratories, North Wales, PA.
authors.forename: Barbara
authors.initials: B
authors.lastname: Haber
authors.affiliation: Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of California, San Francisco Benioff Children's Hospital, San Francisco, CA.
authors.forename: Philip
authors.initials: P
authors.lastname: Rosenthal
authors.affiliation: Washington University School of Medicine, St. Louis, MO.
authors.forename: Yumirle P
authors.initials: YP
authors.lastname: Turmelle
authors.affiliation: Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Indiana University School of Medicine, Indianapolis, IN.
authors.forename: Jean P
authors.initials: JP
authors.lastname: Molleston
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Pediatrics, University of Washington and Seattle Children's, Seattle, WA.
authors.forename: Karen F
authors.initials: KF
authors.lastname: Murray
authors.affiliation: The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Vicky L
authors.initials: VL
authors.lastname: Ng
authors.affiliation: Division of Pediatric Surgery, Children's Hospital, Los Angeles, CA.
authors.forename: Kasper S
authors.initials: KS
authors.lastname: Wang
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Emory University, Atlanta, GA.
authors.forename: Rene
authors.initials: R
authors.lastname: Romero
authors.affiliation: Children's Hospital of Pittsburgh, Pittsburgh, PA.
authors.forename: Robert H
authors.initials: RH
authors.lastname: Squires
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Mount Sinai Medical Center, New York, NY.
authors.forename: Ronen
authors.initials: R
authors.lastname: Arnon
authors.affiliation: Liver Diseases Research Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Averell H
authors.initials: AH
authors.lastname: Sherker
authors.affiliation: Department of Biostatistics, University of Michigan, Ann Arbor, MI.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Moore
authors.affiliation: Department of Biostatistics, University of Michigan, Ann Arbor, MI.
authors.forename: Wen
authors.initials: W
authors.lastname: Ye
authors.affiliation: Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO.
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Sokol
mesh_terms: D001201:Ascites / Q000453:epidemiology; D001656:Biliary Atresia / Q000453:epidemiology / Q000601:surgery*; D001663:Bilirubin / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D002170:Canada / Q000453:epidemiology; D002675:Child, Preschool; D016208:Databases, Factual; D018450:Disease Progression; D004211:Disseminated Intravascular Coagulation / Q000453:epidemiology; D005500:Follow-Up Studies; D006130:Growth Disorders / Q000453:epidemiology; D006801:Humans; D034141:Hypoalbuminemia / Q000453:epidemiology; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation / Q000706:statistics & numerical data; D016015:Logistic Models; D011171:Portoenterostomy, Hepatic; D011379:Prognosis; D011446:Prospective Studies; D014481:United States / Q000453:epidemiology
chemical_list: D015415:Biomarkers; D001663:Bilirubin