title: Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium.
abstract: OBJECTIVE
  To examine the medical status of children with biliary atresia (BA) with their native livers after hepato- portoenterostomy (HPE) surgery.
  
  
  METHODS
  The Childhood Liver Disease Research and Education Network database was utilized to examine subjects with BA living with their native livers 5 or more years after HPE and to describe the prevalence of subjects with BA with an "ideal" outcome, defined as no clinical evidence of chronic liver disease, normal liver biochemical indices (aspartate aminotransferase, alanine aminotransferase, γ-glutamyl transpeptidase, platelet count, total bilirubin, international normalized ratio, and albumin), and normal health-related quality of life 5 or more years after HPE.
  
  
  RESULTS
  Children with BA (n = 219; 43% male) with median age 9.7 years were studied. Median age at HPE was 56 (range 7-125) days. Median age- and sex-adjusted height and weight z-scores at 5-year follow-up were 0.487 (IQR -0.27 to 1.02) and 0.00 (IQR -0.74 to 0.70), respectively. During the 12 preceding months, cholangitis and bone fractures occurred in 17% and 5.5%, respectively. Health-related quality of life was reported normal by 53% of patients. However, only 1.8% met the study definition of "ideal" outcome. Individual tests of liver synthetic function (total bilirubin, albumin, and international normalized ratio) were normal in 75%, 85%, and 73% of the study cohort.
  
  
  CONCLUSIONS
  Cholangitis and fractures in long-term survivors underscore the importance of ongoing medical surveillance. Over 98% of this North American cohort of subjects with BA living with native livers 5 or more years after HPE have clinical or biochemical evidence of chronic liver disease.
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: vicky.ng@sickkids.ca.
authors.forename: Vicky Lee
authors.initials: VL
authors.lastname: Ng
authors.affiliation: Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA.
authors.forename: Barbara H
authors.initials: BH
authors.lastname: Haber
authors.affiliation: Department of Surgery, 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, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Alexander
authors.initials: A
authors.lastname: Miethke
authors.affiliation: Hepatobiliary Program, Seattle Children's Hospital and University of Washington, Seattle, WA.
authors.forename: Karen F
authors.initials: KF
authors.lastname: Murray
authors.affiliation: Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA.
authors.forename: Sonia
authors.initials: S
authors.lastname: Michail
authors.affiliation: Texas Children's Hospital, Houston, TX; Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA.
authors.forename: Saul J
authors.initials: SJ
authors.lastname: Karpen
authors.affiliation: Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA; Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York, NY.
authors.forename: Nanda
authors.initials: N
authors.lastname: Kerkar
authors.affiliation: Pediatric Gastroenterology, Hepatology, and Nutrition, Riley Hospital for Children, Indiana University, Indianapolis, IN.
authors.forename: Jean P
authors.initials: JP
authors.lastname: Molleston
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA.
authors.forename: Rene
authors.initials: R
authors.lastname: Romero
authors.affiliation: Pediatrics Gastroenterology, Hepatology, and Nutrition, University of California San Francisco Benioff Children's Hospital, University of California, San Francisco, CA.
authors.forename: Philip
authors.initials: P
authors.lastname: Rosenthal
authors.affiliation: Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Medical Institutions, Baltimore, MD.
authors.forename: Kathleen B
authors.initials: KB
authors.lastname: Schwarz
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Hospital Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA.
authors.forename: Benjamin L
authors.initials: BL
authors.lastname: Shneider
authors.affiliation: Division of Gastroenterology and Nutrition, Washington University, St. Louis, MO.
authors.forename: Yumirle P
authors.initials: YP
authors.lastname: Turmelle
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Ann and Robert H. Lurie Children's Hospital and Northwestern University, Chicago, IL.
authors.forename: Estella M
authors.initials: EM
authors.lastname: Alonso
authors.affiliation: Liver Diseases Research Branch, NIDDK, NIH, Bethesda, MD.
authors.forename: Averell H
authors.initials: AH
authors.lastname: Sherker
authors.affiliation: Department of Gastroenterology, Hepatology, and Nutrition, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO.
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Sokol
mesh_terms: D001656:Biliary Atresia / Q000601:surgery*; D002170:Canada; D002648:Child; D004766:Enterostomy; D005260:Female; D006304:Health Status; D006801:Humans; D008099:Liver / Q000601:surgery; D008297:Male; D011788:Quality of Life; D017741:Survivors; D013997:Time Factors; D014481:United States