title: Analysis of the prognostic factors of long-term native liver survival in survivors of biliary atresia.
abstract: OBJECTIVE
  Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure.
  
  
  METHODS
  This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis.
  
  
  RESULTS
  The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors.
  
  
  CONCLUSIONS
  The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan. h-sasaki@ped-surg.med.tohoku.ac.jp.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Sasaki
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Hiromu
authors.initials: H
authors.lastname: Tanaka
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Motoshi
authors.initials: M
authors.lastname: Wada
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Takuro
authors.initials: T
authors.lastname: Kazama
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Megumi
authors.initials: M
authors.lastname: Nakamura
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Hironori
authors.initials: H
authors.lastname: Kudo
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Ryoji
authors.initials: R
authors.lastname: Okubo
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Sakurai
authors.affiliation: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Nio
mesh_terms: D001656:Biliary Atresia / Q000401:mortality* / Q000601:surgery*; D004932:Esophageal and Gastric Varices / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D017093:Liver Failure / Q000401:mortality*; D016031:Liver Transplantation; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D015911:Sclerotherapy; D017741:Survivors; D055815:Young Adult
keywords: Biliary atresia; Long-term survivors; Native liver survival; Prognostic factor