title: Liver transplantation following the Kasai procedure in treatment of biliary atresia: a single institution analysis.
abstract: OBJECTIVE
  This study aimed to assess outcomes of liver transplantation (LTx) in patients with biliary atresia (BA).
  
  
  METHODS
  The Kasai procedure was performed for 358 patients at Tohoku University Hospital between January 1955 and December 2013; 64 (17.9%) required LTx. These 64 patients were divided into 4 groups according to their age at the time of transplantation: Group 1, aged <2 years (n = 27); Group 2, aged 2-9 years (n = 16); Group 3, aged 10-19 years (n = 11); and Group 4, aged ≥20 years (n = 10). Clinical parameters were evaluated retrospectively.
  
  
  RESULTS
  Both living-donor (n = 57) and deceased-donor (n = 7) LTx were performed. Indications were irreversible jaundice (n = 53), intractable cholangitis (n = 3), hepatopulmonary syndrome (n = 6), portopulmonary hypertension (n = 1), and intestinal bleeding (n = 1). Jaundice occurred more frequently in Groups 1 and 2 than in Groups 3 and 4 (p = 0.031). Survival rates were 81.5, 100, 90.9, and 80% in Groups 1, 2, 3, and 4, respectively.
  
  
  CONCLUSIONS
  Although the overall LTx survival rate was satisfactory, some adult recipients experienced LTx-related difficulty. Close follow-up, meticulous assessment of physical and social conditions, presence of a multidisciplinary support system, and appropriate time course for LTx are all essential factors in the treatment of BA.
authors: Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan.
authors: Hideyuki
authors: H
authors: Sasaki
authors: Hiromu
authors: H
authors: Tanaka
authors: Motoshi
authors: M
authors: Wada
authors: Takuro
authors: T
authors: Kazama
authors: Kotaro
authors: K
authors: Nishi
authors: Megumi
authors: M
authors: Nakamura
authors: Hironori
authors: H
authors: Kudo
authors: Naoki
authors: N
authors: Kawagishi
authors: Masaki
authors: M
authors: Nio
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001656:Biliary Atresia / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007564:Japan; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000379:methods*; D011183:Postoperative Complications; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult