title: Outcomes in children with biliary atresia following liver transplantation.
abstract: BACKGROUND
  Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia.
  
  
  METHODS
  We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012.
  
  
  RESULTS
  The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively.
  
  
  CONCLUSIONS
  With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.
authors.affiliation: Nankai University School of Medicine, Tianjin 300071, China.
authors.forename: Li-Ying
authors.initials: LY
authors.lastname: Sun
authors.forename: Yun-Sheng
authors.initials: YS
authors.lastname: Yang
authors.forename: Zhi-Jun
authors.initials: ZJ
authors.lastname: Zhu
authors.forename: Wei
authors.initials: W
authors.lastname: Gao
authors.forename: Lin
authors.initials: L
authors.lastname: Wei
authors.forename: Xiao-Ye
authors.initials: XY
authors.lastname: Sun
authors.forename: Wei
authors.initials: W
authors.lastname: Qu
authors.forename: Wei
authors.initials: W
authors.lastname: Rao
authors.forename: Zhi-Gui
authors.initials: ZG
authors.lastname: Zeng
authors.forename: Chong
authors.initials: C
authors.lastname: Dong
authors.forename: Jin-Peng
authors.initials: JP
authors.lastname: Tu
authors.forename: Jian
authors.initials: J
authors.lastname: Wang
authors.forename: Yi-He
authors.initials: YH
authors.lastname: Liu
authors.forename: Yuan
authors.initials: Y
authors.lastname: Liu
authors.forename: Li-Xin
authors.initials: LX
authors.lastname: Yu
authors.forename: Yu
authors.initials: Y
authors.lastname: Wang
authors.forename: Jing
authors.initials: J
authors.lastname: Li
authors.forename: Zhong-Yang
authors.initials: ZY
authors.lastname: Shen
mesh_terms: D000367:Age Factors; D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D002681:China; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D019520:Living Donors; D008297:Male; D011171:Portoenterostomy, Hepatic; D011183:Postoperative Complications / Q000401:mortality; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents