title: Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre.
abstract: OBJECTIVE
  Biliary atresia (BA) has preponderance in Asian populations with Kasai portoenterostomy (KP) regarded as the first-line standard of care. Yet reports from Southeast Asia remain scant. This study reviews the demographics, short- and medium-term outcomes for our cohort, and evaluates prognostic factors for outcome.
  
  
  METHODS
  All patients diagnosed with BA between January 1997 and December 2013 were included. Clinical data were obtained from medical records. Jaundice clearance was defined as total bilirubin < 20 µmol/L within 6 months after KP. Two-year and 5-year native liver survival (NLS) were determined. Prognostic factors examined included gender, ethnicity, associated anomalies, age at KP, post-KP cholangitis and clearance of jaundice within 6 months.
  
  
  RESULTS
  Of 58 patients studied, 31(53.4%) were male. Median age at time of KP was 53 days (range: 28-127). Ethnic distribution showed 32 (55.2%) Chinese, 16 (27.6%) Malays and 10 (17.2%) others. Twenty-one (36.2%) patients achieved jaundice clearance by 6 months. Two-year NLS rate was 36 out of 50 (72%), while 5-year NLS rate was 16 out of 35 (45.7%). Only clearance of jaundice within 6 months had a significant association with NLS (P = 0.006). All other factors showed no significant impact on outcome.
  
  
  CONCLUSIONS
  Our short- and medium-term outcomes after KP for BA are comparable with those reported by most international centres. However, prognostic factors such as age at KP, cholangitis episodes and associated anomalies did not show significant correlation; only clearance of jaundice within 6 months was significantly predictive of NLS.
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Li Wei
authors.initials: LW
authors.lastname: Chiang
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Chuan Yaw
authors.initials: CY
authors.lastname: Lee
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Gita
authors.initials: G
authors.lastname: Krishnaswamy
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Shireen Anne
authors.initials: SA
authors.lastname: Nah
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Ajmal
authors.initials: A
authors.lastname: Kader
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Christina
authors.initials: C
authors.lastname: Ong
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Yee
authors.initials: Y
authors.lastname: Low
authors.affiliation: Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
authors.forename: Kong Boo
authors.initials: KB
authors.lastname: Phua
mesh_terms: D001210:Asia, Southeastern; D001656:Biliary Atresia / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007567:Jaundice, Neonatal; D008099:Liver / Q000503:physiopathology; D008297:Male; D008485:Medical Audit; D017063:Outcome Assessment, Health Care; D011171:Portoenterostomy, Hepatic / Q000379:methods*; D011379:Prognosis; D012189:Retrospective Studies
keywords: Kasai portoenterostomy; biliary atresia; neonatal jaundice