title: Clinical significance of liver histology on outcomes in biliary atresia.
abstract: OBJECTIVE
  Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes.
  
  
  METHODS
  Retrospective chart review of all BA cases between 1999 and 2014. Age at KP, liver biopsy results, use of ursodeoxycholic acid or prophylactic antibiotics and occurrence of PHT and ascending cholangitis post-KP were recorded and related to the primary post-KP outcome measures.
  
  
  RESULTS
  BA was diagnosed in 29 patients. Twenty-four of 29 patients underwent KP. Median age at KP was 68 days (29-104). Fourteen of 24 (58.3%) had bridging fibrosis and 5 of 24 (20.8%) had cirrhosis at time of KP. Median follow-up was 8.4 years (2.08-15.58 years). Bilirubin normalisation within 6 months occurred in 7 of 24 (29.2%) patients and 5-year NLS was 45.8% (11/24). Fourteen of 24 (58.3%) had PHT and 18 of 24 (75%) patients had ascending cholangitis post-KP. Absence of bridging fibrosis in liver histology at KP was the only factor to be significantly associated with improved 5-year NLS. None of the other variables examined had a significant association with either 5-year NLS or bilirubin normalisation by 6 months.
  
  
  CONCLUSIONS
  Five-year NLS in this series was 45.8%. Absence of bridging fibrosis at time of KP was the only factor significantly associated with improved 5-year NLS.
authors.affiliation: Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.
authors.forename: Nicholas L
authors.initials: NL
authors.lastname: Webb
authors.affiliation: School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.|Department of Pediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia.
authors.forename: Ashish
authors.initials: A
authors.lastname: Jiwane
authors.affiliation: School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.|Department of Gastroenterology, Sydney Children's Hospital, Sydney, New South Wales, Australia.
authors.forename: Chee Y
authors.initials: CY
authors.lastname: Ooi
authors.affiliation: Department of Gastroenterology, John Hunter Children's Hospital, Newcastle, New South Wales, Australia.|Discipline of Paediatrics and Child Health, School of Medicine and Public Health, University of Newcastle, Newcastle, New South Wales, Australia.
authors.forename: Scott
authors.initials: S
authors.lastname: Nightinghale
authors.affiliation: School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.|Department of Pediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia.
authors.forename: Susan E
authors.initials: SE
authors.lastname: Adams
authors.affiliation: School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.|Department of Gastroenterology, Sydney Children's Hospital, Sydney, New South Wales, Australia.
authors.forename: Usha
authors.initials: U
authors.lastname: Krishnan
mesh_terms: D001656:Biliary Atresia / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000033:anatomy & histology; D008297:Male; D008485:Medical Audit; D017063:Outcome Assessment, Health Care / Q000379:methods; D011171:Portoenterostomy, Hepatic / Q000379:methods; D012189:Retrospective Studies
keywords: biliary atresia; liver histology; treatment