title: Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008.
abstract: OBJECTIVE
  To examine the outcome of biliary atresia (BA) and to identify prognostic factors using a national database.
  
  
  METHODS
  All children born between January 1987 and December 2008 who underwent the Kasai surgical procedure for BA were retrieved from the Netherlands Study Group on Biliary Atresia Registry database. Outcomes were measured in terms of clearance of jaundice (bilirubin <1.17 g/dL, or 20 μmol/L, within 6 months after surgery) and 4-year transplant-free survival. Two cohorts, one from 1987-1997 and the other from 1998-2008, were compared. Survival rates were determined using Kaplan-Meier analysis, and prognostic factors were tested with univariate and multivariate analyses.
  
  
  RESULTS
  Between January 1987 and December 2008, 214 patients underwent Kasai surgery for BA. In this series, the 4-year transplant-free survival was 46%±4%, and 4-year overall survival was 73%±3%. Clearance of jaundice, surgery within 60 days, and postoperative antibiotic prophylaxis use were independently associated with increased transplant-free survival. The yearly caseload per center (range, 0.5-2.1) was not correlated with transplant-free survival (r=0.024; P=.73).
  
  
  CONCLUSIONS
  During the past 2 decades, outcome parameters have remained constant and are comparable with those reported from other Western countries, despite a relatively low annual caseload per center. Timely surgical correction and postoperative antibiotic therapy were associated with a higher transplant-free survival rate.
authors.affiliation: Department of Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Willemien
authors.initials: W
authors.lastname: de Vries
authors.forename: Zacharias J
authors.initials: ZJ
authors.lastname: de Langen
authors.forename: Henk
authors.initials: H
authors.lastname: Groen
authors.forename: Rene
authors.initials: R
authors.lastname: Scheenstra
authors.forename: Paul M J G
authors.initials: PM
authors.lastname: Peeters
authors.forename: Jan B F
authors.initials: JB
authors.lastname: Hulscher
authors.forename: Henkjan J
authors.initials: HJ
authors.lastname: Verkade
mesh_terms: D001656:Biliary Atresia / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009426:Netherlands; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome