Original ArticleBiliary Atresia in The Netherlands: Outcome of Patients Diagnosed between 1987 and 2008
Section snippets
Methods
The Netherlands Study Group on Biliary Atresia Registry (NeSBAR) database is based on an ongoing joint effort of the Dutch Society for Pediatrics and the Dutch Society for Pediatric Surgeons. The registry contains data for all patients with BA treated in the 6 specialized academic centers in The Netherlands. Each center is independent and offers extensive diagnostic workup for neonatal cholestasis, primary surgical treatment (classical portoenterostomy according to the Kasai procedure or a
Results
Between January 1, 1987, and December 31, 2008, 231 infants (114 females; 49.4%) were diagnosed with BA in The Netherlands. Three patients from overseas (Dutch Antilles) had been operated on in the continental Netherlands, but these were excluded from further analysis because the first presentation and follow-up were not readily accessible for analysis. The overall incidence of BA in The Netherlands during the study period was 1 in 18 619 live births (Statistics Netherlands, http://www.cbs.nl).
Discussion
Our national results from The Netherlands are comparable with those from other Western countries (France, United Kingdom) and remarkably constant over time. Our transplant-free survival was similar to that in the previous Dutch cohort analysis.17 It is striking that although general health care has seen much development over the past 3 decades, transplant-free survival has remained constant. Overall survival has increased slightly over this period, most likely attributed to an increased
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The authors declare no conflicts of interest.
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A list of NeSBAR members is available at www.jpeds.com (Appendix).