Biliary Atresia in The Netherlands: Outcome of Patients Diagnosed between 1987 and 2008

• Published in: The Journal of pediatrics Vol. 160; no. 4; pp. 638 - 644.e2
• Main Authors: de Vries, Willemien, MD, PhD, de Langen, Zacharias J., MD, PhD, Groen, Henk, MD, PhD, Scheenstra, Rene, MD, PhD, Peeters, Paul M.J.G., MD, PhD, Hulscher, Jan B.F., MD, PhD, Verkade, Henkjan J., MD, PhD
• Format: Journal Article
• Language: English
• Published: Maryland Heights, MO Mosby, Inc 01.04.2012; Elsevier
• Subjects: abnormal development; antibiotics; Biliary Atresia - diagnosis; Biliary Atresia - surgery; bilirubin; Biological and medical sciences; children; disease control; Female; General aspects; Humans; Infant; jaundice; Male; Medical sciences; Miscellaneous; multivariate analysis; Netherlands; patients; Pediatrics; Public health. Hygiene; Public health. Hygiene-occupational medicine; Retrospective Studies; surgery; survival rate; Time Factors; Treatment Outcome; Netherlands; Europe; Biliary atresia; Netherlands Study Group on Biliary Atresia Registry; OLT; Orthotopic liver transplantation; BASM; NeSBAR; Pediatric end-stage liver disease; PELD; Biliary atresia splenic malformation syndrome; UDCA; BA; Ursodeoxycholic acid; Human; Biliary tract; 2008; Pediatrics; Prognosis; Digestive system; Digestive diseases; Evolution; Patient; Diagnosis; Public health
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2011.09.061

Objective To examine the outcome of biliary atresia (BA) and to identify prognostic factors using a national database. Study design 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). Conclusion 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.