Biliary Atresia: Swiss National Study, 1994–2004
- 1 March 2008
- journal article
- research article
- Published by Wiley in Journal of Pediatric Gastroenterology and Nutrition
- Vol. 46 (3), 299-307
- https://doi.org/10.1097/mpg.0b013e3181633562
Abstract
Objectives: To determine the epidemiology of biliary atresia (BA) in Switzerland, the outcome of the children from diagnosis, and the prognostic factors. Patients and Methods: The records of all patients with BA born in Switzerland between January 1994 and December 2004 were analyzed. Survival rates were calculated with the Kaplan-Meier method, and prognostic factors evaluated with the log rank test. Median follow up was 58 months (range, 5–124). Results: BA was diagnosed in 48 children. Incidence was 1 in 17,800 live births (95% confidence interval 1/13,900–1/24,800), without significant regional, annual, or seasonal variation. Forty-three children underwent a Kasai portoenterostomy (PE) in 5 different Swiss pediatric surgery units. Median age at Kasai PE was 68 days (range, 30–126). Four-year survival with native liver after Kasai PE was 37.4%. Liver transplantation (LT) was needed in 31 in 48 children with BA, including 5 patients without previous Kasai PE. Four patients (8%, all born before 2001) died while waiting for LT, and 29 LT were performed in 27 patients (28 in Geneva and 1 in Paris). All of the transplanted patients are alive. Four-year overall BA patient survival was 91.7%. Four-year survival with native liver was 75% in patients who underwent Kasai PE before 46 days, 33% in patients operated on between 46 and 75 days, and 11% in patients operated on after 75 days (P = 0.02). Conclusions: Overall survival of patients with BA in Switzerland compares favorably with current international standards, whereas results of the Kasai operation could be improved to reduce the need for LTs in infancy and early childhood.Keywords
This publication has 37 references indexed in Scilit:
- Prognosis of biliary atresia: What can be further improved?The Journal of Pediatrics, 2006
- A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000The Journal of Pediatrics, 2006
- The outcome of the older (≥100 days) infant with biliary atresiaJournal of Pediatric Surgery, 2004
- The Kasai portoenterostomy for biliary atresia: a review of a 27-year experience with 81 patientsJournal of Pediatric Surgery, 2003
- Factors related to the biliary atresia prognosis post-portoenterostomyJornal de Pediatria, 2002
- Anatomy of the Porta Hepatis as a Basis for Extended Hepatoporto-Enterostomy for Extrahepatic Biliary Atresia - A New Surgical TechniqueEuropean Journal of Pediatric Surgery, 2001
- Epidemiology of biliary atresia in France: a national study 1986–96Journal of Hepatology, 1999
- A Multivariable Risk Factor Analysis of the Portoenterostomy (Kasai) Procedure for Biliary AtresiaAnnals of Surgery, 1997
- The Treatment of Biliary Atresia in Europe 1969-1995.The Tohoku Journal of Experimental Medicine, 1997
- Determinants of Life Span after Kasai Operation at the Era of Liver Transplantation.The Tohoku Journal of Experimental Medicine, 1997