Improving outcomes of biliary atresia: French national series 1986-2009.

Abstract

BACKGROUND & AIMS:

This study analyses the prognosis of biliary atresia (BA) in France since liver transplantation (LT) became widely available.

METHODS:

The charts of all BA patients living in France and born between 1986 and 2009 were reviewed. Patients were divided into 3 cohorts according to their years of birth: 1986-1996, 1997-2002, and 2003-2009.

RESULTS:

1107 BA children were identified, 990 born in metropolitan France (incidence 1/18,400 live births). Kasai operation was performed in 1044 (94%), leading to complete clearance of jaundice (total serum bilirubin ≤ 20 μmol/L) in 38% of patients. Survival with native liver (SNL) after Kasai operation was 40%, 36%, and 30% at 5, 10, and 20 years, stable in the 3 cohorts. Median age at Kasai operation was 59 days, unchanged over time. Twenty-year SNL was 39%, 32%, 28%, and 19% after Kasai operation performed in the first, second, third months of life or thereafter (p=0.0002). 588 children underwent 692 LTs. Mortality without transplantation decreased over time: 16%, 7%, and 4% in the 3 cohorts (p<0.0001). Survival after transplantation was 83%, 82%, and 77% at 5, 10, and 20 years in the whole series. Five-year post-transplant survival was 75%, 90%, and 89% in the 3 cohorts (p<0.0001). In the whole series, overall BA patient survival was 81%, 80%, and 77% at 5, 10, and 20 years. Five-year BA patient overall survival increased over time: 72%, 88%, and 89% in the 3 cohorts (p<0.0001).

CONCLUSIONS:

BA patients currently have an 89% live expectancy, and a 30% chance to reach adulthood without transplantation. Early Kasai operation, without age threshold, reduces the need for liver transplantation until adulthood.

• Authors: Christophe Chardot, Chantal Buet, Marie-Odile Serinet, Jean-Louis Golmard, Alain Lachaux, Bertrand Roquelaure, Frédéric Gottrand, Pierre Broué, Alain Dabadie, Frédéric Gauthier, Emmanuel Jacquemin
• Journal: Journal of hepatology (J Hepatol) Vol. 58 Issue 6 Pg. 1209-17 (Jun 2013) ISSN: 1600-0641 [Electronic] Netherlands
• PMID: 23402746 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2013 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Topics

• Adolescent
• Adult
• Biliary Atresia (mortality, surgery)
• Child
• Child, Preschool
• France
• Humans
• Infant
• Infant, Newborn
• Liver Transplantation
• Time Factors
• Treatment Outcome