
Management of Biliary Atresia in France 1986 to 2015
Author(s) -
Fanna Martina,
Masson Guillaume,
Capito Carmen,
Girard Muriel,
Guerin Florent,
Hermeziu Bogdan,
Lachaux Alain,
Roquelaure Bertrand,
Gottrand Frédéric,
Broue Pierre,
Dabadie Alain,
Lamireau Thierry,
Jacquemin Emmanuel,
Chardot Christophe
Publication year - 2019
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000002446
Subject(s) - medicine , biliary atresia , gastroenterology , general surgery , liver transplantation , transplantation
Objectives: This study analyses the prognosis of biliary atresia (BA) in France since 1986, when both Kasai operation (KOp) and liver transplantation (LT) became widely available. Methods: The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed. Results: A total of 1428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin ≤20 μmol/L) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6–199) was stable over time. Survival with native liver after KOp was 41%, 35%, 26%, and 22% at 5, 10, 20, and 30 years, stable in the 4 cohorts. 25‐year survival with native liver was 38%, 27%, 22%, and 19% in patients operated in the first, second, third month of life or later, respectively ( P = 0.0001). Center caseloads had a significant impact on results in the 1986 to 1996 cohort only. 16%, 7%, 7%, and 8% of patients died without LT in the 4 cohorts ( P = 0.0001). A total of 753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. Five‐year patient survival after LT was 76%, 91%, 88%, and 92% in cohorts 1 to 4, respectively ( P < 0.0001). Actual BA patient survival (from diagnosis) was 81%. Five‐year BA patient survival was 72%, 88%, 87%, and 87% in cohorts 1986 to 1996, 1997 to 2002, 2003 to 2009, and 2010 to 2015, respectively ( P < 0.0001). Conclusions: In France, 87% of patients with BA survive nowadays and 22% reach the age of 30 years without transplantation. Improvement of BA prognosis is mainly due to reduced mortality before LT and better outcomes after LT.