Martina Fanna1, Guillaume Masson2, Carmen Capito1, Muriel Girard1, Florent Guerin3,4,5, Bogdan Hermeziu3,4,5, Alain Lachaux6, Bertrand Roquelaure7, Frédéric Gottrand8, Pierre Broue9, Alain Dabadie10, Thierry Lamireau11, Emmanuel Jacquemin3,4,5, Christophe Chardot1. 1. Observatoire Français de l'Atrésie des Voies Biliaires et Centre de Référence Atrésie des Voies Biliaires-Cholestases Génétiques, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris. 2. Unité de Recherche Clinique-Centre d'Investigation Clinique Paris Centre Descartes, Institut Imagine, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris. 3. Hépatologie et Transplantation Hépatique Pédiatriques, Centre de Référence Coordonnateur Atrésie des Voies Biliaires-Cholestases Génétiques, Filfoie, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre. 4. Faculté de Médicine Paris-Sud, Université Paris-Sud/Paris-Saclay, Le Kremlin-Bicêtre. 5. INSERM UMR-S1174, Hepatinov, Université Paris-Sud/ Paris-Saclay, Orsay. 6. Centre de Référence Atrésie des Voies Biliaires-Cholestases Génétiques, Hôpital Femme Mère Enfant, Bron. 7. Centre de Compétence Atrésie des Voies Biliaires-Cholestases Génétiques, Hôpital de la Timone, Marseille. 8. Centre de Compétence Atrésie des Voies Biliaires-Cholestases Génétiques, Hôpital Jeanne de Flandre, CHU de Lille, Université de Lille, Lille. 9. Centre de Compétence Atrésie des Voies Biliaires-Cholestases Génétiques, Hôpital des Enfants, Toulouse. 10. Centre de Compétence Atrésie des Voies Biliaires-Cholestases Génétiques, Hôpital Pontchaillou, Rennes. 11. Unité d'hépato-Gastroentérologie Pédiatrique, Hôpital des Enfants, Bordeaux, France.
Abstract
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.
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.
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