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Management of Biliary Atresia in France 1986 to 2015: Long-term Results

dc.rights.licenseopenen_US
dc.contributor.authorFANNA, M.
dc.contributor.authorMASSON, G.
dc.contributor.authorCAPITO, C.
dc.contributor.authorGIRARD, M.
dc.contributor.authorGUERIN, F.
dc.contributor.authorHERMEZIU, B.
dc.contributor.authorLACHAUX, A.
dc.contributor.authorROQUELAURE, B.
dc.contributor.authorGOTTRAND, F.
dc.contributor.authorBROUE, P.
dc.contributor.authorDABADIE, A.
hal.structure.identifierBordeaux population health [BPH]
dc.contributor.authorLAMIREAU, Thierry
dc.contributor.authorJACQUEMIN, E.
dc.contributor.authorCHARDOT, C.
dc.date.accessioned2020-06-08T11:57:46Z
dc.date.available2020-06-08T11:57:46Z
dc.date.issued2019-10
dc.identifier.issn1536-4801 (Electronic) 0277-2116 (Linking)en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/7812
dc.description.abstractEnOBJECTIVES: 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: 1428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin </=20 mumol/l) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6-199) was stable over time. Survival with Native Liver (SNL) after KOp was 41%, 35%, 26% and 22% at 5, 10, 20 and 30 years, stable in the 4 cohorts. 25-year SNL was 38%, 27%, 22%, 19% in patients operated in the 1, 2, 3 month of life or later, respectively (p = 0.0001). Center caseloads had a significant impact on results in the 1986-96 cohort only.16%, 7%, 7%, 8% of patients died without LT in the 4 cohorts (p = 0.0001).753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. 5-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%. 5-year BA patient survival was 72%, 88%, 87%, 87% in cohorts 1986-96, 1997-2002, 2003-09, 2010-15, respectively (p < 0.0001). CONCLUSIONS: In France, 87% of BA patients 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.
dc.language.isoENen_US
dc.subject.enLEHA
dc.title.enManagement of Biliary Atresia in France 1986 to 2015: Long-term Results
dc.title.alternativeJ Pediatr Gastroenterol Nutren_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1097/mpg.0000000000002446en_US
dc.subject.halSciences du Vivant [q-bio]/Santé publique et épidémiologieen_US
dc.identifier.pubmed31335841en_US
bordeaux.journalJ Pediatr Gastroenterol Nutren_US
bordeaux.page416-424en_US
bordeaux.volume69en_US
bordeaux.hal.laboratoriesBordeaux Population Health Research Center (BPH) - U1219en_US
bordeaux.issue4en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.teamLEHA_BPH
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
hal.exportfalse
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