title: Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center.
abstract: The aim of this study was to review our experience in orthotopic liver transplantation (OLT) for biliary atresia (BA) in children and analyze the survival and prognostic factors, and long-term outcome. We reviewed 332 OLTs performed in 280 children between the years 1986 and 2000. Univariate and multivariate analysis were performed on patient and graft survivals according to recipients' and donors' characteristics as well as intraoperative data. The long-term outcome among the 80 children living at 10 years after OLT was studied according to growth, immunosuppressive therapy, and liver and renal functions. Liver graft status was eventually documented by liver biopsy. Status of rehabilitation was assessed by reviewing school performance and employment. Overall patient survival rates at 1, 5, and 10 years were 85, 82, and 82%, respectively, and the corresponding overall graft survival rates were 77, 73, and 71%. In the multivariate analysis, we identified 4 independent prognostic factors: polysplenia syndrome (P = .03), United Network for Organ Sharing (UNOS) status (P = .05), donor's age (P = .01), and perioperative surgical complications (P = .03). At 10 years after transplant, 80 children were alive and had normal growth rates. Liver histology was abnormal in 73% of these long-term survivors, mainly due to chronic rejection and centrilobular fibrosis. A total of 63 of the 80 children attended normal school and in 55 children (69%) school performance was not delayed. In conclusion, we discovered that a good long-term survival could be achieved after liver transplantation for BA, with a 82% survival rate at 10 years with normal scholastic studies in the majority of recipients.
authors.affiliation: Department of Pediatric Surgery, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Fouquet
authors.forename: Arnaud
authors.initials: A
authors.lastname: Alves
authors.forename: Sophie
authors.initials: S
authors.lastname: Branchereau
authors.forename: Sophie
authors.initials: S
authors.lastname: Grabar
authors.forename: Dominique
authors.initials: D
authors.lastname: Debray
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Jacquemin
authors.forename: Denis
authors.initials: D
authors.lastname: Devictor
authors.forename: Philippe
authors.initials: P
authors.lastname: Durand
authors.forename: Catherine
authors.initials: C
authors.lastname: Baujard
authors.forename: Monique
authors.initials: M
authors.lastname: Fabre
authors.forename: Danielle
authors.initials: D
authors.lastname: Pariente
authors.forename: Christophe
authors.initials: C
authors.lastname: Chardot
authors.forename: Bertrand
authors.initials: B
authors.lastname: Dousset
authors.forename: Pierre-Philippe
authors.initials: PP
authors.lastname: Massault
authors.forename: Denis
authors.initials: D
authors.lastname: Bernard
authors.forename: Didier
authors.initials: D
authors.lastname: Houssin
authors.forename: Olivier
authors.initials: O
authors.lastname: Bernard
authors.forename: Frédéric
authors.initials: F
authors.lastname: Gauthier
authors.forename: Olivier
authors.initials: O
authors.lastname: Soubrane
mesh_terms: D000293:Adolescent; D000328:Adult; D001656:Biliary Atresia / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000401:mortality; D008297:Male; D015999:Multivariate Analysis; D011379:Prognosis; D012086:Reoperation; D016896:Treatment Outcome