title: Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation.
abstract: OBJECTIVE
  To test the hypothesis that risk analysis from the time of listing for liver transplantation (LT) focuses attention on areas where outcomes can be improved.
  
  
  METHODS
  Competing outcomes and multivariate models were used to determine significant risk factors for pretransplantation and posttransplantation mortality and graft failure in patients with biliary atresia (BA) listed for LT and enrolled in the Studies of Pediatric Liver Transplantation (SPLIT) registry.
  
  
  RESULTS
  Of 755 patients, most were infants (age < 1 year). Significant waiting list mortality risk factors included infancy and pediatric end-stage liver disease (PELD) score > or = 20, whose components were also continuous risk factors. Survival posttransplantation (n=567) was 88% at 3 years. Most deaths were from infection (37%). Posttransplantation mortality risk factors included infant recipients, height/weight < -2 standard deviations (SD), use of cyclosporine versus tacrolimus and retransplantation. Graft failure risks included height/weight < -2 SD, cadaveric partial donors, donor age < or = 5 months, use of cyclosporine versus tacrolimus, and rejection.
  
  
  CONCLUSIONS
  Referral for LT should be anticipatory for infants with BA with failed portoenterostomies. Failing nutrition should prompt aggressive support. Post-LT risk factors are mainly nonsurgical, including nutrition, the relative risk of infection over rejection, and the choice of immunosuppression.
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
authors.forename: Elizabeth C
authors.initials: EC
authors.lastname: Utterson
authors.forename: Ross W
authors.initials: RW
authors.lastname: Shepherd
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Sokol
authors.forename: John
authors.initials: J
authors.lastname: Bucuvalas
authors.forename: John C
authors.initials: JC
authors.lastname: Magee
authors.forename: Susan V
authors.initials: SV
authors.lastname: McDiarmid
authors.forename: Ravinder
authors.initials: R
authors.lastname: Anand
mesh_terms: D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery*; D005260:Female; D006084:Graft Rejection; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation; D008297:Male; D011171:Portoenterostomy, Hepatic; D011237:Predictive Value of Tests; D012042:Registries; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome; D014850:Waiting Lists