title: Comparison of outcome after pediatric liver transplantation for metabolic diseases and biliary atresia.
abstract: Metabolic diseases (MD) are the second largest indication group for orthotopic liver transplantation (OLTx) in children after biliary atresia (BA). A better outcome after transplantation can be expected because of a better pretransplant condition and the absence of previous abdominal surgery. To prove this statement, patient survival, graft survival, and morbidity were compared between a group of 24 for MD and 52 for BA consecutively transplanted children. The actuarial one- and five-year patient survival rates for MD were 96% and 84%, and for BA 84% and 70%, respectively (p logrank test = 0.17). Three MD children (13%) and 15 BA children (29%) died. The actuarial one- and five-year graft survival rates for MD were 75% and 58%, and for BA 75% and 64%, respectively (p logrank test = 0.76). Seven MD children (29%) and 11 BA children (21%) were retransplanted. Postoperative bleeding and gastrointestinal complications occurred less frequent (4% vs. 18% and 4% vs. 14%, respectively), whereas biliary complications, viral infections, and acute rejection occurred more frequently (38% vs. 21%, 29% vs. 15%, and 50% vs. 37%, respectively) in MD children. The difference in the incidence of the various postoperative complications between both groups was not statistically significant. The mean ICU and ventilator stay was 7.5 and four days, respectively, in MD children and 16 and 10 days, respectively, in BA children (p = ns). The mean infection, complication, intervention, and retransplantation rate was equal in both groups.
  
  
  CONCLUSIONS
  Mortality and morbidity after pediatric liver transplantation for MD and BA are not different despite the better starting point for children with MD.
authors.affiliation: Liver Transplant Group, University Hospital Groningen, The Netherlands.
authors.forename: P M
authors.initials: PM
authors.lastname: Peeters
authors.forename: E
authors.initials: E
authors.lastname: Sieders
authors.forename: K P
authors.initials: KP
authors.lastname: De Jong
authors.forename: C M
authors.initials: CM
authors.lastname: Bijleveld
authors.forename: H G
authors.initials: HG
authors.lastname: Hendriks
authors.forename: E M
authors.initials: EM
authors.lastname: Ten Vergert
authors.forename: M J
authors.initials: MJ
authors.lastname: Slooff
mesh_terms: D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D007902:Length of Stay; D016031:Liver Transplantation; D008297:Male; D008659:Metabolic Diseases / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome