title: Liver transplantation in children with cystic fibrosis: a long-term longitudinal review of a single center's experience.
abstract: BACKGROUND
  Improved long-term survival in cystic fibrosis (CF) has led to an increased incidence of extrapulmonary complications of this disease. Of these, end-stage liver disease is a significant cause of morbidity and mortality with liver transplantation being the only effective therapy.
  
  
  METHODS
  Records of all CF pediatric liver transplant recipients were reviewed.
  
  
  RESULTS
  Twelve children with CF were the recipients of 16 allografts. The 1- and 5-year survival was 91.6% and 75%, respectively. There were 5 deaths at a mean interval of 6.8 +/- 6.3 years. All of these deaths were related to pulmonary disease. Pulmonary function improved or remained stable in 8 of 9 patients tested. Despite an 83% incidence of positive sputum cultures, there was only one early mortality related to pulmonary sepsis in the setting of primary liver allograft nonfunction.
  
  
  CONCLUSIONS
  Liver transplantation is acceptable treatment for children with CF and end-stage liver disease. Long-term survival is comparable to liver transplantation performed for other indications. Although posttransplant morbidity and mortality is related to lung disease, the authors speculate that as therapeutic improvements prolong the survival in CF, it is expected that longer survival after liver transplantation in this patient population may also be anticipated.
authors.affiliation: Department of Pediatric Transplantation, Thomas E. Starzl Transplantation Institute, Children's Hospital of Pittsburgh, Pittsburgh, PA 15213, USA.
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Fridell
authors.forename: Geoffrey J
authors.initials: GJ
authors.lastname: Bond
authors.forename: George V
authors.initials: GV
authors.lastname: Mazariegos
authors.forename: David M
authors.initials: DM
authors.lastname: Orenstein
authors.forename: Ashokkumar
authors.initials: A
authors.lastname: Jain
authors.forename: Rakesh
authors.initials: R
authors.lastname: Sindhi
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Finder
authors.forename: Ernesto
authors.initials: E
authors.lastname: Molmenti
authors.forename: Jorge
authors.initials: J
authors.lastname: Reyes
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000401:mortality; D005260:Female; D006801:Humans; D017093:Liver Failure / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality; D008137:Longitudinal Studies; D008297:Male; D012086:Reoperation; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D013183:Sputum / Q000382:microbiology; D016019:Survival Analysis