title: Liver transplantation for cirrhosis in cystic fibrosis.
abstract: BACKGROUND
  Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure.
  
  
  OBJECTIVE
  The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population.
  
  
  METHODS
  Five children with CF, at a mean age of 16.5 years, underwent liver transplantation.
  
  
  RESULTS
  All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure.
  
  
  CONCLUSIONS
  Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.
authors.affiliation: Pediatric Gastroenterology, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
authors.forename: T
authors.initials: T
authors.lastname: Lamireau
authors.forename: S
authors.initials: S
authors.lastname: Martin
authors.forename: M
authors.initials: M
authors.lastname: Lallier
authors.forename: J E
authors.initials: JE
authors.lastname: Marcotte
authors.forename: F
authors.initials: F
authors.lastname: Alvarez
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay; D008103:Liver Cirrhosis / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology; D012129:Respiratory Function Tests
chemical_list: D007166:Immunosuppressive Agents