title: Liver transplantation in cystic fibrosis.
abstract: Liver disease is the second most common cause of death in patients with cystic fibrosis (CF). Improvement in surgical techniques, medical management, and imaging modalities has broadened the range of options for treatment of these patients. Medical management with ursodeoxycholic acid and nutritional support may help decelerate the progression of liver disease. A timely evaluation of CF patients with liver involvement for transplantation is important. Such evaluation should not be delayed until signs of hepatic decompensation occur. Combined lung-liver transplant can be considered for patients with advanced pulmonary disease. Pretransplant management of portal hypertension with a portosystemic shunt procedure is an option for patients with well-preserved synthetic liver function. Improvement in lung function after liver transplantation and no significant risk of pulmonary infection with immunosuppressive therapy have been reported. Review of individual center experiences have shown satisfactory survival and improved quality of life for CF patients undergoing liver transplant.
authors.affiliation: Division of Pediatric Gastroenterology and Pediatric Liver Transplant Program, Childrens Hospital Los Angeles, University of Southern California, Los Angeles, California 90027, USA. ygenyk@surgery.usc.edu
authors.forename: Y S
authors.initials: YS
authors.lastname: Genyk
authors.forename: J A
authors.initials: JA
authors.lastname: Quiros
authors.forename: N
authors.initials: N
authors.lastname: Jabbour
authors.forename: R R
authors.initials: RR
authors.lastname: Selby
authors.forename: D W
authors.initials: DW
authors.lastname: Thomas
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications* / Q000601:surgery*; D006801:Humans; D008107:Liver Diseases / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery*; D016031:Liver Transplantation; D016040:Lung Transplantation; D010347:Patient Care Planning