title: Liver and combined lung and liver transplantation for cystic fibrosis: analysis of the UNOS database.
abstract: A proportion of patients with CF develop cirrhosis and portal hypertension. LT and combined LLT are rarely performed in patients with CF. To determine the outcome of LT and LLT in patients with CF. Patients with CF who had LT or LLT between 10/1987 and 5/2008 were identified from UNOS database. A total of 182 children (<18 yr) and 48 adults underwent isolated LT for CF. Seven more children and eight adults with CF underwent combined LLT. One- and five-yr patient and graft survival were not significantly different in patients who underwent LT in comparison with patients who underwent LLT (patient survival: LT; 83.9%, 75.7%, LLT; 80%, 80%; graft survival: LT; 76.1%, 67.0%, LLT; 80.0%, 80.0%, respectively). The two major causes of death after LT were pulmonary disease (15 patients, 22.7%) and hemorrhage (12 patients, 18.2%). Bilirubin was identified as a risk factor for death, and previous liver transplant and prolonged cold ischemic time were identified as risk factors for graft loss in LT patients. LT is a viable option for children and young adults with CF and end-stage liver disease. Outcome of LLT patients with CF was comparable to the outcome of patients with CF who underwent isolated LT.
authors.affiliation: Mount Sinai School of Medicine, Department of Pediatrics, New York, NY, USA. ronen.arnon@mountsinai.org
authors.forename: Ronen
authors.initials: R
authors.lastname: Arnon
authors.forename: Rachel A
authors.initials: RA
authors.lastname: Annunziato
authors.forename: Tamir
authors.initials: T
authors.lastname: Miloh
authors.forename: Maria
authors.initials: M
authors.lastname: Padilla
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sogawa
authors.forename: Linda
authors.initials: L
authors.lastname: Batemarco
authors.forename: Asha
authors.initials: A
authors.lastname: Willis
authors.forename: Frederick
authors.initials: F
authors.lastname: Suchy
authors.forename: Nanda
authors.initials: N
authors.lastname: Kerkar
mesh_terms: D000293:Adolescent; D000328:Adult; D001663:Bilirubin / Q000378:metabolism; D002648:Child; D003550:Cystic Fibrosis / Q000601:surgery / Q000628:therapy*; D016208:Databases, Factual; D005260:Female; D006084:Graft Rejection; D006085:Graft Survival; D006470:Hemorrhage; D006801:Humans; D007511:Ischemia; D016031:Liver Transplantation / Q000379:methods*; D016040:Lung Transplantation / Q000379:methods*; D008297:Male; D012042:Registries; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D001663:Bilirubin