title: Liver transplant in cystic fibrosis: a poll among European centers. A study from the European Liver Transplant Registry.
abstract: Liver Transplant (LTx) has been rarely performed in cystic fibrosis (CF) patients and indications and outcomes are not well defined. A questionnaire was sent to all European CF and LTx centers to collect data on CF transplanted patients. We obtained information regarding 57 CF patients. LTx has been performed prevalently in males and in pediatric age. The main complication of cirrhosis was portal hypertension with hypersplenism. In the majority of cases the decision to transplant was based on the contemporary presence of various factors. Post-LTx survival was high and comparable with that expected for more common pediatric LTx indications. Poor respiratory function was the main risk factor for early death. In the short-term, respiratory function significantly improved after LTx. LTx is the appropriate treatment for patients with advanced CF-related liver disease and preserved pulmonary function (Forced Expiratory Volume at 1 s, FEV(1) >50%). This poll reveals that most European liver centers perform LTx prior to the development of end-stage liver disease or overt pulmonary or other clinical decompensation.
authors.affiliation: Liver Transplant Unit, Ospedali Riuniti, Bergamo, Italy. ctfpediatr@ospedaliriuniti.bergamo.it
authors.forename: Maria L
authors.initials: ML
authors.lastname: Melzi
authors.forename: Deirdre A
authors.initials: DA
authors.lastname: Kelly
authors.forename: Carla
authors.initials: C
authors.lastname: Colombo
authors.forename: Paloma
authors.initials: P
authors.lastname: Jara
authors.forename: Javier
authors.initials: J
authors.lastname: Manzanares
authors.forename: Michele
authors.initials: M
authors.lastname: Colledan
authors.forename: Mario
authors.initials: M
authors.lastname: Strazzabosco
authors.forename: Paola
authors.initials: P
authors.lastname: DeLorenzo
authors.forename: Maria G
authors.initials: MG
authors.lastname: Valsecchi
authors.forename: René
authors.initials: R
authors.lastname: Adam
authors.forename: Bruno
authors.initials: B
authors.lastname: Gridelli
authors.forename: Baroukh M
authors.initials: BM
authors.lastname: Assael
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000401:mortality*; D005060:Europe / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008103:Liver Cirrhosis / Q000401:mortality* / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality*; D008297:Male; D011183:Postoperative Complications / Q000401:mortality; D012042:Registries; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome