Liver transplant in cystic fibrosis: A poll among European centers. A study from the European Liver Transplant Registry

Maria L. Melzi, Deirdre A. Kelly, Carla Colombo, Paloma Jara, Javier Manzanares, Michele Colledan, Mario Strazzabosco, Paola Delorenzo, Maria G. Valsecchi, René Adam, Bruno Gridelli, Baroukh M. Assael

Research output: Contribution to journalArticlepeer-review

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, FEV1 >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.

Original languageEnglish
Pages (from-to)726-731
Number of pages6
JournalTransplant International
Volume19
Issue number9
DOIs
Publication statusPublished - Sep 2006

Keywords

  • Cystic fibrosis
  • Epidemiology
  • Liver transplant
  • Pulmonary function

ASJC Scopus subject areas

  • Transplantation

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