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Liver transplant in cystic fibrosis: a poll among European centers. A study from the European Liver Transplant Registry
- Source :
- Transplant International. 19:726-731
- Publication Year :
- 2006
- Publisher :
- Frontiers Media SA, 2006.
-
Abstract
- Summary 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.
- Subjects :
- Adult
Liver Cirrhosis
Male
medicine.medical_specialty
Cirrhosis
Adolescent
Cystic Fibrosis
Severity of Illness Index
Cystic fibrosis
Pulmonary function testing
Liver disease
Postoperative Complications
MED/12 - GASTROENTEROLOGIA
Internal medicine
medicine
Humans
Respiratory function
Decompensation
Registries
Child
cystic fibrosi
Transplantation
business.industry
Respiratory disease
medicine.disease
Survival Analysis
Liver Transplantation
Respiratory Function Tests
Surgery
Europe
liver transplant
Treatment Outcome
Child, Preschool
Female
business
Follow-Up Studies
Subjects
Details
- ISSN :
- 14322277 and 09340874
- Volume :
- 19
- Database :
- OpenAIRE
- Journal :
- Transplant International
- Accession number :
- edsair.doi.dedup.....20ab40443573f4d287948bfaa9115cfb