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A Journal on Diseases of the Respiratory System
Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index
Minerva Pneumologica 2008 June;47(2):73-91
Lung transplantation and cystic fibrosis
Spencer H., Aurora P.
1 Cardiothoracic Transplant and Respiratory Units Great Ormond Street Hospital for Children London, UK
2 Portex Respiratory Unit Institute of Child Health, UCL, London, UK
Lung transplantation remains the only therapeutic option for children and adults with end stage cystic fibrosis (CF) lung disease who are failing maximal medical therapy. Currently, approximately 2 000 lung transplants are carried out annually worldwide and of those 600 are performed for cystic fibrosis. This review will describe the indication for referral and criteria for acceptance onto the lung transplant list and the pre-, peri- and postoperative management of CF patients undergoing transplantation. There will also be a discussion of the long-term outcomes and current controversies surrounding the potential survival benefits of lung transplantation for children with cystic fibrosis.