title: Lung transplantation in patients with cystic fibrosis: special focus to infection and comorbidities.
abstract: Despite advances in medical care, patients with cystic fibrosis still face limited life expectancy. The most common cause of death remains respiratory failure. End-stage cystic fibrosis can be treated with lung transplantation and is the third most common reason for which the procedure is performed. Outcomes for cystic fibrosis are better than most other lung diseases, but remain limited (5-year survival 60%). For patients with advanced disease lung transplantation appears to improve survival. Outcomes for patients with Burkholderia cepacia remain poor, although they are better for patients with certain genomovars. Controversy exists about Mycobacterium abscessus infection and appropriateness for transplant. More information is also becoming available for comorbidities, including diabetes and pulmonary hypertension among others. Extra-corporeal membrane oxygenation is used more frequently for end-stage disease as a bridge to lung transplantation and will likely be used more in the future.
authors.affiliation: Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Dorgan
authors.forename: Denis
authors.initials: D
authors.lastname: Hadjiliadis
mesh_terms: D001228:Aspergillosis / Q000601:surgery; D019121:Burkholderia Infections / Q000401:mortality / Q000601:surgery; D016956:Burkholderia cepacia; D015897:Comorbidity; D003550:Cystic Fibrosis / Q000453:epidemiology* / Q000276:immunology / Q000382:microbiology / Q000601:surgery*; D005764:Gastroesophageal Reflux / Q000453:epidemiology; D006801:Humans; D016040:Lung Transplantation; D009765:Obesity / Q000453:epidemiology; D016019:Survival Analysis; D009927:Tissue and Organ Procurement; D014181:Transplantation Immunology
keywords: Burkholderia cepacia; Mycobacterium abscessus; cystic fibrosis; cystic fibrosis-related diabetes; extracorporeal membrane oxygenation; lung transplantation