title: Practical guidelines: lung transplantation in patients with cystic fibrosis.
abstract: There are no European recommendations on issues specifically related to lung transplantation (LTX) in cystic fibrosis (CF). The main goal of this paper is to provide CF care team members with clinically relevant CF-specific information on all aspects of LTX, highlighting areas of consensus and controversy throughout Europe. Bilateral lung transplantation has been shown to be an important therapeutic option for end-stage CF pulmonary disease. Transplant function and patient survival after transplantation are better than in most other indications for this procedure. Attention though has to be paid to pretransplant morbidity, time for referral, evaluation, indication, and contraindication in children and in adults. This review makes extensive use of specific evidence in the field of lung transplantation in CF patients and addresses all issues of practical importance. The requirements of pre-, peri-, and postoperative management are discussed in detail including bridging to transplant and postoperative complications, immune suppression, chronic allograft dysfunction, infection, and malignancies being the most important. Among the contributors to this guiding information are 19 members of the ECORN-CF project and other experts. The document is endorsed by the European Cystic Fibrosis Society and sponsored by the Christiane Herzog Foundation.
authors.affiliation: Department of Pulmonary Medicine, German Clinic for Diagnostics (DKD), Wiesbaden, Germany.
authors.forename: T O
authors.initials: TO
authors.lastname: Hirche
authors.affiliation: Department of Chest Medicine, Erasme University Hospital, Brussels, Belgium.
authors.forename: C
authors.initials: C
authors.lastname: Knoop
authors.affiliation: Department of Pediatrics, University Hospital Wuerzburg, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Hebestreit
authors.affiliation: Regional Adult CF Centre, Belfast City Hospital, Belfast, UK.
authors.forename: D
authors.initials: D
authors.lastname: Shimmin
authors.affiliation: Lung Transplant Unit, Hospital Universitario La Fe, Valencia, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Solé
authors.affiliation: Centre for Infection and Immunity, Queens University, Belfast and Adult CF Centre BHSCT, UK.
authors.forename: J S
authors.initials: JS
authors.lastname: Elborn
authors.affiliation: Cystic Fibrosis Center, Department of Pediatrics, Medical University Innsbruck, Innsbruck, Austria.
authors.forename: H
authors.initials: H
authors.lastname: Ellemunter
authors.affiliation: Paediatric Respiratory Medicine and Lung Transplantation, Great Ormond Street Hospital, London, UK.
authors.forename: P
authors.initials: P
authors.lastname: Aurora
authors.affiliation: Institute for Medical Microbiology and Infection Control, Goethe University Hospital Frankfurt, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Hogardt
authors.affiliation: Department of Pneumology, Goethe University Hospital Frankfurt, Germany ; Department of Pneumology, Frankfurt University Hospital, Building 15B,Theodor-Stern-Kai 7, 60590 Frankfurt, Germany.
authors.forename: T O F
authors.initials: TO
authors.lastname: Wagner
authors.affiliation: Department of Pneumology, Frankfurt University Hospital, Building 15B,Theodor-Stern-Kai 7, 60590 Frankfurt, Germany.
mesh_terms: D000075202:Contraindications; D003550:Cystic Fibrosis / Q000601:surgery*; D005112:Extracorporeal Circulation / Q000592:standards; D006084:Graft Rejection / Q000517:prevention & control; D006801:Humans; D016040:Lung Transplantation / Q000009:adverse effects / Q000379:methods / Q000592:standards*; D044623:Nutrition Therapy / Q000592:standards; D010353:Patient Education as Topic; D011182:Postoperative Care / Q000592:standards; D011300:Preoperative Care / Q000592:standards; D011584:Psychology; D012944:Social Support; D009927:Tissue and Organ Procurement / Q000458:organization & administration