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Pulm Med. 2014;2014:621342. doi: 10.1155/2014/621342. Epub 2014 Mar 30.

Practical guidelines: lung transplantation in patients with cystic fibrosis.

Author information

1
Department of Pulmonary Medicine, German Clinic for Diagnostics (DKD), Wiesbaden, Germany.
2
Department of Chest Medicine, Erasme University Hospital, Brussels, Belgium.
3
Department of Pediatrics, University Hospital Wuerzburg, Germany.
4
Regional Adult CF Centre, Belfast City Hospital, Belfast, UK.
5
Lung Transplant Unit, Hospital Universitario La Fe, Valencia, Spain.
6
Centre for Infection and Immunity, Queens University, Belfast and Adult CF Centre BHSCT, UK.
7
Cystic Fibrosis Center, Department of Pediatrics, Medical University Innsbruck, Innsbruck, Austria.
8
Paediatric Respiratory Medicine and Lung Transplantation, Great Ormond Street Hospital, London, UK.
9
Institute for Medical Microbiology and Infection Control, Goethe University Hospital Frankfurt, Germany.
10
Department of Pneumology, Goethe University Hospital Frankfurt, Germany ; Department of Pneumology, Frankfurt University Hospital, Building 15B,Theodor-Stern-Kai 7, 60590 Frankfurt, Germany.
11
Department of Pneumology, Frankfurt University Hospital, Building 15B,Theodor-Stern-Kai 7, 60590 Frankfurt, Germany.

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.

PMID:
24800072
PMCID:
PMC3988894
DOI:
10.1155/2014/621342
[Indexed for MEDLINE]
Free PMC Article

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