%0 Journal Article
%T Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis
%A T. O. Hirche
%A C. Knoop
%A H. Hebestreit
%A D. Shimmin
%A A. Sol¨¦
%A J. S. Elborn
%A H. Ellemunter
%A P. Aurora
%A M. Hogardt
%A T. O. F. Wagner
%A ECORN-CF Study Group
%J Pulmonary Medicine
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/621342
%X 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. This paper is dedicated to Gerd D£¿ring, who was a great researcher and scientist. He was also an enthusiastic participant of the CF Winter School in Obergurgl/Austria, where the ground for this paper was laid in 2012 1. Introduction All aspects of CF care have been optimised over recent decades and survival continues to progressively improve; end-stage respiratory insufficiency at an adult age remains the cause of death in the vast majority of CF patients. CF constitutes the third major indication for lung transplantation (LTX) after emphysema and pulmonary fibrosis. LTX has the potential to significantly extend survival and improve quality of life (QoL) provided that candidates are referred on time to the transplant centre and carefully selected. Since CF is a multiorgan disease, various particularities and CF-specific complications must be taken into consideration before and after LTX, which requires a close cooperation between CF paediatricians/pulmonologists and the transplant team. This paper was initiated in March 2012 in Obergurgl/Austria during a workshop on LTX in CF. A first draft in German was compiled based on the experts' workshop presentations and was subsequently translated into English. This English
%U http://www.hindawi.com/journals/pm/2014/621342/