CONTENTS The Guest Editors vi Preface vii Introduction viii 1. New insights into the pathophysiology of lung disease in cystic fibrosis patients 1 G. Döring 2. Small airways disease in cystic fibrosis 21 K.G. Brownlee 3. Atypical cystic fibrosis 38 K.W. Southern 4. Disease modifier genes in cystic fibrosis 50 H. Grasemann 5. Emerging pathogens in cystic fibrosis 66 N. Høiby, T. Pressler 6. Gene therapy for cystic fibrosis: successes and challenges 79 J.C. Davies, E.W.F.W. Alton 7. Cystic fibrosis transmembrane conductance regulator pharmacotherapy 88 L.J.V. Galietta 8. Cystic fibrosis infection with clonal strains of Pseudomonas aeruginosa: 105 current knowledge and future management A.M. Jones, S.C. Bell 9. Noninvasive ventilation cystic fibrosis 127 B. Fauroux 10. Difficult issues in the selection of cystic fibrosis patients for lung transplantation 139 P.A. Corris 11. Diagnosis and management of cystic fibrosis related low bone mineral density 150 C.S. Haworth, S.L. Elkin The European Respiratory Monograph Number 35 April 2006 Contents MON 35.qxd 02/05/2006 14:31 Page 1