Preface ix Guest Editors xi Introduction xiii Pathophysiology 1. Pathophysiology of cystic fibrosis lung disease 1 Marcus A. Mall and Richard C. Boucher 2. Airway inflammation in cystic fibrosis 14 Cormac McCarthy, M. Emmet O’Brien, Kerstin Pohl, Emer P. Reeves and Noel Gerard McElvaney 3. Airway infection and the microbiome 32 Jiangchao Zhao, J. Stuart Elborn and John J. LiPuma 4. Genetic and environmental modifiers of cystic fibrosis 47 Burkhard Tümmler and Frauke Stanke Diagnosis 5. Newborn screening for cystic fibrosis: opportunities and 65 remaining challenges Anil Mehta, Olaf Sommerburg and Kevin W. Southern 6. Early cystic fibrosis lung disease 77 Stephen M. Stick 7. Imaging the lungs in cystic fibrosis 88 Harm A.W.M. Tiddens 8. End-points and biomarkers for clinical trials in cystic fibrosis 104 Kris De Boeck, Isabelle Fajac and Felix Ratjen Therapy 9. Correcting the basic ion transport defects in cystic fibrosis 116 Emanuela Caci and Luis J.V. Galietta ERS monograph Contents Cystic Fibrosis Number 64 June 2014