Monograph

The ERS Monograph is the quarterly book series from the ERS.

Each Monograph covers a specific area of respiratory medicine, providing in-depth reviews that give clinicians at all levels a concise, comprehensive guide to symptoms, diagnosis and treatment.

Cystic Fibrosis

European Respiratory Society Monograph, Vol. 64. 2014
ISBN: 9781849840514
DOI: 10.1183/1025448x.erm6414

Cystic fibrosis (CF) is one of the most common fatal hereditary diseases. The discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the stage for unraveling the pathogenesis of CF lung disease, continuous refinement of symptomatic treatments and the development of mutation-specific therapies, which are now becoming available for a subgroup of patients. This ERS Monographprovides an update on all aspects of CF lung disease, from infancy to adulthood, including current concepts on disease process, improvements in early diagnosis and monitoring, therapeutic approaches, and patient care. The book highlights important recent developments and discusses the next steps that will be required for further improvement of the life expectancy and quality of life of CF patients. It will be an essential reference for basic and clinical scientists and all members of the CF team.

CME test: ERS Monograph 64: Cystic fibrosis
Preface Tobias Welte Eur Respir Monogr 2014; 64: ix-x
Guest Editors Eur Respir Monogr 2014; 64: xi-xii
Introduction Marcus A. Mall, J. Stuart Elborn Eur Respir Monogr 2014; 64: xiii-xiv
Pathophysiology of cystic fibrosis lung disease Marcus A. Mall, Richard C. Boucher Eur Respir Monogr 2014; 64: 1-13
Airway inflammation in cystic fibrosis Cormac McCarthy, M. Emmet O&Brien, Kerstin Pohl, Emer P. Reeves, Noel Gerard McElvaney Eur Respir Monogr 2014; 64: 14-31
Airway infection and the microbiome Jiangchao Zhao, J. Stuart Elborn, John J. LiPuma Eur Respir Monogr 2014; 64: 32-46
Genetic and environmental modifiers of cystic fibrosis Burkhard Tümmler, Frauke Stanke Eur Respir Monogr 2014; 64: 47-64
Newborn screening for cystic fibrosis: opportunities and remaining challenges Anil Mehta, Olaf Sommerburg, Kevin W. Southern Eur Respir Monogr 2014; 64: 65-76
Early cystic fibrosis lung disease Stephen M. Stick Eur Respir Monogr 2014; 64: 77-87
Imaging the lungs in cystic fibrosis Harm A.W.M. Tiddens Eur Respir Monogr 2014; 64: 88-103
End-points and biomarkers for clinical trials in cystic fibrosis Kris De Boeck, Isabelle Fajac, Felix Ratjen Eur Respir Monogr 2014; 64: 104-115
Correcting the basic ion transport defects in cystic fibrosis Emanuela Caci, Luis J.V. Galietta Eur Respir Monogr 2014; 64: 116-128
Potentiating and correcting mutant CFTR in patients with cystic fibrosis Isabelle Sermet-Gaudelus, Jacques de Blic, Muriel LeBourgeois, Iwona Pranke, Aleksander Edelman, Bonnie W. Ramsey Eur Respir Monogr 2014; 64: 129-149
New horizons for cystic fibrosis gene and cell therapy Uta Griesenbach, Rosanna F. Featherstone, Eric W.F.W. Alton Eur Respir Monogr 2014; 64: 150-168
Improving airway clearance in cystic fibrosis lung disease Judy M. Bradley, Katherine O&Neill, Ruth Dentice, Mark Elkins Eur Respir Monogr 2014; 64: 169-187
Antibiotic treatment of cystic fibrosis lung disease Christopher Orchard, Diana Bilton Eur Respir Monogr 2014; 64: 188-202
Exercise in cystic fibrosis Helge Hebestreit Eur Respir Monogr 2014; 64: 203-218
Extrapulmonary manifestations of cystic fibrosis Barry J. Plant, Michael D. Parkins Eur Respir Monogr 2014; 64: 219-235
Lung transplantation for cystic fibrosis Jens Gottlieb, Mark Greer Eur Respir Monogr 2014; 64: 236-245
Standards of care for patients with cystic fibrosis Malena Cohen-Cymberknoh, David Shoseyov, Eitan Kerem Eur Respir Monogr 2014; 64: 246-261
Using registries to improve cystic fibrosis care Edward F. McKone, Bruce C. Marshall Eur Respir Monogr 2014; 64: 262-271
Transition from paediatric to adult cystic fibrosis care: a developmental framework Anjana S. Madan, Adrianne N. Alpern, Alexandra L. Quittner Eur Respir Monogr 2014; 64: 272-285
Challenges of providing care to adults with cystic fibrosis Scott C. Bell, David W. Reid Eur Respir Monogr 2014; 64: 286-303
Health-economic aspects of cystic fibrosis screening and therapy Jir?í Klimeš, Tomáš Dolez?al, Kater?ina Kubác?ková, Marek Turnovec, Milan Macek Eur Respir Monogr 2014; 64: 304-319