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. 35. 2006

ISBN: 978-1-904097-85-3

DOI: 10.1183/1025448x.erm3506

When CF was first described the disease was found to be affecting children who died as a result of it within the first few years of their life. However, it became clear that the CF phenotype differs between individuals and this may, in parallel with improved treatment,explain why certain affected individuals appear to have a more beneficial prognosis with prolonged survival. The knowledge regarding CF has increased tremendously during the previous decades. It is now clear that the disease is caused by a mutation in the gene coding for CF transmembrane conductance regulator, an ion channel responsible for chlorine transport in epithelial cells. The treatment has improved substantially and lifetime expectancy has increased from approximately 6 months to 30 yrs. Treatment of airway infections and obstructions, nutritional repletion, anti-inflammatory therapy and lung transplantation have contributed to improve survival outcomes with the possibility of gene therapy soon becoming a probable option.

Preface K. Larsson Eur Respir Mon; 2006: 35: vii
Introduction Eur Respir Mon; 2006: 35: viii
New insights into the pathophysiology of lung disease in cystic fibrosis patients G. Döring Eur Respir Mon; 2006: 35: 1–20
Small airways disease in cystic fibrosis K.G. Brownlee Eur Respir Mon; 2006: 35: 21–37
Atypical cystic fibrosis K.W. Southern Eur Respir Mon; 2006: 35: 38–49
Disease modifier genes in cystic fibrosis H. Grasemann Eur Respir Mon; 2006: 35: 50–65
Emerging pathogens in cystic fibrosis N. Høiby, T. Pressler Eur Respir Mon; 2006: 35: 66–78
Gene therapy for cystic fibrosis: successes and challenges J.C. Davies, E.W.F.W. Alton Eur Respir Mon; 2006: 35: 79–87
Cystic fibrosis transmembrane conductance regulator pharmacotherapy L.J.V. Galietta Eur Respir Mon; 2006: 35: 88–104
Cystic fibrosis infection with clonal strains of Pseudomonas aeruginosa: current knowledge and future management A.M. Jones, S.C. Bell Eur Respir Mon; 2006: 35: 105–126
Noninvasive ventilation cystic fibrosis B. Fauroux Eur Respir Mon; 2006: 35: 127–138
Difficult issues in the selection of cystic fibrosis patients for lung transplantation P.A. Corris Eur Respir Mon; 2006: 35: 139–149
Diagnosis and management of cystic fibrosis related low bone mineral density C.S. Haworth, S.L. Elkin Eur Respir Mon; 2006: 35: 150–168