Hodson and Geddes' Cystic Fibrosis

Edited by Andrew Bush, Imperial College and Royal Brompton and Harefield NHS Foundation Trust, London, UK Diana Bilton, Royal Brompton and Harefield NHS Foundation Trust, London, UK Margaret Hodson, Imperial College and Royal Brompton and Harefield NHS Foundation Trust, London, UK

INTRODUCTION: WHAT IS CYSTIC FIBROSIS? Introduction: from the Discovery of the CFTR Gene in 1989 through to 2014. Epidemiology of Cystic Fibrosis. Living with Cystic Fibrosis: The Voices of Patients and Families. BASIC SCIENCE FOR THE CLINICIAN. Molecular Biology of Cystic Fibrosis: CFTR Processing and Functions and Classes of Mutations. Underlying Concepts of the Pathophysiology of Cystic Fibrosis in the Sweat Gland, GI Tract, and Lung. What Have We Learned from Animal Models? Interactions Between Infection and Inflammation in the Cystic Fibrosis Airway. CFTR and Interactions with Modifier Genes and the Environment: Genotype–Phenotype Correlations and Modifier Genes. CFTR and Interactions with Modifier Genes and the Environment: Environment. DIAGNOSTIC ASPECTS OF CYSTIC FIBROSIS. Diagnosis of the Symptomatic Patient. Screening: What Tests? Pathway of Pain: Your "Normal" Baby Has a Life-Threatening Disease. Microbiology of Cystic Fibrosis: Epidemiology of Cystic Fibrosis Pathogens and Clinical Microbiology Laboratory Methods. New Methods for Detecting and Identifying Bacteria. Infection Control. CLINICAL ASPECTS OF CYSTIC FIBROSIS. Respiratory Disease: Infectious Complications. Pulmonary Exacerbations. Respiratory Disease: Noninfectious Complications. Sleep, Lung Mechanics and Work of Breathing, Including Noninvasive Ventilation. Delivering Therapy to the Cystic Fibrosis Lung. Upper Airway Disease. Gastrointestinal Disease in Cystic Fibrosis. The Liver in Cystic Fibrosis. Insulin Deficiency and Cystic Fibrosis-Related Diabetes. Growth and Puberty. Bone Disease. Other Cystic Fibrosis-Related Disease. Sexual and Reproductive Issues. Transplantation. Growing Old with Cystic Fibrosis. MONITORING. Quality Improvement: Using Databases to Improve Cystic Fibrosis Care. Infant and Preschool Lung Function. Role of Bronchoscopy and Bronchoalveolar Lavage in Infants with Cystic Fibrosis. Clinical and Radiological Aspects of Imaging the Lungs. Physiological Monitoring of Older Children and Adults. Exercise: Testing and Use in Therapy. Outcome of Clinical Trials: Electricity, Induced Sputum, and Breath. Chest Computed Tomography and Clinical Trials in Cystic Fibrosis. Outcomes of Clinical Trials: Multiple Breath Washout Tests in Cystic Fibrosis. What Have we Learned Over the Last 5 Years? MULTIDISCIPLINARY CARE. Cystic Fibrosis Center Care. Nursing. Physiotherapy. Nutritional Aspects. Psychology. Palliative and Spiritual Care. CYSTIC FIBROSIS: THE FUTURE. Basic Science: What Will It Deliver? Gene and Stem Cell Therapy. Basic Science: What Will It Deliver? Non-Gene Therapy Treatments. The Future: How Will Management Change? APPENDICES. Appendix A: History of Cystic Fibrosis. Appendix B: How to Manage the Screened Patient. Index.