Full Description
Cystic Fibrosis has seen dramatic advances in treatment since the last edition, including targeted cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators for most CFTR gene abnormalities. This new fifth edition is an update and expansion of the rapid clinical and scientific advances in improving prognosis, and the impact of COVID-19, all of which has transformed conventional models of care. It covers basic science, such as how detailed understanding of the biology of the CFTR gene and protein has led to novel and beneficial therapies, as well as all aspects of clinical management in high-, middle- and low-income settings and the voices of individuals with CF from across the world. It will be a useful reference for clinicians, including all levels of trainees, across the whole multidisciplinary team, scientists and students.
Key Features
• Follows an appealing organization of chapters, by developing fundamental knowledge of the reader before moving on to more complex or developing topics.
• Presents a comprehensive, authoritative and up-to-date text, integrating fundamental science and clinical aspects of cystic fibrosis providing an attractive read for clinicians, trainee doctors and scientists.
• Draws on global expertise and reflects best evidence-based practice from experts conducting cutting-edge clinical and basic science research from around the world.
Contents
Ch 1 Journey from Macromolecular Pathology to Molecular Therapies
Ch 2 Global Epidemiology of CF: High-Income and Low-/Middle-Income Countries (Global Harmonization Registry Countries)
Ch 3 Voices of Patients and Families
Ch 4 Patient Organisations
Ch 5 Molecular Biology of CFTR: From the Gene to the Protein
Ch 6 Biology of the CF Airway Epithelium
Ch 7 The Physiology of Epithelial Ion and Fluid Transport: Beyond CFTR Modulators
Ch 8 Inflammation in Cystic Fibrosis
Ch 9 Model Organisms of Cystic Fibrosis
Ch 10 Systems Biology and the New Omics
Ch 11 Genotype: Phenotype Correlations
Ch 12 Gene Environment Interactions
Ch 13 Demographic, Socioeconomic, and Environmental Contributions to Health in Cystic Fibrosis
Ch 14 Drug Discovery Platforms for CFTR Modulators
Ch 15 CFTR Modulator Drug Discovery and Translation into the Clinic
Ch 16 Newborn and Carrier Screening for CF
Ch 17 Diagnostic Tests: Sweat Testing, Epithelial Potential Differences and Genetic Testing
Ch 18 Immediate Management of the Newly Screened Positive Baby
Ch 19 Diagnosis of the Symptomatic Patient
Ch 20 Blurred Boundaries: CRMS/CFSPID and CFTR Related Disorders
Ch 21 Respiratory Disease across the Lifecourse
Ch 22 Respiratory Effects of the New CFTR Modulators
Ch 23 Epidemiology and Microbiology of Cystic Fibrosis Pulmonary Infections
Ch 24 New Methods for Detecting and Identifying Bacteria
Ch 25 Non-Tuberculous Mycobacterial Infections in Cystic Fibrosis
Ch 26 Fungal Diseases in CF
Ch 27 Molecular Microbiology of the CF Gut and Lung
Ch 28 Pulmonary Exacerbations in Cystic Fibrosis: Epidemiology, Treatment, Outcomes, and Future Research
Ch 29 Infection Prevention and Control in Cystic Fibrosis
Ch 30 Technology in Cystic Fibrosis Therapies
Ch 31 Upper Airway Disease in Cystic Fibrosis
Ch 32 Gastrointestinal Disease in CF
Ch 33 Cystic Fibrosis Liver Disease
Ch 34 Cystic Fibrosis-Related Diabetes
Ch 35 Growth in Cystic Fibrosis: Is Chloride Transport the Key?
Ch 36 Bone Disease in Cystic Fibrosis
Ch 37 Cancer and Cystic Fibrosis
Ch 38 Other Cystic Fibrosis-Related Diseases and Complications
Ch 39 Extrapulmonary Benefits of the New CFTR Modulator Drugs
Ch 40 Sexual Health, Fertility, and Pregnancy in People with Cystic Fibrosis
Ch 41 Mental Health Issues in Cystic Fibrosis
Ch 42 Adherence and Self-Management in Cystic Fibrosis Care
Ch 43 Transplantation
Ch 44: Working with Cystic Fibrosis
Ch 45 Growing Old with Cystic Fibrosis
Ch 46 Imaging of Cystic Fibrosis Lung Disease
Ch 47 Lung Function Testing Including Multiple Breath Washout
Ch 48 Infant Pulmonary Function Tests
Ch 49 Exercise Testing in Cystic Fibrosis
Ch 50 Bronchoscopy and Bronchoalveolar Lavage in Cystic Fibrosis
Ch 51 Patient-Derived Cell-Based Models for Theratyping and Individual CFTR Modulator Assessment
Ch 52 Minimally Invasive Investigations for Biomarkers of Airways Disease
Ch 53 Currrent and Future Interventional Trial Designs to Support the CF Therapeutic Pipeline
Ch 54 Gene and RNA-Bbased Therapies
Ch 55 Cystic Fibrosis in Limited Resource Settings
Ch 56 Traveling with Cystic Fibrosis
Ch 57 Organisation of Cystic Fibrosis Centre Care
Ch 58 The Role of the CF Nurse Specialist
Ch 60 The Role of the Dietitian
Ch 61 The Role of the Pharmacist as Part of the Multidisciplinary Team Caring for CF Patients
Ch 62 The Role of a Psychologist on the Cystic Fibrosis Care Team
Ch 63 Adolescent Health and Transition in Cystic Fibrosis
Ch 64 Palliative and Supportive Care
Ch 65 Using Registries and Databases to Drive up Quality
Ch 66 Digital Transformations within CF Healthcare
Ch 67 Personalized Medicine for Cystic Fibrosis in the 21st Century
Ch 68 CF Research Priorities for the Future
