Hodson and Geddes' Cystic Fibrosis

Andrew Bush, Margarida D. Amaral, Jane C. Davies, Nicholas J. Simmonds, Jennifer L. Taylor-Cousar, Sarath C. Ranganathan, Andrew Bush, Margarida D. Amaral, Jane C. Davies, Nicholas J. Simmonds, Jennifer L. Taylor-Cousar, Sarath C. Ranganathan

• Publisher: CRC Press
• Year: 2023
• Print ISBN: 9781032202204
• eBook ISBN: 9781000988567
• Edition: 5
• Topic: Medicine
• Subtopic: Medical Theory, Practice & Reference

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.

Table of contents

• Cover Page
• Half-Title Page
• Title Page
• Copyright Page
• Dedication Page
• Contents
• Preface
• Acknowledgments
• Editors
• Contributors
• 1 Journey from Macromolecular Pathology to Molecular Therapies
• 2 Global Epidemiology of CF: High-Income and Low-/Middle-Income Countries (Global Harmonization Registry Countries)
• 3 Voices of Patients and Families
• 4 Patient Organisations
• 5 Molecular Biology of CFTR: From the Gene to the Protein
• 6 Biology of the CF Airway Epithelium
• 7 The Physiology of Epithelial Ion and Fluid Transport: Beyond CFTR Modulators
• 8 Inflammation in Cystic Fibrosis
• 9 Model Organisms of Cystic Fibrosis
• 10 Systems Biology and the New Omics
• 11 Genotype: Phenotype Correlations
• 12 Gene Environment Interactions
• 13 Demographic, Socioeconomic, and Environmental Contributions to Health in Cystic Fibrosis
• 14 Drug Discovery Platforms for CFTR Modulators
• 15 CFTR Modulator Drug Discovery and Translation into the Clinic
• 16 Newborn and Carrier Screening for CF
• 17 Diagnostic Tests: Sweat Testing, Epithelial Potential Differences and Genetic Testing
• 18 Immediate Management of the Newly Screened Positive Baby
• 19 Diagnosis of the Symptomatic Patient
• 20 Blurred Boundaries: CRMS/CFSPID and CFTR-Related Disorders
• 21 Respiratory Disease across the Lifecourse
• 22 Respiratory Effects of the New CFTR Modulators
• 23 Epidemiology and Microbiology of Cystic Fibrosis Pulmonary Infections
• 24 New Methods for Detecting and Identifying Bacteria
• 25 Non-Tuberculous Mycobacterial Infections in Cystic Fibrosis
• 26 Fungal Diseases in CF
• 27 Molecular Microbiology of the CF Gut and Lung
• 28 Pulmonary Exacerbations in Cystic Fibrosis: Epidemiology, Treatment, Outcomes, and Future Research
• 29 Infection Prevention and Control in Cystic Fibrosis
• 30 Technology in Cystic Fibrosis Therapies
• 31 Upper Airway Disease in Cystic Fibrosis
• 32 Gastrointestinal Disease in CF
• 33 Cystic Fibrosis Liver Disease
• 34 Cystic Fibrosis-Related Diabetes
• 35 Growth in Cystic Fibrosis: Is Chloride Transport the Key?
• 36 Bone Disease in Cystic Fibrosis
• 37 Cancer and Cystic Fibrosis
• 38 Other Cystic Fibrosis-Related Diseases and Complications
• 39 Extrapulmonary Benefits of the New CFTR Modulator Drugs
• 40 Sexual Health, Fertility, and Pregnancy in People with Cystic Fibrosis
• 41 Mental Health Issues in Cystic Fibrosis
• 42 Adherence and Self-Management in Cystic Fibrosis Care
• 43 Transplantation
• 44 Working with Cystic Fibrosis
• 45 Growing Old with Cystic Fibrosis
• 46 Imaging of Cystic Fibrosis Lung Disease
• 47 Lung Function Testing Including Multiple Breath Washout
• 48 Infant Pulmonary Function Tests
• 49 Exercise Testing in Cystic Fibrosis
• 50 Bronchoscopy and Bronchoalveolar Lavage in Cystic Fibrosis
• 51 Patient-Derived Cell-Based Models for Theratyping and Individual CFTR Modulator Assessment
• 52 Minimally Invasive Investigations for Biomarkers of Airways Disease
• 53 Current and Future Interventional Trial Designs to Support the CF Therapeutic Pipeline
• 54 Gene and RNA-Based Therapies
• 55 Cystic Fibrosis in Limited Resource Settings
• 56 Traveling with Cystic Fibrosis
• 57 Organisation of Cystic Fibrosis Centre Care
• 58 The Role of the CF Nurse Specialist
• 59 Physiotherapy
• 60 The Role of the Dietitian
• 61 The Role of the Pharmacist as Part of the Multidisciplinary Team Caring for CF Patients
• 62 The Role of a Psychologist on the Cystic Fibrosis Care Team
• 63 Adolescent Health and Transition in Cystic Fibrosis
• 64 Palliative and Supportive Care
• 65 Using Registries and Databases to Drive Up Quality
• 66 Digital Transformations within CF Healthcare
• 67 Personalized Medicine for Cystic Fibrosis in the 21st Century
• 68 CF Research Priorities for the Future
• Index