ABSTRACT

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.

chapter 2|10 pages

Global Epidemiology of CF

High-Income and Low-/Middle-Income Countries (Global Harmonization Registry Countries)

chapter 3|8 pages

Voices of Patients and Families

chapter 4|7 pages

Patient Organisations

chapter 5|11 pages

Molecular Biology of CFTR

From the Gene to the Protein

chapter 7|15 pages

The Physiology of Epithelial Ion and Fluid Transport

Beyond CFTR Modulators

chapter 11|14 pages

Genotype

Phenotype Correlations

chapter 17|16 pages

Diagnostic Tests

Sweat Testing, Epithelial Potential Differences and Genetic Testing

chapter 20|11 pages

Blurred Boundaries

CRMS/CFSPID and CFTR-Related Disorders

chapter 26|11 pages

Fungal Diseases in CF

chapter 28|11 pages

Pulmonary Exacerbations in Cystic Fibrosis

Epidemiology, Treatment, Outcomes, and Future Research

chapter 36|7 pages

Bone Disease in Cystic Fibrosis

chapter 45|10 pages

Growing Old with Cystic Fibrosis

chapter 59|24 pages

Physiotherapy