5th edition. — CRC Press, 2024. — 747 p. — ISBN 9781032202204.
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.
Journey from Macromolecular Pathology to Molecular Therapies
Global epidemiology of CF: High income and low/middle income countries (global harmonisation registry countries)
Voices of Patients and Families
Patient Organisations
Molecular Biology of CFTR: From the Gene to the Protein
Biology of the CF airway epithelium
The physiology of epithelial ion and fluid transport: beyond CFTR modulators
Inflammation in Cystic Fibrosis
Model Organisms of Cystic Fibrosis
Systems Biology and the New Omics
Genotype: phenotype correlations
Gene Environment Interactions
Demographic, socioeconomic, and environmental contributions to health in cystic fibrosis
Drug Discovery Platforms for CFTR modulators
CFTR modulator drug discovery and translation into the clinic
Newborn and Carrier Screening for CF
Diagnostic tests: Sweat testing, epithelial potential differences and genetic testing
Immediate Management of The Newly Screened Positive Baby
Diagnosis of the symptomatic patient
Blurred boundaries: CRMS/ CFSPID and CFTR related disorders
Respiratory Disease across the Lifecourse
Respiratory effects of the new CFTR modulators
Epidemiology and microbiology of cystic fibrosis pulmonary infections
New methods for detecting and identifying bacteria
Non-tuberculous Mycobacterial Infections in Cystic Fibrosis
Fungal diseases in CF
Molecular microbiology of the CF gut and lung
Pulmonary Exacerbations in Cystic Fibrosis: Epidemiology, Treatment, Outcomes, and Future Research
Infection Prevention and Control in Cystic Fibrosis
Technology in Cystic Fibrosis Therapies