Cystic Fibrosis: Diagnosis and Management
A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".
Deadline for manuscript submissions: 31 May 2024 | Viewed by 159
Special Issue Editor
Interests: cystic fibrosis; bronchiectasis; airway obstruction; spirometry; lung; pulmonary function test; lung diseases; respiratory physiology; pneumology; ventilation
Special Issue Information
Dear Colleagues,
Cystic Fibrosis (CF) is an autosomal recessive disease caused by mutations in the Cystic Fibrosis Transmembrane Receptor (CFTR) gene that translates into a defective protein with a loss-of-activity mutation. CFTR malfunction causes an impaired transport of chloride, resulting in an accumulation of mucus that causes clinical manifestations compromising respiratory, biliary, pancreatic and gastrointestinal function. For many decades, CF represented a childhood disease, but in the latest years, multifactorial improvements have increased the survival of CF patients. In fact, by 1990, the median survival had increased to 28 years and currently exceeds 40 years. In addition, advances in gene detection, the greater knowledge of pulmonary and extrapulmonary clinical manifestations have caused an increase in diagnoses in adulthood.
The last ten years have also been characterized by an enormous step forward in the therapy and management of patients with CF owing to the development and combination of CFTR modulators. These drugs are very important not only to study their effects on lung function, but also their multisystemic effects.
In this Special Issue, we are looking for reviews and original articles to improve the diagnosis, focusing on the personalized management of pediatric and adult patients and identifying innovative therapies for CF patients.
Dr. Paola Iacotucci
Guest Editor
Manuscript Submission Information
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