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Cystic fibrosis (CF) is an autosomal recessive disease caused by genetic mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene leading to viscous secretions, chronic respiratory infections and airway inflammation. Although survival of patients has been markedly improved due to better care and new treatment options, lung failure is still the major cause of mortality.
Thus, controlling respiratory infection and excessive airway inflammation is an important goal in the treatment of CF lung disease. Interestingly, heterogeneity in disease severity has been noted for a long time and airway inflammation may already be present in young children even in the absence of infection.1 Multiple factors have been proposed to explain the variety of disease severity even in young patients. These factors include differences in CFTR mutations, poor compliance with treatment, external factors like early viral infections and environmental factors like secondhand smoke exposure (SHSe). Many clinicians believe that SHSe is a major contributing factor for disease progression, although the mechanisms associated with increased inflammation and infection due to SHSe in infants and young …
Footnotes
Contributors SZ and DF wrote the manuscript and contributed equally to the critical revision and the final approval.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned; internally peer reviewed.
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