Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease
progression making it an active area of research and important therapeutic target. In this …

Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that
regulates the transport of ions and the movement of water across the epithelial barrier …

Rationale: Proteolysis is a key aspect of the lung's innate immune system. Proteases,
including neutrophil elastase and MMPs (matrix metalloproteases), modulate cell signaling …

This comprehensive State of the Art review summarizes the current published knowledge
base regarding the pathophysiology and microbiology of pulmonary disease in cystic …

The host inflammatory response in cystic fibrosis (CF) lung disease has long been
recognized as a central pathological feature and an important therapeutic target. Indeed …

Chronic lung disease determines the morbidity and mortality of cystic fibrosis (CF) patients.
The pulmonary immune response in CF is characterized by an early and non-resolving …

Rationale: Progressive lung function decline is a defining feature of cystic fibrosis (CF).
Because airway inflammation plays a central role in CF lung disease, inflammatory …

Neutrophilic airway inflammation is a hallmark of cystic fibrosis (CF). As high oxidant
producers, airway neutrophils contribute largely to the systemic redox imbalance seen in CF …

Progressive obstructive lung disease secondary to chronic airway infection, coupled with
impaired host immunity, is the leading cause of morbidity and mortality in cystic fibrosis (CF) …

Rationale: Sputum biomarkers of infection and inflammation are noninvasive measures that
enable quantification of the complex pathophysiology of cystic fibrosis (CF) lung disease …