Cystic fibrosis (CF) remains the most common life-shortening hereditary disease in white
populations, with high morbidity and mortality related to chronic airway mucus obstruction …

Cystic fibrosis (CF) is a monogenic autosomal recessive disorder that affects about 70 000
people worldwide. The clinical manifestations of the disease are caused by defects in the …

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are small
molecules that directly impact the CFTR protein, improving the function of the CFTR chloride …

Nitric oxide (NO) is produced by a family of isoenzymes, nitric oxide synthases (NOSs),
which all utilize l-arginine as substrate. The production of NO in the lung and airways can …

The airway epithelium works as a physical barrier between the external environment and
internal structures, which represent the lung's first defense against inhaled microorganisms …

Cystic fibrosis (CF) is a rare, progressive, multi-organ genetic disease. Ivacaftor, a small-
molecule CF transmembrane conductance regulator modulator, was the first medication to …

Cystic fibrosis (CF) pathophysiology is hallmarked by excessive inflammation and the
inability to efficiently resolve lung infections, contributing to morbidly and eventually the …

Cystic fibrosis (CF) is a life-shortening genetic disease affecting approximately 70 000
individuals worldwide. Until recently, drug development efforts have emphasised therapies …

Airway infections have remained a prominent feature in persons living with cystic fibrosis
(CF) despite the dramatic improvements in survival in the past decades. Antimicrobials are a …

Introduction Cystic fibrosis (CF) is a genetic disease affecting multiple organ systems.
Research and innovations in novel therapeutic agents and health care delivery have …