Background Inhaled hypertonic saline (HS) has been shown to increase mucociliary
clearance (MCC) and improve clinical outcomes in adults and adolescents with cystic …

In the cystic fibrosis (CF) lung, the airway surface liquid (ASL) volume is depleted, impairing
mucus clearance from the lung and leading to chronic airway infection and obstruction …

Cystic fibrosis (CF), is an autosomal recessive disease frequently seen in the Caucasian
population. It is caused by mutations in the cystic fibrosis transmembrane conductance …

The airway surface liquid (ASL) is the thin layer of fluid coating the luminal surface of airway
epithelial cells at an air interface. Its composition and osmolality are thought to be important …

The luminal airway surface is lined with epithelial cells that provide a protective barrier from
the external environment and clear inhaled pathogens from the lung. To accomplish this …

Insufficient hydration at the airway surface can make mucus adherent and poorly cleared.
Cough, the major mechanism of mucus clearance in disease, is ineffective when mucus is …

The thin layer of liquid lining the entire respiratory tract is the first line of defense against the
continuous insult of inhaled bacteria and noxious chemicals. Many chronic obstructive …

The cystic fibrosis transmembrane conductance regulator (CFTR) is a protein kinase A and
ATP-regulated Cl–channel that also controls the activity of other membrane transport …

Activation of the CFTR Cl–channel inhibits epithelial Na+ absorption, according to studies
on native epithelia derived from airways, colon and kidney, and can also be demonstrated in …

Mucin secretion is regulated by extracellular signaling molecules emanating from local,
neuronal, or endocrine sources. Quantifying the rate of this secretion is important to …