Sodium chloride increases the ciliary transportability of cystic fibrosis and bronchiectasis sputum on the mucus-depleted bovine trachea.

Mucus retention in the lungs is an important feature of several respiratory diseases (Regnis, J.A., M. Robinson, D.L. Bailey, P. Cook, P. Hooper, H.K. Chan, I. Gonda, G. Bautovich, and P.T.P. Bye. 1994. Am. J. Respir. Crit. Care Med. 150:66-71 and Currie, D.C., D. Pavia, J.E. Agnew, M.T. Lopez-Vidriero, P.D. Diamond, P.J. Cole, and S.W. Clarke. 1987. Thorax. 42:126-130). On the mucus-depleted bovine trachea, the ciliary transport rate of sputum from patients with cystic fibrosis and bronchiectasis of other causes was slow, but the rate was doubled by increasing the sodium chloride content by 90 mM. Increasing the sputum osmolality by inspissation or by the addition of nonelectrolytes had a similar effect. The viscoelasticity of sputum, but not the bovine ciliary beat frequency, was markedly saline dependent over the pathophysiological range. This suggests that low mucus salinity, not (as is generally assumed) its under-hydration, contributes to its retention in bronchiectasis due to cystic fibrosis and other causes, probably by affecting its rheology. It also indicates how the genetic defect in cystic fibrosis might lead to impaired mucus clearance. Therapies that increase the osmolality of lung mucus might benefit patients with mucus retention.

[1]  M. Knowles,et al.  Effect of uridine 5'-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis. , 1996, American journal of respiratory and critical care medicine.

[2]  M. King,et al.  Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis. , 1996, American journal of respiratory and critical care medicine.

[3]  E. Greenberg,et al.  Cystic Fibrosis Airway Epithelia Fail to Kill Bacteria Because of Abnormal Airway Surface Fluid , 1996, Cell.

[4]  S. Anderson,et al.  Inhalation of hypertonic saline aerosol enhances mucociliary clearance in asthmatic and healthy subjects. , 1996, The European respiratory journal.

[5]  J. Morton,et al.  Short‐term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis , 1996, Pediatric pulmonology.

[6]  L. Kobzik,et al.  Effects of sodium concentration on human neutrophil bactericidal functions. , 1995, The American journal of physiology.

[7]  R. Wilson,et al.  The ciliary transportability of sputum is slow on the mucus-depleted bovine trachea. , 1995, American journal of respiratory and critical care medicine.

[8]  R. Boucher,et al.  Human airway ion transport. Part two. , 1994, American Journal of Respiratory and Critical Care Medicine.

[9]  P. Verdugo Molecular biophysics mucin secretion , 1994 .

[10]  P. Quinton Viscosity versus composition in airway pathology. , 1994, American journal of respiratory and critical care medicine.

[11]  P. Quinton,et al.  Elemental composition of human airway surface fluid in healthy and diseased airways. , 1993, The American review of respiratory disease.

[12]  M. King,et al.  Amiloride inhalation therapy in cystic fibrosis. Influence on ion content, hydration, and rheology of sputum. , 1993, The American review of respiratory disease.

[13]  D. Geddes,et al.  No added benefit from nebulized amiloride in patients with cystic fibrosis. , 1993, The European respiratory journal.

[14]  Matthew P. Anderson,et al.  Cystic fibrosis, CFTR, and abnormal electrolyte transport , 1993 .

[15]  J M Zahm,et al.  Role of the physicochemical properties of mucus in the protection of the respiratory epithelium , 2012 .

[16]  M. King,et al.  A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. , 1990, The New England journal of medicine.

[17]  M. King,et al.  Acute and long-term amiloride inhalation in cystic fibrosis lung disease. A rational approach to cystic fibrosis therapy. , 1990, The American review of respiratory disease.

[18]  P. Cole,et al.  Host-Microbial Interrelationships in Respiratory Infection , 1989 .

[19]  L. Cantley,et al.  Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. , 1986, The Journal of clinical investigation.

[20]  S. Harding,et al.  Polyelectrolyte behaviour in mucus glycoproteins. , 1983, Biochimica et biophysica acta.

[21]  M. J. Dulfano,et al.  Effect of pH, viscosity and ionic-strength changes on ciliary beating frequency of human bronchial explants. , 1983, Clinical science.

[22]  J. Rutland,et al.  NON-INVASIVE SAMPLING OF NASAL CILIA FOR MEASUREMENT OF BEAT FREQUENCY AND STUDY OF ULTRASTRUCTURE , 1980, The Lancet.

[23]  D. Pavia,et al.  Enhanced clearance of secretions from the human lung after the administration of hypertonic saline aerosol. , 2015, The American review of respiratory disease.

[24]  K. Philipson,et al.  Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function. , 1978, Scandinavian journal of respiratory diseases.

[25]  J. L. Potter,et al.  Studies on pulmonary secretions. II. Osmolality and the ionic environment of pulmonary secretions from patients with cystic fibrosis, bronchiectasis, and laryngectomy. , 1967, The American review of respiratory disease.

[26]  J. L. Potter,et al.  STUDIES ON PULMONARY SECRETIONS. I. THE OVER-ALL CHEMICAL COMPOSITION OF PULMONARY SECRETIONS FROM PATIENTS WITH CYSTIC FIBROSIS, BRONCHIECTASIS, AND LARYNGECTOMY. , 1963, The American review of respiratory disease.