Airway surface pH in subjects with cystic fibrosis

The cystic fibrosis (CF) transmembrane conductance regulator protein can transport bicarbonate and may therefore regulate airway surface (AS) pH. Disturbances of AS pH could contribute to the pathophysiology of CF lung disease. Five studies were carried out including the following: study 1) nasal pH measurements were made in 25 CF and 10 non-CF adults using an antimony pH probe. Mean nasal pH was significantly lower in the CF group. Nasal potential difference may have been a confounding factor; study 2) in a fresh cohort of CF and non-CF subjects, no significant difference was found between the two groups using a gold pH probe; study 3) simultaneous nasal pH measurements were made in 15 CF and 15 non-CF adults using both probes. In the CF group, there was a trend for the antimony probe to read lower than the gold probe. In the non-CF group, the antimony probe read higher. The pH difference noted in study 1 related to technical factors; study 4) the effect of acute changes in serum acid/base balance on nasal pH was assessed in five non-CF adults. Nasal pH was not altered by either acute respiratory acidosis or alkalosis; study 5) nasal and lower airway pH was measured in five CF and six non-CF children. No difference was found between the groups. There was a correlation between nasal and lower airway pH. The authors conclude that airway surface pH does not differ between cystic fibrosis and noncystic fibrosis subjects and therefore, cystic fibrosis transmembrane conductance regulator may not play a major role in airway surface pH in vivo.

[1]  L. Taussig,et al.  Bronchial mucous glands in the newborn with cystic fibrosis , 1982, European Journal of Pediatrics.

[2]  Elizabeth Erwin,et al.  Expression and activity of pH-regulatory glutaminase in the human airway epithelium. , 2002, American journal of respiratory and critical care medicine.

[3]  A S Verkman,et al.  Airway surface liquid pH in well-differentiated airway epithelial cell cultures and mouse trachea. , 2001, American journal of physiology. Cell physiology.

[4]  J. S. Tait,et al.  Comparison of nasal pH values in black and white individuals with normal and high blood pressure. , 2001, Clinical science.

[5]  A. Verkman,et al.  Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH. , 2001, The Journal of clinical investigation.

[6]  D. Geddes,et al.  Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence. , 2001, The European respiratory journal.

[7]  C. Haslett,et al.  Temperature-dependent Arrest of Neutrophil Apoptosis , 2000, The Journal of Biological Chemistry.

[8]  B. Gaston,et al.  Endogenous airway acidification. Implications for asthma pathophysiology. , 2000, American journal of respiratory and critical care medicine.

[9]  D. Bush,et al.  Twenty-four hour ambulatory nasal pH monitoring. , 2001, Clinical otolaryngology and allied sciences.

[10]  S. Miller,et al.  Specific lipopolysaccharide found in cystic fibrosis airway Pseudomonas aeruginosa. , 1999, Science.

[11]  I. Fidler,et al.  Constitutive and inducible interleukin 8 expression by hypoxia and acidosis renders human pancreatic cancer cells more tumorigenic and metastatic. , 1999, Clinical cancer research : an official journal of the American Association for Cancer Research.

[12]  E. Sorscher,et al.  CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands. , 1999, American journal of physiology. Lung cellular and molecular physiology.

[13]  Min Goo Lee,et al.  Cystic Fibrosis Transmembrane Conductance Regulator Regulates Luminal Cl−/HCO3 −Exchange in Mouse Submandibular and Pancreatic Ducts* , 1999, The Journal of Biological Chemistry.

[14]  S. Ell,et al.  Nasal pH measurement: a reliable and repeatable parameter. , 1999, Clinical otolaryngology and allied sciences.

[15]  S. Randell,et al.  Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways Disease , 1998, Cell.

[16]  M. Corboz,et al.  Effect of anion secretion inhibitors on mucin content of airway submucosal gland ducts. , 1998, American journal of physiology. Lung cellular and molecular physiology.

[17]  J. Mouroux,et al.  Influence of external pH on ciliary beat frequency in human bronchi and bronchioles. , 1998, The European respiratory journal.

[18]  M. Walshaw,et al.  Tracheal Microaspiration in Adult Cystic Fibrosis , 1998, Journal of the Royal Society of Medicine.

[19]  J. Zweier,et al.  pH dependence of neutrophil-endothelial cell adhesion and adhesion molecule expression. , 1996, The American journal of physiology.

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

[21]  Y Zhang,et al.  Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis. , 1995, The Journal of clinical investigation.

[22]  A. Prince,et al.  Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. , 1995, Proceedings of the National Academy of Sciences of the United States of America.

[23]  L. Tabak In defense of the oral cavity: structure, biosynthesis, and function of salivary mucins. , 1995, Annual review of physiology.

[24]  M. Walshaw,et al.  Twenty-four-hour tracheal pH monitoring--a simple and non-hazardous investigation. , 1994, Respiratory medicine.

[25]  A. Prince,et al.  Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells. , 1993, The Journal of clinical investigation.

[26]  T. Boat,et al.  Sulfate concentrations and transport in human bronchial epithelial cells. , 1993, The American journal of physiology.

[27]  M. Welsh,et al.  cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia. , 1992, The Journal of clinical investigation.

[28]  M. Welsh Abnormal regulation of ion channels in cystic fibrosis epithelia , 1990, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

[29]  J. Ward,et al.  Control of pH of airway surface liquid of the ferret trachea in vitro. , 1990, Journal of applied physiology.

[30]  J. Opitz,et al.  Pathological confirmation of cystic fibrosis in the fetus following prenatal diagnosis. , 1987, American journal of medical genetics.

[31]  D. Geddes,et al.  Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis. , 1987, Thorax.

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

[33]  R. T. Jackson,et al.  Some observations on nasal pH. , 1966, Archives of otolaryngology.