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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Elemental content of airway surface liquid from infants with cystic fibrosis.

We tested the hypothesis that airway surface liquid (ASL) electrolyte composition is altered in infants with cystic fibrosis ( CF) and that the presence of airway inflammation affects ASL composition. We measured the tracheal ASL sodium and chloride concentration and examined bronchoalveolar lavage (BAL) fluid cytology, interleukin-8 (IL-8) concentrations, and quantitative bacterial culture in 19 infants and young children with CF. Seven infants undergoing bronchoscopy for the evaluation of stridor served as non- CF controls. In addition, we measured nasal ASL sodium and chloride concentrations from 10 young adults with CF and from 10 control subjects. On the basis of the BAL findings, the infants with CF were divided into three groups: one with little evidence of pulmonary inflammation (CF-NI, n = 5); one with obvious pulmonary inflammation (CF-I, n = 7); and an intermediate group (CF-MI, n = 7). We found the ASL sodium was not different among any of the four groups (means mM +/- SE, 85 +/- 10 controls; 78 +/- 16 CF-NI; 83 +/- 9 CF-MI, 84 +/- 9 CF-I). In contrast the ASL chloride was lower in the CF-NI group when compared with control subjects (108 +/- 5 control subjects; 77 +/- 7 CF-NI, p < 0.01). In the CF-I and CF-MI groups, the ASL chloride concentrations were of intermediate values (CF-I 95 +/- 10 mM; CF-MI 96 +/- 9 mM) and not significantly different from controls. Results from the nasal ASL analysis showed no significant differences in sodium and chloride concentrations in the CF group compared with control subjects. These results suggest that the primary abnormality of ASL composition is a reduction in chloride concentration. ASL composition appears to be affected by the presence of airway inflammation.[1]


  1. Elemental content of airway surface liquid from infants with cystic fibrosis. Hull, J., Skinner, W., Robertson, C., Phelan, P. Am. J. Respir. Crit. Care Med. (1998) [Pubmed]
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