Epithelial cell dysfunction in cystic fibrosis: Implications for airway disease

T. F. Boat, P. W. Cheng

Research output: Contribution to journalArticlepeer-review

25 Scopus citations


An important pathophysiologic factor in CF airways is the failure to clear poorly hydrated secretions. The water deficit in CF mucous secretions can now be ascribed to a fundamental defect of epithelial cell regulatory processes which promotes sodium reabsorption from surface liquids and interferes with chloride secretion onto the luminal surface. In addition, it is now known that CF airway epithelial cells oversulfate high molecular weight glycoconjugates, both secreted and cell surface-associated. Oversulfation of glycoconjugates may contribute to the altered clearance properties of CF airways mucus and in addition could favor colonization of airways by organisms such as P. aeurginosa.

Original languageEnglish (US)
Pages (from-to)25-30
Number of pages6
JournalActa Paediatrica Scandinavica, Supplement
Issue number363
StatePublished - 1989
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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