Epithelial cell dysfunction in cystic fibrosis: implications for airways disease

Acta Paediatr Scand Suppl. 1989;363:25-9; discussion 29-30. doi: 10.1111/apa.1989.78.s363.25.

Abstract

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. aeruginosa.

Publication types

  • Review

MeSH terms

  • Airway Obstruction / etiology*
  • Biological Transport
  • Chlorides / physiology
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / metabolism*
  • Electrolytes / metabolism
  • Epithelium / metabolism
  • Humans
  • Mucins / metabolism*
  • Respiratory System / metabolism*

Substances

  • Chlorides
  • Electrolytes
  • Mucins