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About CF » Pathophysiology of CF » Airway

Airway

Abnormalities in CFTR impacts the entire upper and lower airway by causing dehydration of the airway surface liquid.1 This results in decreased mucociliary clearance and the build-up of bacteria and particulates. In this situation lungs quickly become infected and inflamed, progressing to bronchiectasis and pulmonary exacerbations.2 This vicious circle of phlegm retention, infection and inflammation is referred to as the pathophysiologic cascade and begins within the first years of life.3

The pathophysiologic cascade

Schematic representation of the CF pathophysiologic cascade

Molecular mechanisms

Discover more about the isotonic volume hypothesis that describes molecular mechanisms of surface airway liquid depletion.

References
  1. Flume P & Van Devanter D. BMC Med 2012;10:88
  2. Elborn J & Bell SC. Thorax 2007;4:288–90
  3. Ratjen FA. Respir Care 2009;54:595–605