Cystic Fibrosis

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Cystic fibrosis (CF) is one of the most common autosomal recessive disorders associated with decreased longevity in the Caucasian population. The disease is classically described as a triad: chronic obstructive pulmonary disease, exocrine pancreatic insufficiency, and elevation of sodium and chloride concentration in sweat. The underlying pathophysiology involves abnormal ion transport due to dysfunction of the CF transmembrane conductance regulator. Altered salt and water movement across epithelia interferes with the hydration and ionic composition of mucus secretions. Abnormal mucus impairs clearance and defense systems leading to inflammation and destruction of the lungs, the pancreas and the developing vas deferens in males.

Original languageEnglish (US)
Title of host publicationInternational Encyclopedia of Public Health
PublisherElsevier Inc.
Pages208-210
Number of pages3
ISBN (Electronic)9780128037089
ISBN (Print)9780128036785
DOIs
StatePublished - Oct 6 2016

Keywords

  • Autosomal recessive disorders
  • CF transmembrane conductance regulator
  • CFTR
  • Cystic fibrosis

ASJC Scopus subject areas

  • Medicine(all)

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