Stephen J. Schueler, M.D.

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CF Underlying Cause

Cystic fibrosis is a hereditary disorder, caused by an abnormal gene.

Cystic fibrosis affects the cells that line organs throughout the body. Organs that are affected include:

  • Skin
  • Pancreas
  • Intestine
  • Lungs
  • Liver

Some of the effects of cystic fibrosis include:
  • The lungs are unable to produce enough mucus to lubricate the air passageways:
    • This causes the mucus to become thick, increasing the risk for pneumonia.
    • Over time, repeated lung infections can damage and scar the lungs.
  • The intestines are unable to produce enough mucus to lubricate the bowels.
    • This can lead to chronic digestive problems and diabetes.
  • The liver and pancreas are unable to secrete digestive enzymes into the intestine.

Continue to CF Genetics

Last Updated: Apr 3, 2009 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed CF References
  1. Aswani N, Taylor CJ, McGaw J, Pickering M, Rigby AS. Pubertal growth and development in cystic fibrosis: a retrospective review. Acta Paediatr. 2003 Sep;92(9):1029-32. [14599064]
  2. Long JM, Fauset-Jones J, Dixon MJ, Worthington-Riley D, Sharma V, Patel L, David TJ. Annual review hospital visits for patients with cystic fibrosis. J R Soc Med. 2001;94 Suppl 40:12-6. [11601158]
  3. Prescott WA Jr, Johnson CE. Antiinflammatory therapies for cystic fibrosis: past, present, and future. Pharmacotherapy. 2005 Apr;25(4):555-73. [15977917]
  4. Schoni MH, Casaulta-Aebischer C. Nutrition and lung function in cystic fibrosis patients: review. Clin Nutr. 2000 Apr;19(2):79-85. [10867724]
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