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Causes
By Mayo Clinic staffIn cystic fibrosis, a defective gene alters a protein that regulates the normal movement of salt (sodium chloride) in and out of cells. This results in thick, sticky secretions in the respiratory and digestive tracts, as well as in the reproductive system. It also causes increased salt in sweat.
The affected gene, which is inherited from a child's parents, is a recessive gene. With recessive genes, children need to inherit two copies of the gene, one from each parent, in order to have the disease. If children inherit only one copy, they won't develop cystic fibrosis, but will be carriers and possibly pass the gene to their own children.
- What is cystic fibrosis? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_all.html. Accessed Dec. 9, 2009.
- Welsh MJ. Cystic fibrosis. In: Goldman L, et al. Cecil Medicine. 23rd ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/175336803-3/0/1492/366.html#4-u1.0-B978-1-4160-2805-5..50094-X_3680. Accessed Dec. 11, 2009.
- Boat TF, et al. Cystic fibrosis. In: Kliegman RM, et al. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/175336803-3/0/1608/945.html#4-u1.0-B978-1-4160-2450-7..50402-3--cesec14_7740. Accessed Dec. 11, 2009.
- Katkin JP. Cystic fibrosis: Clinical manifestations and diagnosis. http://www.uptodate.com/hme/index.html. Accessed Dec. 11, 2009.
- Cystic fibrosis. Genetics Home Reference. National Institutes of Health. http://ghr.nlm.nih.gov/condition=cysticfibrosis. Accessed Dec. 11, 2009.
- What is bronchiectasis? National Heart, Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/brn/brn_whatis.html. Accessed Jan. 4, 2010.
- Simon RH. Cystic fibrosis: Overview of the treatment of lung disease. http://www.uptodate.com/home/index.html. Accessed Dec. 14, 2009.
- An introduction to postural drainage and percussion. Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/treatments/Therapies/Respiratory/PosturalDrainage/An%20Introduction%20to%20Postural%20Drainage%20and%20Percussion%201-2006.pdf. Accessed Dec. 14, 2009.
- Airway clearance techniques. Cystic Fibrosis Foundation. http://www.cff.org/treatments/Therapies/Respiratory/AirwayClearance/. Accessed Dec. 14, 2009.
- Baker RD, et al. Cystic fibrosis: Nutritional issues. http://www.uptodate.com/home/index.html. Accessed Dec. 14, 2009.
- Wenstrom KD. Cystic fibrosis: Prenatal genetic screening. http://www.uptodate.com/home/index.html. Accessed Dec. 14, 2009.
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