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Definition
By Mayo Clinic staff
Cystic fibrosis is a life-threatening disorder that causes severe lung damage and nutritional deficiencies.
An inherited condition, cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. Normally, these secretions are thin and slippery, but in cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs.
Cystic fibrosis happens most often in white people of northern European ancestry, occurring in about 1 out of 3,000 live births. In the past, most people with cystic fibrosis died in their teens. Improved screening and treatments now allow many people with cystic fibrosis to live into their 50s or even longer.
- 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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