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Cystic fibrosis

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Cystic fibrosis

Definition:

Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is the most common type of chronic lung disease in children and young adults, and may result in early death.  

Causes, incidence, and risk factors:

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Cystic fibrosis (CF) is caused by a defective gene which tells the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and the pancreas, the organ that helps to break down and absorb food. This collection of sticky mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and a man's reproductive system.

Millions of Americans carry the defective CF gene, but do not have any symptoms. That's because a person with CF must inherit two defective CF genes -- one from each parent. An estimated 3% to 10% of Caucasians have the CF gene. The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It's more common among those of Northern or Central European descent. 

Most children are diagnosed with CF by their 1st birthday. A small number, however, are not diagnosed until age 18 or older. These patients usually have a milder form of the disease.

References:

US Food and Drug Administration. FDA Approves First DNA-based Test to Detect Cystic Fibrosis. Rockville, MD: National Press Office; May 16, 2005. Press Release P05-23. 

Cystic Fibrosis Foundation. What is CF? Available at http://www.cff.org/about_cf/what_is_cf/. Accessed May 16, 2005.

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