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Cystic Fibrosis

Congenital Disorders, Oral and Gastrointestinal

Cystic fibrosis (CF) is a genetic disease that causes excess mucus production in the lungs and digestive system. This genetic defect makes the mucus thicker than normal, causing it to clog airways and drainage ducts in the body and making it difficult to breathe. It mostly affects the lungs and pancreas, but other areas of the body can be affected. Also, bacteria can be trapped, resulting in frequent infections, which damage the lung tissue and cause respiratory failure. In the pancreas, thick mucus blocks the ability to secrete enzymes that aid digestion and nutrient absorption. This can lead to delayed growth in children due to malnourishment. The known cause of CF is a genetic defect in the CFTR gene. This gene is responsible for the CFTR protein which helps your body move salt and water through the cells. When this gene is defective, the salt is moved to the skin and less is deposited inside the body. Since salt attracts water, not enough gets into the secretions and this causes them to become very thick and sticky. Cystic fibrosis affects over 30,000 people in the U.S and over 70,000 all over the world. It is a progressive lung and digestive disease that can be life-threatening. The majority of cystic fibrosis cases only survive into their teens up to their 20s, but a small number of people with CF may live up to mid-life.

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