Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease which affects the mucus and sweat glands. The disease primarily affects the lungs, pancreas, liver, intestines, sinuses and sex organs. CF causes mucus secretions to be thick and sticky. The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. This can lead to problems such as repeated lung infections and lung damage. Respiratory failure is the most dangerous complication of cystic fibrosis. The specific signs and symptoms of cystic fibrosis can vary with the severity of the disease. For example, one child with cystic fibrosis may have respiratory problems but not digestive problems, while another child may have both. In addition, the signs and symptoms of cystic fibrosis may vary with age. In 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 standard diagnostic test for cystic fibrosis is a sweat test, which measures the amount of sodium or chloride in a person's sweat. During the procedure, a small amount of an odorless sweat-producing chemical is applied to a small area on the arm or leg. An electrode attached to the area stimulates a very weak and painless electric current, causing a tingling or warm feeling. After several minutes, sweat is collected from the stimulated area and sent to a laboratory for analysis. Currently, treatments for cystic fibrosis are aimed at relieving symptoms and complications.

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