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cystic fibrosis

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cystic fibrosis
abies born with CF don't gain weight as expected. They fail to thrive in spite of a normal diet and a good appetite. Mucus blocks the passageways of the pancreas and prevents pancreatic digestive juices from entering the intestines. Without these digestive juices, the intestines can't absorb fats and proteins completely, so nutrients pass out of the body unused rather than helping the body grow. Poor fat absorption makes the stools appear oily and bulky and increases the child's risk for deficiencies of the fat-soluble vitamins (vitamins A, D, E, and K). Unabsorbed fats may also cause excessive intestinal gas, an abnormally swollen belly, and abdominal pain or discomfort.

Because CF also affects epithelial cells in the skin's sweat glands, kids with CF may have a salty "frosting" on their skin or taste "salty" when their parents kiss them. They also may lose abnormally large amounts of body salt when they sweat on hot days.
Cystic fibrosis is the most common cause of pancreatic insufficiency in children, but a condition called Shwachman-Diamond Syndrome (SDS) is the second most common cause. SDS is a genetic condition that causes a reduced ability to digest food because digestive enzymes don't work properly. Some of the symptoms of SDS are similar to those of CF, so it may be confused with cystic fibrosis. However, in kids with SDS, the sweat test is normal.

Because CF produces thick mucus within the respiratory tract, kids with CF may suffer from nasal congestion, sinus problems, wheezing, and asthma-like symptoms. As CF symptoms progress, they can develop a chronic cough that produces globs of thick, heavy, discolored mucus. They also may suffer from repeated lung infections.

As chronic infections reduce lung function, the ability to breathe often decreases. A person with CF may eventually begin to feel short of breath, even when resting. Despite aggressive medical therapy, lung disease develops in nearly all patients with CF and is a common cause of

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