Cystic fibrosis 133

the disease may not show up until adolescence or young adulthood. infants or young children should be tested for CF if they have persistent diarrhea, bulky foul-smelling and greasy stools, frequent wheezing or pneumonia, a chronic cough with thick mucus, salty-tasting skin, or poor growth. Babies born with an intestinal blockage (meconium ileus) also may have CF and should be tested.


CF affects different children in different ways and to varying degrees, although the underlying problem is the same—abnormal glands responsible for controlling sweat and mucus. sweat is responsible for cooling the body, while mucus lubricates the respiratory, digestive, and reproductive systems, and prevents tissues from drying out, protecting them from infection.

Children with CF lose a great deal of salt when they sweat, which can disrupt the balance of minerals in the blood, triggering abnormal heart rhythms. in addition, CF patients have very thick mucus that builds up in the intestines and lungs, leading to malnutrition, poor growth, frequent respiratory infections, breathing problems, and permanent lung damage.

Death in most patients results from lung disease and infections, although CF can cause various other medical problems. These include sinusitis, nasal polyps, rounding and enlargement of fingers and toes, pneumothorax (rupture of lung tissue and trapping of air between the lung and the chest wall), coughing of blood, enlargement of the right side of the heart, abdominal pain and discomfort, gassiness, and protrusion of the rectum through the anus. Liver disease, diabetes, inflammation of the pancreas, and gallstones also are common in some people with CF.

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