Endocrine, nutritional and metabolic diseases
Cystic fibrosis E84
Cystic fibrosis E84-
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- A common hereditary disease in which exocrine (secretory) glands produce abnormally thick mucus. This mucus can cause problems in digestion, breathing, and body cooling.
- A congenital metabolic disorder affecting the exocrine glands, inherited as an autosomal trait. The secretions of exocrine glands are abnormal, resulting in excessively viscid mucus production which causes obstruction of passageways (including pancreatic and bile ducts, intestines, and bronchi). The sweat sodium and chloride content are increased. Symptoms usually appear in childhood and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather.
- An autosomal recessive genetic disease of the exocrine glands. It is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator expressed in several organs including the lung, the pancreas, the biliary system, and the sweat glands. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in airway obstruction; chronic respiratory infections; pancreatic insufficiency; maldigestion; salt depletion; and heat prostration.
- Cystic fibrosis (cf) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses and sex organs. Cf causes your mucus 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. The symptoms and severity of cf vary widely. Some people have serious problems from birth. Others have a milder version of the disease that doesn't show up until they are teens or young adults. Although there is no cure for cf, treatments have improved greatly in recent years. Until the 1980s, most deaths from cf occurred in children and teenagers. Today, with improved treatments, some people who have cf are living into their forties, fifties, or older.
- Most common potentially lethal autosomal recessive disease affecting caucasians; characterized by chronic pulmonary, intestinal, liver, pancreatic, and exocrine gland dysfunction; caused by mutations of the cftr chloride channel.
- E84 Cystic fibrosis
- E84.0 Cystic fibrosis with pulmonary manifestations
- E84.1 Cystic fibrosis with intestinal manifestations
- E84.11 Meconium ileus in cystic fibrosis
- E84.19 Cystic fibrosis with other intestinal manifestations
- E84.8 Cystic fibrosis with other manifestations
- E84.9 Cystic fibrosis, unspecified