Cystic fibrosis (testaferros pancreas, entero-broncho-pancreatic dysphoria, congenital pancreatic steatorrhea) is a hereditary disease mainly affecting the exocrine glands (mucosa, salivary and others). Most often affects the gastrointestinal tract, pancreas, the organs of respiration. Too viscous mucus clogs ductless glands, which leads to secondary changes of the affected organs. The defeat of the pancreas are found in 80% of all cases of cystic fibrosis. Morphologically this is manifested cystic degeneration glands and mucous membrane of the small intestine.
The clinical picture of the disease is variable and depends on the age of the patient. The neonatal cystic fibrosis often revealed in the form of meconium plug obstruction: marked vomiting, pathogenie meconium (see), increasing abdominal distension of the intestinal loops, detected by palpation or visible. Threatening complication meconium plug obstruction is peritonitis. In the absence of surgical intervention disease usually ends lethally.
Another form of cystic fibrosis - intestinal, which is, as a rule, at the age of 1-3 years and due to exocrine insufficiency of pancreas and violations in this regard, the processes of digestion and assimilation of nutrients in the small intestine. Clinical signs of these changes are: rich foul-smelling stools, presence in the stool of a large number of undigested fat, starch, protein. In the study of enzymes duodenal contents revealed a decrease in the activity of trypsin, amylase, lipase. The decreased activity of these enzymes indirectly indicates a negative test for iodine content in saliva and urine after loading yodolipola, insufficient increase in the blood of nitrogen amino acids after intake of gelatin and glucose after intake of starch. Violation of the processes of nutrient absorption in the gastrointestinal tract leads to the development of malnutrition in children. Late complications of cystic fibrosis is the defeat of the islets of Langerhans of the pancreas (see) and the development of symptoms of diabetes. In some cases there are cirrhotic changes in the liver, development of peptic ulcers. Common manifestation of cystic fibrosis, alone or combined with the symptoms of other bodies, may be the defeat of the respiratory system, proceeding by the mechanism of chronic pneumonia with frequent exacerbations. In future this leads to the formation of bronchiectasis with a characteristic clinical and x-ray picture (see Bronhoektatical disease).
Diagnostics of cystic fibrosis is based on clinical symptoms, results of a study of enzymes duodenal juice (reduced activity). An important diagnostic feature of all forms of cystic fibrosis is a higher concentration of electrolytes (potassium, sodium, chlorine) in the sweat and saliva.
Treatment of cystic fibrosis is determined by the form of the disease, but the nature mainly symptomatic therapy. With the development of meconium plug syndrome obstruction in the newborn needs urgent hospitalization and surgical treatment. From the diet of patients with cystic fibrosis children should be excluded rough, indigestible food, limited to a few fat and starch. Children's food must be carefully mechanical and culinary processes. At insufficiency of pancreas appoint enzymes inside: Pancreatin, at low pH - pepsin with hydrochloric acid.
With the defeat of the respiratory, along with the usual methods of treatment applied in cases of chronic pneumonia (see), recommended regimens iontophoresis and Pancreatin, aerosol inhalation of crystalline trypsin (see Aerosol). These procedures promotes segregation and easier Department of mucus from the respiratory tract.