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Signs of cystic fibrosis.

A complex systemic disease, cystic fibrosis is of interest due to its widespread prevalence among diseases of a hereditary nature. The disease is characterized by multiple forms of manifestation of lesions of internal organs as a result of disruption of the glands of external secretion, secreting secretions into the external environment or cavities of internal organs (mucus, sputum, saliva, lacrimal fluid, sweat, etc.). The secretory fluid, as a result of a violation of the transmembrane transport of electrolytes in the cells of the epithelium lining the inner surface of the gastrointestinal tract, lungs, pancreas, liver, reproductive organs, becomes too thick, viscous and cannot perform its functions in full.

To a greater extent, the gastrointestinal tract and the respiratory system suffer from a violation of the functional characteristics of the external secretion, for this reason, several forms of the existence of cystic fibrosis are distinguished: intestinal, pulmonary, mixed. The rest of the forms of cystic fibrosis with damage to other organ systems are worn out, they are classified as atypical manifestations of cystic fibrosis.

Signs of cystic fibrosis in bronchopulmonary form are often camouflaged under various respiratory diseases (chronic bronchitis, obstructive bronchitis, pneumonia, and others). A person develops a painful cough without sputum discharge, wheezing breathing, shortness of breath, cyanosis appears (blue of the red border of the lips), which indicates respiratory failure. The temperature can rise to 38-39 degrees. It is all the fault of clots of secretion (mucus) that accumulate in the lung tissue, without the possibility of natural discharge at the moments of coughing. They form plugs, which in turn contribute to the modification of the lung tissue, its fibrolization, the collapse of the pulmonary alveoli, and the formation of cysts in the lung tissue.

Congestion in the lungs is a factor that provokes the addition of a bacterial infection, since a favorable environment is created for the reproduction of pathogens. This is where the abscess formation of lung tissue originates.

Signs of cystic fibrosis in the intestinal form of its development are similar to the symptoms of bowel disease with enzymatic deficiency. The intestinal form of cystic fibrosis is characterized by the difficulty of enzymatic processing of food that has entered the intestine, as a result of a high degree of viscosity of the intestinal mucous secretion, which interferes with the work of pancreatic enzymes. Against the background of such violations, the assimilation of nutrients is disturbed, which leads to dystrophy, hypovitaminosis, metabolic disorders. Too thick mucous secretion also makes it difficult to evacuate food waste, which causes intestinal congestion, colitis, constipation, and intestinal obstruction.

Disturbances in the work of the digestive system also affect the work of other organ systems, since the lack of minerals and vitamins affects the work of the cardiovascular system, the condition of the skin, reproductive and endocrine activity. It is for this reason that cystic fibrosis is classified as a systemic disease, the treatment of which is difficult.

But, despite the difficulties, cystic fibrosis is a disease, the treatment options for which are of interest in scientific circles.

The treatment of cystic fibrosis is based on drug treatment and diet therapy.

For the treatment of intestinal cystic fibrosis, enzyme replacement therapy is used, which is complemented by a diet full of fats, proteins and carbohydrates, as well as vitamin therapy.

Treatment of pulmonary cystic fibrosis is based on mechanical cleaning (suction) of viscous mucus from the lungs, inhalations with drugs to expand the lumen of the bronchi and antibiotics to extinguish an intrapulmonary infection.