Cystic fibrosis is one of the most common autosomal recessive hereditary diseases common among the European race, which is an actual cause of morbidity and mortality among children and adolescents. In recent decades, the number of patients with cystic fibrosis has increased significantly due to improved diagnostics and a more comprehensive approach to therapy. Cystic fibrosis (CF) is an inherited disease caused by systemic dysfunction of the exocrine glands. In recent years, scientific knowledge about CF, the mechanism of its development at the cellular and molecular levels, has rapidly developed.

The microbial landscape in CF is rather specific. In early childhood, Staphylococcus aureus dominates, then hemophilic bacillus (Haemofilus influaenzae) joins, in adolescence, Pseudomonas aeruginosa appears. In recent years, especially in Western Europe and America, Pseudomonas cepacia, or Burgholderia cepacia, which is resistant to most antibiotics, has been frequently plated. The accession of Pseudomonas aeruginosa can have various consequences for the patient: from asymptomatic carriage to, more often, a significant deterioration in the course of the disease with a pronounced activation of the inflammatory process and deterioration of functional indicators. Based on clinical, functional and microbiological data, the tactics of treating cystic fibrosis are being built.

The main goals of the therapy are:

• reduction of bronchial obstruction;
• fight against infection;
• improvement of the patient's nutritional status.

It is necessary to conduct active treatment of concomitant complications, compensate for the resulting respiratory and heart failure, and provide maximum comfort for the patient in the terminal period of the disease.

New, revolutionary treatments for cystic fibrosis (eg. gene therapy) are under development.

Source: Amelina E.L., Chuchalin A.G. Cystic fibrosis: a modern approach to diagnosis and treatment. RMJ. 1997; 17; 8