Аннотация
Cystic fibrosis (CF) is an autosomal recessive monogenic hereditary disease characterized by dysfunction of the exocrine glands with damage to vital organs and systems, primarily the respiratory and digestive systems. Cystic fibrosis is associated with a reduction in life expectancy, the most common cause of death is progressive lung damage with the development of respiratory failure (RF). It is RF that most often determines the prognosis of CF, so timely diagnosis and correction of this complication is necessary. According to changes in the gas composition of arterial blood, there are 2 types of respiratory failure: hypoxemic and hypercapnic. Taking into account the types of RF, the type of respiratory support is determined: oxygen therapy and / or non-invasive ventilation (NIV). NIV has a positive effect on lung function, arterial blood gas parameters, body weight, duration and quality of life of patients with severe CF. Positive effects of NIV also include the effect of improving sputum drainage due to significant improvement of collateral ventilation. In recent years, due to the introduction of targeted therapy, the clinical picture of patients with CF has changed due to a decrease in the frequency of exacerbations of chronic bronchitis, improvement of functional indicators, and slowing down of the degradation of pulmonary function. However, the use of NIV in patients with CF remains relevant in cases where there is chronic hypercapnic respiratory failure, pronounced bronchopulmonary changes have formed in the form of widespread bronchiectasis, chronic, frequently recurring infectious process and pronounced impairment of sputum drainage. NIV is also indicated when targeted therapy cannot be prescribed due to the absence of corresponding mutations in the CFTR gene or if patients with CF do not have access to targeted therapy. In this regard, the accumulation of experience in NIV in patients with CF is an urgent task.