The article discusses results of a prospective observational study of long-term use of the biosimilar dornase alfa (Tigerase) (Generium, Russia) as part of complex therapy in patients with cystic fibrosis in real clinical practice.The aim was to analyze the outcomes of long-term use of the dornase alfa dornase alfa as part of complex therapy in patients with CF (protocol #DRN-CFR-N01).Methods. The study included patients (n = 165) aged 5 years and older from 11 centers for treatment of cystic fibrosis in the Russian Federation with a confirmed diagnosis of cystic fibrosis who were prescribed dornase alfa by their attending physician.Results. The analysis revealed that exacerbations of chronic pulmonary disease during the treatment with dornase alfa were observed in 29 (17.58%) patients included in the study. At the same time, there were no statistically significant changes in FEV1 and FVC (%) against baseline during the treatment in the study population. Adverse events related to the study drug were recorded in 9 (5.45%) patients.Conclusion. Biosimilar dornase alfa demonstrated a favorable efficacy and safety profile in routine clinical practice, which confirms the results of previously published studies.

Chronic lung infections are a consequence of the disturbance of mucociliary clearance process in cystic fibrosis. For most patients with cystic fibrosis, chronic lung infection is associated with a poor prognosis. The impact of chronic Pseudomonas aeruginosa infection on progressive deterioration of lung function and nutritional status has been established. Timely and effective antibiotic therapy aimed at eradication or control of gram-negative flora affects the duration and quality of life. The purpose of the study . To investigate the safety and efficacy of inhaled administration of sodium colistimethate (Colimistin®). Methods . The study enrolled 42 patients (27 patients aged 5 to 17 years and 15 patients over 18 years) with an established diagnosis of cystic fibrosis, 38 with monoculture of P. aeruginosa or various associations, 4 with Achromobacter spp . culture. Microbial status, external respiratory function, nutritional status, assessment of well-being, adverse reactions, exacerbations, and use of antibiotic therapy during colimistin inhalations were recorded in all patients at baseline and at 3 months. Results . A significant improvement in nutritional status in terms of weight ( p < 0.007) and height ( p < 0.001) was shown in the general patient group and the children’s group. In the group of children, there was a significant increase in weight ( p < 0.034) and height ( p < 0.0001). In the group of patients older than 18 years, there was a significant increase in weight ( p < 0.045) and BMI three months after therapy ( p < 0.013). There were no significant improvements in FVC and FEV1. The treatment efficacy was shown by the assessment of well-being in the general patient group ( p < 0.001) and in the children’s group ( p < 0.002). No significant difference was found in the adult patient group ( p < 0.067). Two patients dropped out of the study due to ADR at the start of therapy. Conclusion . Sodium colistimethate showed efficacy and safety in bronchopulmonary infections caused by P. aeruginosa in monoculture and in association with Achromobacter spp . and may be recommended for use in children and adults with cystic fibrosis.

Aim. To establish the etiological structure and to present clinical and laboratory and instrumental characteristics of bronchiectasis (BE) not associated with cystic fibrosis (CF) in children. Materials and methods. Sixty-seven hospitalised patients with BЕ not related to CF were followed up between 2017 and 2022. Examination methods: clinical-anamnestic method, general clinical laboratory investigations, investigation of allergological and immune status, phagocytosis system, determination of concentration of specific IgE and IgG to fungi of genus Aspergillus, sweat test, radiological examination and computed tomography (CT) of chest organs, bronchoscopy, Bacteriological examination of sputum and/or tracheobronchial aspirates, nasal and/or bronchial ciliary motility, esophagogastroduodenoscopy, 24-hour pH-metry, intra-esophageal combined impedance-pH-metry, genetic study, lung biopsy. Results. Etiologic factors of BЕ not associated with CF in children were severe pneumonia (22%), primary ciliary dyskinesia (22%), bronchial asthma (13%), Williams-Campbell syndrome (7%), bronchial foreign bodies (7%), gastroesophageal reflux disease (6%), Bronchopulmonary dysplasia (6%), postinfectious bronchiolitis obliterans (5%), allergic bronchopulmonary aspergillosis (3%), chronic granulomatous disease (3%), AIDS (1%), prolonged bacterial bronchitis (1%), brain-lung-thyroid syndrome (1%). The clinical picture is characterized by cough (91%), shortness of breath (67%), fever during exacerbation (48%), chest pain (24%), exercise intolerance (55%), drumstick symptom (9%), moist (76%) and dry wheezing (37%). CT-semiotics of BЕ not associated with CF is characterized by localization in one (58%) or several (42%) lobes; traction (42%), non-traction (49%) B and their combination (9%); increased broncho-arterial ratio 0.9; thickening of bronchial wall; "mosaic perfusion"/"air-trap" symptom (9%); more frequent involvement of lower lungs (64%). The main infectious agents in BЕ not associated with CF were Haemophilus influenzae, Pseudomonas aeruginosa, Staphylococcus aureus. Conclusion. On the basis of a multicentre study, the etiological structure, clinical and laboratory and CT-characteristics of non-CF ВE in children were established.

The purpose of the study was to determine the predictors of severe bronchiectasis (BE) not associated with cystic fibrosis (CF) in children and to evaluate the effectiveness of a stepwise complex conservative therapy. Materials and methods of the study: study design - multicenter cohort prospective pilot study. 67 hospitalized pediatric patients aged from 11 months up to 17 years old (52% boys and 48% girls) with BE caused by previous pneumonia (22%), primary ciliary dyskinesia (22%), bronchial asthma (13%), Williams-Campbell syndrome (7%), bronchial foreign bodies (7%), gastroesophageal reflux disease (6%), bronchopulmonary dysplasia (6%), postinfectious bronchiolitis obliterans (5%), allergic bronchopulmonary aspergillosis (3%), chronic granulomatous disease (3%), AIDS (1%), protracted bacterial bronchitis (1%), and brain-lung-thyroid syndrome (1%) were observed. Predictors of severe BE were determined based on a comparison of groups of patients with mild (up to 4 exacerbations of BE per year) and moderate (from 4 to 6 exacerbations) course of BE (n=31) and patients with severe course of BE (>6 exacerbations per year, n=36). At the second stage of the study, in patients with changes in the severity of the course of BE, who were followed in dynamics (n=42), the frequency of exacerbations was assessed during the year after the appointment of a stepwise complex conservative therapy, which included, depending on the severity, daily drainage massage, exercise therapy, auxiliary devices for the respiratory tract clearance, long-term anti-inflammatory azithromycin, inhaled/intravenous antibiotics, and/or respiratory support. Results: the clinical picture of severe BE occurring with >6 exacerbations per year compared with mild/moderate course of the disease (≤6 exacerbations per year) is characterized by a statistically significantly more frequent registration of dyspnea (86% and 45%, p<0.001), exercise intolerance (69% and 39%, p=0.012), wet rales (89% and 61%, p=0.011), finger clubbing (17% and 0%, p=0.027). The prognostic model for determining the likelihood of severe BE not associated with CF in children includes the age of manifestation, a positive result of bacteriological examination of sputum/aspirates from the respiratory tract, localization of BE in the middle lobe of the right lung and/or lingual segments, and dyspnea. The appointment of gradual conservative therapy for BE, depending on the severity/frequency of exacerbations, makes it possible to statistically significantly reduce the severity of BE (p<0.001) and the median of exacerbations during the year from 9.00 [3.25-12.00] before treatment to 2.00 [1.00-3.00] after treatment (p<0.001). Conclusion: BEs are heterogeneous in severity, which determines the choice of therapy. Conservative therapy of BE not associated with CF in children has a stepwise principle depending on the severity of the course. Its appointment could therefore reduce the frequency of exacerbations.

Cystic fibrosis (OF) is characterized by disorders of chloride secretion and sodium absorption in exocrine epithelium. A crucial location of these ion disorders is the respiratory epithelium. Such ion pathology forms a transepithelial electric potential difference. It is hard to measure tracheobronchial electric potential difference, so a method for measuring nasal potential difference (NPD) was created. We measured baseline values of NPD in 100 patients (including 45 OF patients) and in 15 healthy volunteers. More significant negative values of the average baseline NPD were registered in the OF patients (42.2±1.4 mV) compared with healthy and COPD persons (-18.3±1.8 and 19.2±0.6 mV accordingly, p <0.0001). NPD values in 6 (13%) OF patients with typical clinical features, normal or boundary sweat test results and CF gene confirmation were compatible with CF bioelectric profile. Meantime 3 COPD patients had increased sweatiest results and a low NPD level. Under amiloride hydrochloride blocking sodium channels the basal NPD was inhibited greatly (up to 66%) in CF patients, whereas the same value in COPD patients was 36.7%.Therefore, the NPD reflects the principal CF disorder. Its increase under the amiloride influence more than 60% is thought to be used as an additional diagnostic test.

25 patients with CF and 20 with others of COPD were examinated. Ciliary beat was measured by a lifetime TV microscopy of bioptates of respiratory mucous. Material was obtained with the help of brush biopsy during fiberoptic bronchoscopy. The image of ciliary beat from a light microscope was recorded on the hard disk of the personal computer. The specially created program estimated ciliary beat frequency (CBF) and amplitude of ciliary beating. CBF into bronchi of the CF patients have made at impact phase – 6.3+0.33 Hz, raising phase – 5.7+0.36 Hz; in the patients with COPD: 6.5+0.32 Hz and 6.2+0.33 Hz accordingly. The precise tendency to a drop of CBF and amplitude of ciliary beating in the patients with CF on a comparison with the patients with COPD and literary datas was marked. Also, the drop of indexes of beating in the patients with Ps.aeruginosae mucoid were marked.

This article demonstrates high clinical efficacy of long-term treatment of bronchial obstructive syndrome in cystic fibrosis (CF) patients with N-acetylcysteine (Fluimucil, Zambon Group). Lung function parameters, oxygen saturation, sputum viscosity, biochemical parameters of inflammation activity in sputum (elastase, sialic acid and α 1 -antitripsin concentrations) were evaluated. Oral Fliumucil was combined with inhaled N-acetyl-Lcysteine. The therapy resulted in reduction of the sputum viscosity, recovering of the mucociliary clearance, improvement in the lung function, antiinflammatory and antioxidative effects of Fliumucil. This allows to recommend the drug for the long-term basic therapy of bronchoobstructive syndrome in CF patients.

Background: In developed countries there are significant gender differences in lifetime expectancy that can be explained by behavioral risk factors (RF). Objective: The aim of our study was to estimate gender features of behavioral RF in general population of Saint-Petersburg, Russia. Methods: As a part of all-Russian epidemiology survey ESSE-RF a random sampling of 1600 Saint-Petersburg inhabitants (25-64 y.o.) stratified by age and sex was performed. All participants filled in the questionnaire. Anthropometry (weight, height, body-mass index (BMI), waist circumference (WC)) and fasting blood-tests (lipids, glucose by Abbott Architect 8000 (USA)) were performed. Results: There were examined 573 (36%) men and 1027 (64%) women. No gender differences in obesity were found according to BMI criteria — in 178 (31.2%) women and 352 (35.1%) men. Obesity was more often detected in females according to WC criteria: АТРIII — 44.1 vs 30.3%; IDF 51.2 vs 66.4% (p <0.001 for both). Linear regression analysis was performed and age was associated with BMI — 1.6 kg/m 2 /decade, WC in women — 5,2 cm/decade and WC in men — 2.8 cm/decade, р <0.001 for all anthropometric parameters. Optimal level of physical activity was equally documented in both genders — 540 (61.2%) women and 286 (58.9%) men. Daily intake of sweets was lower in men — 228 (39.8%) vs 539 (52.5%) in women (p <0.001). 810 (50,6%) of trial subjects were non-smokers, 395 (24,7%) were former smokers, and 395 (24,7%) were smokers at the moment of trial. The higher number of female smokers was observed — 194 (19.1%). Conclusion: A high prevalence of obesity is observed in sample of Saint-Petersburg inhabitants — it is higher among women according to WC criteria regardless of menopause, possibly due to bigger sweets consumption. Males smoke more often and consume less fresh fruits and vegetables which is accompanied by a higher prevalence of hyperglycemia and hypertriglyceridemia.

It’s well known that neutrophils being fundamental effectors of the host protective from bacteria infection can generate various oxidants and proteins with antibacterial functions such as myeloperoxidase (MPO) and lactoferrin (LF). The aim of our study was to determine the MPO and LF concentrations in serum, sputum and lavage of patients with cystic fibrosis in minimum activity and exacerbation periods of disease, with and without Ps. aemginosa. High levels of MPO and LF had been determined in blood, sputum and lavage fluid in minimum activity of disease. There were no difference in MPO and LF serum concentrations between minimum activity and exacerbation periods of disease, but at the same time concentrations of these proteins were significantly higher (p