Cystic fibrosis (CF) remains one of the most studied inherited multisystem diseases, in which Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein dysfunction leads to progressive respiratory failure, exocrine and endocrine pancreatic insufficiency, and other organs involvement. Over the past decades, approaches to CF diagnosis and treatment have changed significantly due to the neonatal screening introduction, the development of molecular genetic diagnostic methods, and, especially, the advent of targeted therapy – CFTR modulators. Updated clinical guidelines for cystic fibrosis were published in 2025, reflecting not only current scientific data but also Russian clinical experience with the use of CFTR modulators registered in Russia, including the most effective combination of elexacaftor/tezacaftor/ivacaftor + ivacaftor. This updated document aims to clarify diagnostic and therapeutic approaches in the new conditions for the provision of medical care for patients with CF and carriers of pathogenic CFTR gene variants.

The aim is to review the key new additions to the 2025 clinical guidelines (CG) and discuss their practical implications for physicians involved in the care of patients with CF.

Methods. Eighty experts from various specialties worked on the new guidelines using 520 literary sources.

Results. The updated 2025 cystic fibrosis clinical guidelines contain updated sections on molecular genetic and microbiological testing, pulmonary mycobacteriosis treatment, and new antimicrobial agents for treatment of respiratory tract infections. The features of CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor + ivacaftor), off-label drug administration, and post-organ transplantation are described for the first time. Special attention is given to subsections devoted to changes in CF basic therapy during treatment with CFTR modulators, adverse reactions, heterozygous carriers of a pathogenic CFTR variant, and the spectrum of CFTR-related diseases, the course of pregnancy during treatment with CFTR modulators, and monitoring newborns of mothers receiving this therapy.

Conclusion. This updated version of the clinical guidelines incorporates current information and will improve the diagnosis and treatment of cystic fibrosis and standardize the care of pathogenic CFTR gene variants carriers and individuals with CFTR-associated conditions in Russia.

The lack of clinical guidelines for pneumothorax in pediatric practice leaves many questions unanswered. These questions begin with terminology. Key definitions of terms associated with pneumothorax should be outlined in a position paper. There is no pediatric classification of pneumothorax, although a number of diseases occur exclusively in children. Most diseases that include pneumothorax as a symptom are genetically determined. Currently, questions remain regarding the conservative and surgical management of children with pneumothorax in intensive care units and surgical hospitals, as well as subsequent follow-up.

The aim was to prepare a literature review for the development of a working version of clinical guidelines and to identify expert opinions on key complex and controversial issues.

Results. Proposals are made for the etiologic classification of pneumothorax in children, with an emphasis on hereditary forms of the disease. Issues of radiological and molecular genetic diagnostics are discussed. Treatment algorithms and follow-up are discussed.

Conclusion. Therefore, the development of clinical guidelines for pneumothorax is a pressing issue in health­care practice and requires discussion.

Currently, primary ciliary dyskinesia (PCD) is one of the most common hereditary diseases of the respiratory tract. The prevalence of PCD is estimated at approximately 1 case per 10,000 - 20,000, but this figure varies significantly among studies. Despite advances in diagnostics (genetic polymorphism is associated with > 60 genes in PCD), many patients are still diagnosed late, even in adulthood. The exact prevalence of PCD in the Russian Federation is unknown.

The aim of the study was to present the National Registry of Patients with PCD in the Russian Federation, which records health characteristics of the patients, their genetic diversity, assessment of the respiratory tract microbiome, and the organization of treatment.

Methods. The registry included patients (n = 109: 71 children, 38 adults) from 34 regions of the Russian Federation with a genetically confirmed diagnosis of PCD (the presence of 2 pathogenic variants, accounting for the mode of inheritance). A comprehensive diagnostic protocol according to the recommendations of the European Respiratory Society (ERS) was used. The study also included testing of respiratory tract biomaterial samples from patients using classical bacteriological methods with subsequent identification using matrix-assisted laser desorption/ ionization time-of-flight mass spectrometry (MALDI-TOF MS).

Results. The average age at diagnosis was 14.6 ± 13.8 years (median 10 years), indicating a significant diagnostic delay, especially in adults (median 32 years). In total, pathogenic variants were identified in 29 genes. The genes with an autosomal recessive inheritance pattern predominated (95.9%). The most frequent mutations were in the DNAH5 (39.4%) and DNAH11 (9.2%) genes. The most common clinical manifestations of the respiratory tract were chronic rhinosinusitis (90.8%), wet cough (93.6%), and bronchiectasis (76.3% of adults). A study of the etiologic structure of respiratory tract infections revealed that Staphylococcus aureus (53.8%) and Pseudomonas aeruginosa (38.7%) were the predominant bacteria across all age groups.

Conclusion. The study revealed deficiencies in the organization of care for patients with PCD, emphasizing the need for standardized diagnostic algorithms and early molecular genetic diagnosis to improve prognosis and quality of life.

Cystic fibrosis (CF) is a hereditary multisystem disorder characterized by progressive damage to the respiratory and gastrointestinal systems. Data on adult patients with CF are limited in the Republic of Kazakhstan (RK), and the lack of a national registry makes it difficult to evaluate the clinical and functional characteristics of this population.

The aim was to present the genotypic and phenotypic characteristics of adult patients with CF in the RK.

Methods. Data from 29 adult patients (18 years and older) with a confirmed diagnosis of CF from various regions of the RK for 2024 were analyzed. Clinical, anthropometric, functional, laboratory, microbiological, and molecular genetic parameters were assessed according to European Cystic Fibrosis Society Patient Registry (ECFSPR) protocols.

Results. The mean age of patients was 23.3 years, and the median age at diagnosis was 9 years. BMI was below the target values (19.17 kg/m²). Exocrine pancreatic insufficiency was detected in 80% of patients (fecal elastase < 200 pg/g), predominantly in those with “severe” CFTR genotypes. The F508del mutation was detected in 45% of alleles. “Severe” genotypes, associated with earlier diagnosis, low pancreatic function, and high sweat chloride levels, were identified in 72.4% of patients. The mean FEV₁ value was 60.7% predicted, with 35.8% of patients experiencing severe or very severe airflow obstruction. Chronic Pseudomonas aeruginosa infection was detected in 69% of patients. Initiation of CFTR modulator therapy was associated with a reduction in the exacerbation frequency and the need for intravenous antibiotic therapy in most patients.

Conclusion. Adult patients with CF in Kazakhstan are characterized by late diagnosis, decreased nutritional status and respiratory function, and a high rate of exacerbations. Data integration into the ECFSPR is a key step toward developing systemic care and optimizing CF therapy in the country.

Cystic fibrosis is an autosomal recessive disease caused by mutations in the CFTR gene. CFTR modulators are a complex of CFTR protein potenti­ator and correctors that improve chloride channel function. Currently, the most effective targeted therapy drug is the combination of elexacaftor/ tezacaftor/ivacaftor (ETI). However, data on the long-term efficacy and safety of triple combination therapy in real-world clinical practice in the Russian Federation are limited.

The aim is to analyze the efficacy and safety of therapy with the three-component elexacaftor/tezacaftor/ivacaftor based on data from the registry of patients with cystic fibrosis of the Russian Federation for 36 months.

Methods. A retrospective study was conduct­ed using data from the Russian Federation Cystic Fibrosis Patient Registry in the Targeted Therapy section for 2021 - 2024. The study included 900 patients over 6 years old with cystic fibrosis receiving triple targeted therapy with ETI. The following criteria were assessed for the effectiveness of therapy: sweat test, anthropometric data (weight, height, BMI), lung function parameters (FVC and FEV₁ in absolute (L) and relative (% predict­ed) values), at the start of therapy and over 3 years. The following safety criteria were assessed in pairwise comparisons: ALT (U/L), AST (U/L), total bilirubin (^mol/L), systolic and diastolic blood pressure during 3 years of therapy.

Results. Comparison of the sweat test results, FEV1 and FVC, BMI at the start of therapy, after one year, 2 years and 3 years showed a significant decrease in sweat test results, an improvement in anthropometric data (BMI), an increase in absolute and relative indicators of respiratory function (FVC, FEV₁) during the first year of therapy, and stabilization of indicators on during the second and third years of therapy. The safety profile of ETI was characterized by stable median values of ALT, AST, and total bilirubin within the reference values at three-year follow-up. Adverse reactions were reported in 9.4% of cases at the start of therapy. After three years of therapy, the adverse reactions were rare and more related to the mental sphere.

Conclusion. Patients with cystic fibrosis who received three-component ETI therapy showed significant improvement of basic health indicators compared to the initial ones they had while receiving only basic therapy. The long-term efficacy and safety of the elexacaftor/tezacaftor/ivacaftor combination has been demonstrated for three years.

Over the past 40 years, the mean survival age of patients with cystic fibrosis (CF) has increased, driven by advances in diagnosis and treatment. This progress has also been linked to the level of care provided in CF treatment centers.

Aim. To demonstrate the effectiveness of monitoring in a special­ized department/center providing care for patients with CF.

Methods. This study of regular follow-up and microbiological monitoring included 193 patients with cystic fibrosis aged 0 to 17.9 years from the Moscow region. Data for the period from December 2021 to December 2024 were compared. Data from the Russian Federation Cystic Fibrosis Patient Registry for 2021 - 2023 were used in the comparative analysis of the patient health status.

Results. Improved pulmonary function was demonstrated in patients with CF in the Moscow region. Forced expiratory volume (FEV₁) significantly increased from 88.15 ± 20.64%_pred at the end of 2021 to 93.1 ± 18.7%_pred at the end of 2024 (M ± SD; p < 0.00001). Forced Vital Capacity (FVC), increased from 93.23 ± 16.40% to 95.4 ^.± 15.2% in 2024 (M ± SD; p < 0.000^.01). The number of days of intravenous therapy for exacerbations of the bronchopulmonary process decreased from 6 ± 12.25 to 1.3 ± 7.16 (p < 0.00001). Between 2021 and 2024, the proportion of children chron­ically infected with Pseudomonas aeruginosa in the Moscow Region decreased from 18 % to 7.3% against active follow-up care. Similarly, the num­ber of patients with recurrent P. aeruginosa growth decreased from 17.67% to 6.16%. The number of patients with Methicillin-susceptible Staphylococcus aureus (MSSA) growth increased from 76% to 89.2%. Fewer patients tested positive for Methicillin-resistant S. aureus (MRSA) - 8% in 2021 and 3.14% in 2024. The number of patients with Burkholderia cepacia complex (Bcc) growth increased in 2022 compared to 2021, but no Bcc growth was detected in 2024. The number of patients with Achromobacter spp. decreased from 3.03% to 0.52%.

Conclusion.The key disease outcomes vary sig­nificantly in CF patients with different levels of healthcare. CF patients who were followed in specialized centers had better outcomes than patients who were not regularly followed and lacked access to continuous microbiological monitoring. It is necessary to establish cystic fibrosis centers that meet international standards in all regions of the Russian Federation.

Polypous rhinosinusitis (PRS) is one of the main manifestations of cystic fibrosis (CF). Elexacaftor/tezacaftor/ivacaftor (ETI) is a CFTR modulator that improves lung function and prevents the progression of PRS in CF by restoring transmembrane conductance.

The aim of the study was to evaluate the dynamics of the radiographic image of the paranasal sinuses (PNS) and chest organs (CHO) in children receiving the CFTR modulator ETI.

Methods. An examination of children with CF (n = 22) was conducted. X-ray examination was performed in the amount of computed tomography (CT) of the PNS and CHO before and after 12 months of therapy with ETI. The volume of PNS damage was assessed according to the Land - Mackay scale. The volume of lung damage was assessed according to the Brody scale. Lung function parameters (forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) and sweat conductivity) were also determined.

Results. A significant decrease in the Land - Mackay score from 15 to 5.5, marked improvements in the reduction of mucus plugs in the peripheral areas, a decrease in peribronchial infiltration, consolidation of lung tissue, and air traps were observed. An increase in FVC from 88.5% (84.0% - 102.3%) to 101% (92.5% - 102.3%), FEV₁ from 87.5% (77.8% - 106.0%) to 105% (96.0% - 110.5%), and a decrease in sweat test from 113.0 (101.0 - 120.0) mmol/L to 63.0 (50.0 - 77.0) mmol/L were observed.

Conclusion. ETI has been demonstrated to be highly effective in the treatment of PRS by improving lung function and reducing sweat test values.

Pulmonary alveolar proteinosis (PAP) is a rare syndrome associated with abnormal accumulation of (lipo-)protein material in the alveoli, often due to decreased granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling activity, dysfunction of alveolar macrophages, or abnormal surfactant metabolism. Autoimmune PAP accounts for 90% of all cases of PAP and has a prevalence of 7 - 10 cases per million in the general pop­ulation.

The aim of the study is to summarize current approaches to the diagnosis and treatment of pulmonary alveolar proteinosis based on recent clinical guidelines.

Conclusion. Nonspecific clinical presentation, radiographic findings, and normal routine laboratory results often lead to misdi­agnosis of PAP and prolonged path to the diagnosis. Serum testing for GM-CSF autoantibodies has revolutionized the diagnosis of autoimmune PAP, making lung biopsy unnecessary in most cases. Therapy for PAP has evolved significantly in recent years, and European Respiratory Society (ERS) guidelines (2024) proposed the first clear, hierarchical treatment algorithm. Whole lung lavage, historically a first-line treatment, should now be considered alongside inhaled GM-CSF, based on numerous high-quality studies published in recent years. The ERS guidelines clarify the treat­ment sequence for refractory autoimmune PAP, with rituximab considered a third-line treatment and plasmapheresis a fourth-line treatment.

Recent research findings indicate that assessment of the nutritional status of people with cystic fibrosis (CF) should account for not only BMI but also body composition. Estimating fat and lean mass is equally important because these characteristics are associated with respiratory function indicators stronger than traditional anthropometric parameters. A low or normal BMI may mask high fat mass or low lean (muscle) mass. Researchers report a positive effect of targeted therapy on BMI, but the impact on body composition has not been adequately studied.

The aim of this paper was to review the results of studies assessing body composition in patients with cystic fibrosis. The review presents data on methods for assessing body composition, the advantages of body composition assessment over traditional anthropometric measurements, body composition characteristics in patients with cystic fibrosis, the impact of body components, in particular fat and lean mass, on respiratory function, changes in body composition during targeted therapy, and the emergence of cardiometabolic risks.

Methods. We selected publications in the PubMed database using the following keywords: “body composition in patients with cystic fibrosis” and “body composition in patients with cystic fibrosis during targeted therapy”.

Conclusion. Body composition assessment can be an important tool for monitoring patient’s nutritional status, assessing effectiveness of targeted therapy, and guiding dietary modifications and selection of therapeutic nutritional supplements for patients with cystic fibrosis.

Cystic fibrosis (CF) is a hereditary disease caused by mutations in the CFTR gene, which lead to dysfunction of the chloride channel protein. CFTR modulators correct the function of the defective protein and were a breakthrough. Ivacaftor/lumacaftor and elexacaftor/tezacaftor/ivacaftor combinations are registered in Russia, but their use is officially approved only for children aged 2 years and older.

The aim of the study was to present the first clinical case report describing the successful use of elexacaftor/tezacaftor/ivacaftor therapy in a 2-month-old critically ill patient with progressive respiratory failure. The drug was prescribed off-label by a committee decision. The treatment achieved a rapid, significant positive effect without serious adverse reactions, allowing the child to recover from a terminal condition.

Conclusion. This case demonstrates the feasibility and effectiveness of emergency use of modern CFTR modulators in infants with life-threatening CF. This successful experience justifies the need to revise and possibly expand the age-specific indications for targeted CF therapy in the clinical guidelines.

This article discusses the clinical experience of using triple targeted therapy (elexacaftor/tezacaftor/ivacaftor) in a patient with cystic fibrosis complicated by cystic fibrosis-related diabetes. The description includes details on the diagnostic process, the features of the disease course, and treatment approaches. In addition, the work pays special attention to the use of targeted drugs, as well as the need for an integrated approach to managing the condition of patients with combined disorders. To date, there has been insufficient research on the efficacy of the drug elexacaftor/ tezacaftor/ivacaftor and its effects on patients with comorbid conditions, particularly those suffering from cystic fibrosis-related diabetes.

The aim was to highlight modern research on this topic and to assess the effect of triple targeted therapy on the clinical manifestations of the disease.

Results. The patient showed significant improvement in the functional state of the lungs, control of blood glucose levels, and overall quality of life.

Conclusion. The article summarizes the importance of an individualized approach to therapy that considers both genetic and metabolic aspects of the disease. The conclusion emphasizes the importance of early, timely initiation of treatment to achieve optimal results and improve the prognosis of long-term life in patients with cystic fibrosis and related complications.

Cystic fibrosis is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which is expressed on the apical surface of epithelial cells. Advances in the management of cystic fibrosis have significantly improved patient survival. In recent years, CFTR modulators - drugs that target the underlying CFTR defect - have become available, leading to marked clinical improvement. However, data on their efficacy and safety in patients aged 65 years and older are limited.

The aim of this study was to demonstrate the effectiveness of CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor) in an elderly patient with cystic fibrosis.

Conclusion. Treatment with elexacaftor/tezacaftor/ivacaftor over 14 months in an elderly patient led to significant positive clinical, functional, and radiological changes. No significant adverse reactions or worsening of comorbid conditions were observed during the entire observation period.

Patients with cystic fibrosis (CF) have the highest risk of developing pulmonary mycobacteriosis. A pressing issue is the introduction of new drugs, such as bedaquiline, into pediatric clinical practice, as these drugs have shown proven efficacy against nontuberculous mycobacteria (NTM) in microbiological studies.

The aim of this study was to demonstrate the first experience of using bedaquiline in the treatment of pulmonary mycobacteriosis in two 14-year-old boys with CF.

Conclusion. Clinical, laboratory, and radiographic tests demonstrated the efficacy of bedaquiline when included in mycobacteriosis treatment regimens. A good tolerability was noted. Persistent isolation of Mycobacterium abscessus after 12 months of treatment in case 1 and M. intracellulare after 6 months in case 2 were considered carrier states. The use of bedaquiline did not result in the expected eradication of NTM, confirming the need for continued research and the search for new drugs for the treatment of pulmonary mycobacteriosis in patients with CF and implementation of these drugs into clinical practice.

Familial spontaneous pneumothorax (SP) is a rare genetic lung disorder characterized by unilateral or bilateral accumulation of air in the pleural space in patients with a positive family history and no underlying lung disease or prior chest trauma. One of the most common causes of SP is Birt - Hogg - Dubd syndrome (BHDS).

The aim of this study was to demonstrate clinical cases of spontaneous pneumothorax caused by genetic variants in the FLCN gene and the need for DNA testing.

Results. We presented three clinical cases of patients of different genders, ages, and medical histories who contacted our tertiary center to confirm the diagnosis. All patients had no evidence of altered alpha-1 antitrypsin levels, and their radiographic findings revealed pneumothorax. Despite the wide variety of clinical presentations among patients and their relatives, the diagnosis of BHDS was suspected and the pathogenic variant in the FLCN gene was subsequently confirmed in all of them. Two patients underwent surgery for pneumothorax. One patient was diagnosed with kidney cancer, one had fibrofolliculoma-like skin lesions. All patients were recommended dynamic follow-up, and their relatives were recommended further examination.

Conclusion. Since pneumothorax may be the first symptom of a complex multisystem genetic disease, specialists of various specialties should be aware of this disorder at all stages of medical care.

Congenital disorders of bronchopulmonary system are a group of rare diseases characterized by alterations in the morphological structure of the lungs, bronchi, and pulmonary blood vessels with manifestation in childhood. Congenital bronchopulmonary disorders are the third most common type of chronic lung and pulmonary conditions in children. The increase in the number of registered cases is associated with the use of modern imaging techniques, particularly computed tomography (CT). An urgent challenge in modern respiratory medicine is the development of methods for early diagnosis of congenital bronchopulmonary disorders, based on the integration and assessment of the diagnostic significance of key anamnestic, clinical and, potentially, radiological markers.

The aim was to present the most common congenital bronchopulmonary disorders using clinical cases of patients with primary ciliary dyskinesia and bilateral congenital lobar emphysema.

Methods. A retrospective analysis of the primary medical records of patients A and B (outpatient consultation reports and hospital discharge summaries) was performed, including an evaluation of their medical history, clinical presentation and diagnostic findings. Long-term personal follow-up data were also considered.

Conclusion. Raising awareness among primary care paediatricians about congenital bronchopulmonary disorders, including primary ciliary dyskinesia and congenital lobar emphysema, is essential for achieving early diagnosis.

The DNAAF11 gene, also known as LRRC6, follows autosomal recessive inheritance and plays a key role in the assembly of dynein, a protein necessary for the normal functioning of cilia. Mutations in this gene can lead to primary ciliary dyskinesia (PCD), a rare hereditary disease characterized by impaired function of the ciliated epithelium, primarily in the respiratory system but also in other organs. Defects in dynein arms associated with mutations in the DNAAF11 gene disrupt their rhythmic movement, leading to mucus stagnation, chronic inflammatory processes, and increased susceptibility to respiratory tract infections.

The aim of this work is to describe the clinical case of a family consisting of a mother and her son, both with a confirmed diagnosis of PCD. The study revealed a mutation in the DNAAF11 gene in both patients: the mother’s was in a homozygous state (NM_012472.6: c.436G>C), and her son’s was in a compound heterozygous state: one NM_012472.6: c.436G>C variant inherited from the mother (not previously described), and the second, NM_012472.6: c.1011A>G, inherited from the father. The child’s diagnosis was confirmed by segregation analysis.

Methods. The PCD diagnosis included: molecular genetic analysis, segregation analysis, video microscopy, electron microscopy of the ciliated epithelium, air-liquid interface (ALI) cell culture, and immunofluorescence staining.

Conclusion. This clinical case highlights the importance of identifying genetic relationships and features of PCD within families. The newly discovered mutations expand the spectrum of variants in the DNAAF11 gene associated with ciliary defects in PCD and emphasize the need for molecular genetic studies. Early diagnosis contributes to timely treatment initiation, preventing disease progression and improving patients’ quality of life.

Epidemiological data on patients with uncontrolled severe bronchial asthma (SA) in the Russian Federation are limited due to the lack of a unified system for routine monitoring of these patients.

The aim of this study was to describe the clinical and demographic indicators and characterize the routine treatment profile of Russian patients with uncontrolled SA who are not receiving treatment with biological agents.

Methods. This multicenter, observational, cross-sectional study included patients aged 18 years or older diagnosed with uncontrolled SA. Patients received drug therapy, were followed by a pulmonologist or allergist, and had access to follow-up data for 52 weeks or more. As part of the interim analysis, we summarized the demographic characteristics, medical history, and the results of laboratory and instrumental examination of patients (n = 691: 498 (72.1%) - female; mean age of participants - 56.3 ± 13.6 years). Different types of SA therapy, events of healthcare resource utilization and severe exacerbations during 52 weeks preceding enrollment in the study were recorded.

Results. It was established that the median duration of asthma at inclusion was 13 years. Over the previous 3 months, the most frequently used background therapy in 335 (48.5%) patients was a triple combination of inhaled corticosteroid + long-acting e2-agonist + long-acting anticholinergic drug. The most common approach to relief the attacks in 318 (46.0%) patients was monotherapy with a short-acting e₂-agonist. The proportions of patients with 1, 2, and at least 3 severe asthma exacerbations were 47.2%, 12.9%, and 10.1%, respectively. At least one unscheduled outpatient visit, at least one emergency department visit/urgent care call, and at least one hospitalization in the previous 52 weeks were recorded in 317 (45.9%), 103 (14.9%), and 438 (63.4%) patients, respectively. Over 12 months, 79 cases of corticosteroid use were noted in 62 (9.0%) patients. 140 (20.3%) participants were prescribed biologic asthma medications at the enrollment visit.

Conclusion. Patients with uncontrolled SA are characterized by frequent development of severe exacerbations, which create a significant burden on the healthcare system. The identified characteristics of medical care for such patients can serve as a basis for optimizing existing treatment approaches and improving disease outcomes.

Chronic bronchitis (CB) and chronic obstructive pulmonary disease (COPD) are among the most common respiratory diseases and are associated with high morbidity and significant disability. Frequent exacerbations of COPD lead to prolonged deterioration in respiratory function and gas exchange, faster disease progression, a significant decrease in the quality of life, and significant treatment costs. The most affordable and effective local therapeutic agent is inhaled aerosol of thiamphenicol glycinate acetylcysteinate (TGA). However, there is insufficient data in the international and domestic literature on using this drug in real clinical practice.

The aim of this study was to evaluate the clinical and pharmacoeconomic efficacy of TGA compared with systemic antibacterial drugs in the treatment of exacerbations of CB and COPD.

Methods. A retrospective analysis of medical records was carried out, including a total of 1,151 cases of exacerbations of CKD and COPD diagnosed and treated at the Chelyabinsk Regional Pulmonological Center in 2020 – 2024. The clinical and pharmacoeconomic efficacy was evaluated.

Results. The use of the topical antibacterial drug TGA (Fluimucil antibiotic IT®) makes it possible to achieve remission in patients with infectious exacerbations of CB and COPD and increase the time interval to the next exacerbation by 28.09 days (95% CI – 16.57 – 39.6 days, p < 0.01) compared with systemic antimicrobial drugs. The use of TGA leads to a rapid decrease in the main symptoms of exacerbation (decreased cough, shortness of breath, and sputum production). The use of TGA reduces the economic cost per patient, thus reducing the economic burden of CB and COPD.

Conclusion. The use of TGA in clinical practice helps reduce the frequency of exacerbations, increase the time to the next exacerbation and increase the effectiveness of antibacterial therapy, while reducing the costs.