Ceftobiprole medocaril is a fifth-generation cephalosporin antibiotic with expanded activity against Gram-positive bacteria, including methicillin-resistant Staphylococcus aureus, and the activity against Gram-negative bacteria characteristic of third- and fourth-generation cephalosporins. The aim was to determine the role of ceftobiprole medocaril in the treatment of patients with community-acquired and nosocomial pneumonia in the context of increasing antibiotic resistance and post-infectious immune disorders based on an analysis of current literature and our own clinical experience. Results . The first clinical case demonstrates the efficacy of empirical ceftobiprole therapy in a hospitalized patient with community-acquired pneumonia and signs of lung tissue destruction, clinical predictors of challenging pathogens, and predictors of the ineffectiveness of initial antibacterial regimens in the prehospital setting. The second case demonstrates the successful use of the drug in a patient with severe neurological deficit requiring tracheostomy and gastrostomy, recurrent nosocomial lower respiratory tract infections associated with pan-resistant gram-negative bacteria, and exhaustion of various antibacterial treatment options. Ceftobiprole medocaril is included in our clinic’s internal empirical antibacterial therapy protocols for community-acquired and early nosocomial pneumonia, and extensive experience has been accumulated in this patient population. Conclusion . We recommend scaling up our experience in using ceftobiprole in patients with community-acquired pneumonia and the risk of challenging pathogens. The approach described in the second case study can be considered in complex clinical situations associated with exhaustion of antibacterial treatment options and requires further research.
Accurate preoperative assessment of functional reserve in patients with lung diseases is critical for surgical risk stratification. The “gold standard” for functional reserve assessment, cardiopulmonary exercise testing (CPET), has limited availability. The 6-minute walk test (6MWT) is considered a simpler alternative tool; however, its clinically significant thresholds for patients with pulmonary tuberculosis (PTB) require validation. The aim was to determine 6MWT threshold values corresponding to clinically significant levels of peak oxygen consumption (VO 2 peak ) measured by CPET for preoperative risk stratification in patients with PTB and lung cancer (LC). Methods . The study enrolled 81 patients with PTB and 89 patients with LC in the preoperative period. All patients underwent both 6MWT and CPET. The association between 6MWT parameters and VO 2 peak was assessed using low-risk (≥ 20 ml/kg/min) and high-risk (< 15 ml/kg/min) criteria. Threshold values were determined by ROC analysis, with AUC comparisons performed using the DeLong test. Results . The 6MWT distance (m) demonstrated a consistent correlation with VO 2 peak in both groups (r = 0.59 – 0.65; p < 0.05). A distance threshold of ≤ 434 m showed high accuracy for identifying high-risk patients (VO 2 peak < 15 ml/kg/min) with both PTB (AUC 0.91; sensitivity 92%, specificity 87%) and LC (AUC 0.82; sensitivity 89%, specificity 68%). Comparison of ROC curves revealed no significant differences between the groups in AUC for absolute distance (p = 0.19), whereas the percentage of predicted distance showed nosology-specific differences (p = 0.032). Conclusion . Absolute 6MWT distance is a valid screening tool with comparable accuracy in both medical conditions. The threshold of ≤ 434 m may serve as a universal criterion for high functional risk (VO 2 peak < 15 ml/kg/min), enabling optimized patient selection for comprehensive assessment (CPET).
The available scientific research does not sufficiently describe the specific polysomnographic (PSG) characteristics of obstructive sleep apnea (OSA) in obese patients. The aim was to determine the clinical and PSG characteristics of OSA in obese patients. Methods . The study included retrospective analysis of medical histories, assessment of the frequency of concomitant and previous diseases, and analysis of PSG results (Сomet PSG, Grass Technologies, USA). Results . The study included 2 groups of patients: the main group with OSA and BMI > 30 kg/m2 (n = 100) and the control group with OSA and BMI 18 – 25 kg/m2 (n = 44). In the main group, the Charlson comorbidity index (p = 0.012), the likelihood of hypertension (OR 6.4 (95% CI 2.9 – 14.2)), type 2 diabetes mellitus or impaired glucose tolerance (OR 29.9 (95% CI 3.9 – 225.7)), dyslipidemia (OR 2.4 (95% CI 1.1 – 4.9)), and ahistory of pneumonia (OR 3.2 (95% CI 1.1 – 8.9)) were higher than in control group. The PSG analysis revealed that severe OSA was detected in mostpatients (52%) of the main group and in none of the patients in the control group. Duration of SpO 2 < 90% was longer in the main group (42.4 min), than in the control group (0.9 min). The apnea-hypopnea index was increased in the REM sleep stage and in supine position in the main group. Duration of sleep stages was different: stage 1 was longer in the main group and stage 2 was longer in the control group. There were no differences in REM and stage 3. Obese patients had more frequent arousals, due to episodes of apnea and hypopnea and snoring. Conclusion . Our study identified certain characteristics of PSG in OSA and obesity, which may explain the higher incidence of concomitant severe diseases that may be associated with cardio-renal-metabolic syndrome.
Gonadotropin-releasing hormone (GnRH) medications are used to treat a wide range of reproductive disorders. GnRH regulates not only the maturation and growth of the gonads, but also the immune system and metabolism, thereby affecting non-reproductive, somatic tissues. GnRH affects the lungs, but research findings are limited and not generalized. The aim was to summarize the effects of GnRH on the lungs based on a literature review. Results . The presence of specific receptors for GnRH (GnRHR) in various lung cells and in the cells of some forms of cancer allows the use of GnRH-agonists and antagonists for the treatment of pulmonary disease and for both malignant tumors and their metastases to the lungs, especially those originating from the reproductive organs. The presence of GnRHR in tumor cells impels binding GnRH both to antitumor drugs for their targeted delivery and to magnetic nanoparticles for magnetic resonance imaging of cancer. Various forms of cytostatics conjugated with GnRH are less toxic and more effective than the cytostatics themselves. A large surface area with good vascularization and the ability to exchange various substances allow for systemic delivery of GnRH via inhalation into the lungs. Inhalation administration of anticancer drugs associated with a GnRH analogue is promising for the treatment of lung cancer with GnRHR expression. One of the pulmonary complications that develops when using both GnRH analogues and antagonists is interstitial lung disease, which can lead to death. An increased risk of classical pneumonia has been described after androgen deprivation therapy. Also, GnRH can enhance the development of lung pathology during radiation exposure. However, some publications report the absence of negative effects of GnRH on the pulmonary system in the experiments and in clinical practice and report that an increased level of GnRH even protects the lungs from edema and inflammatory damage, reduces the severity of sclerosis and fibrosis. Pulmonary macrophages, type 1 and 2 alveolar cells can be directly involved in the degradation of GnRH and have the necessary enzyme complexes. Conclusion . The obvious scarcity and inconsistency of publications on all aspects of GnRH action on the lungs indicates the need for further continuation of not only applied research, but also fundamental research, due to the high prospects. The GnRH drugs have advantages and disadvantages. Hormonal therapy should be prescribed with caution, taking into account all indications and contraindications, as well as the endocrinologist’s experience, aiming for the most favorable long-term result.
Chronic obstructive pulmonary disease (COPD) remains one of the leading causes of morbidity and mortality worldwide. Over the past decade, international experts have introduced changes in the understanding of the etiology and definition of COPD, particularly with the launch of the Global Initiative for Chronic Obstructive Lung Disease (GOLD). However, the transfer of diagnostic interpretations into domestic practice without proper analysis has led to significant confusion in clinical recommendations and therapeutic decisions. The aim . This position paper examines the historical context of the development of the COPD concept in Russia, analyzes current contradictions between international and domestic approaches to defining the disease, and discusses the practical implications of these differences for patient management. Conclusion . Our position is that there must be a clear distinction between COPD as a nosological entity and the syndrome of reversible or irreversible bronchial obstruction, which may accompany other diseases and conditions.
Ground glass opacity (GGO) foci are a frequent finding on chest computed tomography (CT) scans. These foci may indicate various diseases, including lung cancer (LC), necessitating differential diagnosis of the nature of the changes. The aim of the study was to develop a diagnostic complex of radiological signs that would indicate with high accuracy malignant lung neoplasms in patients with GGO signs on chest CT and magnetic resonance imaging (MRI) scans. Methods . Chest CT (n = 72) and MRI (n = 11) data were analyzed in patients with and without clinical manifestations of acute or chronic lung diseases and with GGO in the lungs. Results . The analysis allowed us to establish the frequency of structural features of benign and malignant GGO foci, which were used to identify 3 GGO groups: predominantly benign, malignant, and of uncertain nature. The most significant predictors of pulmonary leukemia were a multinodular structure, the presence of a soft tissue component (STC) with contrast agent (CA) accumulation, deformed bronchial and vascular structures, and peribronchovascular localization. The probability of malignancy during the initial CT examination (monitoring) was 95%. CT data were supplemented by MRI, which revealed decreased diffusion and CA accumulation in the GGO lesion. Conclusion . CT allows to identify malignancy of the GGO foci in the lungs with high accuracy. Dynamic CT monitoring is essential to clarify the nature of the GGO. The main CT signs of GGO malignancy are multinodular structure, CA accumulation in the GGO area, vacuoles, deformed bronchial and vascular structures, and spicules. MRI is used as an additional method for clarifying the nature of the MS foci in complex cases.
According to the current paradigm of Global Initiative for Asthma (GINA, 2026), the principal goal of treating patients with asthma is to achieve and maintain complete control of the disease. The level of asthma control directly determines the frequency and severity of exacerbations, the majority of which occur in patients with severe, partly controlled, or uncontrolled disease. Severe acute asthma exacerbations (SAAE) account for up to 10 – 20% of all annual asthma exacerbations and usually require hospitalization and complex therapy, including methods of respiratory support. New therapeutic approaches aimed at optimizing the management of patients with SAAE are being actively explored. One promising avenue is the use of a thermal helium–oxygen mixture (t-He/O 2 ) as a component of inhalation therapy. The aim was to evaluate the efficacy and safety of t-He/O inhalations as part of complex therapy in patients with SAAE and a long-standing history of severe asthma, in comparison with SAAE therapy administered in accordance with current clinical guidelines alone. Methods . The study included 26 patients with SAAE: 15 patients of the main group, who received inhalation t-He/O 2 therapy in combination with the standard therapy, and 11 patients of the control group, who received standard therapy alone. Inhalations were performed for 10 – 15 min twice daily as a 10-day course. The dynamics of vital signs (respiratory rate, heart rate, blood pressure, SpO 2 measured by pulse oximetry), clinical symptoms, pulmonary ventilation parameters (peak expiratory flow (PEF), forced vital capacity (FVC), forced expiratory volume in one second (FEV1), FEV1 / FVC), arterial blood gases (PaO 2 , PaCO 2 , SaO 2 ), acidbase balance (pH, HCO 3 –, base excess (BE), lactate), central hemodynamics (deviation of volemic status from normal, stroke index (SI), left ventricular ejection fraction (EF), cardiac output (CO), tidal volume (Vt)) and the oxygen delivery system (CaO 2 , DO 2 I) were assessed before treatment and after completion of the course. The safety and tolerability of t-He/O 2 were evaluated using adverse-event registration logs and individual patient diaries. The Mann – Whitney U-test was used for comparisons between independent groups, the Wilcoxon signed-rank test for within-group dynamics, and Fisher’s exact test for categorical variables. Results . By day 10 of treatment, the main group, which received complex therapy combined with t-He/O 2 inhalations, demonstrated statistically significant differences from the control group in the rate and magnitude of dyspnea reduction and in the improvement of pulmonary function (PEF (p = 0.016), FEV1 (p = 0.026), FVC (p = 0.002)), arterial blood gases (PaO 2 (p < 0.001), SaO 2 (p = 0.026), PaCO 2 (p < 0.001)) and acid–base balance (pH (p = 0.003), lactate (p = 0.038)). Significant between-group differences in favor of the main group were also observed for tidal volume (Vt) and for the oxygen transport system (CaO 2 , DO 2 I), as well as for key hemodynamic parameters (SI), whereas the therapy had no significant effect on cardiac output, left ventricular ejection fraction, or the deviation of volemic status from normal. Treatment with t-He/O 2 was characterized by a high safety profile and good tolerability. Conclusion . The use of t-He/O 2 as part of the complex therapy of SAAE contributes to a more pronounced and more rapid reduction of clinical symptoms, resolution of respiratory failure, effective restoration of pulmonary ventilation and gas exchange, correction of metabolic disturbances, and improved oxygenation and oxygen transport without imposing an additional load on the pumping function of the heart. The inclusion of t-He/O 2 in the complex therapy of SAAE is safe and is characterized by good tolerability.
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.
In real-world clinical practice, mild asthma is the most common type of asthma but selection of the basic therapy for these patients can present certain challenges. Monotherapy with inhaled corticosteroids (ICS) in the form of finely dispersed metered-dose aerosols (e.g., ciclesonide) may prove highly effective in adolescents with poorly controlled mild asthma. The aim of this multicenter, open-label study was to evaluate the efficacy of ciclesonide in different dosing regimens for achieving and maintaining asthma control in adolescents with initially uncontrolled asthma. Methods . An open, prospective, non-comparative study lasting 90 ± 7 days included patients ( n = 129: 69.8% boys; mean age 14.2 ± 1.7 years) with uncontrolled mild atopic asthma (household, epidermal sensitization, and tree pollen in some patients) in 14 centers. The patients received background therapy with ciclesonide 160 μg per day. Asthma control tests (Global Initiative for Asthma (GINA) questionnaires and the Asthma Control Test (ACT) were completed at 3 visits. The number of asthma exacerbations, days with use of emergency medications and/or school absences due to asthma symptoms, and adverse events were taken into account. Spirometry with a salbutamol challenge was performed, and absolute eosinophil blood counts were measured at Visits 1 and 3. Subanalyses were performed for the subgroups of patients who were switched to ciclesonide 80 μg/day at Visit 2 ( n = 26) by the treating physician and for those with tree pollen allergy ( n = 49). Results . The proportion of individuals with controlled asthma increased with regular ciclesonide treatment: 94 (77.7%) at Visit 2 and 111 (95.7%) at Visit 3. No significant differences in asthma control criteria were found between the groups with and without tree pollen allergy. When comparing spirometric indices at Visits 1 and 3, the proportion of patients whose baseline forced expiratory volume in 1 second was > 80% of predicted value increased to 100% ( p = 0.026 compared to Visit 1 – 34.6%), and the proportion of individuals with a negative result in the salbutamol test increased to 83.6% ( p = 0.0001 compared to Visit 1 – 12.4%). No changes in blood eosinophil content were shown between Visits 1 and 3 (Me (Q25; Q75)): 310 (180; 500) and 290 (150; 450) cells/μl. The results of questionnaires, spirometry and the number of emergency inhalations showed maintained asthma control in the subgroup of patients who received ciclesonide at a dose of 80 μg per day after Visit 2. No adverse events of clinical significance or requiring discontinuation of ciclesonide were reported. Conclusion . Background therapy with ciclesonide (Asmalib® Air, metered-dose aerosol for inhalation, LLC PSK Pharma, Russia) in adolescents has a positive effect on asthma control (assessed using the ACT and GINA) and spirometry results. A reduction in the frequency of rescue medication inhalations was also noted.
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.
Expanding the range of biological drugs for the treatment of patients with severe asthma requires rationalizing a personalized approach to treatment to account for the potential risks of adverse events. Information on the development of drug-induced sarcoid-like syndrome during anti-IL-4, -13 therapy is limited to isolated publications and requires a detailed analysis of each specific clinical case. This is necessary to further develop a patient profile associated with higher risks of adverse events related to anti-IL-4, -13 therapy, as well as to develop a rational algorithm for dynamic laboratory and instrumental monitoring and to define its timing. The aim was to investigate the emergence of drug-induced sarcoid-like syndrome associated with anti-IL-4, -13 treatment in a patient with severe asthma and chronic rhinosinusitis with nasal polyps (CRSwNP), as well as to evaluate the safety and efficacy profile of alternative biological therapies. Conclusion . The therapeutic potential of anti-IL-4, -13 therapy in patients with bronchial asthma and CRSwNP is undisputed. Given the risk of adverse events associated with biological therapy, strict frequent monitoring of laboratory and instrumental data is required. Further investigation of the efficacy and safety of biological therapy in asthma and CRSwNP will help determine the optimal timing of treatment response and monitoring intervals, identify possible predictors of adverse events, and develop personalized therapeutic approaches.
Tiotropium bromide is a long-acting antimuscarinic drug that is often referred to as an anticholinergic in clinical practice. Its high affinity for muscarinic receptors and slow dissociation provide a pronounced and prolonged bronchodilatory effect in patients with chronic obstructive pulmonary disease (COPD). Medications containing tiotropium bromide (international nonproprietary name) as the active ingredient have been successfully used in clinical practice for approximately 20 years, with their clinical efficacy and safety confirmed. A novel inhalation delivery of tiotropium bromide via metered-dose inhaler (MDI) has been developed. Aim . This paper presents the results of an open-label, randomized, crossover comparative study of the pharmacokinetics, pharmacodynamics and safety of tiotropium bromide 9 μg/dose MDI (Bronpriva solopharm, Grotex LLC, Russia) and tiotropium bromide 18 μg dry-powder inhaler (Spiriva®, Boehringer Ingelheim International GmbH, Germany). Methods . The study enrolled 70 eligible patients with a diagnosis of COPD with moderate bronchial obstruction (forced expiratory volume in 1 second (FEV 1 ) in the post-bronchodilator test: 50% ≲ FEV 1 < 80%; FEV 1 / forced vital capacity (FVC) < 70%). Patients were randomized into 2 equal groups and received therapy with the tiotropium bromide 9 μg/dose and the tiotropium bromide 18 μg dry-powder inhaler. Pharmacodynamic equivalence was measured based on statistical analysis (ANOVA) of pulmonary distribution parameters (FEV 1 , peak expiratory flow) of tiotropium bromide. Conclusion . Tiotropium bromide 9 μg/dose MDI and tiotropium bromide 18 μg dry-powder inhaler were found to be pharmacodynamically equivalent. Tiotropium bromide delivered via MDI has a favorable safety profile.
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.
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%), FEV 1 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.
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/m 2 ). 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 FEV 1 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.
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 (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.
Comorbidities are important factors influencing the course and prognosis of interstitial lung diseases (ILDs). However, comparative data on the structure and clinical significance of comorbidities in idiopathic pulmonary fibrosis (IPF) and hypersensitivity pneumonitis (HP) remain limited. The aim was to assess the prevalence and clinical significance of comorbidities in patients with IPF and HP. Methods. This single-center prospective observational study included 621 patients (229 with IPF, 392 with HP) with a 12-month follow-up. Clinical, demographic, functional, and radiological characteristics, comorbidities, the Charlson Comorbidity Index (CCI), and the GAP (Gender, Age, Physiology) index were analyzed. All patients underwent pulmonary function testing, the 6-minute walk test (6MWT), echocardiogram, and high-resolution computed tomography scan of the chest. Results. Most patients (66.2%) had 1 – 3 comorbidities, while 19.6% had ≥ 4 comorbidities. Patients with IPF had significantly higher CCI scores than those with HP ( p < 0.001). The most common comorbidities were hypertension, cardiovascular disease (CVD), gastroesophageal reflux disease, pulmonary hypertension, and diabetes mellitus. Emphysema and obstructive airway diseases were more frequent in IPF ( p < 0.05). A higher comorbidity burden and higher CCI scores correlated with higher GAP scores, shorter 6MWT distance, and more severe dyspnea (mMRC scale). The presence of ≥ 2 comorbidities was associated with increased mortality risk (AUC 0.587, p = 0.01), as was CCI ≥ 4 (AUC 0.608, p = 0.002; log-rank p = 0.007). CVD was associated with greater functional impairment and reduced exercise tolerance, while diabetes mellitus was an independent adverse prognostic factor, particularly in HP (OR 2.58, p = 0.016). Conclusion. Comorbidities are highly prevalent in both IPF and HP and significantly influence disease course and prognosis. Assessment of comorbidity burden, including the CCI, may serve as an additional risk-stratification tool in patients with fibrosing ILDs.
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.
