Currently, lung transplantation (LT) is a generally accepted method of treating a wide range of terminal lung diseases that are not amenable to medical or surgical methods of correction. The aim of the study was to determine the frequency and nature of bronchial complications in patients after bilateral LT. Methods. This article presents an analysis of the frequency and the nature of bronchial complications in 49 patients after bilateral LT. All patients underwent bronchoscopy intraoperatively, at the stage of bronchial anastomoses formation, and after the operation. A total of 775 bronchoscopies were performed in 49 patients after lung transplantation. All patients were divided into 2 groups according to the results of bronchoscopy. The first group included 41 patients (83.7%) who had 66 clinically insignificant bronchial complications in the form of ischemia of the bronchial mucosa of I – IV degrees. The second group included 8 patients (16.3%) with 10 clinically significant bronchial complications represented by dehiscence of bronchial anastomoses, anastomotic and non-anastomotic stenoses of the bronchi. Results. Perioperative factors of donors and recipients that could be associated with the development of bronchial complications were analyzed. However, statistically significant differences were not observed for all indicators due to the small number of observations. The analysis showed a direct relationship between the incidence of bronchial complications and the duration of mechanical ventilation in the postoperative period. Conclusion. Thus, according to our experience, the incidence of clinically significant bronchial complications after LT is 16.3%. Improving surgical tactics, timely diagnosis of signs of rejection and infectious complications, early extubation and intraoperative use of extracorporeal membrane oxygenation can play an important role in reducing the incidence of bronchial complications in the post-transplant period.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with a secondary microvasculopathy of vessels < 500 μm, leads to increased pulmonary vascular resistance and progressive right heart failure. The mechanism responsible for the transformation of red clots into fibrotic material remnants has not yet been elucidated. In patients with pulmonary hypertension, the diagnosis is suspected when a ventilation/ perfusion lung scan shows mismatched perfusion defects, and confirmed by right heart catheterisation and vascular imaging. Today, in addition to lifelong anticoagulation, treatment modalities include surgery, angioplasty and medical treatment according to the localisation and characteristics of the lesions. This statement outlines a review of the literature and current practice concerning diagnosis and management of CTEPH. It covers the definitions, diagnosis, epidemiology, follow-up after acute pulmonary embolism, pathophysiology, treatment by pulmonary endarterectomy, balloon pulmonary angioplasty, drugs and their combination, rehabilitation and new lines of research in CTEPH. It represents the first collaboration of the European Respiratory Society, the International CTEPH Association and the European Reference Network-Lung in the pulmonary hypertension domain. The statement summarises current knowledge, but does not make formal recommendations for clinical practice.

PH (pulmonary hypertension) targeted therapy may play an essential role in chronic thromboembolic pulmonary hypertension (CTEPH) patients considered inoperable. Given the limited number of PH-targeted drugs approved for CTEPH, reliable long-term data are necessary on the effects of PH-targeted drugs in patients with inoperable CTEPH. We aimed to evaluate the efficacy and safety of intermittent inhaled iloprost in inoperable CTEPH. 
Methods. The open randomized controlled trial included 22 inoperable CTEPH patients (aged (Me (25%; 75%)) 48,3 (38,4; 59,5) years; 63.6% females; 9.1% with WHO functional class (FC) IV, 72.7% with WHO-FC III, 18.2% with WHO-FC II; 6-minute walking test (6-MWT) distance of 348 (145; 443) m; mean pulmonary artery pressure (mPAP) of 41.8 (29.3; 52.8) mmHg; tricuspid annular plane systolic excursion (TAPSE) of 16.3 (14.5; 18.2) mm; plasma NT-proBNP of 853.8 (562.2; 1124.2) pg/mL). The patients were enrolled 3 – 6 months after acute pulmonary embolism and were randomized 1:1 to receive either standard therapy with vitamin K antagonists and, if indicated, oxygen and diuretics or inhaled iloprost 5.0 µg / inhalation 4 times a day for 2 weeks every 3 months for 2 years in addition to the standard of care. Efficacy endpoints included changes from baseline in 6-MWT, WHO-FC, echo-parameters, inflammatory markers, time to clinical worsening, and all-cause mortality. 
Results. At baseline (prior to therapy), there were no significant differences between iloprost and control groups. Levels of C-reactive protein and the interleukin (IL)-1b, IL-6, IL-8, γ-IF, and TNF-α cytokines were increased. At month 24, a mean 6-MWT distance increased by 215 m (p < 0.001) in the patients receiving inhaled iloprost and by 137 m in the control patients (p < 0.01). The control-adjusted difference was +78 m (p = 0.03). WHO-FC improved by two classes in 63.6% in iloprost group vs 0% in the control group (p = 0.028), by one class in 36.4% vs 30% (p = 0.091), and remained the same in 0% vs 70 % (p = 0.018), respectively. Inhaled iloprost delayed the time to clinical worsening (p = 0.0064). Improvements were noted in control-adjusted changes in ePASP (–18.6 mmHg; p = 0.0065), TAPSE (+2.4 mm; p = 0.028), and plasma NT-proBNP (–256.9 pg/mL; p < 0.01). The levels of inflammation decreased significantly in the iloprost group, while remained unchanged in the control group. Combination therapy with inhaled iloprost was tolerated well. One patient died in the control group (p = 0.093). 
Conclusion. Long-term intermittent inhaled iloprost for patients with inoperable CTEPH may improve their clinical status, hemodynamics, and anti-inflammatory status.

Blood eosinophil count is associated with measurements have been used as biomarkers for eosinophil-related airway inflammation. Eosinophils are found in the airways, tissues, and blood during stable disease or acute exacerbations of COPD (AECOPD). The modified Medical Research Council dyspnea scale (mMRC) and COPD assessment test (CAT) scores have been shown to be useful as novel tools for evaluating these aspects of COPD.
The aim. This study aimed to investigate the relation of blood eosinophil count with the assessment scales and the number of emergency department (ED) admissions in patients with acute exacerbations COPD. 
Study Design. Cross-Sectional Study. Methods. Based on eosinophil count, the patients were divided into two groups: < 300 cells μL−1 and ≥ 300 cells μL−1. For these two groups, the relationship between acute exacerbation and the number of admissions to ED, the number of hospitalizations in the last one year, CAT score, mMRC score, and comorbid diseases were analyzed. 
Results. 166 patients was mean age 69.0, 126 (75.9%) male. Patients with high eosinophil count had fewer ED admission compared with those with low eosinophil count(5 and 10, respectively), Median mMRC score of patients with low eosinophil count was significantly higher compared with that of patients with high eosinophil count(p = 0.022).The difference between the median CAT scores of the two groups was not statistically significant. A statistically significant, negative relationship was found between the eosinophil count and mMRC scores (r = −0.219; p = 0.005). 
Conclusion. High levels of eosinophilia in patients presenting with AECOPD are associated with low mMRC score. Also, although not statistically significant, the number of admissions to the ED may be lower.

Eosinophils play a protective role and are involved in the pathogenesis of exacerbations in chronic obstructive pulmonary disease (COPD). 
The study of the role of eosinophils in pathogenesis of COPD, especially during exacerbations, is relevant to selecting the optimal therapy. The aim was to analyze literature data and compare them with the results of our study in real clinical practice. 
Methods. We analyzed the publications on eosinophil count in COPD in electronic libraries eLIBRARY and National Library of Medicine. In our study, we examined 330 patients, collected their history, and conducted physical examination, spirometry, and laboratory tests. Statistical analysis was carried out using SPSS-18 software.
Results. Analysis of publications showed that percent of patients with COPD with a stable eosinophil count of more than 300 cells/μL varies from 12.3% to 17%. This percentage is variable and can reach 37.5% in patients with exacerbations. As many studies show, eosinophilia is one of the indications for inhaled corticosteroids (ICS) and is associated with better outcomes than eosinopenia. In our study, 18.58% of patients were eligible for ICSs because of their eosinophil count, 53.3% because of > 2 exacerbations, 82.1% because of exacerbations or hospitalizations, and 62.1% because their FEV1  was < 50% of the reference value. Among patients with FEV1  < 50%, 61% had > 2 exacerbations, 87.8% had hospitalizations, and 12.7% had eosinophilia. Only 7.57% of patients with COPD met all three criteria for prescribing ICS. 16.2% of patients had eosinophilia and 2 and more exacerbations within a year. 10.3% of patients had eosinophilia combined with FEV1  less than 50%. 6.7% of patients had eosinophilia, 2 or more exacerbations within a year, and FEV1  < 50%. 
Conclusion. Analysis of literature data and our findings have shown that the eosinophil blood count is one of the important prognostic indicators in COPD. It facilitates selection of the optimal therapy, including triple inhalation therapy.

Bronchiolitis obliterans (BO) is a severe form of chronic graft-versus-host disease (cGVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). 
Aim. The aim of current study was to evaluate the incidence, risk factors, and clinical manifestation of BO after allo-HSCT. 
Methods. The study included 1189 adult patients who received allo-HSCT at R.M.Gorbacheva Research Institute of Pediatric Oncology, Hematology and Transplantation, Academician I.P.Pavlov First Federal Saint-Petersburg State Medical University, Healthcare Ministry of Russia in 2008 – 2019. BO was diagnosed according to the US National Institutes of Health criteria (2014), including pulmonary function test (PFT) and high-resolution computed tomography (HRCT) of the chest. We analyzed the cumulative incidence of BO with competing events, risk factors in the Fine – Gray regression model, clinical symptoms, BO severity, and bronchial obstruction formation dynamics. 
Results. BO was diagnosed in 42 (3.5%) patients. Cumulative incidence of BO was 1.8% (95% CI, 1.2 – 2.7), 3.9 (95% CI, 2.8 – 5.2) и 4.5% (95% CI, 3.2 – 6.1) at 1, 3, and 5 years after allo-HSCT, respectively. The median time of BO onset and diagnosis was 321 (86 – 1 771) and 371 (161 – 2 134) days, respectively. Risk factors were HLAmismatched unrelated and haploidentical donor (ОР – 2.301, 95% CI, 1.247 – 4.246; p = 0.0076), myeloablative conditioning (ОР – 2.544, 95% CI, 1.384 – 4.674; p = 0.0026) and GVHD prophylaxis without posttransplant cyclophosphamide (ОР – 2.152, 95% CI, 1.154 – 4.013; p = 0.0160). The clinical manifestation included cough (88 %) and shortness of breath (90%). Bronchial wall thickening (95%) and expiratory air trapping (79%) were frequent chest HRCT findings. Mild, moderate, and severe BO was identified in 9 (21%), 12 (29%), and 21 (50%) patients, respectively. The median forced expiratory volume in 1 second (FEV1 ) was 39% (20 – 74). The most significant loss of bronchial conductivity was between day + 100 and 1 year after allo-HSCT. 
Conclusion. The large cohort study provided details on current incidence, risk factors, and clinical features of BO after HSCT. The results create the basis for predicting and early diagnosis of BO after allo-HSCT

The aim of the study was to analyze the association of I/D polymorphism of angiotensin-converting enzyme (ACE) gene with the risk of pulmonary sarcoidosis in ethnic Russians of the Republic of Karelia. 
Methods. The study enrolled 242 individuals, including 112 patients of Russian nationality residing in the Republic of Karelia with confirmed pulmonary sarcoidosis and 130 healthy donors as a control group. The distribution of alleles and genotypes of I/D polymorphism of ACE gene (rs4646994) in these groups was investigated. Alleles of this polymorphic marker were identified by the method of PCR-RFLP. 
Results. The comparative analysis showed no statistically significant differences in the distribution of alleles and genotypes of indel locus of ACE gene (rs4646994) between the control group and the group of patients with pulmonary sarcoidosis (χ2  = 0.619; p > 0.05; χ2  = 0.368; p > 0.05, respectively). 
Conclusion. I/D polymorphism of ACE gene is not associated with the risk of pulmonary sarcoidosis in ethnic Russians of the Republic of Karelia

Incidence and prevalence of non-tuberculous mycobacteriosis (NTM) are recorded in many countries, but the rates vary. There are no official statistics on the prevalence and incidence of NTMs in the Russian Federation at the moment. The regulatory and expert clinical database for NTMs has not yet been developed. 
The aim of thе article is to share the experience of the Federal State Scientific Institution “Central Research Institute of Tuberculosis” (FSSI “CRIT”) in working with patients with NTMB over the past 7 years. 
Methods. The study included 190 patients with a confirmed diagnosis of NTMB who were treated at the FSSI “CRIT” in 2011 – 2018. Before the diagnosis of NTMB, all patients were followed for a long time by various specialists (pulmonologists, general practitioners) for various chronic respiratory diseases. NTMs were isolated from the diagnostic material by the culture method. The material was inoculated into a liquid medium in an automated BACTEC MGIT 960 system and into Levenshtein – Jensen dense medium until the culture growth was obtained. The HTM species was determined using DNA strips GenoType® Mycobacterium CM by Hain Lifescience (Germany). 
Results. More than half of the examined patients were registered with a phthisiatrician for various clinical forms of pulmonary tuberculosis (disseminated, fibro-cavernous, focal). In 85.6% of cases, no MBT DNA was found in the sputum and skin tests with recombinant tuberculosis antigen (Diaskintest®) were negative. Nevertheless, the changes in the lungs detected on the chest CT and even a single detection of acid-fast microorganisms (AFB) without typing led to an erroneous diagnosis of tuberculosis. In some patients (6.8%), a combination of pulmonary tuberculosis and NTMB was diagnosed. Bronchiectasis and COPD were the most common chronic nonspecific lung diseases. 66.7% of the patients had centrilobular foci characterized by the involvement of not only the bronchial wall but also the pulmonary vessels. NTMB resistance to a wide range of antibacterial drugs was determined in 118 patients (out of 190). The prolonged use of chemotherapy drugs revealed a wide range of drug intolerance. Side effects were handled in various ways: drug replacement (with consideration to the sensitivity), temporary withdrawal, and drug discontinuation. 
Conclusion. This work reflects the long-term experience of the FSSI “CRIT” staff with patients with NTMs, which can be used in creating national recommendations on NTMs.

The aim of the study was to evaluate the effectiveness of MDR/XDR TB treatment with bedaquiline. 
Methods. The author analyzed the treatment results of 56 patients in Staint Petersburg in 2017 with MDR/XDR TB whose treatment regimen included bedaquiline. All patients were smear/ culture-positive: 43 (67.8%) cases with XDR MBT; 7 (12.5%) with pre-XDR MBT and only 6 (10.7 %) cases with MDR TB. 
Results. 35 (62.5%) patients received full bedaquiline course, 16 received the treatment for a few days to 4 months and did not complete the course, 5 patients with XDR tuberculosis who underwent surgery were treated for more than 6 months. The average duration of bedaquiline course was 4.7 ± 0.3 months. Among the 27 (48.2%) patients who completed the treatment effectively, 22 had XDR TB, 3 – pre-XDR TB, and 2 – MDR TB. The median sputum conversion period was 4.00 ± 0.42 months (CI: 3.14 – 4.86). 13 (23.2%) patients interrupted the treatment, but 9 of them had negative cultures for MBT at this time. These patients received bedaquiline for 1 to 14 months. Treatment failure was recorded in 12 (21.4%) patients, and 4 (7.14%) patients died during the intensive treatment phase. In general, 27 patients with a successful outcome had negative cultures at the end of the intensive phase and 9 patients who effectively completed the intensive phase but interrupted the treatment continuation phase. In total, 36 patients (64.3%) had a successful treatment outcome. 
Conclusion. The analysis showed that routine administration of the drug without consideration for treatment compliance increases the effectiveness of the intensive phase (64.3%) and does not significantly affect general effectiveness of the treatment (48.2%). The high frequency of treatment interruption remains a serious obstacle to improving the treatment effectiveness.

The aim was to study the volume of blood loss and the duration of a surgical session in patients with destructive pulmonary tuberculosis using threedimensional anatomical models of the lungs. 
Methods. The study was conducted in 2020 – 2021 in the State Budgetary Healthcare Institution of the Nizhniy Novgorod region “Nizhny Novgorod Regional Clinical Tuberculosis Dispensary” as part of an internal grant from the Federal State Budgetary Educational Institution of Higher Education “Privolzhsky Research Medical University” of the Ministry of Health of the Russian Federation. The prospective clinical study included 80 patients. All patients were divided into two groups. The main group included 40 patients. Preoperative lung reconstruction was performed for these patients. Thoracic surgeons used the three-dimensional anatomical models of the lungs to study the syntopy of healthy and diseased tissue (visual assessment) and prepare for the surgical session (cutting the model with a scalpel). Threedimensional preoperative lung reconstruction was not performed for the other 40 patients in the control group. In this group, the thoracic surgeons did not use 3D anatomical models of the lungs. Comparison of the results in the control and the main group was carried out using the Mann – Whitney U-test and Spearman and Kendall correlation tests. The obtained data are described with absolute and relative values, boxplots reflecting median (Me) and interquartile interval (IQI), scatter plots with trend lines, and probability density plots. The level of statistical significance of differences for testing the hypotheses was chosen at p < 0.05. 
Results. It was shown that the volume of blood loss (U = 590; p = 0.042) and the duration of surgery (U = 587; p = 0.041) in patients of the main group are smaller than in patients in the control group. A moderate correlation was established between the volume of blood loss and the duration of the surgical session (ρ = 0.54; p < 0.001) in the main group using the Spearman’s test. Kendall’s test also showed a positive correlation (τ = 0.42; p < 0.001). 
Conclusion. The established differences between the main group and control group in the volume of blood loss and the duration of surgery due to the inclusion of polymer models of the lungs in preoperative preparation of thoracic surgeons confirm our hypothesis about the effect of additive technologies on the above parameters and indicate the significance of the applied reconstruction method.

Auscultation of the lungs is one of the oldest diagnostic methods invented by Rene Laenneck. Nowadays, auscultation hasn’t lost its clinical significance. However, the development of science and technical progress have brought a lot of new ideas about the pathogenesis and interpretation of lungs sounds. This, in turn, caused some changes in the nomenclature. Moreover, the nomenclature of lung sounds in several European countries does not differ significantly but does not fully correspond to the recommended terminology for the English language. In addition, the mechanisms underlying some respiratory murmurs are interpreted ambiguously at present. For example, the main respiratory sound is designated both as “vesicular” and as “normal” respiration because of persisting differences in explaining the pathogenesis of this murmur. There is also a considerable difference in the terminology of added sounds such as crackles, wheezing, rhonchi, as well as the difference in understanding of the underlying mechanisms.

Aim. This review analyses the existing differences in the interpretation of lung auscultation and the terminology used to describe respiratory sounds in the Russian and foreign medical literature.

Conclusion. Comparative analysis of Russian and foreign publications concerning the issues of lung auscultation demonstrates the existing differences in the terminology and understanding of the mechanisms that underly the main and adventitious respiratory sounds. Forming the common information space requires the unification of terminology and interpretation of physiological and pathological processes in the lungs responsible for the respiratory sounds. Recording the lung sounds with subsequent computer analysis will make it possible to objectify the auscultation data and classify the sounds more accurately.

Granulomatosis with polyangiitis (GPA) is a disease characterized by necrotizing granulomatous inflammation of small and medium vessels, mainly in the upper respiratory tract, lungs, and kidneys. Verifying the diagnosis can be difficult due to the variety of clinical presentations, possible multiple organ damage, and atypical course. GPA patients may have nonspecific symptoms characteristic of other bronchopulmonary diseases, which often leads to diagnostic errors. 
Methods. The article presents an analysis of literature data on the clinical and radiological aspects of lung damage during GPA and clinical observation of a 26-year-old patient who has been under regular follow-up at the TB department for infiltrative tuberculosis without bacterial excretion and received anti-tuberculosis therapy. At the end of the main course of treatment, the patient underwent surgery. Pulmonary tuberculosis was excluded based on histological and bacteriological examinations, but pneumonia with abscesses was diagnosed. Five years after the surgery, infiltrative changes in the operated right lung were detected again during the fluorography. These findings were regarded as tuberculosis. Tuberculosis treatment was carried out with a change in chemotherapy regimens due to the lack of radiological improvement. 
Results. The patient was referred to the Novosibirsk TB Research Institute. The institute specialists have expressed doubts about the tuberculous etiology because of the long pulmonary disease anamnesis, the previous X-ray findings, and pathological changes in the right lung. These doubts served as the basis to repeat the pathomorphological examination, which led to the diagnosis of granulomatosis with polyangiitis. 
Conclusion. According to the presented clinical observation, the difficulties of timely verification of the diagnosis have been demonstrated, which is associated with the lack of vigilance among doctors regarding GPA.