Lymphangioleiomyomatosis (LAM) is a rare, slowly progressing disease characterized by a tumor-like overgrowth of smooth muscle fibers in the interstitial lung tissue, around blood and lymph vessels, bronchi, bronchioles, in lymph nodes. The disease has mainly been described in women of childbearing age, with a mean age of diagnosis of about 35 years.Methods. This retrospective, cohort study included 16 patients who were diagnosed with LAM at the age of 17 – 53 years (Me – 39, HQ – 42.5, LQ – 30.5). The calculation of the volumetric area of cystic lesion in the entire volume of the lungs was carried out using the GE aws 3.2 Thoracic VCAR software. To assess the area of cystic lesion on histological sections, we calculated area of cystic formations, in the walls of which smooth muscle nodules were found. For statistical analysis, the Kolmogorov–Smirnov test, the Mann – Whitney U-test (p < 0.05), and the Spearman correlation analysis were used.Results. Morphometric analysis of histological preparations of the lungs showed no statistically significant difference between women of young age and older age. A correlation was found between the volumetric area of cystic lesion on CT and FEV1 (correlation coefficient = –0.886). Baseline median area of cysts was larger in patients who later deteriorated, while condition of patients with a smaller area of cysts remained stable (2.677 vs 1.119 mm2 correspondingly).Conclusion. The volumetric area of cystic lesions in LAM correlates with functional parameters. The size of cysts in LAM may determine the prognosis of the course of the disease, because the formation of larger cysts is an unfavorable prognostic factor that increases the risk of developing pneumothorax.

Spirometry is the most common method to assess respiratory function. It is widely used to obtain objective information for the diagnosis of respiratory diseases and monitoring the functional state of the respiratory system. In 2014, the Russian Respiratory Society approved the technical standards for performing spirometry. Improvements in measuring devices have necessitated updating technical standards and standardizing the result interpretation algorithm. Methods. This document on spirometry was prepared by a joint group of experts from the Russian Respiratory Society and the Russian Association of Specialists in Functional Diagnostics, who have experience in performing spirometry in accordance with quality criteria, analyzing the results of the study, and developing national standards. Results. The 2014 Spirometry Technical Standards were revised, including the addition of quality criteria for measurements and test quality assessment. Where necessary, evidence was provided to support the change. The experience and knowledge of the expert group members were used to develop these recommendations. Conclusion. Standards for spirometry and bronchodilator test and the result interpretation algorithm are presented to improve the measurement quality and consistency of data interpretation.

In this lecture, we discussed methods and approaches to the diagnosis of small airways dysfunction, such as multiple breath nitrogen washout test, impulse oscillometry, as well as the calculation of poorly communicating fraction (PCF) as the ratio of total lung capacity to alveolar volume. The detection of the small airways dysfunction with the help of the diagnostic tools listed above makes it possible to establish functional disorders of the respiratory system in the early stages of bronchopulmonary diseases, when the results of traditional pulmonary functional tests remain within normal values. Thus, a well-standardized and the most accessible method for detecting the peripheral airways dysfunction is body plethysmography, which is used to diagnose the presence of “air trappings”. However, in the early stages of bronchopulmonary diseases, the possibilities of this method are limited. The lecture focuses primarily on the results of our own researches which were performed on patients who have suffered from a novel coronavirus infection complicated by viral pneumonia. Spirometry, body plethysmography, diffusion test, impulse oscillometry, multiple breath nitrogen washout test were conducted, and PCF index was calculated in all patients included in this analysis. The description of the PCF index, the method of its calculation, the range of normal values, as well as the possibilities of application in clinical practice are given for the first time in the Russian literature. The approaches to assessing the dysfunction of the small airways described in the lecture are not widely used at present, however, we hope that the knowledge that is currently being applied in scientific laboratories will gradually be introduced into routine clinical practice.

COVID-19 (Coronavirus disease 2019) is an infectious disease caused by the coronavirus SARS-CoV-2, characterized by a high level of morbidity and mortality. A new coronavirus infection affects all organs and systems and often occurs with lung damage and respiratory failure. In a significant part of patients after the acute period of COVID-19, the symptoms of the disease persist, but the most problematic symptoms are the ones regarding lung damage that persists for a long amount of time. This review summarizes the results of our research in 20202021. The purpose of the conducted studies was to estimate the functional parameters of the respiratory system in patients that have been infected by COVID-19. Along with conventional pulmonary function tests (spirometry, body plethysmography and diffusion test), impulse oscillometry, the multiple-breath nitrogen washout test, determination of the maximum inspiratory and expiratory pressures were performed in all patients. Thus, we evaluated the ventilation and gas exchange function of the bronchopulmonary system, the functional state of the peripheral parts of the lungs, including the small airways, the uniformity of pulmonary ventilation and the strength of the respiratory muscles in different periods of recovery after COVID-19, including the follow-up after medical rehabilitation. To sum up, comprehensive functional assessment of the respiratory system is an important clinical tool that allows the planning of pharmacotherapy and to create individual programs of medical rehabilitation of patients who had COVID-19.

Introduction. COVID-19 is a highly contagious infectious disease caused by the novel coronavirus SARS-CoV-2. After the acute phase and discharge from the hospital, convalescent people continue to have respiratory symptoms, changes in pulmonary function (PF) and indicators of chest computed tomography (CT).Aim. To evaluate changes in PF and quantitative CT data in patients after severe coronavirus pneumonia.Materials and methods. A cross-sectional observational study was conducted: 55 patients (including 28 men, aged 32–78 years) with a diagnosis of “lung interstitial process due to a coronavirus infection” were examined. All patients underwent a study of PF (spirometry, body plethysmography, measurement of the diffusion lung capacity: DLCO) in the interval of 24–305 days after discharge from the hospital. CT scan of the chest was performed simultaneously with the functional examination (±30 days), the parameters were calculated: ground glass areas, functional lung tissue volume (FV), affected tissue volume (AV), CovidQ (AV/FV ratio).Results. A decrease in DLCO was found in 28 patients. Restrictive disorders – in 13 patients, obstructive disorders – in 2 patients. At the time of the CT examination, all patients had residual changes in both lungs of varying severity. Functional parameters (FVC, TLC and DLCO) were statistically significantly correlated with the CT data.Conclusion. During the recovery period in patients after COVID-19, lung structural changes, according to CT scans, persist for at least 3 months. Pulmonary ventilation indicators in most patients normalize during this time, half of the patients retained a decrease in diffusion lung capacity. Acknowledgment. The study was carried out as part of the state task on the topic: “The impact of the new coronavirus infection SARS-CoV-2 on the functional parameters of the respiratory system during the convalescence period” (code: “PostCOVID-functional diagnostics”).

The article deals with the application of functional methods for the study of the respiratory system, such as spirometry, bronchodilatation test, stress testing to detect bronchial hyperreactivity, provocative test with metacholine, impulse oscillometry, body plethysmography for the diagnosis, following up and prediction of the course of asthma.

The end of the acute period of COVID-19 does not mean complete recovery. Observation of patients in the post-COVID-19 period showed that a significant number of people experience shortness of breath, fatigue, muscle weakness, sleep disorders, cough, palpitations, so the term post-COVID-19 syndrome was coined. The examination to identify the causes of complaints of COVID-19 convalescents should include lung function assessment.The aim of the study was to assess the dynamics of lung function 4 months after hospitalization for COVID-19.Methods. 31 patients (26 males, the median age was 49 years) underwent traditional pulmonary function tests (PFTs) (spirometry, body plethysmography, test of diffusing lung capacity) and impulse oscillometry (IOS). Results. During the 1st visit, the average PFTs and IOS parameters were within the normal range in the whole group, apart from the diffusing lung capacity (DLCO), which was reduced mildly (on the border with moderate) in 77% of patients. During the 2nd visit, which was conducted on average 102 days after the 1st one, 58% of patients demonstrated abnormal lung diffusion capacity. The second assessment revealed a statistically significant increase in the slow and forced vital capacity (VC and FVC), the forced exhalation volume in 1 second (FEV1), total lung capacity (TLC), DLCO, and a decrease in the ratio of FEV1/FVC and the residual lung volume to TLC ratio.Conclusion. Post-COVID-19 patients show a statistically significant improvement of their lung function 4 months after hospital discharge. A systematic follow-up is essential for such patients to detect lung function abnormalities and correct them.

Respiratory muscles (RM) are a very important part of the respiratory system that enables pulmonary ventilation. This study aimed to assess the post-COVID-19 strength of RM by estimating maximum static inspiratory (MIP or PImax) and expiratory (MEP or PEmax) pressures and to identify the relationship between MIP and MEP and the parameters of lung function. We analyzed the data of 36 patients (72% male; median age 47 years) who underwent spirometry, and body plethysmography, diffusion test for carbon monoxide (DLCO) and measurement of MIP and MEF. The median time between the examinations and onset of COVID-19 was 142 days. The patients were divided into two subgroups. In subgroup 1, as registered with computed tomography, the median of the maximum lung tissue damage volume in the acute period was 27%, in subgroup 2 it reached 76%. The most common functional impairment was decreased DLCO, detected in 20 (55%) patients. Decreased MIP and MEP were observed in 5 and 11 patients, respectively. The subgroups did not differ significantly in MIP and MEP values, but decreased MIP was registered in the second subgroup more often (18%). There were identified no significant dependencies between MIP/MEP and the parameters of ventilation and pulmonary gas exchange. Thus, in patients after COVID-19, MIP and MEP were reduced in 14 and 31% of cases, respectively. It is reasonable to add RM tests to the COVID-19 patient examination plan in order to check them for dysfunction and carry out medical rehabilitation.

The article deals with the pathophysiology of chronic obstructive pulmonary disease (COPD), modern possibilities of functional methods in COPD, including the diagnosis of small airway dysfunction and expiratory air flow limitation with quiet breathing by impulse oscillometry, and also describes the characteristic changes in the indicators of pulmonary functional tests in COPD.

Целью исследования стал анализ частоты протяженных перестроек гена CFTR и клинико-лабораторных характеристик пациентов с протяженными перестройками гена CFTR. В Регистр больных муковисцидозом РФ 2017 г. включены данные 3096 пациентов из 81 региона РФ, у которых выявлено 196 патогенных вариантов гена CFTR. Патогенные варианты обнаруживаются как в кодирующих, так и в интронных областях, и в регуляторных регионах гена CFTR. В гене CFTR относительно мало (около 2,5%) протяженных перестроек, но среди мутантных хромосом, в которых генетические варианты не были идентифицированы стандартными методами, такие перестройки составляют до 20%. По данным Регистра 2017 г. выявлен 21 пациент, несущий в своем генотипе крупные перестройки. Перестройки CFTRdele12,13del16, CFTRdele19-22(17а-19), CFTRdele8(7*), CFTRdele2-8(2-7*) ранее не были описаны в международных базах данных. Клиническая характеристика больных с протяженными перестройками не отличалась по основным признакам от пациентов с «тяжелыми» генотипами. Наличие в генотипе пациентов с протяженными перестройками варианта нуклеотидной последовательности гена CFTR, определяющего сохранную функцию поджелудочной железы, обусловило отсутствие у них панкреатической недостаточности. The aim of the study was to analyze large rearrangements in the CFTR gene in patients with cystic fibrosis in the Russian Federation in 2017. The Cystic Fibrosis Patients Registry of the Russian Federation for 2017 includes data from 3096 patients from 81 regions of the Russian Federation. To date, more than 2,000 mutations or variants of the nucleotide sequence of the CFTR gene have been described. In the Cystic Fibrosis Patients Registry of the Russian Federation for 2017, 196 pathogenic CFTR variants are given. Pathogenic variants are found both in the coding and in the intron regions, and in the regulatory regions of the CFTR gene. The CFTR gene has relatively few (about 2.5%) large rearrangements, but among mutant chromosomes in which genetic variants were not identified by standard methods, such rearrangements account for up to 20%. According to the Registry of 2017, 21 patients were identified that carried large rearrangements in their genotype. The rearrangements CFTRdele12,13del16, CFTRdele19-22 (17a-19), CFTRdele8 (7*), CFTRdele2-8 (2-7*) are not described in international databases. The clinical characteristics of patients with extensive rearrangements in the genotype did not differ in basic characteristics from patients with “severe” genotypes. The presence of a genetic variant in the genotype that determines the preserved function of the pancreas leads to the preservation of gland function in patients with large rearrangements in the genotype.

Background: Small airway dysfunction (SAD) is a functional hallmark of chronic obstructive pulmonary disease (COPD). However, SAD prevalence and its role in COPD pathophysiology are not yet sufficiently studied.Background: Small airway dysfunction (SAD) is a functional hallmark of chronic obstructive pulmonary disease (COPD). However, SAD prevalence and its role in COPD pathophysiology are not yet sufficiently studied.Aim: To assess the prevalence of SAD in COPD patients by various functional diagnostic methods, such as spirometry, body plethysmography, and impulse oscillometry (IOS).Materials and methods: This was an cross-sectional study. Spirometry, body plethysmography and IOS were used in 132 COPD patients in remission under standard anti-COPD treatments. The presence of SAD was confirmed by at least one of the following criteria or their combination: 1) by spirometry: the difference between vital capacity (VC) and forced vital capacity (FVC) > 10%; 2) the presence of air trapping by body plethysmography; 3) identification of the frequency dependence of the resistive resistance at 5 to 20 Hz (R5 - R20 > 0.07 kPa x s/l), as assessed by IOS.Results: Mean forced expiratory volume in 1 s (FEV1) was 42.9% of predicted. Depending on the severity of the obstruction, the patients were divided into 4 groups: 7 patients (group 1) had the obstruction corresponding to GOLD 1 stage, 37 (group 2) to GOLD 2, 49 (group 3) to GOLD 3, and 39 (group 4) to GOLD 4. SAD was found in 96% of COPD patients, whereas in those with severe obstruction (GOLD 3-4), it was present in 100% of the cases. By spirometry, SAD was identified only in 67% of COPD patients, by body plethysmography in 75% of COPD patients (in those with severe obstruction (GOLD 3 and 4) in 88 and 97%, respectively). With IOS, it was possible to identify SAD in 94% of patients and in 100% of those with severe obstruction (GOLD 3-4).Conclusion: With deterioration of obstructive pulmonary ventilation abnormalities in COPD patients, there is a progressive increase in small airway dysfunction. Impulse oscillometry seems to be the most effective method for diagnosis of small airway dysfunction, as it helped to identify SAD in 94% of COPD patients and in 100% of those with severe and very severe obstruction.

An important stage in the study of COVID-19 and its impact on quality of life is the study of lung function during the period of convalescence. Aim . To assess the influence of complex medical rehabilitation (MR) on pulmonary function, dyspnea, muscle weakness, quality of life, anxiety and depression in patients after COVID-19. Materials and methods . The study included 19 patients after COVID-19 (17 men and 2 woman, median age – 45 years), who were treated at the Main Military Clinical Hospital of the Ministry of Defense named after N.N.Burdenko. The second stage of complex MR included medical and physical therapy, and physiotherapy. Before and after the MR pulmonary function tests were performed, also the scores of mMRC, MRSweakness, HADS scales, the scores of EQ-5D Questionnaire were assessed. Results . Before the start of MR, on average, the severity of dyspnea in the group was mild, muscle strength was slightly reduced, the quality of life score was 80 points, anxiety and depression were not detected. The medians of all pulmonary function parameters were within normal values unless anomaly of the lung diffusion capacity in 14 (74%) cases. Repeated examination showed a statistically significant reduction in the degree of dyspnea, increased muscle strength, improved quality of life, increased VC, FVC, FEV 1 , DL CO , with a median increase of 5% pred. or 230 mL, 5% pred. (260 mL), 6% pred. (210 mL) and 6% pred. (1,75 mL/min/mmHg), respectively. Conclusion . Our study demonstrated the importance and effectiveness of complex rehabilitation after COVID-19.

The article summarizes the experience of leading professional communities in organizing the work of lung function laboratories during the COVID-19 pandemic. Recommendations on the choice of methods, indications for pulmonary function testing and infection control measures aimed at minimizing the risk of cross-infection of medical staff and patients are presented.

Pulmonary function after COVID-19 in early convalescence phase. The aim of the study is to investigate the influence of Coronavirus disease 2019 (COVID-19) on pulmonary function in early convalescence phase.Materials and methods. The study included 44 patients (35 male) after COVID-19 without concomitant bronchopulmonary pathology, with a median age of 47.5 years. All patients underwent standard pulmonary function tests (PFTs): spirometry, body plethysmography, diffusion test. Besides, dyspnea on the mMRC scale was assessed, oxygen saturation level (SpO2 ) was measured. Depending on degree of lung damage determined using high-resolution computed tomography (CT), the patients were divided into 2 groups: group 1 (22 patients) — CT 1 and CT 2, group 2 (22 patients) — CT 3 and CT 4.Results. The medians of standard PFTs parameters were in normal values. However, there were statistically significant differences between groups: VC, FVC, FEV1 and TLC were lower in second group. Diffusing capacity was reduced in 52% of patients. Statistical significant correlations were established between lung damage by CT and the parameters of VC, FVC, FEV1 , TLC, IC and DLCO.Conclusion. The degree of functional disorders of lungs depended on the extent of abnormal CT. Impaired diffusing capacity were detected in more than half of the COVID-19 patients in early convalescence phase.

A non-interventional prospective cohort study was conducted on adult patients with cystic fibrosis, who received mucolytic therapy with 7% NaCl solution combined with 0.1% hyaluronic acid (medical product Hyaneb) and 7% NaCl solution for 4 and 8 weeks. Materials and methods: A total of 24 CF patients over 18 years old were enrolled in the study. A comparative analysis of respiratory function, therapy satisfaction, efficacy and safety of treatment was conducted following 4 weeks of therapy in 7% NaCl Solution Group (n = 12) and MP Hyaneb Group (n = 12), and the dynamics of these parameters was assessed against the background of the use of MP Hyaneb for 8 weeks (n = 12). Results: At week 4 of treatment, a significantly smaller number of patients complained of nasal congestion, ears and or accumulation of mucus in the throat and chest, irritation of the throat and unpleasant taste in the MP Hyaneb Group. No significant differences in the onset and increase in the intensity of cough were achieved. No significant differences between the groups in terms of spirometry parameters were identified. Findings: MP Hyaneb showed the best tolerability compared to 7% NaCl solution in adult patients with cystic fibrosis. The use of MP Hyaneb reduces the frequency and severity of irritation of the pharyngeal mucosa, cough and nasal congestion following administration for one month.

Introduction. Impulse oscillometry (IOS) is a novel method of assessing airway resistance. IOS has been introduced as an alternative technique to assess lung function. Aim. To evaluate IOS for lung function assessment in patients with various severe degree of asthma. Materials and methods. The study included patients (n=76) from 16 to 79 years old (mean age was 44±18; 52 (68%) for men and 24 (32%) for women) with asthma of various degree severity. IOS indices were compared with the indices of traditional pulmonary function tests (PFTs) such as spirometry, bodyplethys-mography and diffusing capacity of the lung. Results. There were revealed the following changes of IOS: increasing of respiratory impedance (Zrs), respiratory resistance (Rrs), friction resistance (Rrs), its frequency dependence (Rrs5-Rrs20), square reactance (AX) and resonant frequency (fres), the decrease in reactance (Xrs) in 96% of patients with asthma. 12 out of 18 patients (67%) with traditional PFTs in normal values had deviations of IOS parameters Rrs, Xrs, АХ, (Rrs5-Rrs20). IOS parameters of patients with asthma showed significant correlations with parameters of spirometry and body-plethysmography. Conclusion. IOS can be used for lung function assessment in patients with asthma. IOS may provide objective information in cases in which spirometry was normal.

The aim of this study was to investigate phenotypic features of cystic fibrosis (CF) in patients carrying the complex allele S466X(TGA)-R1070Q. Methods. Data from CF register of Russian Federation, 2014, were used. The Russian CF register includes data of 2,131 patients from 74 regions of Russian Federation. The complex allele S466X(TGA)-R1070Q was found in 17 patients (the study group) and homozygous F508del mutation was found in 170 patients (the control group). The groups did not differ in main clinical, functional and microbiological parameters. Survival was also investigated. Results. Sweat chloride concentration was higher in the study group patients compared to the control group (116.53 mmol/L vs 102.2 mmol/L; р = 0.0341). There were 11.77% of deaths in a combined group of patients with the complex allele vs 3.53% in homozygous F508del mutation group (р = 0.159). Moreover, no significant difference in survival was found between the groups (p = 0.058). FEV1 was higher in the study group (84.69 ± 17.85%pred. vs 64.95 ± 27.16%pred. in the control group: р = 0.0483). Patients did not differ significantly in percentiles of height, weight, and body mass index. Amyloidosis and pneumothorax were seen in the control group, but not in patients with the complex allele S466X(TGA)R1070Q. Polyps were diagnosed in 19 (11.17%) of patients with F508del/F508del genotype and were not found in the study group patients. Prevalence of diabetes mellitus and hepatic cirrhosis was similar in both the groups. Osteoporosis was diagnosed in 10% of control patients and in one (5.88%) of the study group patients. Chronic or intermitted Pseudomonas aeruginosa infection and chronic infection of Staphylococcus aureus were equally seen in both the groups, but Burkholderia cepacia сomplex and Stenotrophomonas maltophilia were found more often in the study group patients compared to controls (23.52% vs 10.58% and 11.76% vs 2.35%, respectively). Conclusion. The results of this study confirmed that the complex allele S466X(TGA)-R1070Q has properties of class I mutation and should be considered as a “severe” mutation.

The aim of this study was to investigate phenotypic features of cystic fibrosis (CF) in patients carrying the complex allele S466X(TGA)-R1070Q. Methods. Data from CF register of Russian Federation, 2014, were used. The Russian CF register includes data of 2,131 patients from 74 regions of Russian Federation. The complex allele S466X(TGA)-R1070Q was found in 17 patients (the study group) and homozygous F508del mutation was found in 170 patients (the control group). The groups did not differ in main clinical, functional and microbiological parameters. Survival was also investigated. Results. Sweat chloride concentration was higher in the study group patients compared to the control group (116.53 mmol/L vs 102.2 mmol/L; р = 0.0341). There were 11.77% of deaths in a combined group of patients with the complex allele vs 3.53% in homozygous F508del mutation group (р = 0.159). Moreover, no significant difference in survival was found between the groups (p = 0.058). FEV1 was higher in the study group (84.69 ± 17.85%pred. vs 64.95 ± 27.16%pred. in the control group: р = 0.0483). Patients did not differ significantly in percentiles of height, weight, and body mass index. Amyloidosis and pneumothorax were seen in the control group, but not in patients with the complex allele S466X(TGA)-R1070Q. Polyps were diagnosed in 19 (11.17%) of patients with F508del/F508del genotype and were not found in the study group patients. Prevalence of diabetes mellitus and hepatic cirrhosis was similar in both the groups. Osteoporosis was diagnosed in 10% of control patients and in one (5.88%) of the study group patients. Chronic or intermitted Pseudomonas aeruginosa infection and chronic infection of Staphylococcus aureus were equally seen in both the groups, but Burkholderia cepacia сomplex and Stenotrophomonas maltophilia were found more often in the study group patients compared to controls (23.52% vs 10.58% and 11.76% vs 2.35%, respectively). Conclusion. The results of this study confirmed that the complex allele S466X(TGA)-R1070Q has properties of class I mutation and should be considered as a “severe” mutation.