Резюме. Целью исследования была оценка роли гипоксемии у больных ХОБЛ в развитии эндотелиальной дисфункции (ЭД) и артериальной ригидности (АР). АР крупных артерий и ЭД измерялись фотоплетизмографическим методом. Рассчитывались 2 индекса: индекс ригидности (SI) и индекс отражения (RI). Индекс SI отражает скорость пульсовой волны в крупных артериях, индекс RI преимущественно связан с тонусом мелких сосудов. Эндотелиальная функция оценивалась по изменению индекса отражения после ингаляции сальбутамола (Δ RISLB). Проводилась комплексная оценка гипоксемии: газовый состав артериальной крови, ночная пульсоксиметрия, базальная сатурация кислородом (SpO2), время сатурации кислородом < 88 %, минимальная сатурация кислородом, 6-минутный шаговый тест (6-МШТ). Все пациенты были мужчинами с ХОБЛ II–IV стадии по GOLD. Их средний возраст составил 64,5 ± 7,6 года, ОФВ1 – 37,7 ± 14,1 %, SaO2 – 90,5 ± 5,5 %, РаО2 – 63,2 ± 11,2 мм рт. ст., РаСО2 – 40,6 ± 5,4 мм рт. ст., ЧДД – 22,4 ± 2,6 в минуту, ЧСС – 80,7 ± 13,4 в минуту, ИМТ – 25,12 ± 5,86 кг / м2, стаж курения – 40 (35–50) пачко-лет. Были обнаружены корреляционные зависимости Δ RISLB с показателями пульсоксиметрии во время 6-МШТ с базальной SpO2 (R = 0,38; p = 0,04), временем SpO2 < 88 % (R = –0,48; p = 0,01), минимальной SpO2 (R = 0,44; p = 0,02); временем SpO2 < 88 % во время сна (R = –0,42; p = 0,04), а также с напряжением кислорода в крови днем, в покое PаО2 (R = 0,42; p = 0,04). У 16 больных с гипоксемией (РаО2 ≤ 60 мм рт. ст.) было отмечено достоверно более выраженное нарушение функции эндотелия, чем у 25 больных с РаО2 > 60 мм рт. ст. (0 [–1,7–1,3] % vs 2,00 [0,35–12,35] %; р ≤ 0,04). Таким образом, с нарастанием уровня гипоксемии у больных ХОБЛ отмечалось достоверное ухудшение функции эндотелия. Так, АР зависела от уровня гипоксемии во время 6-МШТ. Чем ниже было минимальное значение SpO2 и больше времени показатели SpO2 составляли < 88 % во время теста, тем выше был фотоплетизмографический SI. Была проведена комплексная оценка гипоксемии у больных ХОБЛ: определение газового состава артериальной крови, проведение пульсоксиметрии в покое и при нагрузочном тесте днем, а также во время ночного сна. Полученные данные свидетельствуют о важнейшей роли гипоксемии в развитии изменений механических свойств артерий и ЭД у больных ХОБЛ.

Since the beginning of the COVID-19 epidemic, the European cystic fibrosis society (ECFS) has decided to launch a special ECFS-COVID-19 program to collect information on the of COVID-19 characteristics in the patients with cystic fibrosis (CF). The results of the program should help timely and efficiently provide the patients with CF with the necessary care. Initially, it was assumed that COVID-19 would be severe in CF patients. The aim. Тo assess the prevalence and characteristics of COVID-19 in patients with cystic fibrosis (CF) in the Russian Federation (RF). Methods. 6 cases (4 children and 2 adults) of COVID-19 in Russian CF patients were analyzed. Results. There are 405,843 infected with SARS-CoV-2 in Russia, the incidence of coronavirus infection in Russia was 1.4 cases per 1 thousand people. According to the Ministry of Health of the RF, as of December 2019, there were 3,931 patients with CF (2,823 children and 1,108 adults). The incidence of COVID-19 was 1.5 per 1000 patients with CF (1.4 : 1,000 for children and 1.8 : 1,000 for adults). The incidence was not higher than in the General population. The diagnosis of COVID-19 was confirmed in 4 boys and 2 women, 3 of the patients were infected with Pseudomonas aeruginosa and 2 – with Achromobacter spp. Mild disease was seen in 5 patients including all the children. Pneumonia was registered in 3 patients. One child with COVID-19 had abdominal syndrome. 2 patients – 1 adult and 1 child – needed in-patient care. Additional antibiotics were given to 4 patients, 2 of them received i/v antibiotics. One adult patient was on the lung transplantation waiting list. This woman had long-term oxygen therapy and BiPAP noninvasive respiratory support before the infection with SARS-CoV-2, FEV₁ was 24 %_pred. Conclusion. Despite the fact that patients with CF are at risk of severe COVID-19, to date, in the described cases, COVID-19 infection has not led to a significant deterioration of the symptoms of CF. Not a single fatal outcome in Russian patients with CF has been recorded.

Aim. To assess the functional parameters of the respiratory system and their impact on the survival of patients with lymphangioleiomyomatosis (LAM).Materials and methods. A study of lung function (spirometry, bodyplethysmography, diffusion test) was carried out in 131 patients with LAM (mean age 41±10 years), who were under observation and treatment in medical institutions in Moscow (Pulmonology Scientific Research Institute, Sechenov University, Moscow City Clinical Hospital named after D.D.Pletnev). The diagnosis of the disease was made based on the diagnostic criteria for LAM, formulated by a group of experts at the initiative of the European Respiratory Society in the guidelines for the diagnosis and treatment of LAM.Results. The mean VC value was 95±25% pred., FEV₁ /VC − 56±20%, FEV1 − 64±31% pred., DL_CO − 49±24% pred. DL_CO, FEV₁ /VC, FEV₁ and VC were reduced in 87, 76, 70 and 27% of patients, respectively. The obstructive ventilatory defect was detected in the overwhelming majority of cases − in 75%, restrictive ventilatory defect − in 4 patients, mixed defect − in 1 patient. In 28 (21%) patients, there were no pulmonary ventilation disorders. The Cox regression model showed that baseline DL_CO and FEV₁ are the main factors determining the risk of death.Conclusion. In patients with LAM, the obstructive ventilatory defect predominates, the restrictive ventilatory defect is quite rare. The lung diffusion capacity is reduced in 87% of patients with LAM. Parameters of lung function are closely related to survival.

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.

Lymphangioleiomyomatosis (LAM) is a rare disease with a predominant diffuse cystic lesion of the lungs in women. Currently, with the widespread availability of computed tomography (CT), the number of patients with LAM is increasing significantly. All this highlights the importance and significance of knowledge about this orphan disease. In clinical practice, LAM occurs either as an independent disease, sporadic LAM, or as LAM associated with tuberous sclerosis complex (TSC – LAM), a major sign of tuberous sclerosis. The aim was to present an overview of the main common and different manifestations of both variants of LAM.Conclusion. Despite the common clinical, radiological and morphological features, both LAM variants have significant differences. The article discusses in detail the main features of different forms of LAMs.

Lymphangioleiomyomatosis (LAM) is a rare progressive disease that affects women of reproductive age and is characterized by cystic lung destruction, airflow obstruction, and lymphatic dysfunction. Current diagnostic methods are costly or lack sufficient specificity, highlighting the need for novel non-invasive approaches. Exhaled breath analysis using real-time proton mass spectrometry (PTR-MS) presents a promising strategy for identifying disease-specific volatile organic compounds (VOCs). This cross-sectional study analyzed exhaled breath samples from 51 LAM patients and 51 age- and sex-matched healthy controls. PTR-time-of-flight mass spectrometry (PTR-TOF-MS) was employed to identify VOC signatures associated with LAM. Data preprocessing, feature selection, and statistical analyses were performed using machine learning models, including gradient boosting classifiers (XGBoost), to identify predictive biomarkers of LAM and its complications. We identified several VOCs as potential biomarkers of LAM, including m/z = 90.06 (lactic acid) and m/z = 113.13. VOCs predictive of disease complications included m/z = 49.00 (methanethiol), m/z = 48.04 (O-methylhydroxylamine), and m/z = 129.07, which correlated with pneumothorax, obstructive ventilation disorders, and radiological findings of lung cysts and bronchial narrowing. The classifier incorporating these biomarkers demonstrated high diagnostic accuracy (AUC = 0.922). This study provides the first evidence that exhaled breath VOC profiling can serve as a non-invasive additional tool for diagnosing LAM and predicting its complications. These findings warrant further validation in larger cohorts to refine biomarker specificity and explore their clinical applications in disease monitoring and personalized treatment strategies.