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Primary ciliary dyskinesia (PCD) is a rare genetic autosomal recessive disease associated with a defect in the ultrastructure of epithelial cilia. Currently, there is no standard method for diagnosing PCD, so the diagnosis is based on the clinical picture and the results of tests, such as DNA diagnostics, nasal nitric oxide measurements, ciliary beat frequency in a nasal biopsy, ciliary ultrastructure, etc. Diagnosis of PCD can be difficult due to secondary damage to the respiratory epithelium, which often results in undiagnosed or false positive cases. Differential diagnosis with diseases forming widespread bronchiectasis (BE) and upper respiratory tract lesions, especially with cystic fibrosis (CF), is necessary.The aim of this paper is to introduce the difficulties of diagnosis, the appropriate level of detail of the clinical, laboratory and instrumental characteristics over a long period of time, and the organization of care for a patient with PCD. This article describes a clinical case of PCD in a young woman, diagnosed at the age of 17, presents the difficulties and typical mistakes in the management of such patients, and the lack of succession of pediatrician-pulmonologist care.Conclusion. The presented clinical case demonstrates how difficult the diagnosis of PCD is. Such patients need a complex examination, a thorough differential diagnosis to exclude other diseases with a similar clinical picture. Long-term follow-up is carried out by a multidisciplinary team with mandatory microbiological monitoring. The organized care for patients should begin in early childhood and continue in adulthood with proper succession of care and follow-up by of pulmonologist, preferably in specialized centers.

In a retrospective study, we evaluated factors associated with the early development of septic shock in patients with severe COVID-19.We collected medical records of the intensive care unit patients submitted by the local COVID-19 hospitals across Russia to the Federal Center for the Critical Care at the Sechenov First Moscow State Medical University (Sechenov University). Septic shock in crticially ill patients requiring mechanical ventilation was defined as a need in vasopressors to maintain blood pressure.We studied 1078 patients with severe COVID-19 who were admitted to the intensive care units for respiratory support. There were 611 males and 467 females. The mean age was 61.013.7 years. Five hundred twenty five medical records (48.7%) were received from the Moscow hospitals, 159 (14.7%) from the Moscow region, and 394 (36.5%) from the hospitals located in 58 regions of the Russian Federation. In 613 (56.9%) patients, diagnosis of SARS-CoV-2 infection was confirmed by PCR, and in the other cases it was established on the basis of the clinical picture and the results of the chest CT scan. Septic shock developed in 214 (19.9%) of 1078 patients. In the logistic regression model, the risk of septic shock in patients older than 50 years was higher than in patients of a younger age (OR 2.34; 95% CI 1.533.67; p0.0001). In patients with more severe SARS-CoV-2 infection, there was an increase in the prevalence of cardiovascular diseases, including coronary heart disease and atrial fibrillation, type 2 diabetes and malignant tumors. The risk of septic shock in patients with three or more concomitant diseases was higher than in patients without any concomitant chronic diseases (OR 1.76; 95% CI 1.762.70).The risk of septic shock in patients with acute respiratory distress syndrome induced by SARS-CoV-2 is higher in patients older than 50 years with concomitant diseases, although a severe course of the disease is also possible in younger patients without any concomitant disorders.Цель. Анализ факторов, ассоциированных с ранним развитием септического шока у больных с тяжелым течением COVID-19. Материалы и методы. В исследование включены данные всех пациентов, проконсультированных до 1 мая 2020 г. на базе Федерального дистанционного консультативного центра анестезиологии и реаниматологии на базе ФГАОУ ВО Первый МГМУ им. И.М. Сеченова (Сеченовский Университет). Критерием септического шока считали снижение артериального давления, требовавшее введения вазопрессорных средств. Результаты. В исследование включены 1078 пациентов, 611 (56,7%) мужчин и 467 (43,3%) женщин. Средний возраст составил 61,013,7 года; 525 (48,7%) заявок на консультацию получены из стационаров Москвы, 159 (14,7%) Московской области, 394 (36,5%) лечебных учреждений в 58 регионах Российской Федерации. У 613 (56,9%) больных диагноз SARS-CoV-2-инфекции подтвержден с помощью полимеразной цепной реакции, а в остальных случаях установлен на основании клинической картины и результатов компьютерной томографии органов грудной клетки. Развитие септического шока отмечено у 214 (19,9%) из 1078 больных. В логистической регрессионной модели риск его развития у пациентов старше 50 лет выше, чем у больных более молодого возраста (отношение шансов 2,34; 95% доверительный интервал 1,533,67; р0,0001). У пациентов с более тяжелым течением SARS-CoV-2 отмечалось увеличение частоты сердечно-сосудистых заболеваний в целом, ишемической болезни сердца, фибрилляции предсердий, сахарного диабета 2-го типа, частота злокачественных опухолей выше. Риск развития септического шока у пациентов с тремя и более сопутствующими заболеваниями оказался выше, чем у пациентов без сопутствующих болезней (отношение шансов 1,76; 95% доверительный интервал 1,762,70). Заключение. Риск развития септического шока у пациентов с острым респираторным дистресс-синдромом, вызванным SARS-CoV-2, выше у пациентов старше 50 лет, страдающих несколькими сопутствующими заболеваниями, хотя тяжелое течение заболевания возможно и у более молодых людей без какой-либо сопутствующей патологии.

Clinical features of overlap autoimmune hepatitis/primary biliary cholangitis and morphological-proved sarcoid lesions (lungs, lymph nodes, skin) were performed. Data of long-term clinical observation presented in comparison with the results of laboratory datas, instrumental and morphological studies of liver tissue, lungs, skin. The modern aspects of pathogenesis of association autoimmune and granulomatous diseases arediscussed on the example of clinical cases of combination of cholestatic variants of autoimmune hepatitis and generalized sarcoidosis. Keywords: sarcoidosis, autoimmune hepatitis, primary biliary cholangitis, primary biliary cholangitis-autoimmune hepatitis-overlap, extrahepatic manifestations.

Freshly milled wheat flour made from high quality grain gives a low quality bread, especially in the processing of the newly harvested grain. The flour acquires requisite technological properties only after a certain amount of time, which is called flour aging. The strength of wheat flour increases with aging as well as the bread volume, but it takes a lot of time. The main reason behind the increase in strength of the aged wheat flour is change in physical properties of the gluten. The goal of current research is to determine the relationship between stabilization time of wheat flour capacitance and gluten content. The method is based on determining the electrical capacitance of the flour sample which is stabilized depending on gluten content of the flour and correlated with flour aging time. The method involves the use of 15 wheat flour samples with gluten content in the range of 23.0–32.0% and moisture content up to 15.0%. The proposed method allows to use mathematical equations to determine the time of flour aging depending on the gluten content.

The article presents a clinical observation of two patients with generalized sarcoidosis. The woman typical granulomatous changes in the lungs and lymph nodes combined with atrial fibrillation, kidney failure and hereditary thrombophilia, men with atherosclerotic coronary arteries, re-myocardial infarction, cholestasis, tubulointerstitial nephritis. The accession of systemic manifestations was accompanied by increase of level of angiotensin-converting enzyme in the blood serum, morphological examination of lung tissue in both cases there were high histological activity of vasculitis and granulomatous inflammation. Extrapulmonary symptoms regressed when conducting immunosuppressive therapy. Discusses modern aspects of diagnosis of sarcoidosis in clinical practice.

The influence of the polyvinylpyrrolidone (PVP) molecular weight on the stability and spectral luminescence properties of sols of lead sulfide nanocrystals and the related composite coatings has been studied. It is shown that the spectral properties of PbS sols stabilized with low-molecular (PVP) and the related coatings are determined to a great extent by the formation of large particle aggregates in these materials and, accordingly, high level of light scattering. It is effective to use low-molecular PVP for preparing powder materials containing PbS quantum dots (QDs), because it allows one to perform fast powder precipitation and form small semiconductor particles. High-molecular PVP provides high aggregative and sedimentation stabilities of semiconductor nanocrystal sols. This polymer is effective for use in preparing stable QD sols and homogeneous coatings transparent in the visible spectral range.

The use of special digital image processing for removal of the blur which results quite often from a mutual relative motion of the optical sensor and object during the exposure is considered. The problem of blur removal from a digital image belongs to the class of incorrect problems, which means that it is impossible to obtain an exact solution resistant to small perturbations in the input data. Therefore, special methods should be invoked. The use of the Tikhonov regularization methods for solving the problem of blur removal from a digital image is demonstrated.

A study of bend-induced losses in a silica-based single-mode microstructured fiber with a core diameter ranging from 20 to 35 microns and increased relative air content in the holey cladding has been conducted. With the use of the equivalent step-index profile method in approximation of waveguide parameters of microstructured fiber (normalized frequency and normalized transverse attenuation constant) the effect of bending on the spectral position of the fundamentalmode short-wavelength leakage boundary has been analyzed. Upon measurement of spectral characteristics of attenuation in the considered fibers good accordance of numerical and experimental data has been found out. It is shown that increase of the air content in the holey cladding leads to expansion of the mentioned boundary to lower wavelengths for the value from 150 to 800 nm depending on the core size and bending conditions. A single-transverse-mode propagation is achieved on fiber length of 5-10 meters due to a substantial difference in losses of fundamental and higher-order guided modes attained by bending. Optical losses in all studied samples are less than 10 dB/km at the wavelength λ = 1550 nm. The results of the study can be applied in the design of high-power laser systems having such basic requirements as a relatively large mode spot and high beam quality.

The paper deals with design principles of the multi-agent system for prediction and calculation of reliability parameters of heterogeneous, diversified electronic modules. One-parameter models of failures distribution are less accurate and do not take into account the specifics of failures occurring in the base of modules. That’s why the issue has for an object the possibility to use two-parametric models of the timing intensity distribution of failures and determination of parameters for models on the basis of equipment test results on actuating factors. Prediction of reliability parameters for modules is carried out by means of numerous agents, united in a system. It includes heterogeneous agents carrying out information exchange with the user. Developed system structure is presented and the work of separate subsystems is described. The facility to add new agents-prediction techniques is available and they form a library. Prediction results depend on the chosen calculation method of reliability parameters on a specific enterprise, therefore, the developed prediction system use both standard distribution of reliability parameters and received by the method of group assessment of arguments. Selection of models for prediction is done on the basis of the calculated values of standard deviation. The output of work results is performed in a graphical form. Testing carried out on the data received during the electronic module checkout makes it possible to confirm the correctness and effectiveness of the developed approach. The developed system will give the possibility to reduce time for checkout and processing of testing results for complex electronic modules, especially manufactured at the enterprises of electronic industry for the first time.