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Primary ciliary dyskinesia (PCD) (Online Mendelian Inheritance in Man - OMIM - #242650) is a rare hereditary disease, which is based on a defect in the ultrastructure of the cilia epithelium of the respiratory tract which leads to the motor function disorder. Data about health characteristics of patients with PCD in the Russian Federation are incomplete.The aim of the study was to investigate the clinical, laboratory, and instrumental characteristics of patients with PCD.Methods. The data of 90 patients (22 adults (24.4%) and 68 children (75.6%)) from several medical centers were studied. The following methods were used: medical history, spirometry, microbiological examination of the respiratory tract, video microscopic analysis of the functional activity of the nasal mucosa ciliated epithelium, transmission electron microscopy of the ciliated epithelium, and DNA testing.Results. The median age at diagnosis was 17.0 years for adults and 5.0 years for children. Kartagener syndrome was detected in 23 (27%) people, including 6 (26.0%) adults. Hearing loss was noted in 5 (26.3%) adult patients and 15 (26.8%) children. Light microscopy of the ciliated epithelium was performed in 14 (82.3%) children and 3 (17.7%) adults. In 12 patients, cilia motor activity was not registered at each of the magnifications (x 100, x 400, x 1,000). Transmission electron microscopy showed that absence ofinternal and external dynein handles (51%) and absence of internal dynein handles (17.9%) were the most common disorders. DNA testing was performed in 55 (61.2%) patients: 16 (29.1%) adults and 38 (70.9%) children. The most common genetic variants were found in the DNAH5 and HYDIN genes. Lung function was reduced in both adults and children, but a significant decrease was noted in adult patients. P. aeruginosa predominated in the culture and accounted for 21.3% (intermittent detection in 13.2%, persistent detection in 9%). It has increased resistance to antibiotics.Conclusion. The results correlate with the European data. Infection caused by P. aeruginosa with the increased resistance to antibiotics was prevalent in patienths with PCD.

MEHMO syndrome (OMIM: 300148; ORPHA: 85282) is a disease appears by mental retardation, epilepsy seizures, hypogonadism, microcephaly, and obesity. Pathology is associated with mutations in the EIF2S3 gene located on the X chromosome and leads usually to serious disability of patients. The article presents a clinical observation of the case of the syndrome in two male cousins with microcephaly, manifested by a complex of endocrinopathies (hyperinsulinemic hypoglycemia, multiple adenohypophysis hormone deficiency) and accompanied by severe neurological abnormalities (epilepsy, spastic tetraparesis, optic nerve atrophy). The complexity of the diagnostic due to the rarity of this syndrome, is described.

This work summarizes the current understanding of the mechanisms providing mucociliary clearance and their disorders in respiratory diseases. It is noted that currently the understanding of the drainage function of the respiratory tract and cough formation in bronchopulmonary pathology has significantly expanded, and therefore much attention is paid to the search for opportunities to correct these parts of pathogenesis. The use of drugs improving sputum rheology and decreasing inflammatory mucosal edema are of great importance in this aspect. The article summarizes the data on the possibilities of pathogenetic correction of respiratory disorders with the emphasis on the use of expectorants and mucolytic drugs made from herbal raw materials. For many of them a sufficiently rich clinical experience has been accumulated, and modern manufacturing techniques have significantly increased their quality and safety. The mucolytic and expectorant properties of modern herbal remedies are in many cases as good as those of synthetic drugs. An important feature of herbal remedies is that they contain a combination of many components with a complex therapeutic effect, whereby the quality of herbal remedies is ensured by the most modern manufacturing control mechanisms. The paper reflects the main therapeutic effects of herbal remedies, which depend on the plant species and are determined by their content of biologically active substances: alkaloids, glycosides (including anthraglycosides, cardiac glycosides, saponins, flavonoids), phytoncides, essential oils, coumarins, tannins, resins, etc. The results of own observations on the use of herbal remedies in routine clinical practice are presented. The high adherence of patients to the use of herbal syrups and drops based on ivy extract in the therapy of cough syndrome in adults and children is shown.

During the pandemic, an urgent task has become to develop new vaccine platforms that will help fight the infection caused by SARS-CoV-2 and quickly respond to newly emerging pathogens. Plant viruses can make a significant contribution to the solution of this problem. Phytoviruses, having the properties of any viral particles (self-assembly, immunogenicity, nanosize), are safe for humans since plants and mammals have no common infectious agents. As a result of thermal rearrangement of the tobacco mosaic virus, spherical particles of a protein nature have been obtained, which have unique immunostimulation and adsorption properties and can play the role of a universal adjuvant platform to create vaccines. Based on these particles, a scheme for obtaining vaccine preparations is proposed. This technology resembles a toy construction set for children. The basis is spherical particles, on the surface of which there are toy blocks-antigens. The "blocks" can be removed, added, or replaced, and this does not take much time and resources. Based on spherical particles, a polyvalent vaccine candidate against COVID-19 has been created as an adjuvant platform.

The article analyzes the regional features of the provision of medical personnel in Russia in the context of various subjects and federal districts. P u r p o s e of the study is to analyze the features of staffing of medical organizations in the constituent entities of the Russian Federation in modern conditions and the main directions of policy to improve the staffing situation at the regional level. M a t e r i a l s a n d m e t h o d s . According to the official statistics, an analysis of the staffing of medical and paramedical personnel was carried out in the context of the constituent entities of the Russian Federation; in the course of the study, methods were used such as: analytical, statistical, study and generalization of experience. R e s u l t s . The analysis showed that the provision of doctors and the provision of emergency medical services for different regions of the Russian Federation is different. The lowest values of the indicator of the provision of doctors in 2019 were recorded in the Ural FD, the Southern FD and the North Caucasian FD, while in the constituent entities of the Russian Federation the most pronounced tendency towards a decrease in the level of provision nursing staff was noted in Moscow, Tyumen region, Chukotka Autonomous Okrug, Republic of Kalmykia, Udmurt Republic, as well as in Yaroslavl, Murmansk, Novgorod and Kirov regions, which emphasizes the need to take appropriate management measures, both at the local and federal levels. F i n d i n g s . The existing imbalance in the country between the provision of the population with doctors and nurses in various regions (the difference between low and high rates is more than 2.3 times), as well as between the provision of medical workers in the city and rural areas is the main problem of staffing the system. To overcome this situation, further implementation of new measures of social support and moral incentives for medical personnel in Russia is required.

Health status of the younger generation in Russia is of particular relevance, since it is this period that forms the development potential of future generations.Objective. To study and analyze regional features of the incidence rates of the adolescent population in Russia.Material and methods. The authors have used various literature sources, statistical reports; the method of studying and generalizing experience. For the statistical analysis of the morbidity dynamics they used the indicators of absolute and relative growth, they assessed the chain and basic growth rates. Also the authors generalized the characteristic of the incidence dynamics by means of regression analysis.Results. The paper provides a detailed analysis of the incidence of the adolescent population in the Russian Federation in 2012–2018. It describes the dynamics and structure of both primary and general morbidity, identifies classes of diseases with a stable trend of growth, and compares with similar indicators in other countries; the authors analyze the regional characteristics of the incidence of adolescents; compare the level of morbidity in the Russian Federation for all classes of diseases.Conclusion. The authors revealed classes of diseases, characterized by a stable increase in indicators of both primary and general morbidity: neoplasms, endocrine diseases (obesity), eye diseases, injuries. Thus, the article identifies the directions for the health care system that require special attention and development of specific measures to reduce the level of these diseases. The results of the study demonstrate a low medical activity of adolescents, which should be taken into account when developing a set of measures aimed at increasing the coverage of adolescents with medical examinations.

Results of experimental study of the temperature field of coatings deposited under different conditions are presented. Revealed in this paper are the basic technical options of the control of the thermal state of coatings and determination of the noncrystalline solidification regimes of melt.

An autonomous wearable patient monitor with the function of broadcasting the measured parameters via wireless channels of computer networks to the server (to the cloud), and from there to the central console of the department, remote computers, tablets and doctors’ smartphones was tested. It was used to monitor the vital parameters of the patient’s body during rehabilitation in the inpatient facility. Various load programs have been tested: exercise therapy, kinesiotherapy, mechanotherapy, and verticalization.

Introduction. Extending professional longevity, preventing diseases and improving the quality of life of railway transport workers are the priority areas of activity of RZhD-Meditcina medical institutions. However, the infl uence of production factors on the prevalence and course of skin and subcutaneous tissue diseases in employees of this important branch of the national economy has not yet been suffi ciently studied. The aim of the study is to study the hygienic features of working conditions in railway transport workers that aff ect the course of skin and subcutaneous tissue diseases.Materials and methods. Materials for hygienic and statistical research were sanitary and hygienic characteristics of working conditions of railway transport workers and statistical reports of the private healthcare institution «Clinical hospital» RZhD-Meditcina « of Barnaul for 2014-2018. A retrospective analysis of the incidence of temporary disability, the number of days of disability, and the number of exacerbations in employees of harmful professions in the Altai region of the West Siberian railway with chronic skin and subcutaneous tissue diseases was conducted.Results. According to the sanitary and hygienic characteristics of working conditions, the most unfavorable working conditions of all railway specialties are employees of locomotive crews and track fi tt ers. Combined eff ects of chemical, meteorological, and physical factors of the production environment and the severity of the labor process belong to class 3 (with a high risk of occupational diseases). Th is leads to a signifi cantly higher (p=0.010944) number of days of disability (27.8±0.83) and the number of exacerbations of chronic skin diseases in railway transport workers than in the rest of the adult population not in contact with occupational hazards (25.32±1.2).Conclusions. Th e combined impact of factors of the production environment and the severity of the labor process, despite the systematic comprehensive prevention, leads to a more severe course of chronic dermatoses in railway transport workers.

In this work, a model of the cathode spot of a vacuum arc in the framework of the drift-diffusion representation has been considered. The use of the drift-diffusion approximation eliminates the need to calculate the particle energy distribution functions and take into account various energy reactions that are not accompanied by a change in the particle charge. It has been shown that the field strength of the plasma column is significantly reduced. The column is surrounded by a space charge of slow positive ions, providing a high field strength in front of it. At the stage of the formed plasma boiler, the ionization of metal vapor occurs mainly in a thin layer in front of the ion cloud, and ion recombination prevails in the column.

A method for synthesis of copper oxide nanoparticles in a reactor for low-pressure arc discharge synthesis is presented, and the effect that the oxygen content of the gas mixture fed into the reactor has on the ratio between CuO and Cu2O phases is studied. The crystalline structure of synthesized copper oxide nanoparticles is investigated by X-ray diffraction (XRD), and the particle morphology is characterized using transmission electron microscopy. The growth of nanoparticles during synthesis in arc discharge plasma is investigated using optical emission spectroscopy. XRD analysis shows that the proportion of the Cu2O phase decreases with increasing oxygen content of the gas mixture. The photocatalytic properties of the prepared Cu2O nanopowder are evaluated by carrying out the decomposition reaction of methyl orange dye in an aqueous medium.

Ferrite Fe3O4 nanoparticles have been formed in low-pressure arc discharge plasma. It has been shown that the obtained nanoparticles have an average size of 9.4 nm and a blocking temperature of 89 K, crystallize in the magnetite phase, and are superparamagnetic at room temperature. The features in the behavior of nanoparticles in a magnetic field related to their large specific surface are discussed.

Specific features of the structure formation in rapidly quenched high-cobalt alloy coatings formed by plasma sputtering have been investigated. The structural state of the investigated coatings has been identified by the X-ray diffraction technique. The sequence and kinetics of structural transformations occurring in the specified time-temperature heating regimes have been examined using differential thermal analysis. It has been established that heat generation and crystallization temperature depend on the conditions of formation of bulk amorphous coatings.

During surgery on the thyroid gland, injury to the recurrent nerve is possible with the development of a clinical picture due to impaired mobility of the vocal fold. A disturbance of the mobility of the opposite vocal fold in the form of a paradoxical spasm may join these manifestations if the parathyroid glands were affected during surgery, followed by a decrease in the level of ionized calcium in the blood. Management of such patients presents certain difficulties. A clinical case of examination and treatment of a patient with bilateral impairment of mobility of the vocal folds resulting from thyroid surgery is presented. The peculiarity of this observation is that because of our treatment, we managed to stop the spasm of one vocal fold, and then restore the mobility of the other vocal fold, due to which a complete restoration of the respiratory and vocal function was achieved.При проведении операций на щитовидной железе возможна травма возвратного нерва с развитием клинической картины, обусловленной нарушением подвижности голосовой складки. К данным проявлениям может присоединяться нарушение подвижности противоположной голосовой складки в виде парадоксального спазма, если во время операции были затронуты паращитовидные железы, с последующим снижением уровня ионизированного кальция в крови. Ведение подобных больных представляет определенные трудности. Представлен клинический случай обследования и лечения пациентки с двусторонним нарушением подвижности голосовых складок, возникшим вследствие операции на щитовидной железе. Особенность данного наблюдения заключается в том, что в результате проведенного нами лечения удалось добиться купирования спазма одной голосовой складки, а затем восстановить подвижность другой голосовой складки, благодаря чему было достигнуто полное восстановление дыхательной и голосовой функции.

The disability is one of the main indices of public health, along with medical and demographic characteristics, morbidity and physical development that characterizes level of social economic development and is a major problem of health and social care authorities. The availability of statistical data and analysis of problem of population disability will allow to properly plan activities of various administrative bodies. The article presents trends of persistent disability in the Russian Federation during the studied period. In recent years, total number of disabled people has been gradually decreasing. In Russia, most of disabled people have disability group II. At the same time, the percentage of persons with group III increases and proportion of disabled persons with the groups I and II gradually decreases. The children disability in Russia tends to increase. In the Russian Federation, the analysis of regional differences in disability is presented. The analysis and assessment of regional characteristics of disability are necessary for planning and forecasting measures of medical and social support of persons with disability. The analysis of available statistical data permitted to established interdepartmental dissociation on this issue. The results of the study permitted to conclude that the need of increasing attention to the problem of disability results in strengthening interdepartmental interactions and developing of unified register of disabled people.

A device for modifying the granular high-temperature superconducting ceramics in the plasma of a low-pressure arc discharge has been considered. The particular features of the design and operational principle of this device have been described. The device made it possible to combine the synthesis of that play a role of additional pinning centers and simultaneous deposition of these nanoparticles on microgranules in a single processing cycle. The experimental results on the effect of additional pinning centers on an increase in the critical current thanks to the formation of self-assembled structures in the form of whiskers have been considered.

In recent years, treatment approaches in patients with HL are actively discussed. The actual issue remains the combination therapy or possible avoiding of radiation therapy to reduce toxicity and the occurrence of secondary tumors in the future. In a number of studies, the prognostic role of interim and end of treatment PET/CT is investigated as a disease prognostic risk factor. In this article, we presented analysis of interim-PET/CT in patients with primary HL as a prognostic marker. It was proved, that results of interim PET/CT show a highly predictive of the response to the first line therapy and could predict progression free survival (PFS).

Objective. To verify oxidative stress as an indicator of metabolic impairments in the complex cascade of the pathogenesis of hemorrhagic (HS) and ischemic (IS) stroke. Materials and methods. A total of 50 patients with HS and 50 with IS, along with 20 healthy subjects, were studied. All patients underwent complex biochemical blood tests, assessment of free radical oxidation (ketodienes, diene conjugates, malondialdehyde, dityrosine, and Schiff bases), and evaluation of the endogenous antioxidant defense system (vitamin E, reduced glutathione, total antioxidant activity, superoxide dismutase, glutathione peroxidase, glutathione reductase, catalase) – a total of 12 indicators. Results. In patients with IS and HS, in contrast to the control group, complex biochemical investigations revealed an increase in the intensity of free radical oxidation of lipids and proteins, along with a functional imbalance in the nonenzymatic and enzymatic branches of the endogenous antioxidant defense systems. Conclusions. These results identify two factors in the formation of oxidative stress in IS and HS, widening our knowledge of the pathogenesis of this severe vascular process.

The influence of the quench rate on structuring in spinel ferrites has been studied. It has been found that, when the quench rate is increased, the equilibrium spinel structure gradually becomes disordered. At the first stage, the statistically homogeneous (or almost homogeneous) redistribution of cations over crystal lattice sites has been observed. Then, the fcc lattice of the anion framework breaks down, the translational symmetry disappears, and topological chaos arises. The resulting cluster structural state is thermodynamically unstable, and heating of quenched ferrites causes stepwise energy liberation. As a result, the activity of ferrite powders in solid-state and catalytic reactions rises.

Quasi-crystalline Al65Cu23Fe12 powders have been obtained by the method of plasma-chemical synthesis. Coarse particles have a spherical form and an inhomogeneous structure because of the laminar growth of crystallites toward the surface of the particles. The influence of heat treatment on phase formation and structuring in Al–Cu–Fe alloys has been studied. It has been found that the heat treatment of as-prepared powders changes the phase relationship in the quasi-crystalline alloy.

When low-frequency stimulated Raman scattering (SRS) is excited in the tobacco mosaic virus (TMV) suspension, an anomaly in the scattering line frequency shift is detected, i.e., the dependence on the concentrations of TMV and tris-buffer as the TMV concentration increases. An explanation of the phenomenon, based on the idea of the formation of the hydration shell of the virus capsid is proposed.

Thanks to their strong immunostimulating properties and safety for humans, plant viruses represent an appropriate basis for the design of novel vaccines. The coat protein of Alternanthera mosaic virus can form virus-like particles that are stable under physiological conditions and have adjuvant properties. This work presents a recombinant human rotavirus A antigen based on the epitope of rotavirus structural protein VP6, using Alternanthera mosaic virus coat protein as a carrier. An expression vector containing the gene of Alternanthera mosaic virus (MU strain) coat protein fused to the epitope of rotavirus protein VP6 was designed. Immunoblot analysis showed that the chimeric protein was effectively recognized by commercial polyclonal antibodies to rotavirus and therefore is a suitable candidate for development of a vaccine prototype. Interaction of the chimeric recombinant protein with the native coat protein of Alternanthera mosaic virus and its RNA resulted in the formation of ribonucleoprotein complexes that were recognized by anti-rotavirus antibodies.

Mucocele of the paranasal sinuses is a rare disease not infrequently associated with massive bone destruction, orbital and intracranial complications. The authors report a patient presenting with the giant post-traumatic frontal mucopyocele associated with the erosion of the posterior and inferior sinus walls that was clinically manifested as hypo- and exophthalmos and has caused the inferolateral eye deviation and orbital decompression. The patient displayed the ophtalmological symptoms 8 years after the frontal sinus fracture. The mucopyocele was treated effectively with the combination of the endoscopic trans-ethmoidal and open trans-frontal approaches. The noticeable intraoperative finding in this patient was the absence of complaints as well as the combination of dura mater defects in the form of its erosion and the leakage of the cerebrospinal fluid together with the presence of purulent discharge in the sinus in the absence of intracranial complications.Мукоцеле околоносовых пазух - редко встречающаяся патология, которая может приводить к массивным разрушениям, орбитальным и внутричерепным осложнениям. Нами представлено клиническое наблюдение посттравматического мукопиоцеле лобной пазухи с разрушением задней и нижней стенок, клинически проявляющееся выраженным гипо- и энофтальмом. В представленном наблюдении описана успешно примененная хирургическая методика трансэтмоидальной и трансфронтальной декомпрессии орбиты. Особенностью данного случая является длительное отсутствие жалоб, а также интраоперационно обнаруженное сочетание дефекта твердой мозговой оболочки и гнойного содержимого в пазухе.

This publication was designed to report the experience gained by the Department of ear, nose and throat diseases at I.M. Sechenov First Moscow State Medical University in the matters concerning professional medical education in the framework of the clinical residency. The main factors that contribute to the adequate shaping of the future ENT-doctor are considered. These include the continuity of generations and conservation of the traditions in the combination with the development of the leading areas of scientific, clinical and educational work.В данной статье представлен опыт коллектива кафедры болезней уха, горла и носа Первого МГМУ им. И.М. Сеченова по обучению в клинической ординатуре. Выделены факторы, влияющие на формирование 'облика' будущего врача-оториноларинголога на кафедре болезней уха, горла и носа. К ним относятся: преемственность поколений, сохранение традиций в сочетании с развитием ведущих направлений научной, клинической и педагогической работы.

The palliative care is determined as "a complex of medical interventions targeted to deliverance from pain and relief of other severe manifestations of disease, for improving quality of life of terminally ill citizens" (Art. 36 of the Federal Law of 21.11.2011 № 323FZ) on the stage of uncontrolled progression of disease. The actual status and accessibility of palliative care was analyzed, including development of this issue in both the Russian Federation and number of foreign countries. According to the WHO, in Russia, 360-460 out of 100,000 population need palliative care. Out of them, 200 individuals with oncologic diseases, 150 individuals with non-malignant diseases and 12 individuals with AIDS/HIV. The health authorities face the task of significant increasing of scope of palliative care to make it more accessible. In recent years, issues of palliative care were intensively considered at the level of patients, of public organizations and legislators, as well as at the level of physicians, the Ministry of Health Care officials and others. Despite the great attention of researchers to issues of palliative care, the majority of physicians confirm its low accessibility to patients, as testify the results of survey of health workers carried out by the Foundation of Independent Monitoring of Medical Services "Zdorovie". The article also considers issues of normative base regulating palliative care provision and application of narcotic analgesics. The analysis of study results permits to conclude that despite considerable attention to palliative care, the issue of its accessibility continoue to be very actual.