Search

Abstract Spin-Orbit Torque (SOT) Magnetic Random-Access Memory (MRAM) devices offer improved power efficiency, nonvolatility, and performance compared to static RAM, making them ideal, for instance, for cache memory applications. Efficient magnetization switching, long data retention, and high-density integration in SOT MRAM require ferromagnets (FM) with perpendicular magnetic anisotropy (PMA) combined with large torques enhanced by Orbital Hall Effect (OHE). We have engineered a PMA [Co/Ni]3 FM on selected OHE layers (Ru, Nb, Cr) and investigated the potential of theoretically predicted larger orbital Hall conductivity (OHC) to quantify the torque and switching current in OHE/[Co/Ni]3 stacks. Our results demonstrate a ~30% enhancement in damping-like torque efficiency with a positive sign for the Ru OHE layer compared to a pure Pt layer, accompanied by a ~20% reduction in switching current for Ru compared to pure Pt across more than 250 devices, leading to more than a 60% reduction in switching power. These findings validate the application of Ru in devices relevant to industrial contexts, supporting theoretical predictions regarding its superior OHC. This investigation highlights the potential of enhanced orbital torques to improve the performance of orbital-assisted SOT-MRAM, paving the way for next-generation memory technology.

Combining wind and hydropower facilities makes it possible to solve the problems caused by power supply shortages in areas that are remote from the central energy system. Hydropower plants and highly manoeuvrable hydroelectric units successfully compensate for the uneven power outputs from wind power plants, and the limitations associated with them are significantly reduced when they are integrated into the regional energy system. Such an integration contributes to increasing the efficiency of renewable energy sources, which in turn reduces our dependence on fossil resources and decreases their harmful impact on the environment, increasing the stability of the power supply to consumers. The results of optimisation calculations show that a consumer load security of 95% allows the set capacity of RESs to be used in the energy complex up to 700 MW. It is shown here that the joint operation of HPPs and WPPs as part of a power complex and hydraulic energy storage allows for the creation of a stable power supply system that can operate even in conditions of variable wind force or uneven water flow. The conclusions obtained allow us to say that the combination of hydro- and wind power facilities makes it possible to solve the problem of power supply deficits in the regions of Kazakhstan that are remote from the central power station. At the same time, hydroelectric power plants and highly manoeuvrable hydroelectric units successfully compensate for the uneven power output from wind power plants and significantly reduce the limitations associated with them during their integration into the regional energy system.

According to Rosstat statistics, in recent years there has been a tendency in the Russian Federation to increase the intensity of construction, while both the number of buildings put into operation and their total area are increasing. The assessment of the potential radon hazard of land plots for construction provides the possibility of timely inclusion of the necessary protective (preventive) measures against radon in the design of buildings. This is a legal requirement in cases where the density of radon flux from the ground surface within the building area exceeds the established hygienic standards (action levels). The paper presents a review of Russian and foreign approaches to assessing the potential radon hazard of land plots conducted within the framework of engineering and environmental surveys. The current regulatory requirements to radiation safety indicators of land plots for construction of residential, public and industrial buildings and facilities in the Russian Federation are analyzed. The main drawbacks of the algorithm for determining the potential radon hazard of land plots, used in the current guidelines MU 2.6.1.2398-08 approved at the federal level more than 15 years ago, are described. Critical remarks (the unsuitability of the value of density of radon flux from the ground surface for designing radon protective and mitigation (remedial) measures, lack of consideration of seasonal variations of radon flux density, etc.) accumulated over the years as a result of practical application of these guidelines are presented. Benefits and drawbacks of foreign approaches to assessing the potential radon hazard of the territory based on the results of measurements of radon concentration in soil gas, as well as the very possibility of a transition in Russian regulations from the density of radon flux from the ground surface to the radon concentration in soil gas, are analyzed. Some rational proposals of various Russian research teams on improving the algorithm for determining the potential radon hazard of land plots are considered.

Abstract The current study aimed to evaluate short‐ and long‐term complication rates and functional outcomes in a substantial cohort of patients undergoing ileal ureter interposition at two high‐volume medical centres. Materials and methods A retrospective single‐arm analysis was conducted on patients who underwent ureter reconstruction using ileum between 2003 and 2022 at the University Clinic of Cologne and the National Cancer Institute of Ukraine. Data on aetiology, surgical techniques, pre‐ and postoperative kidney function changes, readmission rates and complication management were collected. Postoperative complications were classified according to Clavien–Dindo, and estimated glomerular filtration rate (eGFR) was calculated using the CKD‐EPI formula. Results Results revealed 107 cases with consistent data. Within 90 days post‐surgery, 53% experienced complications, mainly graded as I–II. Grade III complications were seen in 13%, with two cases of grade IV complications leading to extended hospitalisation and patient death. The 90‐day mortality rate was 1.8%. Over a mean follow‐up of 52 months, clinically significant vesico‐renal refluxes occurred in 28%, with only 5.4% leading to persistent urinary tract infection. Antireflux techniques appeared to reduce urine upflow incidence compared with conventional interposition. Anastomosis stricture occurred in 15% of patients, with 63% requiring permanent re‐stenting and 37% needing re‐anastomosis. Metabolic acidosis was clinically significant in 7.5% of cases. A slight improvement in renal function was observed during the first year post‐surgery (average postoperative eGFR = 76 ± 22 ml/min; Mann–Witney U test, p = 0,0198). Affected kidney function improved in 56 (52%), was stable in 41 (38%) and deteriorated in 10 (9.3%). Loss of kidney function on the surgery side was seen in 4 (3.7%) patients and resulted in nephrectomy in 3 (2.8%) cases. Conclusion Ileal ureter interposition demonstrated a favourable safety profile and functional outcomes. This surgical intervention provides an effective tension‐free bypass, irrespective of healthy ureter length.

BACKGROUND: Molecular genetic testing (MGT) is increasingly accessible, improving diagnosis of monogenic diabetes (DM), particularly maturity-onset diabetes of the young (MODY). While most MODY research focuses on pediatric populations, diagnosis is possible after age 18. The Federal Diabetes Registry (FDR) offers unique insights into real-world management of MODY patients.AIM: To analyze the clinical features of DM onset, carbohydrate metabolism, complications, and hypoglycemic therapy (HT) in patients with the main types of MODY based on the FDR data.MATERIALS AND METHODS: A cross-sectional analysis of the FDR was conducted. All patients with registered MODY diagnoses (MODY-1, MODY-2, MODY-3, or other) as of June 1, 2023, were included. The specified MODY type was considered indicative of prior MGT. Direct MGT results are not recorded in the FDR.RESULTS: The study included 640 patients. MODY2 was the most prevalent type (69.4%), followed by MODY1 (18.2%) and MODY3 (12.4%). The median age of DM diagnosis was 19 years for MODY1, 10 years for MODY2, and 14 years for MODY3. The majority of patients (71.4%) were diagnosed with MODY before the age of 18 years.While 61% of MODY2 patients received monotherapy with diet, others received various ADT. Sulfonylureas were commonly prescribed for MODY3 patients (45.8%), and for a smaller portion of MODY1 patients (14.1%). Insulin therapy was more frequent in MODY1 and 3 (35.9% and 31.2%, respectively). The target glycated hemoglobin level was achieved in 82% of MODY2 patients and in 50.7% and 52.9% of MODY1 and 3 patients, respectively.Diabetic complications were observed in 6.04% of MODY2 patients, 23.0% of MODY1 patients, and 22.0% of MODY3 patients. Specific complications included diabetic retinopathy (5.75%, 1.21%, and 3.39% in MODY1, MODY2, and MODY3, respectively), diabetic nephropathy (10.3%, 2.11%, and 11.9%), and diabetic polyneuropathy (14.9%, 4.53%, and 15.3%).CONCLUSION: The FDR analysis revealed real-world practice patterns in MODY management, highlighting a lack of standardized treatment approaches and potentially unnecessary insulin use. These findings, coupled with an expected rise in MODY diagnoses, underscore the need for clinical guidelines for this population.

The first Russian book in forty years summarizing the information on the history of Korean art was published by the MIF publishing house as part of the book series The Main Things in History. But is the art of Korea known to us enough to highlight the “main things” in it? The publisher clearly understands the audience of readers the product is intended for, and in serial publications it offers navigation designed for the usual quick search for introductory information; however, the requirements of brevity and ease of presentation prevail over scientific fundamentality. The competence of the author, Korean Art historian Elena Khokhlova, her passion for the material, and courage in the selection of ‘key monuments’ made it possible, even within constraints, to create a serious work that awakens interest in the Oriental culture and in the history of art in general.

Summary: The loss of cerebellar Purkinje cells is a hallmark of neurodegenerative movement disorders, but the mechanisms remain enigmatic. We show that endocytic adaptor protein complex 2 (AP-2) is crucial for Purkinje cell survival. Using mouse genetics, viral tracing, calcium imaging, and kinematic analysis, we demonstrate that loss of the AP-2 μ subunit in Purkinje cells leads to early-onset ataxia and progressive degeneration. Synaptic dysfunction, marked by an overrepresentation of parallel fibers (PFs) over climbing fibers (CFs), precedes Purkinje cell loss. Mechanistically, AP-2 interacts with the PF-enriched protein GRID2IP, and its loss triggers GRID2IP degradation and glutamate δ2 receptor (GLURδ2) accumulation, leading to an excess of PFs while CFs are reduced. The overrepresentation of PFs increases Purkinje cell network activity, which is mitigated by enhancing glutamate clearance with ceftriaxone. These findings highlight the role of AP-2 in regulating GRID2IP levels in Purkinje cells to maintain PF-CF synaptic balance and prevent motor dysfunction.

ABSTRACT Prediction of body weight (BW) using biometric measurements is an important tool especially for animal welfare and automatic phenotyping tools that needs mathematical models. In this study, it was aimed to predict the BW using body length (BL), chest girth (CG) and width of the waist (WW) for rabbits of the maternal form of Hyla NG. The standard rabbit‐raising practices were applied for the animals. A highly efficient gradient‐boosting decision tree (LightGBM), eXtreme gradient‐boosting (XGBoost) and support vector machine (SVM) algorithms were evaluated and compared to the prediction of BW. The coefficient of determination, root mean square error and mean absolute error values were used as comparison criteria. The results showed that LightGBM, XGBoost and SVM algorithms were well fit for the BW using the biometric measures with over 95% accuracy for both train and test sets. The BL was determined as the most explanatory variable on body weight.

For deposition protective coatings of Ta/Nb/Sub (Sub – steel grade 0HN3MFA) on the inner surface of a caliber 30 mm full-size barrel simulator using high power impulse magnetron sputtering (HiPIMS) technology, the optimal design of a magnetron evaporator and its magnetic system was developed, construction documentation was developed, and a test vacuum installation stand was manufactured on this basis.Single- and double-layer Nb and Ta films were produced on the manufactured stand with different technological parameters. Studies of their physical and mechanical properties have shown that two-layer Ta/Nb/Sub coatings with a tantalum thickness of 20 μm or more have high wear resistance. Tantalum niobium coatings have Young's modulus values closer to gun steel than galvanic chromium, which increases the resistance of these coatings to cyclic thermal and mechanical stresses and improves the protection of gun steel from the aggressive effects of powder gases. It has been found that the probability of cracking in Ta/Nb/Sub coatings is almost half less than in chromium coatings.

The article is dedicated to the study of issues related to multiple citizenship in the context of international law and security. In the current conditions of increasing globalization, the regulation of multiple citizenship is becoming ever more relevant, particularly in terms of national security and state sovereignty. The article examines key challenges associated with the involvement of individuals with multiple citizenship in armed forces during military conflicts, potential conflicts of interest that may arise in such cases, and the risks of other states interfering in internal affairs under the pretext of protecting the rights of their citizens. Special attention is given to the analysis of legal practices for the protection of citizens in cases of multiple citizenship, specifically through the lens of the “effective citizenship” concept formulated by the International Court of Justice in the „Notteboom“ case. The article also explores the economic aspects of multiple citizenship, including issues of double taxation and possible ways to resolve these issues through international agreements. Based on an analysis of national and international legal systems, the authors conclude that there is a need to improve legal regulation of citizenship to avoid legal conflicts and ensure a stable political situation in the context of globalization. Particular attention is given to aspects of state sovereignty, which are increasingly under threat due to the growing number of individuals with multiple citizenships. The article offers recommendations to enhance international legal regulation to protect state sovereignty and the rights of individuals with multiple citizenship in the face of modern global challenges. In the context of martial law, the issue of maintaining ties with the Ukrainian diaspora around the world and preserving social capital is examined. Emphasis is placed on multiple citizenship, which is often seen as a result of conflicts between the laws of different countries. One of the risks to national security, both for the state of residence and the state of origin, is the issue of preserving state secrets and ensuring the inviolability of borders for individuals with multiple citizenships. To maintain close contact with Ukrainian refugees, the host countries and Ukraine must cooperate to strengthen financial ties, promote the reduction of remittance costs, and prevent double taxation. The need for effective cooperation between Ukraine and other states to create a legal framework for legal migration and cross-border mobility of Ukrainians after the war is substantiated. It has been proven that for the sake of security, control over migration processes, and post-war reconstruction after the Russia-Ukraine war, the legalization of multiple citizenship in Ukraine is the optimal step.

Recently, the production of metal products has become more adaptable and rapid because of the use of 3-D technologies based on layer-by-layer manufacturing. The quality of products produced using selective laser melting (SLP technology) depends on many factors, which can be divided into main groups: equipment, materials, processes, details, and finishing. To ensure high-quality products, a systematic approach is necessary. Subject of research. Patterns of influence of changes in combinations of the main parameters of the manufacturing process (scanning strategy, distance between tracks, specific power of the process, scanning speed, power) of various elements (main body, border, post-border) of products manufactured using SLP technology on their quality (integrity and surface roughness). Purpose. Development of recommendations for border and post-border printing modes for 316L stainless steel with a layer thickness of 30 μm at different distances between tracks and with varying laser beam scanning speeds. Methods. For research purposes, samples of 10×10×5 mm were prepared using SLP technology from AISI 316L austenitic steel powder. The main body of the test samples was produced with constant parameters: layer thickness 30 μm, power 100 W, scanning speed, 1700 mm/s; distance between tracks, 0.05 mm; calculated specific energy density is 39.21 J/mm3, continuity ~ 99, 9%. The test samples of groups A, B, and C differed according to the scanning strategy (Out2In or In2Out) and the distance between the boundary tracks. Test samples of groups D, E, and F were made with constant power parameters of 100 W and the distance between the border tracks of 0.05 mm and with variable scanning speed parameters for group D 2.6...1.75 m/s; for group E 2.6...1.55 m/s; for group F 3.0...1.55 m/s. Samples were printed on an Alfa-150 3D printer by ALT Ukraine LLC. Etching of the structure was carried out in Kalling’s solution, and analysis of the microstructure was carried out using an AxioVert 200MMat optical microscope. Roughness control was performed using BioBase 220R with a constant control zone of 1.25 mm. Results. It was established that the borders of samples of groups A and C printed in the range of specific energy density ≈27-33 J/mm3 have thin borders that do not protrude and have no visible deviations. Roughness control showed that test samples A3, A4, C4, D5, E1, E2, and F6 had roughness values in the range of 9.7...11.2 microns. When comparing the obtained data with the manufacturing parameters and microstructure analysis, it was established that to achieve high continuity with minimal roughness in the finished products on the surface, it is necessary to apply the parameters in such a way that the post-contour manufacturing parameters energetically fluctuate in the range of ± 4 J/mm3 from the specific energy density main body.

The paper presents comments, remarks and opinions on the material presented in the publication “Radon regulation crisis in Russia: scale of the problem and proposals for remediation” by Tsapalov A., Miklyaev P., Petrova T., Kuvshinnikov S., published in the Russian peer-reviewed journal “ANRI” (“Radiation measurement equipment and news”), 2024, No. 1.

The paper (in two parts) presents an overview of the new guidelines MR 2.6.1.0333-23 (approved on 01 December 2023) that supersede guidelines MU 2.6.1.2838-11, which were used for organizing radiation surveys of residential, public and industrial buildings and facilities and their sanitary assessment in terms of radiation safety indicators over the past 12 years. Due to a large number of critical comments on the document received during this period, a need for significant revision emerged. The scope of the revised document was expanded and now covers all stages of the life cycle of buildings and facilities: commissioning; operation period; overhaul and reconstruction; demolition. In the first part of the paper, the structure and legal status of the new guidelines are considered, as well as the main innovations in terms of measuring the ambient dose equivalent rate of gamma radiation, searching and identifying local radiation anomalies and sites of radioactive contamination, measuring surface contamination levels, and determining the minimum number of premises to be surveyed. The reasons for the introduction of certain changes are outlined. The issues of evaluation of measurement uncertainty, reporting of measurement results and registration of test reports are covered in detail. In the second part of the paper, numerous innovations will be considered in terms of estimating the indoor average annual equilibrium equivalent concentration of radon isotopes.

The paper presents the results of analysis of the Ivanovo Regional databank of radiation doses to the pub-lic from exposure to natural and technologically enhanced radiation background for 2008–2022. Despite the fact that the average individual annual effective dose of public exposure due to natural sources of radiation in the Ivanovo region calculated for the whole 15-year period (4.63 mSv/year) does not qualify as increased (i.e. does not exceed the level of 5 mSv/year), in some years of the period under review the average dose repeatedly exceeded this level, reaching 7.50 mSv/year. The analysis of the structure of the doses of public exposure due to natural sources of radiation in the Ivanovo region showed that the contribution of the dose from internal exposure to radon, thoron and progenies according to measurement results from 2008–2022 ranged from 65.98 to 81.47%. The paper provides examples of buildings of different types in settlements of the Ivanovo region, in which the indoor radon isotopes equilibrium equivalent concentrations are more than 1.5 times higher than the established action level for existing dwellings and public buildings (200 Bq/m3). Despite the significant amount of measurement data in the Regional databank, it is necessary to obtain additional information on the indoor radon levels in existing wooden and other low-rise dwellings and public buildings in the most radon-prone areas of the region. For this purpose, a radon survey is planned for 2024-2026 in the cities of Ivanovo and Kohma, Privolzhsky, Gavrilovo-Posadsky, Teykovsky and Zavolzhsky districts of the Ivanovo region within the framework of a joint project of Saint-Petersburg Research Institute of Radiation Hygiene after Professor P.V. Ramzaev, the Directorate of the Federal Service for Surveillance on Consumer Rights Protection and Human Wellbeing in the Ivanovo region, and the Center of Hygiene and Epidemiology in the Ivanovo region.

Introduction. The development of a comfortable living environment for persons with disabilities is one of the most important tasks in the modern world. To a large extent, its implementation depends on housing conditions of wheelchair users, as well as principles of their settlement in multi-apartment residential buildings in cities. Physical barriers in the architectural environment are the most pressing problem hindering rehabilitation and socialization of disabled persons. Uncomfortable housing conditions can hinder therapeutic and preventive care aimed at health improvement, and physical barriers in housing and yard spaces can hamper the socialization of disabled residents. The problem is more relevant than ever, because due to the ongoing Special Military Operation, the number of physically challenged persons is growing fast. With this in mind, it is important to continue adapting residential buildings for limited mobility population groups and creating a more comfortable environment for all urban residents.Materials and methods. The article analyzes (1) the way wheelchair users take up residence in large-scale development areas in the post-war Krasnodar, (2) the settlement of wheelchair users in urban districts, (3) the residence of wheelchair users in buildings with a different number of storeys. Contradictions are identified between current urban development conditions and surrounding architecture, on the one hand, and the needs of limited mobility residents, including their socialization, on the other hand. Criteria for selecting design solutions are developed; recommendations are provided for the reconstruction of existing residential buildings, equipping and modernizing the urban environment, raising awareness of problems to be tackled by disabled persons.Results. A list of steps is proposed to solve problems of housing conditions and principles of resettlement of disabled persons. In particular, factors impeding the treatment, preventive care and socialization of wheelchair users are considered, and their elimination plan is proposed. The article provides a theoretical justification for techniques and principles of architectural design of buildings, taking into account the accessibility of standard residential buildings to limited mobility persons.Conclusions. The article describes methods and techniques for solving several most important problems of wheelchair users in Krasnodar, in particular, their accessible living environment. The appropriateness of the settlement of disabled persons in the existing urban development and reconstruction of this architectural environment were analyzed; methods for designing buildings that meet the housing requirements of limited mobility persons were presented. Also, a social approach to developing a comfortable living environment for such members of society was formulated and analyzed to ensure their integration into the life of society, equal access to infrastructure, work and education.

The purpose of the current work was to search for sources of winter wheat varieties’ resistance to the most common pathogens of the area under artificial infectious backgrounds and recommend them for inclusion in the breeding process. The study was carried out on the experimental plots of the laboratory for plant immunity and protection of the FSBSI Agricultural Research Center “Donskoy” in 2021–2023. In order to expand the genetic diversity in immunity of newly developed varieties, it is necessary to use new sources of resistance. The objects of study were 78 winter wheat varieties from interstation variety testing, representing various breeding institutions in Russia and some foreign varieties. The study material was presented by the North Caucasian populations of pathogens of such wheat leaf diseases as powdery mildew, brown and yellow rust, and leaf spot (Septoria). Infectious backgrounds were formed according to generally accepted methods, using spore material of pathogens both stored and collected from crops in the previous year (types of rust) and collected from overwintered plants in the spring (powdery mildew, leaf spot). Weather conditions varied across the years of study (mainly in the autumn), but the damage to susceptible test varieties in the experiments was maximum. As a result of the study of the varieties on their resistance to one pathogen, there have been identified 23 varieties resistant to powdery mildew, 56 ones to leaf rust, 47 ones to yellow rust, 8 varieties to leaf spot. There has been given a characteristic of varieties according to various degree of resistance or susceptibility to each pathogen. On the resistance to two pathogens, there have been identified 17 varieties resistant to brown and yellow rust, 2 varieties resistant to brown rust and powdery mildew, 2 varieties resistant to yellow rust and powdery mildew, 1 variety resistant to brown rust and leaf spot. Ten varieties have shown resistance to no one pathogen, but they have had moderate resistance or moderate susceptibility to several other pathogens. 20 varieties were resistant to three diseases in various combinations, and 16 of them were resistant to brown, yellow rust, and powdery mildew. One variety ‘MV Nador’ from Hungary showed resistance to all 4 pathogens. All resistant varieties identified in the study can replenish the supply of winter wheat sources to the complex of leaf diseases for breeding purposes or be used in integrated crop protection against leaf diseases.

Aim: to develop a model for predicting preeclampsia (PE) based on the clinically most significant differentially expressed plasma microRNAs.Materials and Methods. A prospective observational comparative study was conducted with 62 women, divided into two parallel groups: 32 patients with PE and 30 clinically healthy women with uncomplicated pregnancy. Transcriptomic analysis was performed to identify differentially expressed blood plasma microRNAs using next generation sequencing (NGS).Results. Calculation of risk ratios for PE development allowed to identify 14 plasma microRNAs that influence the development of PE pathology. PE-associated microRNAs hsa-miR-103a-3p, hsa-miR-451a and hsa-miR-516a-5p have a high diagnostic value when combined to assess their blood plasma expression level in early pregnancy stages.Conclusion. The developed prognostic model can be applied to pregnant women at risk for PE development, which may further reduce obstetric complications and improve perinatal outcomes.

Abstract: Numerical modeling of the propagation of high-power femtosecond laser beams in a diamond crystal in the self-focusing mode is carried out. The wavelength of the modeled beams is varied in a wide range (400 nm, 532 nm, 800 nm, and 1064 nm), which covers the most common ultrashort laser systems. For all the wavelengths analyzed, a limitation in the growth of laser energy density inside the diamond crystal with increasing pulse energy is found. This effect has a certain similarity to the effect of optical limitation in laser filaments, but manifests itself outside the filament formation zone and is capable of equalizing the laser energy density, as well as the electron density, inside the extensive pre-focal region.

The building of the Kazan Cathedral Mosque is still a promising architectural project, but it already has its own history, which includes the 2022 international sketch competition, held within the anniversary of the adoption of Islam in the Volga region, the struggle for the preservation of the Kyrlay park, where the foundation stone was laid, the decision to move the construction site, the introduction of new projects out‐of‐competition in 2023, and the discussion of one of such projects, unexpectedly “announced approved by the authorities”, with the Muslim religious leaders of Tatarstan... The protracted design of such an iconic object allows evaluating the starting points, the development direction, and problems of modern mosque architecture in Russia, the searches for the image of a religious building and the options to combine the traditional and the modern in Muslim architecture. The fate of the Kazan Cathedral Mosque is comparable to the “Kocatepe mosque incident” — the construction of the Ankara Cathedral Mosque, which for decades consolidated the neo‐Ottoman trend in Muslim architecture. The project owners of the Kazan mosque declared rejection of the tiresome adapted neo‐Ottoman model, which substituted the search for truly national forms. However, architectural bureaus are not yet ready to propose projects that would equally satisfy the requirements for the preservation of the traditional and the compliance with the trends of modern architecture, and often replace creative searches with combinations of quotations of famous monuments or elements of earlier competition projects.

Objective: The study aimed to evaluate the epidemiological, clinical, and molecular data of mucopolysaccharidosis type II (MPS II) patients and their outcomes using the national registry of patients in the Russian Federation (RF). Materials and Methods: In the retrospective cohort study, the authors included data from the Russian national registry of MPS II. Results: The prevalence of MPS II in RF is 0.62 per 100 000 live births or 0.09 per 100 000 population with the majority of patients in the Central (n = 36) and the Volga Federal District (n = 35). Males were 157 (99.4%), positive MPS II family history had 47 (29.7%) patients. The median age of the first symptoms was 1.8 (0.8-2.6) years, ranging from 0.1 to 19 years, and the age of diagnosis was 4.0 (2.5; 5.9) years, ranging from 0.1 to 38.9 years. A genetic study was available for the analysis in 116 (73.4%) patients. Single nucleotide variants in the IDS gene were found in 98/116 (84.5%) patients, and 18 further patients (15.5%) had gross rearrangements. About 59/98 (60.2%) patients had missense, 15/98 (15.3%) had frame-shift variants, 12/98 (12.2%) had splice site, and 11/98 (11.2%) had nonsense variants. One (1.0%) patient out of 98 patients had a small deletion. Pathogenic, likely pathogenic variants, and variants with uncertain significance were found in 54 (55.1%), 36 (36.7%), and 8 (8.2%) patients, respectively. About 138 (87.3%) patients received enzyme replacement therapy. Conclusion: The prevalence of MPS II in the RF is higher than that in some European countries and closer to the Asian population. The registry is a convenient tool for disease epidemiology and monitoring.