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This paper aims to examine the impacts of career guidance interventions on school students' career-related skills, knowledge and beliefs by combining relevant empirical studies conducted in the last 10 years. A random-effects meta-analytic technique was employed for this purpose. After screening, electronic databases using pre-defined eligibility criteria, nine studies involving a total of 1,433 participants were included in the final meta-analysis. The analysis yielded a weighted mean effect size of 0.42 (95% confidence interval = 0.19, 0.65; z = 3.61, p < 0.01) which may be construed as a moderate-to-high effect size with a significant difference between the treatment and control conditions at post-treatment. As a result, post-test career-related outcomes in students who received career guidance were significantly higher than in non-guidance groups. The results suggest that career interventions may provide some modest developmental progression in school-age children and adolescents particularly through improving learners' career decidedness and attitudes such as future time perspective. These findings might have strategic implications for policy and practice. This paper extends past research on career guidance effectiveness by identifying the combined effect size of relevant career interventions.

The article deals with results of airborne laser scanning, which was undertaken with drone above selected locations of kurgans of the Sargat culture in the middle Irtysh river basin (Omsk region) and its correlation with total station data of terrain survey. The selected scanning area covers imposing “Princely kurgans” situated on a plowed field and small mounds inside birch forest. The scanning results were processed with various filters for further creation of a digital terrain model (DTM), and then based on DTM, a topographical relief of the area under study was presented with a step of 1.0 m and of 0.1–0.2 m for sites of kurgan groups’ locations. Structures visible on DTM are coincided in quantity, size and relative position with previously recorded kurgan mounds. Besides already known archaeological objects DTM allows identifying structures similar to small kurgans mapped during terrain prospection, but requires further clarification in a course of electromagnetic survey or excavation. Combination of field and cartographic approaches provides estimation of topography particularities and hierarchy of kurgans various in size and remoteness. Small kurgans are mapped at lower hypsometric levels and distinctly close to the origins of the ravines. “Princely kurgans” were accommodated to dominant heights of foremost river terrace what was decisive in landscape exploitation, i.e. new territory markers were erected and thus transformation of landscape into social sense occurred. The particularity of the graveyard functioning was reconstructed based on data recorded during excavation of various kurgans in different sides of the area under study.

Background. Gamma rhythm plays an important role in enabling cognitive processes. Studies confirm the association of gamma activity with visual and auditory perception, time perception, attention, consciousness and processing of semantic information, inner speech, and memory.Objective. The purpose of the study is to evaluate gamma activity in patients with hemispheric ischemic stroke in the acute and acute periods in comparison with cognitive and anxiety-depressive disorders.Materials and methods. A study was carried out on 32 patients with hemispheric ischemic stroke. All patients underwent comprehensive clinical-neurological, laboratory-instrumental and neurophysiological studies. The EEG was analyzed visually and using mathematical analysis. Using the method of mathematical analysis, we assessed the average values of the gamma rhythm power spectrum in the range of 30-45, 50-70 and 80-100 Hz in all leads and the peak frequency of the γ rhythm of the background EEG. The NIHSS, Rankin, Rivermead, Montreal Cognitive Assessment Scale, Hospital Anxiety and Depression Scale, Spielberger – Hanin Reactive and Personal Anxiety Scales, and Beck Depression Scales were used.Results. Mathematical analysis of bioelectrical activity of the brain of patients with hemispheric ischemic stroke showed deviations in the gamma rhythm in the range of 30-100 Hz, compared with the control group. Statistically significant correlations were established between cognitive, anxiety-depressive disorders and the gamma rhythm index in the frontal and central-temporal regions in the frequency range of 30-100 Hz.Conclusion. Mathematical analysis of the bioelectrical activity of the brain, along with clinical and neuropsychological studies, is recommended to be used for the diagnosis and identification of cognitive and anxiety-depressive disorders in patients with hemispheric ischemic stroke already in the acute and acute periods, which is especially important for prescribing adequate, pathogenetically based therapy and determining the prognosis of the disease.

Background. Predicting the success of rehabilitation in patients who have suffered an ischemic stroke (IS) is determined by factors of rehabilitation potential (RP). Assessment of RP is necessary to develop an effective rehabilitation program and predict the degree of restoration of impaired functions.Objective. The purpose of the study is to evaluate the effectiveness of rehabilitation in patients who have undergone IS.Materials and methods. A prospective study was carried out on 177 patients who were treated in the neurological department for patients with acute cerebrovascular accident and the neurorehabilitation department of the State Budgetary Healthcare Institution of the Republic of Bashkortostan of the Clinical Emergency Hospital in Ufa. All patients underwent clinical neurological, neuroimaging, laboratory and instrumental studies. The NIHSS, Rankin, Rivermead, Montreal Cognitive Function Scale, Hospital Anxiety and Depression Scale, A. M. questionnaire were used. Wein, T. Ehlers test and A. M. Schubert technique. The assessment of the RP was carried out using the analysis of data from the "Rehabilitation Sheet".Results. The majority of patients with IS were diagnosed with cognitive, anxiety-depressive disorders, and dysfunction of the autonomic nervous system. During treatment, the number of patients with mild neurological deficits, mild disabilities and an average level of mobility increased significantly. Based on a combination of factors, 74% of patients had medium and high levels of RP. In our study, according to the T. Ehlers test, 82.9% of patients before treatment had a medium to high degree of motivation to succeed. Conclusion. An integrated and individualized approach to the correction of various pathological disorders in patients who have undergone IS, taking into account RP, is the key to the effectiveness of restorative treatment at the stages of rehabilitation.

In a modern large city, it is mandatory to have playgrounds in residential areas. The level of social well-being and well-being of urban space is also assessed by the presence of a children’s playground in each residential courtyard. However, from a city-ecological point of view, is it advisable to organize parking on boulevards, in small squares, in courtyards next to car parks? In order to understand this, the state regulatory requirements and standards for the organization of children’s playgrounds in a large industrial city were studied; The territories of playgrounds were identified, the location of which raises doubts about the correctness of their placement; a geochemical analysis of natural environments was performed – the surface layer of air, soils and soils, snow cover on the territory of playgrounds and adjacent zones. An assessment was made of the compliance of of the geochemical analysis results with existing standards for the placement of playgrounds in urban space and urban planning solutions were proposed to optimize the locations of playgrounds created contrary to existing standards.

The article discusses the accounting of inventories received by the company without documentary support. The authors of this study set a goal — to propose a method that is justified from the point of view of theoretical, normative and practical aspects for reflecting such uninvoiced supplies in the accounting system, since the current official industry recommendations do not provide clear explanations for such business transactions. In the course of the work, the content of the concept of “uninvoiced supplies”, the history of its origin and modern interpretation in the theory and practice of the organization’s economic activity were examined. The current state of accounting at enterprises is characterized by a tendency to optimize the costs of its maintenance through the use of rational tools in the field of internal control, safety of material resources, generation of data for tax accounting, collection and synthesis of statistical information, etc. Accordingly, the creation of a universal accounting and analytical base used for the purposes of managing the economic objects of an enterprise becomes relevant. This circumstance is an important criterion when organizing the accounting of uninvoiced supplies. The authors of the study used methods such as a systems approach, analysis and synthesis, abstraction and modeling. The analysis of the problem and the search for optimal solutions were carried out on the basis of the requirements of regulations and methods of accounting theory. The material in the article can be used when creating an enterprise’s accounting policy, preparing local acts regulating economic accounting of the movement of material assets and their safety. The results of the studied issue are aimed at ensuring the efficiency of activities and sustainable development of business entities.

Objective: to investigate in a multicenter study relationship between type 2 diabetes mellitus (DM) and clinical manifestations of osteoarthritis (OA). Material and methods. The study involved 767 patients aged 40–75 years with a confirmed diagnosis of stage I–III knee OA who had signed an informed consent form. The mean age of patients was 57.9±9.6 years, body mass index (BMI) was 30.8±6.4 kg/m2 and median duration of disease was 5 [2; 11] years. An individual record card was filled out for each patient, it contained anthropometric parameters, medical history and clinical examination data, knee pain assessment using a visual analogue scale (VAS), WOMAC, parameters of KOOS questionnaire and patient's global assessment of health. Results and discussion. DM was detected in 17.2% of cases. Patients were categorized into two groups according to the presence or absence of DM. In the presence of DM, more severe clinical manifestations of OA were noted. However, patients with DM were older, had higher BMI values, longer disease duration and were more frequently diagnosed with radiological stage III OA. After stratification by age and disease duration, individuals with DM retained worse pain scores according to VAS compared to patients without DM (median 51.5 [41.5; 70] mm versus 36 [25; 50] mm, p=0.049), total WOMAC index (1047.5 [792; 1260] mm versus 823 [536; 1145] mm; p=0.005) and its components (pain – 200 [160; 254] mm versus 155 [108; 230] mm, p=0.002; stiffness – 90 [50; 115] mm versus 60.5 [35; 100] mm, p=0.03; functional impairment – 765 [550; 918] mm versus 595 [350; 820] mm, p=0.009). Conclusion. Type 2 DM is common in patients with OA (in 17.2% of cases) and is associated with more severe clinical manifestations: greater pain intensity according to VAS and higher WOMAC scores (total index and its components).

The aim of the work was to determine the pheno- and genotypic features of the aquatic CTX+ strain of Vibrio cholerae isolated in 2023 and a comparative bioinformatic analysis of whole-genome sequencing data.Materials and methods. Whole-genome sequencing was performed on MiSeq (Illumina) and MinIon Oxford Nanopore Technologies (ONT) platforms; hybrid assembly of the whole genome was carried out using the Trycycler algorithm; assembly errors were eliminated by means of the Medaka algorithm and the Pilon program. Dendrogram construction and bioinformatics analysis were carried out with the help of the scipy and Graphviz packages, BioEdit, BLASTN, BLASTP, CARD, ICE Genotyper, and Vector NTI programs. The ability to produce cholera toxin was tested using the GM1ELISA.Results and discussion. The isolated strain was identified as V. cholerae O1 Ogawa, sensitive to most antibiotics. Based on the totality of genetic properties, it was classified as the first genovariant, distinct from the typical El Tor strains only by the presence of ctxB1 gene of classical type instead of ctxB3 of the El Tor type. It has been established that it contains a tandemly duplicated CTX prophage on the small chromosome and a tandem of two copies of RS1 prophage on the large chromosome. Thereat, the rstR gene of the CTX prophage belonged to the classical type, and the RS1 prophage – to the El Tor type. The remaining criteria of epidemic hazard – tcpAelt, rtxA1 and intact VSP-II did not differ from the prototypes. The genome of the strain carries the ICE element VchBan11, which contains the trimethoprim resistance gene dfrA1, and phenotypically the strain is resistant to this antibiotic. Under in vitro conditions, the strain did not produce cholera toxin, as shown by ELISA results. This may be due to the presence of a deletion within the toxR regulatory gene. Strains similar to the 2023 isolate are mainly attributed to the second wave of the seventh pandemic. Currently, they are almost replaced by new genovariants, but occasionally can emerge and even cause diseases. Therefore, their importation into Russian territory potentially pose a threat to public health.

Background. Non-pharmacological treatments based on collagen as a dietary supplement are emerging as a new area of interest to support preventive or therapeutic effects in patients with osteoarthritis (OA). Aim. In a multicenter, prospective, double-blind, placebo-controlled, randomized study, to evaluate the effectiveness and safety of the use of the Artneo complex containing undenatured chicken collagen type II in patients with OA of the knee joints. Materials and methods. The study enrolled 212 outpatients from 12 centers in the Russian Federation with knee OA, stages II and III according to the Kellgren–Lawrence classification. The participants included 171 women (80.7%) and 41 men (19.3%), with an average age of 60.2±9.0 years (range: 40 to 75 years). The study population was randomly allocated in equal proportions into two groups using an interactive web response system (IWRS). Group 1 (Artneo) consisted of 106 patients who took one capsule of the drug once daily for 180 days. Group 2 (Placebo) also had 106 patients, with the dosage form and regimen identical to Group 1. During the treatment period, the following outcomes were assessed: WOMAC index, KOOS, pain according to VAS, quality of life using the EQ-5D questionnaire, and the need for NSAIDs. All patients underwent a clinical blood test, general urine analysis, biochemical blood test, and ultrasound examination of the affected knee joint. Results. In a prospective, double-blind, placebo-controlled, randomized study, it was demonstrated that the Artneo combination, containing undenatured chicken collagen type II, has a positive effect on all clinical manifestations of OA: it effectively reduces pain, stiffness, and improves the functional state of joints and quality of life. It has a good safety profile and is superior to placebo in all parameters studied. Conclusion. The results of the study confirm the good effectiveness and safety of the Artneo combination in patients with OA of the knee joints.

Aim. To evaluate the relationship of hypercholesterolemia (HCE) with clinical, instrumental, and laboratory parameters in osteoarthritis (OA) in a multicenter, cross-sectional study. Materials and methods. The study included 183 patients aged 40–75 years, with a confirmed diagnosis of stage I–III OA (ACR) of the knee joints, who signed an informed consent. The mean age was 55.6±10.7 years (40 to 75), body mass index was 29.3±6.3 kg/m2, and disease duration was 5 [1; 10] years. For each patient, a case record form was filled out, including anthropometric indicators, medical history, clinical examination data, an assessment of knee joint pain according to VAS, WOMAC, KOOS and comorbidities. All patients underwent standard radiography and ultrasound examination of the knee joints and laboratory tests. Results. HCE was detected in 59% of patients. Depending on its presence or absence, patients were divided into two groups. Patients were comparable in body mass index, waist and hip measurement, and disease duration but differed significantly in age. Individuals with elevated total cholesterol levels had higher VAS pain scores, total WOMAC and its components, an overall assessment of the patient's health, a worse KOOS index, and ultrasound findings (reduced cartilage tissue). HCE patients showed high levels of cholesterol, low-density lipoproteins, triglycerides, STX-II, and COMP (p0.05). However, after stratification by age, many initial intergroup differences became insignificant, and differences in the WOMAC pain score persisted. Conclusion. The results of the study confirmed the high prevalence of HCE in OA patients (59%). Patients with OA and increased total cholesterol have more intense pain in the knee joints.

Objective: to investigate the efficacy and safety of olokizumab (OKZ) in patients with knee osteoarthritis (OA) with synovitis, persistent pain and ineffectiveness of previous conservative therapy. Material and methods. The study included 15 patients with stage II–III knee OA who fulfilled the ACR criteria and had pain ≥50 mm on a visual analogue scale (VAS), synovitis and treatment failure. The age of patients ranged from 54 to 75 years; the duration of the disease was from 1 to 23 years. The duration of the study was 12 weeks, during which the patients received 3 subcutaneous injections of OKZ at a dose of 64 mg. The effectiveness of the treatment was assessed by the dynamics of pain intensity according to VAS, WOMAC and KOOS indices, the values of the DN4 questionnaire and the quality of life according to EQ-5D. In addition, the general assessment of the patient's health (GHA) according to VAS, the assessment of treatment efficacy by doctor and patient and the need for non-steroidal anti-inflammatory drugs (NSAIDs) were considered. All patients underwent laboratory testing. Results and discussion. During treatment, there was a significant decrease in pain intensity according to VAS, a statistically significant improvement in the KOOS and WOMAC indices (p

The objective is to determine the complete nucleotide sequence and conduct a phylogenetic analysis of genome variants of the Puumala virus isolated in the Saratov region. Materials and methods. The samples for the study were field material collected in the Gagarinsky (formerly Saratovsky), Engelssky, Novoburassky and Khvalynsky districts of the Saratov region in the period from 2019 to 2022. To specifically enrich the Puumala virus genome in the samples, were used PCR and developed a specific primer panel. Next, the resulting PCR products were sequenced and the fragments were assembled into one sequence for each segment of the virus genome. To construct phylogenetic trees, the maximum parsimony algorithm was used. Results. Genetic variants of the Puumala virus isolated in the Saratov region have a high degree of genome similarity to each other, which indicates their unity of origin. According to phylogenetic analysis, they all form a separate branch in the cluster formed by hantaviruses from other subjects of the Volga Federal District. The virus variants from the Republics of Udmurtia and Tatarstan, as well as from the Samara and Ulyanovsk regions, are closest to the samples from the Saratov region. Conclusion. The data obtained show the presence of a pronounced territorial confinement of strains to certain regions or areas that are the natural biotopes of their carriers. This makes it possible to fairly accurately determine the territory of possible infection of patients and/or the circulation of carriers of these virus variants based on the sequence of individual segments of their genome.

The current paper has presented the three-year study results (2021–2023) on recording the population dynamics of wheat and predatory thrips during the spring-summer vegetation period of winter common wheat in the forest-steppe of the Samara region. The purpose of the study was to determine the dynamics of the seasonal development of wheat and predatory thrips according to the phenophases of the development of winter common wheat in the forest-steppe of the Samara region. For this purpose, there were carried out the calculations using the mowing method with an entomological net on winter wheat crops in different phenophases of development, and the population of species and thrips were determined in the laboratory under a binocular. According to weather conditions, 2021 and 2023 were characterized as dry and hot, 2022 was characterized as cool with plenty of precipitation in the first half of the winter wheat vegetation period. The population dynamics of wheat and predatory thrips varied depending on the weather conditions of the spring-summer period of the year. In the hot years of 2021 and 2023 there were 2 periods of increase in population. In 2021, the period of maximum population of wheat thrips was observed from a heading stage to a flowering stage (1016 and 1048 specimen per 100 sweeps of the net) and in the phase of milky-wax ripeness (426 specimen per 100 sweeps of the net). In 2022, the population of wheat thrips on plants increased gradually, the peak was noted once during the flowering stage, amounting to 1084 specimen per 100 sweeps of the net. In 2023, a population increase was observed in the heading stage (1782 specimen per 100 sweeps of the net) and the phase of milky-wax ripeness (421 specimen per 100 sweeps of the net). In the hot years of 2021 and 2023 there were 2 periods of increase in predatory thrips’ population; the maximum number of entomophages was observed during the heading stage and amounted to 87 and 61 specimens per 100 sweeps of the net, respectively. In the cool year of 2022, there was one population increase during the flowering stage and amounted to 14 specimens per 100 sweeps of the net. In the phases of heading and flowering of plants, there were predominantly adult insects (imago), and in the phases of milky and milky-wax ripeness, there were predominantly found larvae of different ages. The weather conditions in 2021 and 2023 turned out to be more favorable for the development of wheat and predatory thrips than in 2022.

BACKGROUND: In 2014, germline signal transducer and activator of transcription (STAT) 3 gain-of-function (GOF) mutations were first described to cause a novel multisystem disease of early-onset lymphoproliferation and autoimmunity. OBJECTIVE: This pivotal cohort study defines the scope, natural history, treatment, and overall survival of a large global cohort of patients with pathogenic STAT3 GOF variants. METHODS: We identified 191 patients from 33 countries with 72 unique mutations. Inclusion criteria included symptoms of immune dysregulation and a biochemically confirmed germline heterozygous GOF variant in STAT3. RESULTS: Overall survival was 88%, median age at onset of symptoms was 2.3 years, and median age at diagnosis was 12 years. Immune dysregulatory features were present in all patients: lymphoproliferation was the most common manifestation (73%); increased frequencies of double-negative (CD4-CD8-) T cells were found in 83% of patients tested. Autoimmune cytopenias were the second most common clinical manifestation (67%), followed by growth delay, enteropathy, skin disease, pulmonary disease, endocrinopathy, arthritis, autoimmune hepatitis, neurologic disease, vasculopathy, renal disease, and malignancy. Infections were reported in 72% of the cohort. A cellular and humoral immunodeficiency was observed in 37% and 51% of patients, respectively. Clinical symptoms dramatically improved in patients treated with JAK inhibitors, while a variety of other immunomodulatory treatment modalities were less efficacious. Thus far, 23 patients have undergone bone marrow transplantation, with a 62% survival rate. CONCLUSION: STAT3 GOF patients present with a wide array of immune-mediated disease including lymphoproliferation, autoimmune cytopenias, and multisystem autoimmunity. Patient care tends to be siloed, without a clear treatment strategy. Thus, early identification and prompt treatment implementation are lifesaving for STAT3 GOF syndrome.

The functional responsibilities of general practitioners (family doctors) (GPs) have changed significantly over the past decade. The number of GP functions has expanded. Despite the increasingly complex professional activities of GPs, there is no scientific evidence for the qualification characteristics of GPs expressed in knowledge, skills, abilities, and competencies in the theory and practice in training. In this regard, a study was conducted to determine the priority of GP competencies that make up the qualification characteristics of a specialist. Aim. To analyze, generalize and rationale the research results, as well as to develop preliminary guidelines for GP training. Material and methods. The information base for the study was the results of ranking competencies according to their importance in the professional activities of GPs in Russia. It was obtained during the ascertaining experiment, implemented using the Delphi method as a method of expert assessment and strategic planning of training specialists. Conclusion. The analysis of competencies developed and agreed upon by experts showed its compliance with the job functions of GPs, the requirements of the Federal State Educational Standard for the General Medical Practice (Family Medicine) and the WONCA guidelines. A summary of the study results to determine the priority of competence in GP activities showed the following: — all competencies (25) were defined as necessary and constitutive of GP qualification; — 21 competencies were considered significant and highly significant for GPs; — 4 competencies were considered as of moderate significance and below moderate significance. The analysis of the priority of competences revealed insufficient methodological support for GP training in accordance with the social order. The results obtained showed the need to continue the study within the project "Quality Management of Training of General Practitioners (Family Doctors)".

The .sup.87Sr/.sup.86Sr strontium isotope ratio in skeletal tissues (teeth and bones) gives valuable information about the migrations and origin of humans and animals. In this study, we assess the suitability of various structural elements (enamel and dentin) of a human tooth sample from a burial mound (Early Iron Age, Omsk oblast) for strontium isotope analysis using inductively coupled plasma mass spectrometry (ICP-MS), laser ablation, and electron probe microanalysis. The microelemental mapping of a tooth fragment was conducted. The results indicate the absence of significant diagenetic changes in the tooth enamel, for which the .sup.87Sr/.sup.86Sr isotope composition was determined by multicollector ICP-MS after chromatographic separation. This research represents the first isotopic study of materials from archaeological excavations of Sargat culture burial mounds. Furthermore, this approach offers promise in reconstructing taphonomic conditions of burial environments, contributing to the understanding of conditions facilitating the preservation of skeletal tissues and archaeological information., Author(s): D. V. Kiseleva [sup.1] , M. V. Chervyakovskaya [sup.1] , V. S. Chervyakovskii [sup.1] , T. G. Okuneva [sup.1] , N. G. Soloshenko [sup.1] , V. A. Bulatov [sup.1] [...]

Introduction. Beside the excretion of metabolic wastes, the lymphatic system in CNS play a crucial role in the regulation of intracranial pressure that is vital for the organism. One of the possible pathways of cerebrospinal fluid (CSF) drainage is its flow through the foramen of the cribriform plate (CP) into the nasal cavity. Despite the significant contribution of the nasal tract to the overall dynamics of the liquor, not much is known about the mechanisms of this process and how it is regulated. Due to its influence on the tone of blood vessels and peristalsis of lymphatic vessels, nitric oxide (NO) is a powerful modulator of liquor outflow, but its effects on nasal CSF outflow have not been studied yet. Aim and Methods. Using diffusion-weighted magnetic resonance imaging (DW MRI), we characterized the changes in CSF outflow through the CP of C57Bl/6 mice influenced by intranasal application of NO synthesis modulators. Results. In our study, using DW MRI and computer tomography (CT) mapping of the CP, we detected significant CSF outflow through its large dorsal and ventral foramen located along the nasal septum. At the same time, the CSF flow rate through the dorsal orifices of the CP was the highest. In addition, we showed that intranasal introduction of NO donor after 30 min leads to a significant decrease of water diffusion through the CP whereas application of a nonspecific NO synthase inhibitor into the nasal cavity enhances nasal outflow. The effects of the NO modulators did not have any significant spatial patterns; CSF outflow was significantly altered across all CP orifices regardless of their size or localization. Conclusion. The obtained results demonstrate the potential possibility of noninvasive local regulation of liquor dynamics, which may be used in the development of new approaches to the therapy of intracranial hypertension of various etiologies and methods of CNS detoxification.

High-pressure high-temperature (HPHT) crystalline monosulfide solid solution (Mss) phases (FexNi1−xS, x = 0.90, 0.75, 0.50, 0.25), troilite (FeS I), and α-NiS of the Fe-Ni-S system were synthesized at 7 GPa and 900–1550 °C. The structural parameters of the obtained phases were refined by XRD using the Rietveld method. Factor group analysis revealed the number of active Raman modes for FeS I and α-NiS. Raman spectra of troilite, α-NiS, and Mss phases were obtained. It was shown that the Raman spectra of Mss phases and α-NiS have a similar topology. The Raman spectra of the experimental phases in the Fe-Ni-S system were analyzed with non-negative matrix factorization, which provided a meaningful concentration dependence of the spectral patterns. The spectral components were assigned to the FeS I and α-NiS structures. The structural and spectroscopic studies show linear dependencies of unit cell parameters and spectral components on composition and confirm the existence of a series of monosulfide solid solution FexNi1−xS.

The recombination-activating genes (RAG) 1 and 2 are indispensable for diversifying the primary B cell receptor repertoire and pruning self-reactive clones via receptor editing in the bone marrow; however, the impact of RAG1/RAG2 on peripheral tolerance is unknown. Partial RAG deficiency (pRD) manifesting with late-onset immune dysregulation represents an 'experiment of nature' to explore this conundrum. By studying B cell development and subset-specific repertoires in pRD, we demonstrate that reduced RAG activity impinges on peripheral tolerance through the generation of a restricted primary B cell repertoire, persistent antigenic stimulation and an inflammatory milieu with elevated B cell-activating factor. This unique environment gradually provokes profound B cell dysregulation with widespread activation, remarkable extrafollicular maturation and persistence, expansion and somatic diversification of self-reactive clones. Through the model of pRD, we reveal a RAG-dependent 'domino effect' that impacts stringency of tolerance and B cell fate in the periphery.

Background and aimsThere is an increased risk of lymphomas in inborn errors of immunity (IEI); however, germline genetic testing is rarely used in oncological patients, even in those with early onset of cancer. Our study focuses on a child with a recombination-activating gene 1 (RAG1) deficiency who was identified through a screening program for Slavic founder genetic variants among patients who died with malignancy at an early age in Belarus.ResultsWe identified one homozygous founder RAG1 variant out of 24 available DNA samples from 71 patients who developed lymphoma aged

BackgroundTrichothiodystrophy-1 (TTD1) is an autosomal-recessive disease and caused by mutations in ERCC2, a gene coding for a subunit of the TFIIH transcription and nucleotide-excision repair (NER) factor. In almost half of these patients infectious susceptibility has been reported but the underlying molecular mechanism leading to immunodeficiency is largely unknown.ObjectiveThe aim of this study was to perform extended molecular and immunological phenotyping in patients suffering from TTD1.MethodsCellular immune phenotype was investigated using multicolor flow cytometry. DNA repair efficiency was evaluated in UV-irradiation assays. Furthermore, early BCR activation events and proliferation of TTD1 lymphocytes following DNA damage induction was tested. In addition, we performed differential gene expression analysis in peripheral lymphocytes of TTD1 patients.ResultsWe investigated three unrelated TTD1 patients who presented with recurrent infections early in life of whom two harbored novel ERCC2 mutations and the third patient is a carrier of previously described pathogenic ERCC2 mutations. Hypogammaglobulinemia and decreased antibody responses following vaccination were found. TTD1 B-cells showed accumulation of γ-H2AX levels, decreased proliferation activity and reduced cell viability following UV-irradiation. mRNA sequencing analysis revealed significantly downregulated genes needed for B-cell development and activation. Analysis of B-cell subpopulations showed low numbers of naïve and transitional B-cells in TTD1 patients, indicating abnormal B-cell differentiation in vivo.ConclusionIn summary, our analyses confirmed the pathogenicity of novel ERCC2 mutations and show that ERCC2 deficiency is associated with antibody deficiency most likely due to altered B-cell differentiation resulting from impaired BCR-mediated B-cell activation and activation-induced gene transcription.

Diagnosing skin diseases in children can be a complex interdisciplinary problem. Incontinentia pigmenti (IP), also known as Bloch-Sulzberger syndrome, is a rare hereditary genodermatosis related to a mutation in the IKBKG gene. We present a family case of IP described from the perspective of various specialists, including dermatologists, oncologists, geneticists, dentists, and trichologists. The peculiarity of this case is the development of squamous cell carcinoma (SCC) on the shin of a 10-year-old female patient with IP. The patient had a positive family history: her mother and two sisters also displayed clinical manifestations of IP with involvement of skin, teeth and hair. The presence of exons 4–10 deletion in the IKBKG gene in all affected females was confirmed by detailed genetic evaluation using long-range PCR, and also high degree of X-chromosome inactivation skewing was demonstrated. The family underwent a comprehensive examination and was followed up for 2 years with successful symptomatic treatment of dermatologic manifestations. Recommendations were also made regarding dental and hair problems. By the end of the follow-up period, patients had stabilized, with the exception of a 36-year-old mother who developed generalized morphea. The study demonstrates the varying expressiveness of clinical symptoms among family members and emphasizes the importance of timely diagnosis for effective management of patients with IP.

Aim. To assess awareness of blood pressure (BP) level, adherence to therapy and achievement of target BP in residents of Russian regions as part of the 2023 hypertension (HTN) screening campaign.Material and methods. During May 2023, 4613 participants from 23 cities of Russia took part in the screening. Participation was voluntary without restrictions on sex. All patients aged over 18 years. During screening, BP and pulse were measured three times using automatic or mechanical BP monitors, and a questionnaire about behavioral risk factors, concomitant diseases and therapy was filled out. HTN was diagnosed with systolic BP ≥140 mm Hg and/or diastolic BP ≥90 mm Hg and/or taking antihypertensive therapy (AHT).Results. The analysis included data from 4585 respondents aged 18 to 95 years with an average age of 45 [28;59] years, of which 1729 were men (37,7%). The proportion of hypertensive patients was 49,6%. Among them, 62,8% took AHT and achieved target BP in 76,6% and 45,7% with the target BP criterion

The article deals with a complex study of the materials obtained during archaeological excavation of the kurgan Novopokrovka 16 in the middle Irtysh river basin (Omsk region). The site, which is attributed to the Sargat culture (5th century BC – second half of the 3rd century AD), was located farmost on the right bank of the river Irtysh, nearby the group of “Princely kurgans”. Despite almost total disturbance by robbers and agricultural activity, common scholars’ efforts demonstrate high informative capacity of the multidisciplinary approach, while modern level of undertaken research is not just being declared but enables to reconstruct seemingly lost information. The kurgan erection stages and mound structure features have been decoded using the results of a geophysical survey and soil morphology data. The paleoanthropological study of the incomplete skeletal remains does not support multiple or inlet interment inside the central grave, and provides sexing and aging of the buried individuals as well as some paleopathological observations. Apart from species examination, archaeozoological data testify that a warm period from spring to early autumn was the season of animal slaughter. Characteristics of mortuary rituals and direction of intercultural contacts of the ancient forest-steppe groups have been completed by new details based on the results of strontium isotope analyses (dental enamel of humans and animals) and evidence non-local origin of the individual buried under the excavated kurgan, what is different from local origin of horses butchered for funereal feasts. The proposed hypothesis supports previously suggested archaeological, paleoanthropological and paleogenetic facts on the origin of the forest-steppe population, indicating an involvement of various population groups with notable external components. Based on the archaeological materials, the kurgan under study might be dated back to mid-4th – 3rd centuries BC.

Nowadays, the importance of efficiency in providing qualified care in the field of dentistry is gaining relevance. The literature available to us indicates that 70–90 % of the child population and 100 % of the adult population of Russia suffer from various types of disease in the field of dentistry. The purpose of the study is analytical assessment of managerial and or-ganizational decisions in building up the Russian dental service over the period from 2010 using data available in Russian literature. The organization of dental care in Russia is provided by the Ministry of Health of the Russian Federation in accordance with the territorial-administrative affiliation of the population. In connection with the transition of the Russian Federation to a market economy, the level of budget financing of the dental industry has decreased, which has led to a significant increase in the incidence of diseases and their complications. Thus, it is necessary to develop the most rational and appropriate options for the management of dental services at the level of each individual region of Russia and a specific situation in it taken into account. These options must necessarily consider the distinctive specific features of socio-economic development of a particular region, the current level of dental morbidity of the local population and its main trends, the provision of with all necessary resources, financial stability, as well as other socio-hygienic risk factors, which have a direct impact on the overall state of dental care.