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This article presents modern data on epidemiology, etiology, and clinical manifestations of mucopolysaccharidosis (MPS) type I in children. MPS develops due to deficiency of particular lysosomal enzyme which determines the disease type. The article considers in details disease's pathogenesis and classification. Evidence-based approaches to diagnosis (differential diagnosis included) are covered, moreover, special attention is paid to pathogenetic, symptomatic, and surgical treatment of MPS.

The basis of the trilogy about Vitalin by A. A. Grigoriev is the diary entries of the autobiographical character. The writer consciously refers to this form of narration, since it allows the most complete disclosure of the inner world of the protagonist, conveying his thoughts, feelings, emotions, showing the complexity and inconsistency of his nature. Thanks to fragments of the diaries, the themes of the stories expand: from love experiences to philosophical reflections, reflections on the truth, the meaning of life, and the search for happiness. The protagonist’s diary also influences the chronotope of stories, expanding the space and time of the narrative, introducing a life background, background of events into the work, and at the same time enriching our understanding of the character. The author of the article briefly dwells on the autobiographical elements in the life of Arseny Vitalin, notes his resemblance to the character of the novel by M. Yu. Lermontov — Pechorin. Special attention is paid to the genre originality of Grigoriev’s works, their simultaneous isolation and inclusion in the cycle, trilogy. Besides the article discusses the diary fragment “The Dreamer’s Diary” and its meaning in the text. Psychologically convincingly, the author shows that Vitalin, immersed in love experiences, but unable to share his thoughts with someone close to him, finds an outlet for his pent-up feelings in keeping a diary, on the pages of which he gives free rein to his feelings and actions.

We study the optical flux and polarization variability of the binary black hole blazar OJ 287 using quasi-simultaneous observations from 2015 to 2023 carried out using telescopes in the USA, Japan, Russia, Crimea, and Bulgaria. This is one of the most extensive quasi-simultaneous optical flux and polarization variability studies of OJ 287. OJ 287 showed large amplitude, ∼3.0 mag flux variability, large changes of ∼37% in degree of polarization, and a large swing of ∼215° in the angle of the electric vector of polarization. During the period of observation, several flares in flux were detected. Those flares are correlated with a rapid increase in the degree of polarization and swings in electric vector of polarization angle. A peculiar behavior of anticorrelation between flux and polarization degree, accompanied by a nearly constant polarization angle, was detected from JD 2,458,156 to JD 2,458,292. We briefly discuss some explanations for the flux and polarization variations observed in OJ 287.

The task being undertaken can influence orthographic, phonological and semantic processes. In linguistic research, two tasks are most often used: a task requiring a decision in relation to the presented word and a passive reading task which does not require a decision regarding the presented word. The results of studies using these different tasks are not always consistent. This study aimed to explore brain responses associated with the process of recognition of spelling errors, as well as the influence of the task on this process. Event-related potentials (ERPs) were recorded in 40 adults during an orthographic decision task to determine correctly spelled words and words written with errors that did not change the phonology and during the passive reading. During spelling recognition, the early stages up to 100 ms after the stimulus were automatic and did not depend on the requirements of the task. The amplitude of the N1 component (90–160 ms) was greater in the orthographic decision task, but did not depend on the correct spelling of the word. Late word recognition after 350–500 ms was task dependent, but spelling effects were similar across the two tasks: misspelled words evoked an increase in the amplitude of the N400 component related to lexical and semantic processing regardless of the task. In addition, the orthographic decision task modulated spelling effects, this was reflected in an increase in the amplitude of the P2 component (180–260 ms) for correctly spelled words compared with misspelled words. Thus, our results show that spelling recognition involves general lexico-semantic processes independent of the task. Simultaneously, the orthographic decision task modulates the spelling-specific processes necessary to quickly detect conflicts between orthographic and phonological representations of words in memory.

The objective: to determine possibility of prescribing and safety of various anti-tuberculosis drugs and their combinations as part of a therapeutic or preventive course of anti-tuberculosis therapy (ATBT) in patients with end-stage chronic kidney disease (CKD) receiving hemodialysis replacement therapy in the settings with high prevalence of drug resistant strains of Mycobacterium tuberculosis.Subjects and Methods. 42 patients receiving hemodialysis replacement therapy were followed-up by Central Tuberculosis Research Institute: of them, 31 (73.8%) had active tuberculosis and 11 (26.2%) had residual post-tuberculous changes in the respiratory organs. During the follow-up, the nature and severity of adverse reactions to anti-tuberculosis drugs were assessed.Results. In the patients with end-stage CKD suffering from respiratory tuberculosis, drug resistant strains prevailed – 68.2%. Some of adverse events developed when taking anti-tuberculosis drugs were as follows: deterioration of uremia in 20.5% of cases, cardiovascular complications – in 17.9%, and neurotoxic reactions – in 15.4%. Progression of uremia occurred more often in the followed-up patients taking anti-tuberculosis drugs versus hepatotoxic reactions (p = 0.02). When treating tuberculosis patients with end-stage CKD, isoniazid was discontinued due to adverse events in 12.8% of cases and pyrazinamide in 12.8% of cases, other drugs were discontinued less frequently. Compilation of individual regimens of anti-tuberculosis drugs with the consideration of drug susceptibility testing results, contraindications, and concomitant diseases made it possible to provide an adequate anti-tuberculosis therapy for patients with end-stage CKD with tuberculosis infection in 92.9% of cases for a period of more than 6 months.

Background Conventional radiography, magnetic resonance imaging, nuclear medicine scintigraphy are the imaging techniques currently in use for the evaluation of diabetic neuroosteoarthropathy (DN) with computed tomography being not commonly employed even for cases complicated by chronic osteomyelitis. The objective was to explore MSCT-semiotics of anatomical and radiomorphological changes in foot bones in patients with DN complicated by chronic osteomyelitis (CO) to determine most common manifestations of CO in diabetic foot. Material and methods Single-centre retrospective study. A series of cases. Preoperative radiological findings, MSCT (multislice computed tomography) were examined in 14 patients with DN complicated by chronic osteomyelitis. Results The patients showed impaired structure of the cancellous bone of various severity depending on the nature and localization of foot bone destruction. Overall bone density was higher in cancellous bone of the distal tibia, talus, calcaneus with the local density ranging within significant limits and was maximum at some points of the subchondral tibia and talus and minimum in the intertrabecular areas of the calcaneus and the distal tibia. MSCT scans showed medial calcinosis of the arteries in 5 (35.7 %) from 14 patients. Conclusion MSCT as one of the most objective method for the qualitative and quantitative assessment of the bone is practical for identifying anatomical and topographic relationships of the foot bones and the ankle joint to facilitate reconstructions in three planes and VRT, which is important for assessing the foot in patients with DN complicated by chronic osteomyelitis. The technique allows measurement of the foot bone density in Hounsfield units determining the severity of osteoporosis, the extent of architectonics impairment to facilitate preoperative planning.

A great share of workers employed at polymetallic ores mining have to face harmful working conditions at their workplaces. To provide safe working conditions for them and to preserve their health is a vital task occupational medicine has to tackle. Polymetallic ore mining enterprises employ certain common technological processes; nevertheless, there are specific features depending on ore mining techniques and the mineralogical composition of different ores. These features determine differences both in working conditions and in occupational risks of developing occupational morbidity (OM) and work-related morbidity (WRM). By now, there have been enough studies on peculiarities of occupational health risks for workers employed at sulfide ore mines, copper-nickel ore mines and ferruginous quartzite mines. Considerably less attention has been given to assessing occupational risks for workers dealing with mining and processing copper-zinc ores. We performed complex clinical and hygienic examinations at a major copper-zinc ore mining enterprise located in the Southern Urals. The research results gave grounds for determining a category of working conditions, establishing formation peculiarities and the structure of occupational and work-related diseases among workers from various occupational groups. Occupational risks were assessed considering hygienic and medical-biological indicators. We established the highest occupational health risks for shaft sinkers, followed by drilling unit operators, timberers, excavator drivers, load haul dumper (LHD) operators and underground self-propelled machine (USPM) operators. The research results gave grounds for developing a conceptual model of assessing and managing occupational risks in the branch. The urgency of developing and implementing activities aimed at risk mitigation should be determined depending on how validated a risk is and on its rates established for specific occupational groups.

The paper summarizes the results of the authors of the study on the impact of the established tariffs within the marketing activities of the dominant market subject to the level of competition in the region. Studies have shown that marketing actions (including as a tool for retaining existing and attracting new subscribers) made regional operator of information-communication services is the dominant subject in the target market did not lead to a restriction of competition in the region.

This article considers the basic techniques for creating television works (videoshooting, lighting, edit- ing), allowing to form the virtual space and virtual time through television text.

Introduction. Ovarian cancer (OC) is the third most common gynecological cancer with the worst prognosis and highest mortality rate. The progression of OC can be accompanied by the detrimental functions of the components of the tumor microenvironment, including tumor-associated macrophages (TAMs).The purpose of the study to analyze distribution and morphological phenotype of TAMs in tumor tissue of patients with high-grade serous ovarian cancer (HGSOC).Material and methods. Formalin fixed paraffin embedded tissue sections were obtained from ovarian cancer patients after tumor resection. The protein expression of general macrophage marker CD68 and M2-like markers CD206, CD163 and stabilin-1, belonging to scavenger receptors, was analysed by immunohistochemical staining in tumor tissue. Histological assessment of TAM distribution was performed by pathologist. Immunofluorescent analysis/confocal microscopy was applied to establish the co-expression of CD68 with the main macrophage scavenger receptors.Results. We were able to find giant CD 68-positive macrophages with foamy cytoplasm in ovarian tumor tissue. The accumulation of these tams was specific only for patients with advanced stage (IIIC and IV stages). The presence of foamlike tams had a statistical tendency to be associated with ovarian cancer progression, including metastasis and recurrence. The distribution of stabilin-1-positive macrophages was matched to CD68 expression in almost all cases, as was shown by IHC. Confocal microscopy confirmed that stabilin-1 was expressed in at least 50 % of giant tams. If analysis of tumor samples also demonstrated co-expression of other scavenger receptors, CD163 and CD36, in foam-like cells. Similar to IHC, in most samples the expression of CD206 in tams of foam-like morphology was limited.Conclusion. For the first time we demonstrated the accumulation of giant macrophages with fluffy foam cytoplasm in the tumor tissue of treated patients with advanced ovarian cancer. Such macrophages express diverse scavenger receptors (stabilin-1, CD163, CD 36), thus indicating a high clearance activity of giant TAMs.

The article presents an overview of domestic research devoted to the development of students’ emotional intelligence by means of art technologies. The relevance of the problem of developing emotional intelligence in modern students is determined by the objectives of higher education. Despite the serious experience gained in Russia in the use of art in teaching and personal education, the active penetration of art technologies into the education system began to occur relatively recently, which explains the time frame of the review study – 2019–2021. The analysis was performed in December 2021 using the national bibliographic database of scientific citation. The research was conducted using such combinations of keywords as “emotional intelligence”, “development of emotional intelligence of students”, “art therapy”, “pedagogical art therapy”, “art technology”, “art therapy at the university” in Russian for the period of 2019–2021. Out of 902 publications devoted to art therapy, the problem of using art technologies in higher education, the development of emotional intelligence through art technologies, the authors selected 49 publications. The analysis of the selected publications showed that the problem of the development of emotional intelligence arouses increased interest among researchers. However, the development of emotional intelligence in students is not a priority. In the system of professional education, the topic of emotional intelligence is not given enough attention, which is unacceptable and does not meet the requirements for the level of training of modern specialists. In recent studies, the possibilities of using art technologies in the development of students’ emotional intelligence have not been sufficiently disclosed. There is a need for further development of a program for the development of students’ emotional intelligence, presentation and description of art therapy techniques and techniques, their place and role in the development of emotional intelligence. Undoubtedly, building up the evidence base and substantiating methods for verifying the effectiveness of various art technologies will be a promising direction.

An aluminum oxide (AlOx)/silicon nitride (SiNy) dielectric stack was developed using an industrial plasma‐enhanced chemical vapor deposition (PECVD) system with low‐frequency (LF) plasma source for the surface passivation of undiffused textured p‐type crystalline silicon. The median recombination current density is 4.3 fA cm−2 as determined from photoconductance decay lifetime measurements and numerical device modeling. To the best of our knowledge, this is the first time to present a high‐quality LF‐PECVD AlOx/SiNy passivation stack on undiffused textured p‐type crystalline silicon wafers, which are cleaned with industrial processes using HF, HCl, and O3. The simulation agrees well with the measured effective carrier lifetime if the velocity parameters of 5.6 cm s−1 for holes and 803 cm s−1 for electrons are applied with a fixed negative charge density of −3 × 1012 cm−2. The process integration of developed AlOx/SiNy dielectric stack is successfully demonstrated by fabricating p‐type back junction solar cells featuring a poly‐Si‐based passivating contact at the cell rear side. As the best cell efficiency, we achieve 24.2% with an open‐circuit voltage of 725 mV on a M2‐sized Ga‐doped p‐type Czochralski‐grown Si wafer as independently confirmed by ISFH CalTeC. [ABSTRACT FROM AUTHOR]

This article examines the dual impact of artificial intelligence (Al) on the EU's economic security, focusing on its opportunities and threats: While Al contributes to economic growth and stability, it also creates new vulnerabilities. The paper discusses the role of Al in fighting economic crime, improving risk management, and fostering innovation. At the same time, it also highlights the risks of Al misuse in cyberattacks and manipulations, as well as potential losses for businesses. The report's conclusions emphasize the need for international cooperation and national cybersecurity strategies to effectively use Al and overcome potential challenges. [ABSTRACT FROM AUTHOR]

In this work, the effect of high-entropy alloy powder preparation on the coatings deposited via high-velocity oxygen fuel sprayings was studied. The powders of FeNiCoCrMo0.5Al1.3 composition were prepared by milling and gas atomization. The structures, porosity, phase composition, and microhardness of the coatings produced from mechanically alloyed and gas-atomized powders were compared. The influence of milling parameters on the powder phase composition and morphology was studied. Milling at 600 rpm for 1.5 h allowed the production of mechanically alloyed powder with a homogeneous distribution of Fe, Ni, and Al and thin lamellas enriched with Co, Cr, and Mo. Despite the difference in the feedstock powders' phase compositions, the phase compositions of the coatings deposited from mechanically alloyed and gas-atomized powders are the same consisting of BCC, FCC solutions, and oxide. The amount of FCC solutions and oxide in the coating depends on the size distribution of the sprayed powder. It was found that the phase composition and the properties of the coatings deposited from the mechanically alloyed and gas-atomized powders of similar sizes are similar. [ABSTRACT FROM AUTHOR]

Background: Previous studies have claimed that pharyngeal teeth in medaka (Oryzias latipes) are induced independent of retinoic acid (RA) signaling, unlike in zebrafish (Danio rerio). In zebrafish, pharyngeal tooth formation depends on a proper physical contact between the embryonic endodermal pouch anterior to the site of tooth formation, and the adjacent ectodermal cleft, an RA‐dependent process. Here, we test the hypothesis that a proper pouch–cleft contact is required for pharyngeal tooth formation in embryonic medaka, as it is in zebrafish. We used 4‐[diethylamino]benzaldehyde (DEAB) to pharmacologically inhibit RA production, and thus pouch–cleft contacts, in experiments strictly controlled in time, and analyzed these using high‐resolution imaging. Results: Pharyngeal teeth in medaka were present only when the corresponding anterior pouch had reached the ectoderm (i.e., a physical pouch–cleft contact established), similar to the situation in zebrafish. Oral teeth were present even when the treatment started approximately 4 days before normal oral tooth appearance. Conclusions: RA dependency for pharyngeal tooth formation is not different between zebrafish and medaka. We propose that the differential response to DEAB of oral versus pharyngeal teeth in medaka could be ascribed to the distinct germ layer origin of the epithelia involved in tooth formation in these two regions. Key Findings: Pharyngeal tooth formation in medaka depends on the retinoic acid (RA)‐controlled establishment of physical pouch‐cleft contacts.Retinoic acid sensitivity for pharyngeal tooth formation is conserved across teleost fish.Differential response to RA inhibition of oral versus pharyngeal teeth in medaka could be ascribed to the distinct germ layer origin of the epithelia involved. [ABSTRACT FROM AUTHOR]

The coordination environment of magneto-luminescent Dy3+-based Single-Molecule Magnets (SMM) is a crucial factor influencing both magnetic and luminescent properties. In this work, we explore how triphenylmethanolate (Ph3CO−), in combination with other ligands, can modulate the structure and, therefore, the magnetic properties of Dy3+-based SMM. Using triphenylmethanolate in combination with THF and pyridine (Py) as co-ligands, we synthesized a series of mononuclear cis-[Dy(OCPh3)2(THF)4][BPh4]·(2,6-Me2C5H3N) (1), trans-Dy(OCPh3)3(THF)2 (2), fac-Dy(OCPh3)3(py)3 (3) and dinuclear [(Ph3CO)Dy(THF){(μ2–Cl)2Li(THF)2}μ2–Cl]2 (4) complexes where the Dy3+ ion presents five- or six-coordinate geometries. Dinuclear compound 4 exhibits a genuine SMM behavior with a relatively high energy barrier of 421 cm−1, while mononuclear complexes 1–3 are field-induced SMM. These complexes also present Dy3+-characteristic luminescence, highlighting their multifunctional character. [ABSTRACT FROM AUTHOR]

Brain-derived neurotrophic factor (BDNF) is a secreted molecule that plays an important role in the survival and growth of nerve cells. BDNF undergoes complex post-translational processing with cellular proteases. Pro- and mature BDNF forms bind to different receptor types in the brain. BDNF is prominent in the neonatal cerebral cortex. The neonatal period is critical for the proper development of the brain and nervous system. Disruptions in these critical periods can have long-lasting effects on behavior and mental health. Individuals who experience adverse effects in the neonatal period have demonstrated a predisposition to depression and other neurobehavioral disorders. In this work we studied the influence of transient expression (P3–P8) elevation of pro-, mature and mutant forms of BDNF that could not be processed with cellular convertases in the neonatal medial prefrontal cortex (mPFC) on anxiety and depressive-like behavior in adolescents. Elevated expression of mature BDNF (LV-BDNF) increased anxiety and depressive-like behavior at P30. Only immobility in the tail suspension test was increased after expression of mutant BDNF (LV-pBDNF mut). Using our RNA-seq data and available online sn-RNAseq results, we investigated transcriptomic changes in the neonatal mPFC at P8 that underlie subsequent behavioral changes. Mature BDNF expression caused an increased transcriptional response in perivascular stromal cells (PSC) with such genes: Ptgds, Slc6a13, Slc22a6, Bnc2, Slc13a4, Aldh1a2. Based on GWAS data, Ptgds is a candidate gene associated with ADHD and bipolar disorder Pujol-Gualdo et al. (2021); Marín-Méndez et al. (2012); Munkholm et al. (2015). LV-pBDNF mut caused a complete opposite set of transcriptional changes in the PSC compared to LV-BDNF. The observed similar behavioral phenotype after expression of mature and mutant forms of BDNF together with the detected genes related to bipolar disorder underpinned that Bdnf could play a substantial role in the pathogenesis of this neurobehavioral disorder. [ABSTRACT FROM AUTHOR]

Due to its peculiar and highly variable nature, the blazar 3C 454.3 has been extensively monitored by the WEBT team. Here, we present for the first time these long-term optical flux and color variability results using data acquired in B , V , R , and I bands over a time span of about two decades. We include data from WEBT collaborators and public archives such as SMARTS, Steward Observatory, and Zwicky Transient Facility. The data are binned and segmented to study the source over this long term when more regular sampling was available. During our study, the long-term spectral variability reveals a redder-when-brighter trend, which, however, stabilizes at a particular brightness cutoff of ∼14.5 mag in the I band, after which it saturates and evolves into a complex state. This trend indicates increasing dominance of jet emission over accretion disk (AD) emission until jet emission completely dominates. Plots of the variation in spectral index (following F _ν ∝ ν ^− ^α ) reveal a bimodal distribution using a one-day binning. These correlate with two extreme phases of 3C 454.3, an outburst or high-flux state and a quiescent or low-flux state, which are respectively jet- and AD-dominated. We have also conducted intraday variability studies of nine light curves and found that six of them are variable. Discrete correlation function analysis between different pairs of optical wave bands peaks at zero lags, indicating cospatial emission in different optical bands.