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Background. The participation of immune responses in the pathogenesis of degenerative-dystrophic diseases of the synovial joints determines the feasibility of using more rational approaches to assessing the immune status of patients with this pathology. Despite the ongoing immunological studies in patients with degenerative-dystrophic diseases of the hip joint, there are questions related to the functioning of the immune system as a whole, which require additional research.The aim of the study was to establish the features of the relationship between the indicators of the immune status and to identify the factors that determine the nature and severity of immunological disorders in patients with coxarthrosis.Material and methods. The study is based on the results of immunological parameters in 73 patients (mean age – 45.3 ± 2.56 years) with stage III dysplastic coxarthrosis. The data obtained using cluster analysis made it possible to distribute patients into two groups. The first clinical group included 46 patients, the second one – 27 patients. The control group consisted of 28 clinically healthy individuals. Mathematical processing of the results was carried out using the Statistica 6.0 software package (StatSoft Inc., USA) and the mathematical package Mathcad 2001 Pro (MathSoft Inc., USA).Results. The levels of CD4+ and CD8+ cells and IgA were determined as the classification features that make the greatest contribution to the division of the studied groups. Model experiments have shown that a specific combination of the magnitude of each of the noted factors with a certain degree of probability makes it possible to assess the state of the initial immunological reactivity and verify the nature and severity of immunological disorders.Conclusion. The information obtained is of great importance, since it allows predicting the course of the recovery period after surgery and assessing the risk of developing possible postoperative complications in patients with severe articular pathology.

The paper is devoted to a detailed study of cathodic processes, their influence on the anode process, and electrolysis performance. The polarization of a steel cathode in a CaCl2–BaCl2–NaCl melt at t = 610 °C was measured. The polarization curve clearly shows the potentials and current densities of the formation of a saturated sodium solution in the electrolyte (Esat = –2.97 V, ic = 0.04 A/cm2, lgic = –1,4), and the occurrence of sodium metal on the cathode (ENa = –3.22 V, iNa = 0.12 A/cm2, lgiNa = –0.92).The value of Esat was used to calculate the concentration of sodium in the electrolyte at t = 610 °С (1.3·10–4 mol. fr.). The values of Esat, ENa, and their difference (E = 0,25 В) were confirmed by long-term electrolysis. These fundamental characteristics are the basis for process control and management. During long-ter 3 regions close to rectilinear ones were revealed: the discharge of sodium ions from supersaturated solutions at E more negative than Esat (from ENa to Esat), from mixtures of supersaturated and saturated solutions (at a constant E equal to Esat), from diluted solutions (with E more positive than Esat). The activity coefficients of sodium in supersaturated solutions are close to 1, which ensures their increased reducing ability. Maximum degrees of supersaturation (>100) are created at formation and decomposition on the cathode of metallic sodium nuclei, which are sufficient to intensify and prolong electrolysis, to lower the lower temperature limit of its realization from 600 to 350 °С. The formation of metallic titanium in the near-anode layer is explained by the disproportionation of Ti2+ ions entering the near-anode electrolyte from the anode surface and from the near-cathode melt.

Introduction. Down syndrome (DS) is one of the most common chromosomal abnormalities. Children with DS have an increased risk of developing acute lymphoblastic leukemia (ALL). Standard therapy is usually used to treat ALL in children with Down syndrome, but the outcome is worse than in the general population. The high toxicity of therapy is a particular problem.The purpose of the study – in this study we presents a comparative analysis of the results of therapy for children with DS and ALL (DS-ALL) who received therapy according to the ALL-MB 2008 and ALL-MB 2015 protocols.Materials and methods. The analysis included primary ALL patients, aged 1 to 18 years, who received therapy in Russian and Belarusian clinics participating in the Moscow–Berlin study from January 2008 to December 2020. To analyze the treatment results of DS-ALL patients, a “comparison group” was formed from all patients with ALL registered in the database, using the matched-pair method. Survival was calculated using the Kaplan–Meier method, toxicity analysis and clinical-genetic parameters were investigated using nonparametric statistical methods.Results. The results of therapy both among patients with DS-ALL who received therapy according to ALL-MB 2008 and ALL-MB 2015 in comparison with “sporadic” ALL (non-DS-ALL) are unsatisfactory. The event-free survival rate of patients with DS-ALL in the ALL-MB 2008 group was 61 ± 7 % versus 85 ± 4 % among non-DS-ALL (p = 0.001), in the ALL-MB 2015 group – 67 ± 7 % versus 84 ± 4 % respectively. Overall survival in the ALL-MB 2008 group was 70 ± 7 % in children with DS versus 88 ± 4 % in non-DS (p < 0.001), in the ALL-MB 2015 group – 78 ± 6 % versus 92 ± 3 % respectively (p < 0.001). The risk of therapy-related death was higher in patients with DS: 20.6 ± 6.1 % versus 4.6 ± 2.2 %; p < 0.001 in the ALL-MB 2008 group and 18 ± 4.1 % versus 3.3 ± 1.3 %; p < 0.001 in the ALL-MB 2015 group, without a significant increase in the risk of relapse. The effectiveness of induction therapy among patients with DS treated according to ALL-MB 2008 versus children with DS-ALL treated according to ALL-MB 2015 was 80 % versus 92 % respectively (p = 0.018). The probability of achieving continuous complete remission was also lower in the ALL-MB 2008 group compared to ALL-MB 2015 – 57 % versus 75 %; p < 0.001 respectively. Thus, the results of treatment of DS-ALL according to the ALL-MB 2015 protocol were better than those according to the ALL-MB 2008.Conclusion. The results of therapy for patients with DS-ALL are still unsatisfactory today, this circumstance dictates the need for new approaches to optimize therapy. The main problem for these patients remains the high toxicity of therapy and the associated lethality. Further progress in the treatment of DS-ALL may be associated with the development of new approaches to concomitant therapy, the use of molecular-targeted drugs and immunotherapy, as well as with the study of the molecular genetic characteristics of this subgroup of patients.

This study presents the clinical and laboratory data of 50 patients with ataxia-telangioectasia syndrome (AT) (Louis-Bar syndrome) treated at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology (Moscow, Russia) between 2012 and 2021. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. We found that the patients experienced a significant diagnostic delay (the median delay in diagnosis was 4.5 years), although the first typical symptoms of AT were present at an earlier age (the median age was 1.5 years). The majority of patients showed laboratory signs of immunodeficiency, yet only 24% of the children developed severe infections. However, lung infections resulted in bronchiectasis in 16% of the patients and were the cause of death in 4/10 cases. Fifty-two percent of the patients had autoimmune complications, including interstitial lung disease and skin granulomas, and 24% of the patients developed malignant neoplasms. Of patients who underwent testing, 85% had KREC and/or TREC levels below the cutoff values used for neonatal screening of primary immunodeficiency disorders in Russia, which suggests that the majority of AT cases could be diagnosed by neonatal screening. Early diagnosis, multidisciplinary approach and high clinical suspicion for neoplastic manifestations are crucial for the successful management of AT.

Over the two years that have passed since the WHO announced on March 11, 2020, a pandemic of the new coronavirus disease COVID-19, more than 460 million cases of the disease have been detected in the world, of which more than five million have been fatal. During the natural evolution of the COVID-19 pathogen, dominant variants emerge that account for most new infections. The WHO constantly monitors coronavirus mutations that potentially pose an epidemiological danger. Currently, the WHO divides modified variants of the SARS-CoV-2 virus into

Purpose. To carry out marking of existing deposits of brown coal in accordance with domestic and international classifications. To give a comparative assessment of the distribution of balance reserves of brown coal in accordance with different classification systems. Methodology. Reserves and quality indicators data for brown coal in Ukraine were taken from the site of the State Research and Production Enterprise State Information Geological Fund of Ukraine and on the basis of our own research. Some quality indicators were calculated according to the formulas given in the periodical scientific literature. Ukraines brown coal reserves were estimated using Ukrainian and international brown coal classifications. Findings. It is confirmed that the balance reserves of brown coal in Ukraine are located in Dnipropetrovsk, Zhytomyr, Zakarpattia, Kirovohrad, Kharkiv and Cherkasy regions and are (thousand tons): categories A + B + C1 2,593,359; category C2 299,181. Among the existing classifications, the most suitable for the brown coal distribution in Ukraine is the International Code System of Brown Coal, which is regulated in ISO 2950:1974 Brown coal and lignites Classification by types on the basis of total moisture content and tar yield. Originality. For the first time, a range of changes in brown coal quality indicators in Ukraine has been established, and its reserves have been marked using Ukrainian (DSTU 3472:2015) and international (ISO 2950:1974; International Classification of Coal in Reservoirs; ISO 11760:2005; ASTM D38812) classifications. Comparative assessment of coal reserves will allow choosing the optimal ways of their processing, depending on the predicted properties of raw materials. Practical value. The reserves of brown coal deposits by regions and the degree of industrial development up to 01.01.2021 have been described, and the production of brown coal from 2011 to 2020 has been shown. Determining the quality of brown coal deposits in Ukraine allows us to develop the most effective methods for using the coal.

The work reports implementation of the “COVID-19.rf: information against the pandemic” project.Since 2020, dozens of projects have been implemented to create collections of national COVID-19 publications and populate national COVID-repository platforms.The Russian project “COVID-19.rf: information against the pandemic” is among them. The following tasks were realized during the development of the COVID-19.rf repository: the program software to create and populate the project platform; automated data exchange with previously created Russian platforms; monitoring global citation indexes: Web of Science, Scopus, Dimensions and the Lens and automatic population of the created platform: possibility for access and downloading the project data via API under the OAI PMH protocol; project advancement and user training; indexing of the project content on the international integrator platforms: CORE, OpenAire: COVID-19 Open Research Gateway and Google Academy.As a result, algorithms and technical implementation of automatically replenishing of repositories within the legal framework of publishers’ license have been developed.By the beginning of 2022, metadata about 7380 Russian COVID publications were uploaded to the project platform, 3923 articles being in full text. These indicators put the Russian project on a par with the largest national projects such as of the Great Britain, Spain and Brazil.

Ataxia-telangiectasia (АТ) represents a typical syndromic form of primary immunodeficiencies. Besides progressive neurological features, patients with AT have a high risk of malignancies, as well as autoimmune complications, with interstitial lung disease being one of the most frequent. AT is also characterized by a significant diagnostic delay, which was demonstrated in our case report. The patients’ parents gave their consent to the use of their children’s data, including photographs, for research purposes and in publications.

SUMMARY We present new radiometric ages and full-vector palaeomagnetic data from São Vicente Island, Cape Verde. Samples were obtained from a sequence of 11 flows. A K-Ar age determination was performed on the lowermost flow of the sequence, yielding an upper Miocene age of 6.07 ± 0.16 (2σ) Ma. Considering the normal polarity observed in all flows, the age of the sequence could be narrowed down to an interval between 6.03 and 6.23 Ma. The mean palaeomagnetic direction of the studied sequence was compared with the expected direction, calculated from the African Apparent Polar Wander Path (5 Ma). While inclinations approximately agree, the palaeodeclination appears westwardly deviated by 26.5° ± 2.9°. Moreover, nearly no scatter of virtual geomagnetic poles around their mean value is observed and its comparison with the scatter observed in palaeomagnetic directions obtained from geomagnetic field model SHA.DIF.14k suggests that the sequence was very rapidly emplaced in only a few centuries. Thus, the obtained anomalous palaeomagnetic direction seems to reflect the effect of non-averaged secular variation, although a local vertical-axis counter-clockwise rotation of the sequence, cannot be entirely ruled out. Thellier–Coe palaeointensity experiments on 66 specimens yielded 22 successful determinations and multispecimen (MS) palaeointensity experiments on 11 samples yielded 8 successful determinations. In the upper part of the sequence, Thellier–Coe determinations fail due to the presence of multidomain (MD) grains, but the MS method yields successful results in several of these flows, demonstrating that this method can provide reliable palaeointensity determinations on samples with a higher MD grain fraction. A mean palaeointensity BSV = (18.5 ± 1.9) µT is obtained from the joint analysis of the results from both methods. This value lies clearly below the present-day value BP = 33.1 μT. The virtual axial dipole moment (VADM = 4.3 × 1022 Am2), however, cannot be considered anomalous if compared to available upper Miocene–Pliocene data and represents a new high-reliability contribution to the very limited African palaeointensity database.

Abstract In this paper, we consider disturbances in the airglow of the Earth’s upper atmosphere caused by a launch of the Yamal-601 satellite on May 30, 2019. For this purpose, we used the data acquired with an optical system of the Institute of Solar−Terrestrial Physics, Siberian Branch, Russian Academy of Sciences (ISTP SB RAS). The measurements were carried out at the Geophysical Observatory (GPO) of the ISTP SB RAS (~52° N, ~103° E) with a complex of optical instruments—a whole-sky camera, a spectrograph, and a Fabry–Pérot interferometer. According to the data of the KЕО Sentinel camera, an extensive area of airglow was observed along the flightpath; it appeared approximately 2−4 min after the spacecraft’s passage over the GPO and existed for ~20 min. A transversal scale of the spacecraft’s flightpath is estimated approximately as 95–110 km if the spontaneous-emission altitude is assumed to be at ~150 km (and it is about 190–220 km if the spontaneous-emission altitude is ~300 km). We discuss the mechanisms that may result in forming the observed area of airglow, including the mechanisms connected with the physical and chemical interaction of fuel products with atmospheric components and the influence of propagation of a shock wave or short-period internal gravitational waves. Probable causes of the delay observed in appearing the airglow after the spacecraft’s flyby are analyzed.

There are only limited data coming from isolated case reports regarding the real-world use of emicizumab for the treatment of children with hemophilia A and inhibitors (HAI) in Russia. The aim of the study was to evaluate the efficacy and safety of emicizumab prophylaxis in children with severe HAI. Ethical approval was not required since the study only involved the use of anonymized and generalized retrospective data obtained during routine clinical practice. We retrospectively analyzed medical records of children with HAI who had been treated with emicizumab at 11 institutions located in Russia, taking into consideration such parameters as annualized bleeding rates (ABR), annualized spontaneous bleeding rates (ASBR), annualized joint bleeding rates (AJBR) and annualized bleeding rates for bleeds requiring additional therapy (ABRRT), as well as the presence and severity of adverse events during the treatment. The median age of patients at the time of initiation of emicizumab prophylaxis was 65 (11–170) months. Before the treatment, ABR was 19.9 (95% confidence interval (CI), 15.4–26.1), ASBR – 13.6 (95% CI, 10.6–17.8), AJBR – 6.6 (95% CI, 4.7–9.7), ABRRT – 16.6 (95% CI, 12.4–22.7). After the initiation of the treatment, bleeding rates changed dramatically: ABR decreased by 98.6% (95% CI, 96.7–99.4), AJBR – by 99.4% (95% CI, 95.3–99.9), ABRRT – by 98.8% (95% CI, 96.8–99.6); and there were no signs of spontaneous bleeding during 10 (1–32) months of treatment. No adverse events leading to the interruption or discontinuation of the treatment with emicizumab were reported. The use of emicizumab in children with HAI in the real-world clinical setting results in a significant (> 98%) and safe reduction in bleeding episodes without any signs of spontaneous bleeding.

The research is intended to develop a technology for the production of finely dispersed (10 to 100 μm) powders of titanium and its alloys suitable for use in additive technologies after classification and spheroidization. A eutectic mixture was used as electrolyte, mole fractions: BaCl2 – 0.16, CaCl2 – 0.47, NaCl – 0.37, melting point of 452 °C. Electrolytes with a similar composition are used in industry for the electrolytic production of sodium with high current efficiency. No titanium salts were added to electrolyte. Sodium losses due to evaporation, corrosion, and ion recharge were replenished by a periodic increase in electrolysis current. A VT1-0 titanium plate was used as an anode. The walls of a steel crucible served as a cathode. Sodium was released on these walls and dissolved in electrolyte. Titanium ions were reduced in the bulk of electrolyte and in the anode layer. It is the first time that the results obtained were interpreted using the data on the electrode potentials of Ti3+/Ti, Ti2+/Ti, Ti3+/Ti2+ systems. It was shown that the concentration of slowly moving complex Ti3+ ions increases in the anode layer, and sodium dissolved in electrolyte reduces mainly Ti2+ ions in the electrolyte volume in the first 12 min of electrolysis. Starting from the 20th min, the concentration of Ti2+ ions in the anode layer begins to increase rapidly according to the reaction: 2Ti3+ + Ti = 3Ti2+ as titanium powder accumulates in the electrolyte volume. At the same time, the proportion of sodium consumed for the reduction of Ti3+ ions to Ti2+ decreases, which contributes to an increase in current efficiency and cathode potential stabilization for 30 minutes at –2.963 V. After the 50th min, the reactivity of the salt melt begins to decrease, the concentration of Ti3+ ions increases steadily until it levels off with the concentration of Ti2+ ions at the 85th min. This sharply increased the current consumption for ion recharge and made it necessary to stop electrolysis after switching on a current of 12 A for a short time (for 40 s). After 10 s, judging by the change in the cathode potential, sodium dissolved in electrolyte was almost completely consumed for titanium ion reduction. After 6 min, the potentials of electrodes returned to the initial anode potential value indicating that the system returned to its original state with the near-zero content of titanium salts and dissolved sodium. 95 % of powder was obtained in the electrolyte volume. Current efficiency was 84.0 % and turned out to be close to the value calculated from the average valence of titanium ions and the loss of anode weight (87.0 %). After ultrasonic dispersion, more than 80 % of powder was in the 10–100 μm range with a maximum at 36 μm. X-ray phase analysis showed that this is practically pure α-titanium (93.06 %) and oxygenated α-titanium (5.45 %). The originality of the research consists in the use of a volumetric, intensive, electrolytic method for producing finely dispersed titanium powders with no dissolved sodium and titanium chlorides in the initial and final electrolytes, in a stepwise increase in the current and potentiometric process control. The uniqueness of the research consists in the titanium powder obtained where the major part is in the melt volume in the form of intergrowths that are easily crushed by ultrasonic dispersion into individual crystals. Over 80 % of these crystals were in the range of 10–100 μm required for additive technologies with an average size of 36 μm.

Purpose. To determine and evaluate technogenic risks within the water purification systems of TSs and TPSs during normal operation in terms of electrocoagulation plant. Methodology. It is proposed to apply a fault tree method for the analysis of various operation failures of water purification facilities in the heat power industry. Additional analysis method, applied at stages one and two of technogenic risk determination, is suggested for its use. The method is based upon the construction of matrix combining states of the system elements. Findings. The aggregation of the combination matrix and fault tree method makes it possible to derive a new grapho-analytical procedure to analyse probabilities of technogenic risk initiation in the context of any water purification system operation both at the stage of its design and at the stage of its work. Originality. Non-routine operation of a water purification system may depend upon certain internal reasons as well the external ones. The reasons pose risks to a situation that at the output of the system, water will turn out being insufficiently purified. It has been identified that in terms of the non-routine operation of water purification system, risk probability is worth analysing with the help of the fault tree serving as graphical representation of causal relationships obtained while considering dangerous situations in reverse order to determine probabilities for their initiation. Practical value. The procedure helps obtain quantitative, qualitative, and causal-consequential indicatorsfacilitating control of technogenic risk initiation in water purification systems. Software has been developed to calculate rapidly the probabilities of running of block elements or water purification system elements in an operation mode or in a failure mode, and see clearly the poorest combinations in terms of an electrocoagulation water purification system.