СТРУКТУРА И ФАЗОВЫЙ СОСТАВ МНОГОСЛОЙНЫХ РЕНТГЕНОВСКИХ ЗЕРКАЛ W-Si

Методами рентгеновской дифрактометрии в жесткой области (l~0,154 нм) исследована фазовая структура, состав и строение многослойных рентгеновских зеркал (МРЗ) W/Si с толщиной слоев вольфрама tW2,7 нм слои вольфрама имеют поликристаллическую (ОЦК) структуру, а при tW<1,9 нм они аморфны. При помощи sin2Y-метода установлено, что в тонких кристаллических слоях вольфрама (tW<10 нм) может содержаться более 3 ат.% Si. Растягивающие напряжения в слоях кристаллического вольфрама не превышают 1,1 ГПа. Построение функций радиального распределения атомов позволило установить, что аморфные слои вольфрама имеют расположение атомов, близкое к b-W. Во всех образцах за счет взаимодействия на межфазных границах наблюдается формирование силицидных прослоек, в результате чего реальная толщина слоев вольфрама меньше номинальной. Аморфные силицидные прослойки, обязательно формирующиеся на стадии изготовления МРЗ, содержат дисилицид вольфрама. В зависимости от скорости осаждения дисилицид может иметь расположение атомов, близкое либо к тетрагональной фазе, t-WSi2 (~0,6 нм/с.), либо к гексагональной фазе, h-WSi2 (~0,15 нм/с.). Представлена уточненная модель строения аморфных МРЗ W/Si. Предложены механизмы формирования силицидных прослоек, согласно которым нижние силицидные прослойки (W-на-Si) формируются преимущественно за счет баллистического перемешивания атомов вольфрама и кремния, а верхние – вследствие диффузионного перемешивания. Сделана оценка коэффициентов взаимной диффузии, которые позволили установить, что осаждаемая поверхность слоев может быть разогрета, по меньшей мере, на 250° выше температуры подложки. Предложены пути снижения межфазного взаимодействия

Activated phosphoinositide 3-kinase δ syndrome: Update from the ESID Registry and comparison with other autoimmune-lymphoproliferative inborn errors of immunity

Background: Activated phosphoinositide-3-kinase d syndrome (APDS) is an inborn error of immunity (IEI) with infection susceptibility and immune dysregulation, clinically overlapping with other conditions. Management depends on disease evolution, but predictors of severe disease are lacking. Objectives: This study sought to report the extended spectrum of disease manifestations in APDS1 versus APDS2; compare these to CTLA4 deficiency, NFKB1 deficiency, and STAT3 gain of-function (GOF) disease; and identify predictors of severity in APDS. Methods: Data was collected from the ESID (European Society for Immunodeficiencies)-APDS registry and was compared with published cohorts of the other IEIs. Results: The analysis of 170 patients with APDS outlines high penetrance and early onset of APDS compared to the other IEIs. The large clinical heterogeneity even in individuals with the same PIK3CD variant E1021K illustrates how poorly the genotype predicts the disease phenotype and course. The high clinical overlap between APDS and the other investigated IEIs suggests relevant pathophysiological convergence of the affected pathways. Preferentially affected organ systems indicate specific pathophysiology: bronchiectasis is typical of APDS1; interstitial lung disease and enteropathy are more common in STAT3 GOF and CTLA4 deficiency. Endocrinopathies are most frequent in STAT3 GOF, but growth impairment is also common, particularly in APDS2. Early clinical presentation is a risk factor for severe disease in APDS. Conclusions: APDS illustrates how a single genetic variant can result in a diverse autoimmune-lymphoproliferative phenotype. Overlap with other IEIs is substantial. Some specific features distinguish APDS1 from APDS2. Early onset is a risk factor for severe disease course calling for specific treatment studies in younger patients. (J Allergy Clin Immunol 2023;152:984-96.

Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries

The Directions of Improving the Management Efficiency of Financial-Economic Security of Industrial Complex of Region

The publication substantiates the directions of improving the management efficiency of financial-economic security of industrial complex of region. It is indicated that efficiency of the Ukrainian economy depends to a large extent on the level of industry development in the regions, which today are marked by significant sectoral, territorial, and functional differentiation. The existence of a close relationship between the actors of financial relations in the system of financial-economic security is proved. Factors of financial-economic security of industrial complex of region are allocated and divided into several groups. The general ways of overcoming problems of financial-economic security of regions together with measures on increase of efficiency of use of own economic potential are allocated. It is indicated that the main importance of the system of financial-economic security of industrial complex of region should consist in its preventive nature. Key objectives of the financial-economic security management of industrial complex of region are defined

The social risk forecast indicator of the road traffic accidents in the territory of Khabarovsk and the Khabarovskiy krai

The paper analyzes the road traffic safety in the territory of Khabarovsk in 2011-2020 according to the following indicators: the severity of the accidents, the severity of the consequences, the social risk, the forecast indicator of the social risk in the city of Khabarovsk by 2024. The analysis is based on the exponential smoothing method using the statistical data of 2011-2020. The forecast indicator of the social risk is compared to the value established by the Road Safety Strategy in the Russian Federation for 2018 - 2024, as a target by 2024. An assessment of achieving the mortality rate reduction in the Khabarovskiy krai is also given. The impact of the pandemic consequences and the socio-economic situation on reducing the road accidents mortality are examined. The objective data on the costs increasing to support the population and business being evaluated, the target values are set by the national project in 2020 and the subsequent years. For the analysis, the empirical methods are used, such as: examining the results of the previous activities; the expert assessments; the methods of studying an object in time: retrospective, forecasting. The socio-economic factors are considered the most susceptible to reduce the traffic accidents mortality

Investigation of structure formation and tribotechnical properties of steel plasma coatings after chemical-heat treatment and liquid-phase impregnation

The paper is focused on the studies of the microstructure development and physical and mechanical properties of metal-matrix composite coatings based on steel 11Cr18MoWCu deposited using plasma and galvanoplasma methods. The expediency of combining gas-thermal spraying processes of plasma coatings with open porosity up to 16%–18%, with their subsequent thermodiffusion saturation (chromium plating) or liquid-phase impregnation with eutectic alloys of previously applied Ni–B galvanic layer, is shown. The study of the tribotechnical properties of the proposed coatings showed a significant improvement in their performance under conditions of various types of intensive wear, as well as in corrosive environments

VRN1 genes variability in tetraploid wheat species with a spring growth habit

Abstract Background Vernalization genes VRN1 play a major role in the transition from vegetative to reproductive growth in wheat. In di-, tetra- and hexaploid wheats the presence of a dominant allele of at least one VRN1 gene homologue (Vrn-A1, Vrn-B1, Vrn-G1 or Vrn-D1) determines the spring growth habit. Allelic variation between the Vrn-1 and vrn-1 alleles relies on mutations in the promoter region or the first intron. The origin and variability of the dominant VRN1 alleles, determining the spring growth habit in tetraploid wheat species have been poorly studied. Results Here we analyzed the growth habit of 228 tetraploid wheat species accessions and 25 % of them were spring type. We analyzed the promoter and first intron regions of VRN1 genes in 57 spring accessions of tetraploid wheats. The spring growth habit of most studied spring accessions was determined by previously identified dominant alleles of VRN1 genes. Genetic experiments proof the dominant inheritance of Vrn-A1d allele which was widely distributed across the accessions of Triticum dicoccoides. Two novel alleles were discovered and designated as Vrn-A1b.7 and Vrn-B1dic. Vrn-A1b.7 had deletions of 20 bp located 137 bp upstream of the start codon and mutations within the VRN-box when compared to the recessive allele of vrn-A1. So far the Vrn-A1d allele was identified only in spring accessions of the T. dicoccoides and T. turgidum species. Vrn-B1dic was identified in T. dicoccoides IG46225 and had 11 % sequence dissimilarity in comparison to the promoter of vrn-B1. The presence of Vrn-A1b.7 and Vrn-B1dic alleles is a predicted cause of the spring growth habit of studied accessions of tetraploid species. Three spring accessions T. aethiopicum K-19059, T. turanicum K-31693 and T. turgidum cv. Blancal possess recessive alleles of both VRN-A1 and VRN-B1 genes. Further investigations are required to determine the source of spring growth habit of these accessions. Conclusions New allelic variants of the VRN-A1 and VRN-B1 genes were identified in spring accessions of tetraploid wheats. The origin and evolution of VRN-A1 alleles in di- and tetraploid wheat species was discussed

Control of the direction of energy transfer in associates of colloidal quantum dots Ag2S/TGA and dye molecules

The possibilities for changing the direction of energy transfer in hybrid associates of colloidal Ag2S quantum dots (QDs) and thionine molecules are analyzed. The studies were performed by transmission electron microscopy, absorption and luminescence spectroscopy, PL decay study (time correlated single photon counting). An increasing of the average QDs size from 1.8 nm to 5.5 nm, and also a shift of the luminescence band from 630 nm to 950 nm, were found at using of TGA molecules and sodium sulfide as a sulfur precursor. Hybrid association of QDs (1.8 nm) with TH+ molecules leads to quenching of QDs luminescence with a simultaneous reduction of the luminescence lifetime from 13.7 to 6.5 ns. An association of QDs with a luminescence band maximum of 950 nm with TH+ molecules leads to quenching of TH+ luminescence and a reduction in its lifetime of luminescence from 0.43 to 0.3 ns. It was concluded that the reduction of lifetime of luminescence caused by the resonant nonradiative energy transfer between the components of the associates. An increasing in the average size of QDs leads to a change in the direction of energy transfer between the components of the associates