Репозиторий Университета

© 2018, © 2018 Informa UK Limited, trading as Taylor  &  Francis Group. Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study. Results of study showed high risk of life-threatening complications during pregnancy and delivery, especially the risk of hemorrhage and cardiovascular complications. In all the patients, we observed the progression of bleeding or development of bleeding in new localizations (epistaxis in 27 patients, easy brushing in 22, skin and mucosa telangiectasia in 20, gastrointestinal bleedings in 4, hemoptysis in 4, hematomas for minor traumas in 14, conjunctivas hemorrhages in 5). Conclusion: The pathogenesis of bleeding in such patients has mixed pattern: besides vascular wall pathology coagulation deficiency plays some role. The preferred delivery method for such patients is caesarean section. Deep vaginal ruptures and serious hemorrhage accompany vaginal delivery.

© 2019 Most researchers associate the increase in the permeability of lipid bilayers of artificial and biological membranes observed in various experiments with the formation of hypothetical hydrophobic and hydrophilic pores. Although the existence of hydrophobic defects, as the first stage of the formation of a hydrophilic pore, was hypothesized decades ago from electroporation experiments, the difficulty of describing this stage is determined by the lack of experimental data confirming the existence or at least associated with hydrophobic pores. We explored the increase in the current variance through the lipid membrane, observed when approaching the phase transition from the side of high temperatures, and have associated it with capacitive currents arising in response to the formation of hydrophobic pores. Assuming that the number of hydrophobic pores in a membrane follows a Poisson distribution, and thus, the mean number of hydrophobic pores is equal to the variance of that number, we used the measurements of the membrane current variance to evaluate the number of hydrophobic pores. Analysis of experimental data within this model allows us to estimate the number of hydrophobic pores at or above the phase transition and shows that the number of hydrophobic pores in a membrane close to the phase transition increased 20 times compared to the number of hydrophobic pores existing in the membrane far from the melting transition.

© 2019 Elsevier B.V. Fabry disease (FD [MIM:301500]) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficient activity of its product, lysosomal enzyme α-galactosidase A (α-Gal A), leads to excessive accumulation of glycosphingolipids in cells of multiple organs. The establishing of the diagnosis is challenge in female patients because of milder clinical manifestation and normal α-Gal A activity. The globotriaosylsphingosine (lysoGb3) is described as a more sensitive diagnostic biomarker for females with pathogenic mutation in the GLA gene. Thus, the aim of this study is to improve the biochemical diagnostic efficiency for FD in females. Here we report the α-Gal A/lysoGb3 ratio as the novel biochemical criteria for diagnosis of female patients with FD, using dried blood spots (DBS) as test samples. It showed 100% sensitivity in distinguishing our group of 35 female patients from control (n = 140). Whereas measurement of α-Gal A and lysoGb3 alone showed 8.6% and 74.4% respectively. A new approach of using the ratio of α-Gal A activity to lysoGb3 concentration in DBS may provide a more accurate screening tool for identification of FD females.

© 2019 Elsevier B.V. A high-performance thin-layer chromatography (HPTLC) method combined with effect-directed-analysis (EDA) was developed to screen the antioxidant, neuroprotective and antidiabetic effects in essential oils derived from lavender flower, lemon myrtle, oregano, peppermint, sage, and rosemary leaves (Lamiaceae family). HPTLC hyphenated with microchemical (DPPH•, p-anisaldehyde, and ferric chloride) derivatizations, was used to evaluate antioxidant activity, presence of phytosterols and terpenoids, and polyphenolic content, while the combination with biochemical (α-amylase and acetylcholine esterase (AChE) enzymatic) derivatizations was used to asses α-amylase and AChE inhibitory activities. The superior antioxidant activity of oregano leaf extract is attributed to the presence of high levels of aromatic compounds, like polyphenolic acids. The strongest α-amylase inhibition was observed in lemon myrtle and rosemary plus extracts due to the presence of monoterpenes. Rosemary and sage extracts exhibit the highest AChE inhibition activity, with 1 μL essential oils being more potent than the recommended daily dose of donepezil. This superior neuroprotection was attributed to the presences of di- and triterpenes that displayed strong AChE inhibition and antioxidant potential in DPPH• free radical assay. Antioxidant activity was related to phenolic content (R = 0.49), while α-amylase inhibitory activity was positively related to antioxidant activity (R = 0.20) and terpenoid/sterol content (R = 0.31). AChE inhibitory activity was correlated (R = 0.80) to the combined effect of phenolics and terpenoids. Thus, the superior AChE inhibitory and neuroprotection potential of rosemary and sage essential oils could be attributed to joint effects of main phenolic and terpene constituents. The hyphenated HPTLC method provided rapid bioanalytical profiling of highly complex essential oil samples.

Eukaryotic cells can harbour mitochondria with markedly different transmembrane potentials. Intracellular mitochondrial quality-control mechanisms (e.g. mitophagy) rely on this intracellular variation to distinguish functional and damaged (depolarized) mitochondria. Given that intracellular mitochondrial DNA (mtDNA) genetic variation can induce mitochondrial heterogeneity, mitophagy could remove deleterious mtDNA variants in cells. However, the reliance of mitophagy on the mitochondrial transmembrane potential suggests that mtDNAs with deleterious mutations in ATP synthase can evade the control. This evasion is possible because inhibition of ATP synthase can increase the mitochondrial transmembrane potential. Moreover, the linkage of the mtDNA genotype to individual mitochondrial performance is expected to be weak owing to intracellular mitochondrial intercomplementation. Nonetheless, I reason that intracellular mtDNA quality control is possible and crucial at the zygote stage of the life cycle. Indeed, species with biparental mtDNA inheritance or frequent 'leakage' of paternal mtDNA can be vulnerable to invasion of selfish mtDNAs at the stage of gamete fusion. Here, I critically review recent findings on intracellular mtDNA quality control by mitophagy and discuss other mechanisms by which the nuclear genome can affect the competition of mtDNA variants in the cell. This article is part of the theme issue 'Linking the mitochondrial genotype to phenotype: a complex endeavour'.

Eukaryotic cells can harbour mitochondria with markedly different transmembrane potentials. Intracellular mitochondrial quality-control mechanisms (e.g. mitophagy) rely on this intracellular variation to distinguish functional and damaged (depolarized) mitochondria. Given that intracellular mitochondrial DNA (mtDNA) genetic variation can induce mitochondrial heterogeneity, mitophagy could remove deleterious mtDNA variants in cells. However, the reliance of mitophagy on the mitochondrial transmembrane potential suggests that mtDNAs with deleterious mutations in ATP synthase can evade the control. This evasion is possible because inhibition of ATP synthase can increase the mitochondrial transmembrane potential. Moreover, the linkage of the mtDNA genotype to individual mitochondrial performance is expected to be weak owing to intracellular mitochondrial intercomplementation. Nonetheless, I reason that intracellular mtDNA quality control is possible and crucial at the zygote stage of the life cycle. Indeed, species with biparental mtDNA inheritance or frequent 'leakage' of paternal mtDNA can be vulnerable to invasion of selfish mtDNAs at the stage of gamete fusion. Here, I critically review recent findings on intracellular mtDNA quality control by mitophagy and discuss other mechanisms by which the nuclear genome can affect the competition of mtDNA variants in the cell. This article is part of the theme issue 'Linking the mitochondrial genotype to phenotype: a complex endeavour'.

Eukaryotic cells can harbour mitochondria with markedly different transmembrane potentials. Intracellular mitochondrial quality-control mechanisms (e.g. mitophagy) rely on this intracellular variation to distinguish functional and damaged (depolarized) mitochondria. Given that intracellular mitochondrial DNA (mtDNA) genetic variation can induce mitochondrial heterogeneity, mitophagy could remove deleterious mtDNA variants in cells. However, the reliance of mitophagy on the mitochondrial transmembrane potential suggests that mtDNAs with deleterious mutations in ATP synthase can evade the control. This evasion is possible because inhibition of ATP synthase can increase the mitochondrial transmembrane potential. Moreover, the linkage of the mtDNA genotype to individual mitochondrial performance is expected to be weak owing to intracellular mitochondrial intercomplementation. Nonetheless, I reason that intracellular mtDNA quality control is possible and crucial at the zygote stage of the life cycle. Indeed, species with biparental mtDNA inheritance or frequent 'leakage' of paternal mtDNA can be vulnerable to invasion of selfish mtDNAs at the stage of gamete fusion. Here, I critically review recent findings on intracellular mtDNA quality control by mitophagy and discuss other mechanisms by which the nuclear genome can affect the competition of mtDNA variants in the cell. This article is part of the theme issue 'Linking the mitochondrial genotype to phenotype: a complex endeavour'.

Eukaryotic cells can harbour mitochondria with markedly different transmembrane potentials. Intracellular mitochondrial quality-control mechanisms (e.g. mitophagy) rely on this intracellular variation to distinguish functional and damaged (depolarized) mitochondria. Given that intracellular mitochondrial DNA (mtDNA) genetic variation can induce mitochondrial heterogeneity, mitophagy could remove deleterious mtDNA variants in cells. However, the reliance of mitophagy on the mitochondrial transmembrane potential suggests that mtDNAs with deleterious mutations in ATP synthase can evade the control. This evasion is possible because inhibition of ATP synthase can increase the mitochondrial transmembrane potential. Moreover, the linkage of the mtDNA genotype to individual mitochondrial performance is expected to be weak owing to intracellular mitochondrial intercomplementation. Nonetheless, I reason that intracellular mtDNA quality control is possible and crucial at the zygote stage of the life cycle. Indeed, species with biparental mtDNA inheritance or frequent 'leakage' of paternal mtDNA can be vulnerable to invasion of selfish mtDNAs at the stage of gamete fusion. Here, I critically review recent findings on intracellular mtDNA quality control by mitophagy and discuss other mechanisms by which the nuclear genome can affect the competition of mtDNA variants in the cell. This article is part of the theme issue 'Linking the mitochondrial genotype to phenotype: a complex endeavour'.

© 2020 by the authors; licensee Growing Science, Canada. The article reviews methods of transportation for various types of imported medicinal plant materials of ginseng roots from China to the Russian Federation, with due consideration for risks. Logistic supply chains for ginseng roots have been developed and proposed based on the systemic method of research and the information analytical method. The optimal method for transportation of pharmaceutical substances of plant origin is chosen on the basis of the priority criteria for the cargo owner, as well as with due consideration for the peculiarities of the type of medicinal plant materials of ginseng roots.

© Springer Nature Switzerland AG 2020. The author considers an important problem of using the Huff model for obtaining quantitative estimates of trade turnover of the shopping center. The aim of the article is to identify the actual limitations of the specified model, which when conducting a practical assessment of potential trade turnover of large objects of commercial real estate in a significant way affect the quantitative results of the calculation and the quality of the conclusions. In accordance with the goal, the author solved the following tasks: –economic and mathematical structure of the Huff model is considered; –the calibration parameters of the model are revealed, the accuracy of which determines the accuracy of calculations and the correctness of conclusions; –an algorithm for estimating the potential trade turnover of the trading center based on a combination of the Huff model and econometric methods is constructed. The hypothesis of the research: For correct application of the Huff model in practice it is necessary to use econometric methods of estimation of calibration parameters. The article shows that the evaluation of calibration parameters of the model significantly affects the accuracy of the evaluation. As a result, the necessity of using empirical data and constructing econometric model based on them is proved by obtaining accurate quantitative estimates. The author presents the algorithm of correct estimation of potential trade turnover of the shopping center using econometric estimation of parameters of the Huff model, empirical and expert data and a calculation example. Conclusions on the use in practice of the Huff model with its methodological and actual limitations are formulated.

© Springer Science+Business Media, LLC, part of Springer Nature 2020. This chapter reports a library preparation protocol for efficient high-throughput sequencing of double-stranded RNA viruses. The protocol consists of four main steps, viz., enzyme treatment, precipitation using lithium chloride, full-length amplification of cDNAs, and tailing adapters for high-throughput sequencing. This protocol will be useful for all double-stranded RNA viruses and for all of the high-throughput sequencing platforms.

© Springer Science+Business Media, LLC, part of Springer Nature 2020. We describe here the Oncobox method for scoring efficiencies of anticancer target drugs (ATDs) using high throughput gene expression data. The method rationale, design, and validation are given along with the examples of its practical applications in biomedicine. The method is based on the analysis of intracellular molecular pathways activation and measuring expressions of molecular target genes for every ATD under consideration. Using Oncobox method requires collection of normal (control) expression profiles and annotated databases of molecular pathways and drug target genes. Both microarray and RNA sequencing profiles are acceptable, although the latter type of data prevails in the most recent applications of this technique.

© 2020, Springer Nature Switzerland AG. The chapter is devoted to the attacks modeling for Cyber-Physical systems of industrial enterprises with regard to risk assessment. In this chapter the analysis of corporate systems of industrial is held; systems’ attacks and risk assessment techniques were studied; software for attacks modeling are compared. Information models of corporate networks and attacks are developed; describes the design and basic functions of the module for assessing the risks of attacks in the corporate system. Corporate networks of more than 70% of industrial enterprises are potentially vulnerable to hacker attacks. Today, according to research by Positive Technologies analysts, hackers can cross the perimeter and get into the corporate network of 73% of the companies in the industrial segment. In 82% of companies, penetration from the corporate network to the technological one is possible. One of the main opportunities for obtaining unauthorized access to the enterprise network turned out to be administrative control channels. Solving the problem of ensuring the information security of Cyber-Physical systems is an urgent task today.

© Springer Science+Business Media, LLC, part of Springer Nature 2020. DNA mutations govern cancer development. Cancer mutation profiles vary dramatically among the individuals. In some cases, they may serve as the predictors of disease progression and response to therapies. However, the biomarker potential of cancer mutations can be dramatically (several orders of magnitude) enhanced by applying molecular pathway-based approach. We developed Oncobox system for calculation of pathway instability (PI) values for the molecular pathways that are aggregated mutation frequencies of the pathway members normalized on gene lengths and on number of genes in the pathway. PI scores can be effective biomarkers in different types of comparisons, for example, as the cancer type biomarkers and as the predictors of tumor response to target therapies. The latter option is implemented using mutation drug score (MDS) values, which algorithmically rank the drugs capacity of interfering with the mutated molecular pathways. Here, describe the mathematical basis and algorithms for PI and MDS values calculation, validation and implementation. The example analysis is provided encompassing 5956 human tumor mutation profiles of 15 cancer types from The Cancer Genome Atlas (TCGA) project, that totally make 2,316,670 mutations in 19,872 genes and 1748 molecular pathways, thus enabling ranking of 128 clinically approved target drugs. Our results evidence that the Oncobox PI and MDS approaches are highly useful for basic and applied aspects of molecular oncology and pharmacology research.

© 2019 Elsevier Ltd Background: Single studies suggest that nursing students perceive caring as more an instrumental than expressive behaviour and indicate some differences between caring perceptions in junior and senior nursing students. However, there are limited studies investigating caring perceptions in nursing students across multiple cultures. Objective: To determine perceptions of caring in Slovene, Croatian, Chinese and Russian nursing students and explore whether there are statistically significant differences in perceptions of caring between countries and between first and third-year nursing students. Design: A cross-sectional descriptive study design was used. Settings and participants: The study included 604 nursing students enrolled in first and third year in seven different nursing faculties in four countries: Slovenia; China; Croatia; and the Russian Federation. Methods: The 25-item Caring Dimension Inventory (CDI-25) was used to measure caring perceptions. We also included demographic questions regarding age, gender, country, year of study and type of study. Demographic data were analysed using descriptive analysis while a two-way analysis of variance (ANOVA) adjusted for unequal sample sizes was performed together with a post hoc analysis of the results. Results: The results of two-way ANOVA showed that both main effects (country and year of study) were statistically significant, as well as their interaction at the 0.05 significance level. The main effect for country was F(3, 596) = 3.591, p < 0.0136 indicating a significant difference in CDI-25 between Slovenia (M = 108.9, SD = 9.2), Russian Federation (M = 107.1, SD = 8.2), China (M = 102.8, SD = 9.7) and Croatia (M = 110.0, SD = 8.6). Conclusions: Perceptions of caring in nursing students differ across countries, probably due to different educational systems, curricula, cultural differences and societal values. Implementing caring theories in nursing curricula could help students to cultivate caring during their education.

© 2019 The Author(s) We report a Astrakhan ricketsiosis fever in woman who came from Astrakhan. On admission she had fever, intoxication syndrome, exanthema. In complex examination of blood serum by ELISA were reveled IgM and IgG to Rickettsia conorii on the 15th day of the disease.

© 2019 The essence of our hypothesis is quantum properties of conscious processes as well as the possibility to measure changes in conscious attention by using quantum double-slit experiment. We supposed that the act of observing in state of focused attention cause a wave function collapse in double-slit diffraction. In order to test the hypothesis, 26 participants took part in the study divided into physical and physiological parts of the experiment. The purpose of the physical system was to reproduce the brain quantum process via hypothesized quantum entanglement. The physical part consisted of a red laser source, neutral density filter, double-slit diaphragm, and linear couple charged camera, while the physiological part of the experiment was significant for the physiological quantifying state of attention. Physiological data were collected by using 29 channel electrophysiological unit with 21 channel electroencephalograph. The study had control and experimental group according to dependent variables measured in the physical part of the experiment. The data in the experimental group were collected over ten studies (sessions). Results obtained in hypothesis testing showed significant increases in corpuscular properties of the electromagnetic wave as well as significant quantum entanglement between the brain and external double-slit quantum system. Our results also offer insight into the connection between the chaotic dynamic of the electroencephalographic signals and uncertainty in the physical system due to focused attention effect. We also hypothesized that the state of concentrated attention was highest during the first several seconds. The last hypothesis considered possible backward time referral effect of cognitive evoked potential p300.

© 2019, The Author(s). Background: Sudden cardiac death (SCD) refers to unanticipated death as a consequence of cardiac failure which takes place over a brief time period (typically within an hour of the commencement of symptoms) in an individual, who may or may not be known to be subject to cardiac disease, but in whom no fatal condition has been diagnosed. The objective of this investigation was to identify the potential cause of SCD in the young population in Moscow. Result: Analysis revealed that the mean age of the deceased amongst men was 24.6 years, and amongst women was 29.1 years. Seventy-six percent of sudden deaths were found to result from some previously undiagnosed pathology of the cardiovascular system, and cardiomyopathy is the most common cause of death in young people. Coronary heart disease is the most common cause of death in people above 40 years old. Conclusion: Investigation to diagnose the causes of SCD constitutes a relevant, comprehensive, and multidisciplinary undertaking, which ought to be founded upon the application of novel and current diagnostic methodologies. An integrated strategy to the quest for the underlying reason for sudden death ought to be founded upon the outcomes of biochemical, anatomical, genetic, pathophysiological, and other investigations. The search and development of objective risk factors will permit timely therapeutic intervention for patients with cardiovascular pathology to minimise and prevent the risk of sudden cardiac death.

© 2019, Springer-Verlag GmbH Germany, part of Springer Nature. Purpose: An anterior glottic web consists of the formation of a bridge of scar tissue covered by epithelium between the anterior free edges of the true vocal cords and represents one of the most common complications of laryngeal endoscopic surgery for tumors involving the anterior commissure. Endoscopic surgery is the therapy of choice, but simple section of the web is burdened by a high recurrence rate. Topical application of mitomycin C, intracordal stents, and the use of mucosal microflaps have been proposed to improve outcomes. We report our experience with the use of single and double mucosal microflaps (sliding technique) during the management of iatrogenic anterior glottic web (IAGW). Methods: From November 2010 to December 2018, 30 patients (29 males, 1 female, mean age 65 years, range 47–87 years) were observed for IAGW, and 11 of these patients (36.7%) required surgical treatment. The Voice Handicap Index (VHI) and the GRBAS were used for the perceptive evaluation of pre- and post-operative voice quality. Results: A reduction of the web length was observed in all cases, and we did not observe any residual web at the mid-third of the glottis. The mean post-operative VHI score decreased from 45 to 24, and the mean post-operative GRBAS values were reduced from 2.8, 2.4, 2.3, 2.1, and 1.1 to 1.9, 1.4, 1.3, 1.1, and 0, respectively. Conclusions: The microflap technique represents an effective and reproducible one-step procedure that, in expert hands, allows to obtain good anatomical and functional results in a high percentage of cases.

© 2019 Objective: According to one of the hypotheses, somatoform disorders (SD) are related to disturbed perception of bodily signals. We suspect that the disturbances and abnormalities of multisensory integration dynamics may be an additional SD mechanism. The objective of the research was to investigate multisensory bodily illusion dynamics in SD patients. Methods: A clinical group of SD patients (n = 16) and a control group (n = 17) participated in experiments aimed at eliciting a visual-tactile rubber hand illusion (RHI) and a visual-kinesthetic virtual hand illusion (VHI). Results: For both illusions studied, the illusion dynamics in SD patients differed from those of healthy subjects. The visual-tactile illusion on the subjective level appeared to be less strong in SD patients, and no proprioceptive drift (PD) was detected. The subjective dynamics of the visual-kinesthetic illusion in patients were similar to those of the control group, but the PD dynamics were different: after the termination of stimuli from the artificial limb, the proprioceptive system did not return to its initial state; on the contrary, PD continued to increase. Conclusion: In comparison with the norm, patients with somatoform disorders display differences in multisensory mechanisms underlying the development of body perception. The results suggest that the disturbance of visual-tactile integration of the stimuli represents one of the mechanisms of body perception distortion in somatoform disorders. It may be assumed that one of the reasons for persistent symptom perception in SD patients is the rigidity of multisensory stimuli perception, at least in the visual-motor domain.