bluuebunny/crossref_metadata_2025 huggingface dataset:
Regulation of autophagosome biogenesis by OFD1‐mediated selective autophagy
Authors: Morleo Manuela , Brillante Simona , Formisano Umberto , Ferrante Luigi , Carbone Fabrizia , Iaconis Daniela , Palma Alessandro , Buonomo Viviana , Maione Angela Serena , Grumati Paolo , Settembre Carmine , Franco Brunella
Published: December 2020

Autophagy is a lysosome‐dependent degradation pathway essential to maintain cellular homeostasis. Therefore, either defective or excessive autophagy may be detrimental for cells and tissues. The past decade was characterized by significant advances in molecular dissection of stimulatory autophagy inputs; however, our understanding of the mechanisms that restrain autophagy is far from complete. Here, we describe a negative feedback mechanism that limits autophagosome biogenesis based on the selective autophagy‐mediated degradation of ATG13, a component of the ULK1 autophagy initiation complex. We demonstrate that the centrosomal protein OFD1 acts as bona fide autophagy receptor for ATG13 via direct interaction with the Atg8/LC3/GABARAP family of proteins. We also show that patients with Oral‐Facial‐Digital type I syndrome, caused by mutations in the OFD1 gene, display excessive autophagy and that genetic inhibition of autophagy in a mouse model of the disease, significantly ameliorates polycystic kidney, a clinical manifestation of the disorder. Collectively, our data report the discovery of an autophagy self‐regulated mechanism and implicate dysregulated autophagy in the pathogenesis of renal cystic disease in mammals.
Modeling by disruption and a selected‐for partner for the nude locus
Authors: Li Jian , Lee Yun‐Kyoung , Fu Wenyu , Whalen Anne M , Estable Mario C , Raftery Laurel A , White Kristin , Weiner Lorin , Brissette Janice L
Published: December 2020

A long‐standing problem in biology is how to dissect traits for which no tractable model exists. Here, we screen for genes like the nude locus (Foxn1)—genes central to mammalian hair and thymus development—using animals that never evolved hair, thymi, or Foxn1. Fruit flies are morphologically disrupted by the FOXN1 transcription factor and rescued by weak reductions in fly gene function, revealing molecules that potently synergize with FOXN1 to effect dramatic, chaotic change. Strong synergy/effectivity in flies is expected to reflect strong selection/functionality (purpose) in mammals; the more disruptive a molecular interaction is in alien contexts (flies), the more beneficial it will be in its natural, formative contexts (mammals). The approach identifies Aff4 as the first nude‐like locus, as murine AFF4 and FOXN1 cooperatively induce similar cutaneous/thymic phenotypes, similar gene expression programs, and the same step of transcription, pre‐initiation complex formation. These AFF4 functions are unexpected, as AFF4 also serves as a scaffold in common transcriptional‐elongation complexes. Most likely, the approach works because an interaction's power to disrupt is the inevitable consequence of its selected‐for power to benefit.
How do People with Disabilities Evaluate the Quality of Social Services?
Authors: Čiapaitė Laurita , Vaitkevičienė Asta
Published: December 2020

In this article we analyse the quality of social services from the perspective of people with disabilities and we analyse other social services quality studies that use the EQUASS system or the SERVQUAL model. We show the contrast between the high quality ratings reported by social workers and unmet expectations of the services’ recipients. It is argued that the difference may be due to methodological errors in choosing one or another research method, and the phenomenon of social workers’ conformism, which can be supported also by the peculiarities of Lithuania’s long-term social policy. It is proposed that the SERWQUAL model be used for the study of the quality of social services, which is flexible with regards to the needs of service users. Triangulation of research methods was used to analyse the data. We show evaluation of the quality of social services according to the 5 dimensions of the SERWQUAL model: tangibles, reliability, responsiveness, assurance (certainty) and sensitivity (empathy).
Semantics and Ideography of Dostoevsky's Handwritten Text: from Handwriting to Meaning
Authors: Tarasova Nataliya , Panyukova Tatyana
Published: December 2020

The paper analyzes the material from two workbooks (1864–1867) that belonged to Feodor Dostoevsky (Russian State Archive of Literature and Art. Collection 212, inventory 1, storage units 4 and 5). The research was conducted in three main areas: selection of information for the letter style alphabet; comparative analysis of sources (manuscripts and publications of handwritten text) for the purpose of identifying and correcting errors in its reading; a review of calligraphy and graphics of Feodor Dostoevsky. The alphabet of letter styles allows to establish typical and atypical letter styles in the handwriting of Feodor Dostoevsky. This made it possible to compile a classification of letters, describe their features, and use this information in the study of difficult-to-read and previously undecipherable records. The latter may contain valuable information that allows to clarify the facts in the creative history of the works of Feodor Dostoevsky, biographical information, chronology of the creative process, and so on. The second area of research includes the comparative analysis of manuscript sources and their published versions — is of particular value for ascertaining the original author's text and eliminating the inaccuracies of research interpretations that often appear in the process of manuscript publishing. There is a direct connection between these areas of analysis — the letter style alphabet is a tool that allows to read the handwritten text more accurately and correct the mistakes of the publishers. The third direction, dedicated to the study of calligraphy, expands the range of tasks related to the study of graphics in the writer's texts and allows to draw preliminary conclusions about the nature of its function in the handwritten text. The scientific novelty of the work consists in the systematization of data on the writer's handwriting in manuscripts of the specified period, on the features of his creative work, and in correcting the errors made by publishers of handwritten materials and restoring the true meaning of the author's notes. The practical value of the work consists in collecting information about the writer's handwriting, which is relevant for the textual analysis of his works, publication of his texts, and use of the collected information for subsequent scientific work.
DIGITAL BUSINESS ETIQUETTE IN THE DISTANCE FORMAT OF HIGHER EDUCATION
Authors: Mamina Raisa , Piraynen Evgeniya
Published: December 2020

The article highlights the essential characteristics and specifics of Digital Business Etiquette. The role and importance of digital business etiquette as a tool for effective communication in the internet space of the university environment are shown within the framework of the problem areas of the di-station format of higher education, which were updated in the context of the pandemic. The study is based on the application of the methodology of cultural-philosophical, axiological and interdisciplinary approaches to the consideration of digital etiquette as a new communicative phenomenon of a new reality.
THE PROBLEM OF LACK OF TEACHING STAFF IN THE REPUBLIC OF KARELIA: PROFORIENTATION ASPECT
Authors: Skoropadskaya Anna , Efremov Nikita
Published: December 2020

The article reveals the topical issue of the lack of teaching staff in the Republic of Karelia. Despite the fact that traditionally the education sector is one of the main in terms of the share of employed workers for the republic, the need for teaching staff remains high. At the federal and republican levels a number of socio-economic measures are being taken to level the situation: incentive payments and various kinds of allowances for teachers (including beginners), benefits, federal programs. The authors of the article propose to look at the personnel problem from the standpoint of the attractiveness of the teaching profession for applicants and students. The statistical surveys carried out within the framework of the study among the students of Petrozavodsk State University in the areas of training related to pedagogy, showed that students are not sufficiently interested in future employment in the studied specialty. This is largely correlated with the other existing studies. Based on the revealed fact that future students become interested in the profession of a teacher during the school period, the authors conclude that one of the ways to consolidate this interest is thoughtful career guidance, which should be carried out not only with schoolchildren but also with students.
Protein bodies of the endoplasmic reticulum in Arabidopsis thaliana (Brassicaceae): origin, structural and biochemical features, functional significance
Authors:   M.G. Kholodny Institute of Botany, National Academy of Sciences of Ukraine, Romanchuk S.M.
Published: December 2020

History of the discovery, formation, structural and biochemical traits of the protein bodies, derivatives of the granular endoplasmic reticulum (GER) that are known as ER-bodies, are reviewed. The functions of ER-bodies in cell vital activity mainly in Arabidopsis thaliana are reported. The highly specific component of ER-bodies, β-glucosidase enzyme, is described and its protecting role for plants under effect of abiotic and biotic factors is characterized. Based on the analytical review of the literature, it is shown that ER-bodies and the transcription factor NAI2 are unique to species of the family Brassicaceae. The specificity of the system GER – ER-bodies for Brassicaceae and thus the fundamental and applied importance of future research of mechanisms of its functioning in A. thaliana and other Brassicaceae species are emphasized.
Co-occurrence of depressive disorders and cardiovascular diseases – selected aspects
Authors:   Zakład Ratownictwa Medycznego, Wydział Nauk o Zdrowiu, Uniwersytet Jagielloński – Collegium Medicum, Kraków, Polska, Zalewska Magdalena
Published: December 2020

Depressive disorders are a common problem in patients with cardiovascular diseases. They are associated with increased mortality, disability, increased healthcare expenditure and reduced quality of life. Depression occurs in 1 in 5 patients with coronary artery disease, peripheral arterial disease or heart failure. It significantly complicates the optimal management of a patient with cardiovascular disease, primarily by reducing compliance with healthy lifestyle principles and therefore reducing the effectiveness of recommended therapeutic methods. The mechanisms responsible for unfavorable prognosis in patients with cardiovascular disease and depression are associated with lifestyle factors, autonomic dysfunction, neuroendocrine disorders, inflammation, immune system dysfunction, insulin resistance and increased platelet reactivity. These mechanisms significantly interact in the regulation of both cardiovascular and central nervous system functions. Therefore, it is important to perform prompt and complete diagnosis of depression in a particular patient with cardiovascular disease, and to implement optimal therapeutic management from the psychiatric and cardiological point of view. In recent years, interest in the effect of antidepressants on cardiac parameters in patients with depression has increased. The assessment of the safety and efficacy of antidepressant therapy in the treatment of cardiac patients with depression is also important due to the fact that depression in these patients is often accompanied by other significant comorbidities such as diabetes, hypertension and tobacco addiction. The aim of this study was to review the most important aspects necessary in the cooperation of a psychiatrist and cardiologist that may enable the most effective treatment of patients with depressive disorders coexisting with selected cardiovascular diseases.
Examples of mental health campaigns targeted at men
Authors:   Uniwersytet Marii Curie-Skłodowskiej w Lublinie, Lublin, Polska, Stradomska Marlena ,   Polskie Towarzystwo Suicydologiczne, Warszawa, Polska, Kielan Aleksandra ,   Polskie Towarzystwo Suicydologiczne, Warszawa, Polska,   Zakład Zdrowia Publicznego, Wydział Nauki o Zdrowiu, Warszawski Uniwersytet Medyczny, Warszawa, Polska, Walewska-Zielecka Bożena ,   Zakład Zdrowia Publicznego, Wydział Nauki o Zdrowiu, Warszawski Uniwersytet Medyczny, Warszawa, Polska
Published: December 2020

Men commit suicide more frequently than women in nearly all parts of the world. This is due to a number of factors, including the fact that women tend to seek mental help relatively more often than men. Many developed countries have attempted to implement suicide prevention programmes and initiatives, but only a small proportion of these have targeted men directly. The aims of this paper are to highlight preventive measures focusing on mental problems experienced specifically by men, and provide a detailed description of these measures. Consequently, the paper has both theoretical and practical dimensions, outlining the challenges and presenting potential solutions. The study discusses selected examples of mental health actions targeting men which were carried out in several countries around the world including Japan (“Daddy, have you slept well?”), UK (“Heads Up” campaign), United States (“Real men. Real depression”), Canada (“HeadsUpGuys” campaign), and Australia (Movember), as well as social media campaigns (Instagram: #HereForYou). Men are often reluctant to reveal their mental problems (stress, low mood, insomnia, suicidal ideation), which can be attributed to the so-called macho culture existing in societies. The man is traditionally considered head of the family, a guardian and leader, a strong personality, and a person to rely on. This perception contributes to the fact that men tend not to admit their weaknesses in front of family or friends. It is recommended that preventive actions take into account such aspects as the transfer of knowledge, skills and competences in the areas of identification of mental problems, and abilities to cope with a crisis situation. Mental health actions should be launched in areas frequently visited by men including workplaces, buses, pubs, bars, sports clubs, stadiums or other meeting places, so that the information can reach the largest possible target group.
Comparative Analysis of Centrality Measures of Network Nodes based on Principal Component Analysis
Authors: Eremeev Igor , Tatarka Maxim , Shuvaev Fedor , Tsyganov Andrey
Published: December 2020

. The analysis of networks of a diverse nature, which are citation networks, social networks or information and communication networks, includes the study of topological properties that allow one to assess the relationships between network nodes and evaluate various characteristics, such as the density and diameter of the network, related subgroups of nodes, etc. For this, the network is represented as a graph – a set of vertices and edges between them. One of the most important tasks of network analysis is to estimate the significance of a node (or in terms of graph theory – a vertex). For this, various measures of centrality have been developed, which make it possible to assess the degree of significance of the nodes of the network graph in the structure of the network under consideration.

The existing variety of measures of centrality gives rise to the problem of choosing the one that most fully describes the significance and centrality of the node.

The relevance of the work is due to the need to analyze the centrality measures to determine the significance of vertices, which is one of the main tasks of studying networks (graphs) in practical applications.

The study made it possible, using the principal component method, to identify collinear measures of centrality, which can be further excluded both to reduce the computational complexity of calculations, which is especially important for networks that include a large number of nodes, and to increase the reliability of the interpretation of the results obtained when evaluating the significance node within the analyzed network in solving practical problems.

In the course of the study, the patterns of representation of various measures of centrality in the space of principal components were revealed, which allow them to be classified in terms of the proximity of the images of network nodes formed in the space determined by the measures of centrality used.
Theology in a Post-Secular Context: Origins, Problems, and Prospects
Authors:   Volgograd State University, Javorskiy Dmitriy
Published: December 2020

The article reconstructs the cultural conditions of the possibility of theology as a specific intellectual practice. The author proceeds from the understanding of the divine as non-anthropic, that is, beyond the control of man, but at the same time exerting an irresistible influence on him. In this context, the divine appears as unintelligible, which casts doubt on the project of theology as a form of cognition of the divine. However, despite this, in the ancient Greek Poleis, the divine becomes the subject of theology as a contemplative practice; it is the contemplative attitude to the deity that allows making the divine an object of cognition. A contemplative attitude to the divine has accompanied theology throughout its history. However, it is supplemented by a practical (liturgical) attitude. The secularization of Western European culture led to the separation of theology from religious practice. In modern times, there is a specific form of theology (crypto-theology) that allows thinking about the divine and its attributes, regardless of the experience of communion with God. Besides, extra-institutional theology is being formed, free from dogmatic restrictions and even a kind of amateurish theology, whose representatives did not have special, "school" training. All these transformations eventually led to the crisis of theology and the decline of its influence. At the same time, at the beginning of the XIX century, there were conditions for the emergence of a "modern theology" that responds to the challenges of secularism. In the second half of the twentieth century, the topic and problems of modern theology were also influenced by the programs of "overcoming metaphysics" (M. Heidegger) and "deconstruction" (J. Derrida). Modern theology basically positions itself as post-metaphysical and generates more or less radical projects of phenomenological theology (J.-L. Marion, J. Manoussakis) and negative theology (J. Derrida).
Modern Youth of Kalmykia: Attitude to Agricultural Labor (Based on the Materials of the 2019)
Authors:   Kalmyk Scientific Center of the Russian Academy of Sciences, Namrueva Ludmila
Published: December 2020

The development of rural settlements is interrelated with the state of population reproduction. Various aspects of life in rural areas, including the development of its economic and social sphere, are usually described by demographic indicators. The deterioration of economic and social conditions in rural areas increases the migration behavior of rural residents. Young people aged 18–35 are most susceptible to migration aspirations. The negative social changes that have been taking place in the Republic of Kalmykia for three decades (1990–2010) have significantly affected the irrevocable migration of young people to other regions of Russia, as well as to foreign countries. Youth unemployment, growing social stratification, and a prolonged lack of social elevators increase the gap between actively advertised opportunities for success, a career in 2–-30 years, and the social experience of the younger generation. Rural youth of the Republic of Kalmykia have long been deprived of the necessary resources to realize themselves on their native land. This article deals with the problems related to the desire / unwillingness of rural youth of the Republic of Kalmykia to work in the agricultural sector, migration behavior, and life strategies of young people. Natives of the village, for the most part, do not want to engage in agricultural work, to continue the traditions of their ancestors in raising livestock. The article concludes that the measures taken in the region to address the personnel policy in the agricultural sector are ineffective. Young livestock and plant breeders need state support, as they ensure the country's food security not only in the near future, but also in the long term. The author uses materials of the Republican newspaper "Halmg unn" for 2019, which reveal the problems of young villagers, their plans to engage in animal husbandry – the traditional type of work of the Kalmyks.
Development of Tools for Conducting Content Analysis of Federal and Regional Mass Media in Russia on the Issues of Reflecting the Demographic Situation and Politics
Authors:   Institute for Demographic Research – Branch of the Federal Center of Theoretical and Applied Sociology of the Russian Academy of Sciences, Rostovskaya Tamara , Vasilieva Ekaterina ,   Volgograd State University, Knyazkova Ekaterina ,   Institute for Demographic Research – Branch of the Federal Center of Theoretical and Applied Sociology of the Russian Academy of Sciences, Danilova Elena ,   Volgograd State University
Published: December 2020

At the present stage, the development of scientific tools that allow for a qualitative and independent assessment of the current demographic situation and the socio-political measures taken to strengthen, stabilize and develop it is particularly relevant. The efforts of Federal, regional and local authorities aimed at increasing population reproduction, increasing the birth rate, reducing mortality, increasing life expectancy, and supporting young families and families with children should be publicized, developed as public initiatives, and resonated with the population. The content analysis method as a method of qualitative and quantitative study of information sources, which implies search, registration, numerical processing of semantic units of analysis, interpretation of the obtained data, and has successfully proven itself in the sociopolitical sphere, can be used for demographic research, including identifying the level of loyalty of public institutions (including the media) to the demographic policy. In the context of media policy, it is important that information related to the demographic situation, especially in Federal and regional media, be objective, reliable, and consistent. This article presents a method of content analysis developed for the purpose of analyzing Federal and regional media in Russia on the issues of reflecting the demographic situation and taking measures of demographic policy. The article provides an overview of the research tools: sample, indicators and keywords, instructions for research and analysis, units of analysis (revealing demographic problems in the Russian Federation, measures to support families at the birth of children, assistance to the older generation and other demographic problems in the mass media).
Biologic Therapy of Moderate and Severe Forms of Atopic Dermatitis in Children
Authors: Murashkin Nikolay N. , Namazova-Baranova Leyla S. , Opryatin Leonid A. , Epishev Roman V. , Materikin Alexander I. , Ambarchian Eduard T. , Ivanov Roman A. , Fedorov Dmitriy V. , Kukoleva Daria S.
Published: December 2020

Atopic dermatitis (AD) is the disease with chronic inflammation, epidermal barrier dysfunction and microbial dysbiosis. AD is widespread, including pediatric population. The article discusses the disease’s pathogenesis: skin barrier deficiency, immunological causes of chronic inflammation, characteristics of normal skin microbiome and its disorders on both affected and unaffected skin of children with AD. Main principles of systemic treatment for moderate and severe forms of disease are considered. Features of targeted therapy with dupilumab (IL 4/IL 13 inhibitor) in children with moderate and severe forms of AD are discussed. The overview of the research results on the dupilumab efficacy and safety is presented.
Psoriasis and Psoriatic Arthritis in ChildhoodPsoriasis and Psoriatic Arthritis in Childhood
Authors: Murashkin Nikolay N. , Materikin Alexander I. , Ambarchian Eduard T. , Epishev Roman V. , Opryatin Leonid A. , Ivanov Roman A. , Kukoleva Daria S. , Kuptsova Daria G. , Pomazanova Marina U.
Published: December 2020

Psoriasis is chronic inflammatory skin disease that can develop at any age. Approximately 20–30% of all patients report about first rashes before the age of 18. Psoriatic arthritis is one of psoriasis comorbid conditions. Its signs can range from mild to extremely severe destructive forms. Arthralgia, joint stiffness and swelling are the most common symptoms. Early psoriatic arthritis treatment onset allows to control joint damage which usually occurs during the first 2 years of the disease. The moderate and severe course of psoriasis and psoriatic arthritis may require systemic therapy, however, there is not much data on the efficacy and toxicity of systemic agents in the pediatric practice. This article provides the review of studies on etanercept efficacy and safety in children with psoriatic arthritis.
Psoriasis in Pediatrics
Authors: Bakulev Andrey L. , Talnikova Ekaterina E.
Published: December 2020

Psoriasis is one of the most debatable topics in modern dermatology among the wide range of other dermatological diseases in childhood. The article provides current data on the epidemiology of psoriasis, its clinical features in different age groups, its triggering factors, differential diagnostics, possible comorbid conditions, as well as the quality of life of patients and their relatives.
Pruritus and Atopic Dermatitis: from Etiological Features to Management
Authors: Murashkin Nikolay N. , Opryatin Leonid A. , Epishev Roman V. , Materikin Alexander I. , Ambarchian Eduard T. , Ivanov Roman A. , Fedorov Dmitriy V. , Kukoleva Daria S.
Published: December 2020

Pruritus is one of the main clinical manifestations of atopic dermatitis, and it significantly reduces the quality of life of patients in childhood. Scientific images on its pathophysiological basis have now undergone significant changes. The histamine exceptional role in pruritus development was confounded, as well as data on immune system involvement in its maintenance was given. This article presents current data on differential approaches to pruritus management depending on its etiopathogenetic characteristics. The role of dermocosmetics in restoration of the skin barrier as the first stage prevention of pruritus in atopic dermatitis was considered. The results of clinical studies showing efficacy of topical agents (innovative component with anti-pruritic action — STIMU-TEX) application are presented.
Prevention of Transcutaneous Sensitization to Cow Milk Proteins in Infants with Atopic Dermatitis: Cohort Study
Authors: Murashkin Nikolay N. , Makarova Svetlana G. , Grigorev Stepan G. , Fedorov Dmitri V. , Ivanov Roman A. , Ambarchian Eduard T. , Epishev Roman V. , Materikin Alexander I. , Opryatin Leonid A. , Savelova Alena A.
Published: December 2020

Background. Malformations in epidermal barrier in children with atopic dermatitis (AD) can cause transcutaneous sensitization with further development of allergic diseases that can worsen the AD course and significantly reduces patients’ quality of life.

Objective. The aim of the study was to determine the effect of topical treatment and maintenance therapy with pimecrolimus 1% cream (PIM) and topical glucocorticosteroids (tGCS) in infants with AD on reducing the risk of developing transcutaneous sensitization (due to the levels of specific IgE to the cow milk protein over time) and on reducing the disease severity (by the EASI scale).

Methods. The study included children aged from 1 to 4 months with early manifestations of moderate and severe AD. The severity of AD was estimated via the EASI scale at start of observation, then at 6, 9 and 12 months of life. The class and level of specific IgE to cow milk proteins (CMP) were determined by the ImmunoCAP method at the point of enrolment and at the ages of 6 and 12 months. Statistical analysis of studied indicators dynamics and their comparison in research groups was carried out using multifactorial dispersion analysis.

Results. The study included 36 patients. All patients have received standard tGCS therapy in combination with emollients (wet wrap) for 10 days. The maintenance therapy was prescribed in postacute period. It included topical calcineurin inhibitor PIM 2 times/day for 3 months, then double application (morning/evening) 3 times/week up to the age of 1 year old (group 1). Other group had maintenance therapy — tGCS2 times/week for 3 months, and then at AD aggravation (group 2). Group 1 has shown lower level of sensitization to CMP at the age of 6 and 12 months and more significant decrease in AD severity according to EASI scale compared to group 2.

Conclusion. The treatment with PIM is effective in therapy of AD and prevention of transcutaneous sensitization in infants.
Multimorbidity in Pediatric Dermatology: Clinical Case
Authors: Murashkin Nikolay N. , Materikin Alexander I. , Ambarchian Eduard T. , Epishev Roman V. , Opryatin Leonid A. , Ivanov Roman A. , Kukoleva Daria S. , Kuptsova Daria G. , Pushkov Alexander A. , Pomazanova Marina Yu. , Kozyr Yana V.
Published: December 2020

Background. Nowadays, dermatoses with mixed clinical picture and resistant to classical management become more common. The presence of various genetic disorders typical for most chronic dermatoses may indicate possible combination of several nosologies.

Clinical Case Description. The article presents the clinical case of multimorbid condition in 10 years old patient who has nucleotide variants in CARD14 and EXPH5 genes. Mutations in CARD14 gene are typical for patients with type 2 psoriasis and pityriasis rubra pilaris (autosomal dominant type), while mutations in EXPH5 gene are typical for patients with non-specific epidermolysis bullosa (autosomal recessive type). Mutation in the TGM1 gene that is described in patients with congenital ichthyosis (autosomal recessive type), pathogenic mutations in KRT74 gene typical for ectodermal dysplasia, hypotrichosis and uncombable hair syndrome, and mutations in the KRT86 gene typical for monilethrix were also revealed. Medical history taking and histological examination as well as clinical data evaluating are crucial for correct diagnosis. They allow to understand the absence of the such manifestations in relatives and reveal various pathological processes in the epidermis. Molecular genetic testing with new generation sequencing (NGS) helps to finally establish the diagnosis and determine the further tactics for patient management.

Conclusion. Multidisciplinary approach and use of high-technology methods of examination and treatment (such as molecular genetic testing and biological therapy) are required for final diagnosis in severe forms of chronic dermatosis resistant to treatment and for decision on correct tactics for the further management of such patients.
Anaplastic Large Cell Lymphoma with Generalized Skin Lesion in Childhood: Clinical Case
Authors: Valiev Timur T. , Belysheva Tatyana S. , Varfolomeeva Svetlana R.
Published: December 2020

Background. Anaplastic large cell lymphoma (ALCL) represents 15% of all non-Hodgkin lymphomas (NHL) in children, and it is characterized by aggressive course and involvement of various organs and systems (including skin) in the pathological process. Skin tumors at NHL in childhood are rare. Thus, cases of skin localization are more oftenly diagnosed at ALCL among all morphoimmunologic variants of NHLs. The description of any new case of skin lesions in patients with ALCL has undeniable interest and scientific and practical significance due to its extremely rare frequency.

Clinical Case Description. Patient G., 11 years old, has firstly noted inflammatory nodes on the skin of scrotum. The generalization of skin lesions on tibia, face, lymph nodes and bones was recorded due to late admission to the hospital. The inflammatory-necrotic process has been revealed at skin histological examination. Local antiseptic and antibacterial therapies were ineffective. The ALCL was diagnosed on morphoimmunologic re-examination of the skin. Life expectancy after disease manifestation to patient’s death was 6 months.

Conclusion. Rare cases of skin involvement at ALCL require mandatory histological and immunohistochemical examinations. Second biopsy with reconsideration of histological preparations in the reference center may be necessary due to clinical-laboratory picture of the inflammatory process accompanying the tumor itself.
Alopecia Areata vs Trichotillomania. Differential Diagnosis According to Trichoscopy
Authors: Gadzhigoroeva Aida G.
Published: December 2020

Alopecia areata is one of the most common forms of hair loss in children. Meantime, its clinical picture is similar to trichotillomania, thus, it leads to incorrect diagnosis and management. The article provides major differential diagnosis signs for these related forms of alopecia and describes in details trichoscopic features of alopecia areata and trichotillomania.
¿Qué es una ocurrencia? Sobre humanidades y ciencias
Authors: Oyarzún Pablo
Published: December 2020

En primer término, se aborda la relación asimétrica de humanidades y ciencias en las instituciones contemporáneas de conocimiento sugiriendo la inconveniencia de acentuar la diferencia entre unas y otras, en la medida en que ambos órdenes epistémicos están sometidos hoy a los mismos condicionamientos estructurales. El efecto que estos tienen sobre las humanidades es analizado a través de dos aspectos críticos. En segundo término, se discute la significación epistémica de la ocurrencia como principio común de ciencias y humanidades apelando a la obra de Georg Christoph Lichtenberg a manera de caso ejemplar. En esta, experimentalismo y ensayo, singularidades, ficción y variación dan cuenta de un mismo brote epistémico en humanidades (y literatura) y ciencias.
La importancia del auto-engaño en la Fenomenología del espíritu
Authors: Von dem Bussche Rivera Matías
Published: December 2020

El presente estudio tiene por objetivo demostrar lo siguiente: que no es posible comprender suficientemente, al menos en un aspecto, el método de ejecución(Methode der Ausführung) de la Fenomenología del espíritu como “la exposición del saber que aparece” (die Darstellung des erscheinenden Wissens), si es que no se comprende la relación que tiene dicho método de ejecución con la temática del auto-engaño. Este objetivo se lleva a cabo de la siguiente manera: 1) se propone el tipo de relación que se busca establecer entre el concepto de “auto-engaño” y el método de ejecución, 2) se presentan las notas que les corresponden a ambos y 3) se argumenta en orden a demostrar que el método de ejecución no se puede comprender rectamente sino en relación con la temática del auto-engaño.
"Nadie sabe lo que puede un cuerpo". Historia de la inversión de un error
Authors: Aguilar Claudia
Published: December 2020

La frase “nadie sabe lo que puede un cuerpo” se la retoma generalmente para plantear la indeterminación de los cuerpos o el poder ilimitado de ellos. Sin embargo, algo que no se tiene en cuenta es que uno de los puntos centrales de la filosofía spinoziana es la doble atribución; por lo que mente y cuerpo son una sola y misma cosa vista desde distintos atributos de la única sustancia. Para explicar esta frase, que nunca aparece formulada de esa manera en las obras de Spinoza, considero que es crucial la noción de individuo. Mi hipótesis es que dicha afirmación es inadecuada si partimos de la doble atribución y si consideramos las caracterizaciones del individuo. Mis objetivos son dos: (1) desarrollar las caracterizaciones del individuo como cuerpo complejo y conatus para abordar cómo estas caracterizaciones responden a la pregunta por lo que puede un cuerpo y (2) analizar el escolio que dio lugar a la frase del título, frase que invierte erróneamente un viejo error.
Phýsis krýptesthai phileî. Naturaleza y metafísca en la crisis de Ser y tiempo
Authors: Grasset Jean-Paul
Published: December 2020

En este artículo, expondré el concepto heideggeriano de phýsis entre los años 1927-30. Este concepto aparece en la doctrina de la Welteingang des Seienden, en el marco de una ‘radicalización de la ontología’, proyecto metafísico que luego hará crisis y llevará a Heidegger a reinterpretar la pregunta por el sentido del ser como pregunta por la verdad del Ser. Mostraré que la cuestión de la phýsis aporta elementos en esa crisis y posterior cambio de enfoque, en el ámbito filosófico-histórico y en el fenomenológico. Mi hipótesis es que el empleo del concepto de phýsis en el contexto de la doctrina de la Welteingang des Seienden es un recurso especulativo que pretende aclarar el sentido en que el ente natural es, pero sin aparecer dentro del mundo, en términos de ‘tránsito’ desde el completo ocultamiento (phýsis) al desocultamiento (alétheia). Esto tendrá consecuencias respecto a la evaluación de la tradición metafísica, entendida como un preguntar por la phýsis en el doble sentido de ente en totalidad y ser de lo ente o esencialidad.
On the Study of Nonstandard Mineral Vocabulary in the Russian Language: Articulation of the Issue
Authors:   Perm State University, Berezovich Elena L.
Published: December 2020

This article introduces the reader to the variety of Russian vocabulary that denotes minerals, mineraloids, rocks, which lies outside the framework of the official scholarly nomenclature. The author suggests that the term nonstandard mineral vocabulary be used to refer to this layer of vocabulary; in a narrow sense, it includes the names of minerals themselves, and in a broader sense, it can include words of adjacent thematic groups naming processes and phenomena related to the existence of minerals in nature and culture, their extraction and processing, etc. The author identifies sociolinguistic layers that make up nonstandard mineralogical vocabulary in its narrow sense: dialect; professional vocabulary and slang; argotic; colloquial words. A special place is occupied by names that have emerged as a result of artificial nomination (in particular, trade names). The article also lists some thematic groups related to mineral vocabulary in a broad sense (a group of words denoting features of the structure of minerals; peculiarities of their occurrence; designations of people who mine and process stones; nominations of stone products; vocabulary from the sphere of mythology and beliefs related to stones, etc.). Each classification position is illustrated by language material from lexicographic and book sources; also data from the Vocabulary, Toponymy, Ethnography of Minerals card file, currently formed by the staff of the Department of the Russian Language, General Linguistics, and Speech Communication of Ural Federal University, is introduced into scholarly circulation for the first time. The author focuses on issues around nonstandard mineral vocabulary that exist in linguistics today: it seems to lie “in the blind zone” of linguistics as it is insufficiently collected, poorly introduced into scholarly circulation, and understudied. There is a need to fill this gap in a systematic way.
Decline of the Byzantine Thalassocracy
Authors:   Ural Federal University, Kushch Tatiana V.
Published: December 2020

After 1204, the Byzantine Empire lost control over the Eastern Mediterranean. The restoration of the Empire in 1261, however, did not recover its sea dominance. The Latins, especially the Venetians and the Genoese, who had possessions in the Aegean and the Black Seas and conducted active maritime trade there, established themselves in the region. The importance of sea routes for Byzantium increased dramatically given the territorial dispersion of the Byzantine possessions, the high activity of Europeans in the region, and the growing threat of an Ottoman conquest. This article analyses the specifics of Byzantine sea communications and their role in the fate of the Empire during the period of geopolitical changes between the fourteenth and fifteenth centuries. Based on written sources, the author examines the condition of the Byzantine fleet, the role of foreigners in ensuring movement within and outside the Empire, and the meaning of the sea communication during the Ottoman sieges of Byzantine cities. The author reveals that the Empire could not provide stable and regular sea contacts between separate parts of its territories and external relations with the West on its own. The decline of the fleet and the lack of material resources forced the Byzantines to use foreign ships as means of transportation. The Italians, especially the Venetians, provided transport to the Byzantine emperors and diplomats who made official trips to the West, transported people and goods within the region, and provided food for the inhabitants of besieged cities. It is concluded that the transport dependence of Byzantium on the Italian maritime republics testified to its economic and political weakness. The loss of control over the sea routes in the Eastern Mediterranean and the degradation of its own transport system contributed to the decline and fall of Byzantium in 1453.
Existential Sentences as a Characteristic Syntactic Feature of I. A. Bunin’s Poetic Texts
Authors:   Bunin Yelets State University, Selemeneva Olga A.
Published: December 2020

In this article, existential sentences are examined as a syntactic dominant of I. A. Bunin’s lyrical poetry. The interest in the originality of the syntax of Nobel laureate’s literary texts is due to the lack of research on this issue, linguists’ focus on the aesthetic salience of the vocabulary, its expressive properties and combination potential, as well as stylistic figures and tropes. Meanwhile, it is the writer’s selection of specific syntactic structures for the implementation of the idea, the representation of key ideas and concepts that reflect his personality and the peculiarities of his perception of the surrounding world. The author refers to Bunin’s poems from 1886–1917 and 1918–1953 published in Bunin’s collected works in 9 volumes. In the writer’s poetic oeuvre, existential sentences are regularly used. Despite the traditional structure that underlies them and is represented by three meanings (‘the object of being’, ‘being’, and ‘area of being’), the richness of the lexical content of each of the main components stands out. As a result, existential sentences become a universal form used to represent completely different situations in the author’s individual worldview: the existence of natural objects in space, meteorological phenomena, events, time periods, artifacts, etc.; physical states of the surrounding world; psychological states of the subject. Acting as a semantic core of a poetic text, existential sentences do not have a fixed place in it, and are used as a lyrical beginning, an interposed element, or an ending in its structure. In each position, their use is conceptually significant. It is established that the peculiarity of existential sentences in Bunin’s lyrical poetry is their syntactic unconditionality (attachment to three registers of speech, i.e. reproductive, informative, and generative) and polyfunctionality (performing the pictorial, characterising and concluding, and generalising functions).
Energy resource efficient designs of small-sized devices for recycled water cooling
Authors: Bondar K.E. , Shulaev N.S. , Ivanov S.P. , Laponov S.V.
Published: December 2020

Introduction. To use natural sources in rational way, plantsof continuous cooling of closed systems of recycling water supply are used. The paper presents designs of small-sized devices for recycling water cooling which are energy resource effective due to twisted motion of air flow, moving countercurrent to the cooled water. Heat and mass transfer is a nanotechnological process that occurs at the intermolecular level. Methods and materials. Countercurrent mini cooling towers are widely used in all industries,but there are some disadvantages, the main of which is the insufficient interaction time of the moving phases. Screw motion of air flow is created by the tangential supply of cooling air in the bottom part of cylindrical small-sized cooling tower. The rate of rotary motion decreases as air flow moves up in cooling towers, and vertical parameter of the rate – increases. Such scheme of the air flow motionmakes it possible to decrease average vertical parameter of the rate and to increase phases contact time. Laboratory research. To determine the technological and hydroaerothermal characteristics, as well as to estimate the efficiency of cooling recycled water, and to carry out mass-heat exchange at the intermolecular stagean experimental facility of small-sized cooling tower with twisted air flow has been developed. Conclusions. In accordance with the exponential law it is shown that the rotational component decreases at increasing height, and in accordance with the power law the vertical component increases component with the exponent ~1,79. It is determined that moisture content x and air temperature tv in the volume of the height of the sprinkler varies according to a power law, in particular for a screw cooling tower proportionally x ~ h0,83, t в ~ h1,25. It was determined that the coefficients of mass transfer βxv and heat transfer αv of a mini cooling tower with twisted air flow at the intermolecular level with equal irrigation densities are 20% higher than the coefficients of a mini cooling tower with counter-current flow. Also it has been determined experimentally a dependence of aerodynamics resistance coefficient of the twisted irrigator of the cooling tower on criterium Refor air flow, and it was determined that it decreases like Re–K2 as the exponent K2 varies in the range 0.114÷0.193 depending on the irrigation density
A New DNA sequencing alignment methodology using the Longest Common Subsequence technique
Authors: Chowdhury Debkumar , Sau Kartik , Mishra Sanjukta
Published: December 2020

The goal of this paper is to perform DNA sequence alignment using the Longest Common Sub-sequence Algorithm to obtain the longest common subsequence from the two strings X and Y for matching DNA in molecular biology etc. This paper would seek to establish the differences between the
2 species DNA and thereby conclude on their genetic similarity. The paper will be implemented in terms of space and time using the optimized longest common subsequence algorithm. This paper acts as a bridge between computer science and genomics, exploring the interdisciplinary area of computational
biology and helping us to consider the very fundamental concepts of life and our relationship with other species.
Infuence of seasonal changes on performance of swimmers
Authors: Saha Limon Kumar , Banerjee Manas
Published: December 2020

The purpose of the study was to investigate the effect of seasonal changes on performance of swimmers. For this study, 20 State level male swimmers specialized in 50 meter and 100 meter backstroke swimming from different clubs of West Bengal were considered as subjects. Subjects were
selected purposively and the age of the subjects ranged from 15 to 17 years. To find out the differences in relation to the performance of swimmers between summer and winter season, t test was employed. To analysis the collected data, level of significance was set at 0.05 level. The result
showed that there was significant difference of 50 meters and 100 meter backstroke Swimming Performance in winter as well as in summer.