bluuebunny/crossref_metadata_2025 huggingface dataset:
Five Adventure Stories: Americans in Stalin’s Moscow (Lapina, Galina. Americans in Moscow, 1930–1940. Moscow: Litfakt Publ., 2023. 206 p.)
Authors:   A.M. Gorky Institute of World Literature of the Russian Academy of Sciences, Panova Olga Yu. ,   Lomonosov Moscow State University
Published: December 2023

The monograph by Russian-American researcher, translator and critic Galina Lapina Americans in Moscow: 1930–1940 is dedicated to pre-war Soviet-American theatrical, educational, cinematic, diplomatic, literary contacts. Among the heroes of the book are American Ambassador Joseph E. Davis and his chauffeur Charles Ciliberti, writers Langston Hughes and Dorothy West, playwright Sophie Treadwell, actress Blanche Yurka and other writers, artists, diplomats, journalists who came to the USSR in the 1930s and early 1940s. The book consists of five chapters; each of them is devoted to a certain episode of Soviet-American connections: the failed film project Black and White; The Promised Land, Sophie Treadwell's play about Soviet life; Moscow theater festivals of 1933–1937; history of the Anglo-American Institute (1933–1935), Moscow summer school for American students; a comparison of two books about the USSR — Joseph E. Davis’ Mission to Moscow (1943) and Charles Siliberti’s Backstage Mission to Moscow (1946), that show Soviet reality from the opposing points of view. The book that is, in fact, a collection of articles, is however marked by unity and integrity; it reconstructs the general context of the period in question on the basis of the extensive material. Memoirs, correspondence, archival documents, Soviet and American press form a vivid picture of Soviet-American relations in the pre-war decade. Many sources revealing American attitudes towards the USSR used by the author, have not yet been translated or published in Russia. Galina Lapina’s book encourages further research and publishing projects.
El Tungsteno by César Vallejo: 90 Years After (Fernández, Carlos, and Gianuzzi, Valentino. Sobre “El Tungsteno” de César Vallejo. Manchester: Trafalgar Square, 2021. 170 p.)
Authors:   A.M. Gorky Institute of World Literature of the Russian Academy of Sciences, Popova Victoria Yu.
Published: December 2023

The review focuses on the book On “El Tungsteno” by César Vallejo (Sobre “El Tungsteno” de César Vallejo) by Carlos Fernández and Valentino Gianuzzi, published in 2021 in honor of the 90th anniversary of the publication of the novel in Spain. César Vallejo at the turn of the 1920s–1930s collaborated with the USSR, and his novel El Tungsteno became one of the key texts of his creative biography, especially its Soviet page. The book reconstructs the history of the writer's work on the text, identifies possible sources of the novel’s idea, and studies the book's reception in Spain, Latin America and the USSR. The novel El Tungsteno is considered in the context of César Vallejo’s literary contacts with the Soviet Union, his activities in Spain, and interaction with publishers. The second part of the book contains reviews of the novel from Latin American, Spanish and Soviet periodicals, as well as prefaces to the Soviet and Ukrainian editions translated into Spanish. The book by C. Fernández and V. Gianuzzi makes a significant contribution to the study of the work of C. Vallejo, to the study of his Soviet literary connections, outlines further trajectories of development of César Vallejo studies and fills many gaps in Latin American literary studies in Latin America, Europe and Russia.
Archipelagic Turn in American Studies (Brian R. Roberts. Pogranichnye vody: posredi arkhipelagovykh shtatov Ameriki. Durham & London: Duke University Press, 2021. 392 p.; Brian R. Roberts. Pogranichnye vody: posredi arkhipelagovykh shtatov Ameriki [Borderwaters: Amid the Archipelagic States of America], translated by Maria Bykova. St. Peterburg: Academic Studies Press / Bibliorossika Publ., 2023. (In Russ.)
Authors:   Saint-Petersburg State University, Golovacheva Irina V.
Published: December 2023

The review focuses on the book by Brian R. Roberts Borderwaters: Amid the Archipelagic States of America (2021) published in Russian translation in 2023. Roberts suggests reconsidering the US culture post-continentally by viewing it as an archipelagic nation. Dispelling the ‘continental dominance’ in American studies, Roberts explores the rich imagery of borderwaters by drawing on fiction (e. g. Mark Twain’s and Zora Neale Hurston’s works) and visual arts (Miguel Covarrubias’s representations of the Caribbean). The reviewer analyzes the role of the ‘archipelagic turn’ in American studies, as well as the specific effects of the critic’s rhetoric strategy.
“A Garden of Forking Paths”: Reimagining Dreiser (Dreiser’s Path: A View With a Modern Lens. Edited by Irina V. Morozova. Moscow: RSUH Publ., 2023. 206 p.)
Authors:   Russian State University for the Humanities, Martirosian Georgii E.
Published: December 2023

The review focuses on the collective monograph Dreiser’s Path: A View with a Modern Lens (2023). The scholarly work reevaluates Dreiser's significance as a classic of American literature and offers a contemporary perspective on his legacy through the lens of modern methodologies such as critical race theory, postcolonial theory, gender theory, etc. The attention of prominent researchers in American literature and culture from Russia, the USA, Canada, and the UK is drawn to Dreiser's contradictory and hermetic view of society and its artistic projection. The monograph reevaluates Dreiser's creative legacy, considering both major and well-known works as well as minor forms. The researchers featured in the monograph highlight the reasons why certain laws operate in Dreiser's artistic world, align Dreiser's value system with the social norms prevailing during his time, typologize the components of Dreiser's texts, and theorize them using contemporary methodological tools. They also juxtapose Dreiser's texts with other artifacts of American culture and demonstrate the evolution of Dreiser's perception from the times of the USSR to the present day. The monograph was conceived in 2021 as a special tribute to Theodore Dreiser to mark his 150 anniversary.
Features of creating an interactive mapping web application for the analysis of space images
Authors: Matviienko Oleksii , Kurach Tamara
Published: December 2023

In the era of the high level of technological achievements of remote sensing of the Earth and the high level of development of web cartography, interactive web applications for the analysis of data of remote sensing of the Earth are gaining more and more popularity. Considering the large amount of space image data, and the complexity and heterogeneity of the analysis tools that need to be developed, the question arises of quickly and efficiently creating an interactive web application for the analysis of space images that will help scientists monitor and study changes in the Earth's surface and predict optimal ways of territory development with minimal expenditure of time and resources. In solving such a question, the specialized web platform Google Earth Engine (GEE) can help. The purpose of the article is to highlight the characteristics and advantages of the GEE platform by creating an interactive web application using the example of analyzing changes in the NDVI index. Analyzing GEE allows us to conclude that this web-platform provides a comprehensive solution for creating interactive cartographic web applications for the analysis of space images with a built-in IDE. It will help to significantly reduce development time and costs, allowing for quick and efficient analysis of Earth remote sensing data.
Importance of Health Policy and Systems Research for Strengthening Rehabilitation in Health Systems
Authors: Frontera Walter R. , DeGroote Wouter , Ghaffar Abdul
Published: December 2023

Abstract missing (Editorial)
Modelo de valoración de activos de capital o capital Asset Pricing Model (CAPM) en economías emergentes
Authors:   Universidad Internacional del Ecuador (UIDE), Analuisa Aguiar Andrés , Estrella Herrera JC ,   Instituto Superior Tecnológico Quito Metropolitano, Ruales Villacreses Sandra ,   Centro de Idiomas de la Universidad Nacional de Educación
Published: December 2023

Una de las mayores preocupaciones de los inversores de cualquier parte del mundo ha sido determinar una tasa que sirva como referencia para identificar el rendimiento esperado de cualquier activo financiero en el que estén dispuestos a invertir. El objetivo es determinar la factibilidad de aplicar el modelo Capital Asset Pricing Model (CAPM) mediante la realización de distintos ajustes a sus elementos para obtener el rendimiento requerido por los inversionistas que presentan interés en este tipo de mercados que no cumplen de manera integral la hipótesis de mercados eficientes. Para el desarrollo y elaboración se utiliza un proceso de experimentación, contraste y selección que consta de 5 fases: a) Plantear el modelo clásico CAPM b) Identificar sus componentes c) Diseñar ajustes al modelo CAPM d) Medir los valores resultantes e) Identificar y seleccionar el mejor ajuste. En cuanto a los resultados, se puede evidenciar que los hallazgos más significativos recaen en que existen cuatro ajustes principales que se pueden realizar con el fin de adaptar el modelo a la realidad de un país emergente. Con esto, se concluye que los principales ajustes deben ser en las variables coeficiente beta y tasa libre de riesgo, ya que dichos elementos se calculan en base a la información inexistente de un mercado emergente.
Modelo de empleabilidad integral: creando oportunidades propias para parroquias rurales
Authors:   Instituto Superior Tecnológico Quito Metropolitano, Sanguña Katherine
Published: December 2023

En la actualidad los estudios sobre empleabilidad adquieren importancia al comprender y abordar los diferentes desafíos que enfrentan las parroquias rurales en un contexto de cambios tanto económicos, tecnológicos y sociales, lo que genera que el tema de acceso a empleos adecuados se convierta en elementos importantes para el bienestar de la población que reside en cualquier zona geográfica. La presente investigación tiene por objetivo diseñar un modelo de empleabilidad integral tomando como eje central la parroquia de Calderón en Quito-Ecuador, mediante la determinación de la tasa de desempleo de los sectores de mayor demanda laboral, las habilidades y competencias más valoradas por los empleadores y el impacto de la educación en la empleabilidad de los residentes para el fortalecimiento de las oportunidades de empleo en parroquias rurales. La metodología aplicada consta de tres fases: i) recopilación de la información; ii) procesamiento e interpretación de información cuantitativa; iii) creación del modelo integral. Como resultados obtenidos se logró crear el modelo de empleabilidad con los rangos de edades adecuados, tendencias en niveles educativos, habilidades que más valoran los empleadores todo esto con la finalidad de crear a largo plazo políticas o estrategias sostenibles de empleabilidad y la inserción laboral. Concluyendo que, los modelos de empleabilidad pueden contribuir a reducir las tasas de desempleo al facilitar la transición de los desempleados hacia empleos adecuados, enfatizando la importancia de la igualdad de oportunidades y el desarrollo de habilidades en grupos minoritarios y desfavorecidos.
narrativa do dilúvio em Gênesis e a ciência
Authors: Silva Robson Barbosa
Published: December 2023

O presente artigo trata das possibilidades no que tange à interpretação da narrativa bíblica relativa ao dilúvio em face dos conhecimentos concernentes às ciências da Terra. Busca mostrar que a descrição do cataclismo em Gênesis está condicionada à cosmologia dos antigos hebreus, e que insistir na exatidão do que lá é dito só faz sentido com o compromisso com tal cosmologia. Trata brevemente dos percalços da “busca pelo dilúvio histórico”, assim como da tese do dilúvio do Mar Negro, o qual pode estar na origem da narrativa bíblica e das várias outras descrições de um dilúvio no mundo antigo. Também discorre sobre como o entendimento acerca da natureza do mito e da revelação pode oferecer interessantes perspectivas para a leitura e interpretação do relato bíblico. Este, sendo possivelmente referência a uma grande catástrofe em meio à qual muitos podem ter visto manifestos juízo e salvação divinos, guarda analogia com acontecimentos como a queda de Jerusalém, quando o juízo de Deus cai sobre a nação impenitente, ao mesmo tempo que há a expectativa da revelação da graça em favor de um remanescente, com a restauração de sua pátria e culto. Também é paradigma de toda e qualquer situação onde se experimentem juízo e graça.
Clinical Supervision to Improve the Performance of Elementary Teachers in Generation Alpha
Authors:   Universitas Lampung, Hestiningtyas Widya , Nurzafira Istiqomah , Rosidin UU , Hariri Hasan , Sowiyah Sowiyah
Published: December 2023

The purpose of this study was to describe the application of clinical supervision at the elementary school stage in Lampung Province. This research focuses on combining innovate approaches to clinical supervision with a focus on improving the performance of elementary school teachers in facing the unique challenges of Generation Alpha. The research was conducted in three areas of Lampung Province, namely elementary schools in Bandar Lampung City, Metro City and South Lampung Regency. The research was conducted with a qualitative approach. Sources of research data are school principals and teachers. Data collection techniques use interviews, observation, and documentation techniques. The results of research related to the problems experienced by elementary school teachers are the teacher's lack of interest in using learning media, students' disinterest in learning media, and the teacher's lack of mastery of technology, information, and communication (TIK). Provision of solutions by supervisors, namely in the form of skills training to develop teacher learning media, communicate learning media plans, and learning training based on TIK.
Remodelling Business UMK “Bakso Sip”: Ten Types of Innovation
Authors:   Universitas Muhammadiyah Gresik, Hidhayah Anysha Nur , Rahmi Vembri Aulia , Kurniawan Aries , Ismanto Hadi
Published: December 2023

This study aims to determine how the innovation was used Business model canvas and Ten Types Innovation methods. The research uses a qualitative-descriptive method. Data collection by interview, observation and documentation with data analysis techniques used the Ten Types innovation method approach. "Bakso SIP" is located in Pekauman Village, South Banjarmasin District, Banjarmasin City, South Kalimantan Province. The results showed that the owner of "Bakso SIP" has made three innovations in ten types of innovation, namely process innovation, channel innovation, and customer engagement, so that the business model of "Bakso SIP" has changed (remodeling) the Business model canvas on the element of Customer segments which used to only reach residents of the Pekauman area, Banjarmasin then more widely reach residents of Banjarmasin City. Customer relationships that used to be only with direct transactions then make reservations in advance via WhatsApp Business, Instagram. The value proposition is a change in service online and key partnes also joined with online application.
The Influence Of Need For Achievement, Locus Of Control And Self Efficacy On Interest In Entrepreneurship
Authors:   Universitas Siliwangi, Nabilah Salma , Sadiah Ati , Srigustini Astri
Published: December 2023

This research aims to determine the influence of Need for Achievement, Locus of Control and Self Efficacy on Entrepreneurial Interest. This research uses a survey research method with a quantitative approach. The population of students at SMK Negeri 2 Tasikmalaya is 2,649 people. The sampling technique used was the Proportionate Stratified Random Sampling technique. So the total sample taken was 348 people according to the researchers' considerations. Data collection techniques in this research used questionnaires, statistical tests used multiple linear regression analysis. The research results show that: 1) Need for Achievement has a positive and significant effect on students' entrepreneurial interest. 2) Locus of Control has a positive and significant effect on students' interest in entrepreneurship. 3) Self Efficacy has a positive and significant effect on students' interest in entrepreneurship. 4) Need for Achievement, Locus of Control and Self Efficacy have a positive and significant effect on students' interest in entrepreneurship
The Influence of Technology, Financial Literacy, and Ease of Using E-Wallets on Consumers of Mall Boemi Kedaton Supermarket
Authors:   Universitas Lampung, Candrakanta Cheryl , Pujiati Pujiati , Rufaidah Erlina
Published: December 2023

The development of the times has influenced changes in various areas of life, one of which is finance. The financial field that has undergone changes is transaction activities. In the past, transaction payment activities were carried out by exchanging goods or barter, but at this time it is developing using digital-based transaction payments. These changes occur due to technological advances, financial understanding, and the development of the digital economy. This study aims to determine the influence of technological advances, financial literacy, and perceptions of ease of use of e-wallets on consumers of Chandra supermarket Mall Boemi Kedaton Bandar Lampung. This research is quantitative using descriptive verifiative methods with ex post facto approach and surveys. Sampling in this study used a non-probability sampling method with purposive sampling techniques. The population in this study was consumers of Chandra supermarket Mall Boemi Bandar Lampung with 75 consumers as a sample. The data analysis method uses SPSS version 25 by testing the hypothesis through a simple linear regression test and multiple regression test. This research reveals that technological advancements, financial literacy, and perceived convenience have a positive effect on the use of e-wallets. These three factors play an important role in increasing the tendency of consumers to choose and utilize e-wallets in their digital transactions.
Implementasi Bahasa Pemrograman Python untuk Path analysis
Authors:   Sistem Informasi, STMIK Palangkaraya, Kaestria Rommi , Himmah Elok Faiqotul ,   Sistem Informasi, STMIK Palangkaraya
Published: December 2023

Path analysis merupakan bagian dari Structural Equation Modelling (SEM) yang sering digunakan untuk menyelesaikan masalah diberbagai bidang ilmu. Penyelesaian masalah dengan path analysis membutuhkan bantuan perangkat lunak ataupun bahasa pemrograman untuk mempermudah pengujian model dan analisis data. Penelitian ini bertujuan untuk mengimplementasikan bahasa pemrograman Python untuk menyelesaikan masalah path analysis. Studi kasus dilakukan berdasarkan penelitian yang sebelumnya telah dilakukan penulis yaiu mengenai path analysis untuk menentukan pengaruh mata kuliah prasyarat terhadap mata kuliah lanjutannya dengan menggunakan perangkat lunak statistika, R. Penulis menyelesaikan studi kasus tersebut dengan menggunakan bahasa pemrograman Python kemudian melakukan perbandingan terhadap hasil yang diperoleh dengan menggunakan Python dan R. Penelitian ini merupakan penelitian eksperimen yang meliputi tahap persiapan, pelaksanaan, analisis, dan pengambilan kesimpulan. Studi kasus dilakukan terhadap 94 sampel yaitu mahasiswa yang telah menempuh dan lulus mata kuliah prasyarat dan mata kuliah lanjutannya. Penulis menyelesaikan kasus tersebut dengan menggunakan Python kemudian menganalisis dan membandingkan hasil yang diperoleh ini dengan hasil yang diperoleh dengan menggunakan R pada penelitian sebelumnya. Hasil penelitian menunjukkan bahwa penggunaan bahasa pemrograman Python juga bisa menyelesaikan permasalahan pada path analysis ini dibuktikan dengan hasil yang diperoleh dengan Python tidak jauh berbeda dengan hasil yang diperoleh dengan R.
Estimation of the Amount of the Vaporized Substance from the Surface of an Accidental Spill of a Heated Flammable Liquid
Authors:   FSBEI HE KNRTU, Galeev A.D. , Chistov Yu.S. ,   FSBEI HE KNRTU
Published: December 2023

Petrochemical and oil refining industries are characterized by the handling of large quantities of flammable liquids in a heated state in the technological processes. An adequate assessment of the consequences of accidental spills and leaks of the heated flammable liquids within the procedure of accident risk analysis requires a correct calculation of the mass of evaporated liquid from the surface of the accidental spill. The process of evaporation of the heated liquids received little attention, both in domestic and foreign literature, which cause the need in the further experimental and numerical studies of this phenomenon. Numerical analysis of the evaporation of heated liquids from the surface of accidental spills was performed. A number of substances are selected for the study (benzene, toluene, ethylbenzene, cumene), covering a wide range of liquid properties such as molecular weight and boiling point. In the numerical simulation, the heat exchange of the spill with the underlying solid layer was considered. The influence of the spill thickness and the liquid properties on the mass of the evaporated substance is shown. The results of numerical simulation are compared with the results of calculating the evaporated mass without taking into account the cooling of the liquid over time. As a result of a numerical analysis, it was established that calculating the consequences of accidental spills of the heated flammable liquids without taking into account the cooling of the liquid over time can lead to an overestimation of the evaporated mass by several tens of times. The importance of taking into account heat removal during the evaporation of a heated liquid when estimating the evaporated mass is higher, the higher the boiling point of the liquid and the smaller the thickness of the spill. Based on the results of numerical experiments, a generalizing formula was obtained that allows to quickly estimate the mass of evaporated liquid for the considered series of substances.
Draft Classifier of the Causes of Accidents and Incidents at Hazardous Production Facilities in the Oil and Gas Industry
Authors:   Peter the Great St. Petersburg Polytechnic University, Sazanova N.V. , Kolesnikov E.Yu. ,   Peter the Great St. Petersburg Polytechnic University, Burlov V.G. ,   Peter the Great St. Petersburg Polytechnic University, Rumjanceva N.V. ,   Peter the Great St. Petersburg Polytechnic University
Published: December 2023

Technical investigation of accidents and incidents is carried out in order to establish the technical and organizational causes of accidents at hazardous production facilities. The legislation of the Russian Federation established the mandatory nature of this procedure, which is regulated by Order № 503 adopted by Rostechnadzor. This regulatory document describes the procedure for conducting an investigation and other organizational issues, but there is no more detailed information on the distribution of causes by groups. As the analysis of the materials of such investigations conducted by the authors of this article shows, there is a problem of uniformity in their identification (various experts formulate the causes of accidents and classify them as technical or organizational in different ways, based on their education, their own experience and other factors). Having analyzed more than 100 «lightnings» (information sheets indicating the causes after an accident investigation), posted on the official website of Rostechnadzor in the section Lessons learned from accidents, the authors of the article found some inconsistencies in assigning causes to a specific group, for example, the causes those related to strictly technical ones were correlated with the group of organizational ones. In this regard, there is a need to adopt an appropriate regulatory legal act, which in its content would have a clearer and, as far as possible, detailed classification of the causes of accidents and incidents for hazardous production facilities in the oil and gas industry. In order to ensure the unity of formulations of the causes of accidents and incidents by different experts and to facilitate their machine processing, a draft classification of them is proposed, set out in the article.
Setting and Solving the Problem of Developing an Integrated Safety System at the Explosion- and Fire-hazardous Production Facilities of the Enterprises
Authors:   National Research Moscow State University of Civil Engineering, Gvozdev E.V.
Published: December 2023

The article presents the idea of a new category of complex risk of exposure to hazardous events (accidents and fires). Adjustment of the required reserve should consider the dependence of the influence of services (labor safety, industrial safety and fire safety) on the integrated safety system created at explosion-hazardous production facilities of the enterprises. Attention is focused on the explosion and fire hazards of production facilities of the enterprises categorized by explosion and fire hazards, where flammable liquids and gases are processed, handled, transported, and stored. Enlarged areas were formed that have scientific results on risk assessment in the integrated safety system created at the enterprises under consideration. A model is presented that makes it possible to develop a new scientific and methodological apparatus (methods, methodology, software and hardware) for its use in practice. The goal and tasks requiring solutions to achieve it are formulated. To improve safety at the enterprises under consideration, it was required to solve 2 tasks. During the theoretical solution of problem 1, the conclusions were drawn that the existing and projected integrated safety systems, when compared in the form of mathematical models, are characterized by the presence of many parameters requiring calculations for all risks. It was required to develop a scientific and methodological apparatus for the formation of such a required reserve intended for the maintenance and development of subsystems (labor safety; industrial safety; fire safety), which would contribute to improving the state of the integrated safety system at the enterprises under consideration. In the course of solving the 2nd problem, an example of an experimental verification calculation was demonstrated. It substantiates the positive dynamics in the development of an integrated safety system at the enterprises in question using scientific and methodological apparatus. The author of the article shows the possibility of reducing the risk of damage from accidents and fires by 18 % during the period allocated by the management of production enterprises for the development of the integrated safety system created at them. Solving these problems allows to talk about a significant contribution to the socio-economic development of Russia.
A Method for Controlling Induced Seismicity in the Development of Mineral Deposits
Authors:   OFRC UrO RAN, Vladov Yu.R. ,   Orenburg State Univercity, Nesterenko M.Yu. ,   OFRC UrO RAN, Vladova A.Yu. ,   IPU RAS,   Financial University under the Government of the Russian Federation, Belov V.S. ,   OFRC UrO RAN
Published: December 2023

In conditions of intensive development of mineral deposits, a significant induced component arises, which significantly increases the overall seismicity of the region. This entails the relevance of the problem on a global scale. Scientific and patent analysis of domestic and foreign research shows that there is no information in the open press in the field of induced seismicity management. The proposed and tested method for controlling induced seismicity largely solves this problem. The coefficient characterizing the increase in the cost of a facility planned for construction in a regional context in a seismically active area using the developed method is reduced by 3–5,8 %. This provides a significant technical and economic effect on a large scale. Thus, a new method for controlling induced seismicity in the development of solid mineral deposits using drilling and blasting technologies is more than technically feasible and economically beneficial.
KIDNEY DAMAGE IN CHILDREN WITH A NEW CORONAVIRUS INFECTION CAUSED BY SARS-CoV-2: A SYSTEMATIC REVIEW
Authors:   Chita State Medical Academy, Chita, Zabaykalsky Krai, Russia, Bataeva E.P. , Bogomolova I.K. ,   Chita State Medical Academy, Chita, Zabaykalsky Krai, Russia, Dolina A.B. ,   Chita State Medical Academy, Chita, Zabaykalsky Krai, Russia, Oparina A.G. ,   Chita State Medical Academy, Chita, Zabaykalsky Krai, Russia
Published: December 2023

This bibliographical review is based on studies published in the PubMed Central® (PMC) database between Mar. 2020 and Dec. 2022 using the keywords as follows: “SARS-CoV-2,” “COVID-19,” “kidney damage” and “children.” Information on clinical criteria, laboratory symptoms, possible pathogenetic response pathways and expected long-term outcomes of kidney damage associated with SARS-CoV-2 (Severe Acute Respiratory Syndrome Coronavirus) infection in children was analyzed. An attempt was made to trace the features of manifestations of the new coronavirus infection depending on previous renal pathology. The polyetiological nature of renal tissue lesion is evident, which is the result of direct effect of SARS-CoV-2 virus on the renin-angiotensin-aldosterone system (RAAS), ischemia, hypercoagulation, cytokine storm, rhabdomyolysis, pulmonary-renal pathogenic reaction etc. Kidney damage in the pediatric population has been found to be more frequent in the severe COVID-19 in children with multisystem inflammatory syndrome (MIS) in the setting of admission at the intensive care unit (ICU). Manifestations range from minor urinary syndrome to the development of acute kidney injury (AKI). A number of consequences and cases of the course of some diseases after coronavirus infection in children as well as clinical and laboratory features of coronavirus infection in patients with previous renal pathology are described. The findings initiated the need for the long-term follow-up of children with COVID-19 both with and without renal insufficiency.
Diabetes mellitus and the cognitive functions in childhood and adolescence
Authors:   Siberian State Medical University (SSMU), Tomsk, Russia, Samoilova Yu.G. , Matveeva M.V. ,   Siberian State Medical University (SSMU), Tomsk, Russia, Yun V.E. ,   Siberian State Medical University (SSMU), Tomsk, Russia, Koshmeleva M.V. ,   Siberian State Medical University (SSMU), Tomsk, Russia, Kudlay D.A. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia,   M.V. Lomonosov Moscow State University, Moscow, Russia,   National Research Center Institute of Immunology of the Russian Federal Biomedical Agency, Moscow, Russia, Galyukova D.E. ,   Siberian State Medical University (SSMU), Tomsk, Russia, Kachanov D.A. ,   Siberian State Medical University (SSMU), Tomsk, Russia, Trifonova E.I. ,   Siberian State Medical University (SSMU), Tomsk, Russia, Sivolobova T.V. ,   Siberian State Medical University (SSMU), Tomsk, Russia, Mutalimi V.E. ,   Siberian State Medical University (SSMU), Tomsk, Russia
Published: December 2023

Type 1 diabetes mellitus (DM1) is widespread among children and adolescents in Russia. In addition to the pretty well-studied microvascular complications of the disease, scientific sources also describe the brain damage that is manifested by cognitive impairment. Despite the problem being studied actively by researchers Worldwide, they have reported mixed and sometimes contractionary results. Thus, as of now there are disputes about the etiology, pathogenesis, clinical manifestations and factors affecting the development of the abovementioned complications. Changes in the brain are registered at all age periods with special attention being fairly paid in childhood and adolescence because such disorders of carbohydrate metabolism during active growth can lead not only to functional, but also to structural changes in the brain. This is manifested by memory impairment and decreased attention concentration, which in its turn negatively affects the academic performance of schoolchildren and therefore reduces the quality of life of patients. Further study of this problem would help developing an algorithm for early diagnosis of cognitive impairment among children and adolescents with DM1 in order to increase compliance among patients, reduce the risk of microvascular complications and possible disability in older age. A promising direction in the diagnosis of cognitive disorders are neuroimaging methods, which, as opposed to neuropsychological tests, are helping to identify morphofunctional changes in the brain. This bibliographical review describes the etiology of cognitive disorders in children and adolescents with DM1, the main variants of cognitive dysfunction, the effect of the disease on schooling performance and neuroimaging disorders of the brain.
Autoinflammatory diseases in the pediatric neonatologist’s and physician’s practices: series of clinical cases
Authors:   Novosibirsk State Medical University, Novosibirsk, Russia, Belousova T.V. ,   Novosibirsk Oblast Regional Clinical Hospital, Novosibirsk, Russia,   Novosibirsk Oblast Regional Perinatal Center, Novosibirsk, Russia, Loskutova S.A. ,   Novosibirsk State Medical University, Novosibirsk, Russia, Zatolokina A.O. ,   Novosibirsk State Medical University, Novosibirsk, Russia,   Novosibirsk Oblast Regional Clinical Hospital, Novosibirsk, Russia, Svershchevskaya T.Yu. ,   Novosibirsk Oblast Regional Perinatal Center, Novosibirsk, Russia, Kolotova E.N. ,   Novosibirsk Oblast Regional Perinatal Center, Novosibirsk, Russia
Published: December 2023

Molecular genetic analysis in the current clinical practice is a kind of must-do standard in the diagnosis of rare (orphan) diseases and syndromes in pediatric patients, which gives the opportunity for making a diagnosis at an early stage, early period of life and followed by the initiation of a targeted pathogenetic therapy, if and when needed, that in its turn is able to improve the prognosis not only for the health, but also for the quality of life and in some cases for the survival of a patient. As for now, this is an overall accessible diagnostic method the use of which is nevertheless often associated with certain difficulties, such as the need for going through the path of a complex differential diagnosis and the awareness and targeted qualification among pediatric practitioners and neonatologists. Authors presents the description of the two clinical cases of a rare autoinflammatory disease, cryopyrin-associated periodic syndrome (CAPS) in children as a demonstration of the way to verify a clinical diagnosis in childhood and the features of diagnosing orphan diseases in a Russian regional hospital. Conclusion: the presented clinical observations demonstrate the capabilities of modern medicine in making a clinical diagnosis of an orphan disease from the group of autoinflammatory diseases, CAPS in particular, starting from the neonatal period of life of a patient.
Allogeneic hematopoietic stem cell transplantation in a pediatric patient with alpha-mannosidosis
Authors:   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Machneva E.B. , Mezentseva A.V. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Burya A.E. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Olkhova L.V. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Kuritsyna M.S. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Nikolaeva Yu.А. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Pristanskova E.A. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Mikhailova S.V. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia, Skorobogatova E.V. ,   Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia, Moscow, Russia
Published: December 2023

Alpha-mannosidosis is a rare autosomal recessive hereditary disease from the group of lysosomal storage diseases with diverse clinical manifestations, the most typical of which are neurodegenerative process, hearing loss, skeletal deformities and immunodeficiency. The only radical treatment for this disease currently is allogeneic hematopoietic stem cell transplantation (allo-HSCT). Successfully performed allo-HSCT not only allows stoppage of the disease progression, but also leads to partial improvement in previously developed complications. The Article provides general information on the disease and demonstrates the Authors’ own experience of performing allo-HSCT in a patient with alpha-mannosidosis as well.
Rare hereditary congenital heart defects and skeletal malformations syndrome: Russia’s first description of a clinical case
Authors:   Research and Clinical Institute of Pediatrics named after Academician Yuri Veltischev with the Pirogov Russian National Research Medical University, Moscow, Russia, Demikova N.S. ,   Russian Medical Academy for Continuous Professional Education, Moscow, Russia, Kotalevskaya Yu.Yu. ,   M.F. Vladimirsky Moscow Oblast Regional Scientific and Research Clinical Institute (MONIKI), Moscow, Russia
Published: December 2023

Russia’s first description of the congenital heart defects and skeletal malformations syndrome (CHDSKM, https://www.omim.org/entry/617602) is presented. The disease is inherited in an autosomal dominant type and is caused by missense variants in the ABL1 gene in most cases, which is a proto-oncogene, which in its turn is encoding the cytoplasmic and nuclear protein tyrosine kinase. The data on the clinical manifestations of the syndrome is under accumulation as yet with only 19 clinical cases described in scientific sources that are forming a spectrum of frequently encountered symptoms. The description of the case facilitates to the accumulation of clinical diversity of symptoms of the disease contributing to its spectrum expansion and confirming the multisystemic nature of the disease’s clinical manifestations.
Genetically determined progressive cardiac conduction disease in a child with SCN5A misregulation
Authors:   Research and Clinical Institute of Pediatrics named after Academician Yuri Veltischev with the Pirogov Russian National Research Medical University, Moscow, Russia, Polyakova E.B. , Yakshina A.Yu. ,   Research and Clinical Institute of Pediatrics named after Academician Yuri Veltischev with the Pirogov Russian National Research Medical University, Moscow, Russia, Bereznitskaya V.V. ,   Research and Clinical Institute of Pediatrics named after Academician Yuri Veltischev with the Pirogov Russian National Research Medical University, Moscow, Russia, Shcherbakova N.V. ,   Research and Clinical Institute of Pediatrics named after Academician Yuri Veltischev with the Pirogov Russian National Research Medical University, Moscow, Russia
Published: December 2023

Progressive cardiac conduction disease (PCCD) refers to a group of genetic disorders that are extremely rare and can be caused by mutations in various genes, including SCN5A. In addition to PCCD, genetic variants of SCN5A can have other diverse phenotypic manifestations in forms of sick sinus syndrome, also known as sinus node dysfunction (SND), atrial fibrillation (AF), Brugada syndrome, long QT syndrome etc. Authors present a clinical case of genetically determined PCCD that had combination of SND (tachy-bradycardia syndrome), AF and intraventricular conduction disorders in a child with histologically confirmed atria fibrosis. Two variants in SCN5A were revealed in the female pediatric patient in a compound heterozygous state during a molecular genetic study. The patient was implanted with a permanent pacemaker, but further progression of the disease led to the manifestation of ventricular tachyarrhythmias and her death. Histological examination confirmed significant replacement of the atrial myocardium with fibrous tissue. Further accumulation of knowledge about the pathogenesis, the course and prognosis of genetically determined cardiac arrhythmias would help identify patients with life-threatening disorders among all children with SCN5A misregulations.
Guidelines for Practitioners: Duchenne Muscular Dystrophy (DMD). Becker Muscular Dystrophy (BMD). Early diagnosis. Therapy with the use of drugs that are currently not registered properly in Russia/by the Ministry of Healthcare of Russia
Authors:   Charity Foundation for the development of systemic care for patients with Duchenne Muscular Dystrophy “Gordey,” Moscow, Russia, Gremyakova T.A. , Anisimova I.V. , Artemyeva S.B. , Baybarina E.N. , Baranov A.A. , Belousova E.D. , Brzhezinskaya L.B. , Butry S.A. , Varnakhina O.A. , Vashakmadze N.D. , Vdovenko I.Yu. , Vladimirova N.N. , Vlodavets D.V. , Gnetetskaya V.A. , Dvornikova T.A. , Groznova O.S. , Guzeva V.I. , Gusakova E.V. , Dadali E.L. , Gremyakova O.I. ,   Charity Foundation for the development of systemic care for patients with Duchenne Muscular Dystrophy “Gordey,” Moscow, Russia, Zhurkova N.V. , Zakharova E.Yu. , Zinina E.V. , Izhevskaya V.L. , Karpacheva N.G. , Kuzenkova L.M. , Kutsev S.I. , Lavrova A.E. , Liseicheva E.A. , Mikhaylova S.V. , Nazarenko L.P. , Namazova-Baranova L.S. , Nikitin S.S. , Paunova S.S. , Pechatnikоva N.L. , Podkletnova T.V. , Polevichenko E.V. , Polyakov A.V. , Ragozin A.K. , Romanenko E.V. , Rudenko D.I. , Tsvetkova E.M. , Sakbaeva G.E. , Sapego E.Yu. , Smirnova N.S. , Smirnova O.Ya. , Stepanov A.A. , Suslov V.M. , Tozliyan E.V. , Rumyantsev A.G. ,   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia,   Russian Academy of Sciences (RAS), Moscow, Russia,   State Duma of the Federal Assembly of Russia, Moscow, Russia,   Editorial of the Journal PEDIATRIA named after G.N. Speransky, Moscow, Russia, Shakhovskaya N.I. , Shreder E.V.
Published: December 2023

Supplement to the 2023 Issue 6 (Volume 102) of the Journal PEDIATRIA named after G.N. Speransky. Guidelines for Practitioners: Duchenne Muscular Dystrophy (DMD). Becker Muscular Dystrophy (BMD). Early diagnosis. Therapy with the use of drugs that are currently not registered properly in Russia/by the Ministry of Healthcare of Russia. DOI: 10.24110/0031-403X-2023-102-6-217-256. https://doi.org/10.24110/0031-403X-2023-102-6-217-256
Hereditary forms of prion disease in pediatric practice: bibliographic review and series of clinical cases
Authors:   Academician N.P. Bochkov Research Centre of Medical Genetics (RCMG) of the Ministry of Science and Higher Education of the Russian Federation, Moscow, Russia, Voskanyan A.E. , Semenova N.A. ,   Academician N.P. Bochkov Research Centre of Medical Genetics (RCMG) of the Ministry of Science and Higher Education of the Russian Federation, Moscow, Russia, Golovteev A.L. ,   Epilepsy Center, LLC, Moscow, Russia, Zhmurova-Kriventsova A.A. ,   “Fantasy” Children’s Clinic, Moscow, Russia, Titova O.N. ,   Federal Research Centre for Nutrition, Biotechnology and Food Safety, Moscow, Russia, Stepanova A.A. ,   Academician N.P. Bochkov Research Centre of Medical Genetics (RCMG) of the Ministry of Science and Higher Education of the Russian Federation, Moscow, Russia, Ismagilova O.R. ,   Academician N.P. Bochkov Research Centre of Medical Genetics (RCMG) of the Ministry of Science and Higher Education of the Russian Federation, Moscow, Russia, Shchagina O.A. ,   Academician N.P. Bochkov Research Centre of Medical Genetics (RCMG) of the Ministry of Science and Higher Education of the Russian Federation, Moscow, Russia, Dumova S.V. ,   Pirogov Russian National Research Medical University, Moscow, Russia, Chugunova O.L. ,   Pirogov Russian National Research Medical University, Moscow, Russia
Published: December 2023

As for now, there is very limited and surely insufficient scientific data published Worldwide on the manifestation of hereditary forms of prion disease in childhood. Authors provide brief overview of PRNP-associated prion diseases and the description of their own two clinical cases of this disease with manifestation in childhood. Both of the observed patients had many similar clinical manifestations at the onset of the disease at the age of 15 to 16 y/o, such as: predominance of neuropsychiatric symptoms at the onset, rapid progression of the disease, early development of dementia and the lethal outcome within 1 to 1.5 years. Genotype that had been identified by the results of a molecular genetic study was also similar in both patients: the repeatedly described mutation D178N in combination with the M129M polymorphism in the PRNP gene. Prion disease in all cases initially occurred under the guise of mental disorders, making it difficult to suspect a diagnosis at an early stage. Considering the extreme rarity of the manifestation of hereditary forms of prion disease in childhood, the Article is drawing attention to the current problematics connected with low alertness and delayed diagnosis of this pathology in pediatric practice.