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5. Clinical and functional spectrum of RAC2-related immunodeficiency

Author

Donkó, Ágnes, Sharapova, Svetlana O., Kabat, Juraj, Ganesan, Sundar, Hauck, Fabian H., Bergerson, Jenna R. E., Marois, Louis, Abbott, Jordan, Moshous, Despina, Williams, Kelli W., Campbell, Nicholas, Martin, Paul L., Lagresle-Peyrou, Chantal, Trojan, Timothy, Kuzmenko, Natalia B., Deordieva, Ekaterina A., Raykina, Elena V., Abers, Michael S., Abolhassani, Hassan, Barlogis, Vincent, Milla, Carlos, Hall, Geoffrey, Mousallem, Talal, Church, Joseph, Kapoor, Neena, Cros, Guilhem, Chapdelaine, Hugo, Franco-Jarava, Clara, Lopez-Lerma, Ingrid, Miano, Maurizio, Leiding, Jennifer W., Klein, Christoph, Stasia, Marie José, Fischer, Alain, Hsiao, Kuang-Chih, Martelius, Timi, Sepännen, Mikko R. J., Barmettler, Sara, Walter, Jolan, Masmas, Tania N., Mukhina, Anna A., Falcone, Emilia Liana, Kracker, Sven, Shcherbina, Anna, Holland, Steven M., Leto, Thomas L., and Hsu, Amy P.

Published
2024
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6. Regional Features of Digital Transformation During the Pandemic

Author

Petrov, Igor V., Plyasova, Svetlana V., Kolomytseva, Olga Y., Yakovleva, Irina K., Kuzmenko, Natalia I., Pisello, Anna Laura, Editorial Board Member, Hawkes, Dean, Editorial Board Member, Bougdah, Hocine, Editorial Board Member, Rosso, Federica, Editorial Board Member, Abdalla, Hassan, Editorial Board Member, Boemi, Sofia-Natalia, Editorial Board Member, Mohareb, Nabil, Editorial Board Member, Mesbah Elkaffas, Saleh, Editorial Board Member, Bozonnet, Emmanuel, Editorial Board Member, Pignatta, Gloria, Editorial Board Member, Mahgoub, Yasser, Editorial Board Member, De Bonis, Luciano, Editorial Board Member, Kostopoulou, Stella, Editorial Board Member, Pradhan, Biswajeet, Editorial Board Member, Abdul Mannan, Md., Editorial Board Member, Alalouch, Chaham, Editorial Board Member, O. Gawad, Iman, Editorial Board Member, Nayyar, Anand, Editorial Board Member, Amer, Mourad, Series Editor, Buchaev, Yakhya G., editor, Abdulkadyrov, Arsen S., editor, Ragulina, Julia V., editor, Khachaturyan, Arutyun A., editor, and Popkova, Elena G., editor

Published
2023
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14. Management of Corporate Rights of the State: Evaluation of Efficiency and Directions of Reforming

Author

Koliada Tetiana A. and Kuzmenko Natalia V.

Subjects
management of corporate rights of the state, efficiency of corporate management, revenues to the budget from the management of the state property, Business, HF5001-6182
Abstract

The article is aimed at defining the essence of corporate management, evaluating the efficiency of corporate management, disclosing the factors of influence and substantiating the main directions of reforms in this sphere in Ukraine. It is proved that an appropriate consideration of the efficiency of management of corporate rights of the State be made through the prism of revenues received to the budget and/or the public goods together with services provided. Enterprises with a State-shared ownership in the statutory capital pay taxes to the budget under the general tax system, same as enterprises with other forms of ownership. At the same time, part of the net profit and dividends in the form of non-tax payments are additionally transferred to the budget. Based on the study of dynamics of the share of revenues to the State Budget of Ukraine from the management of corporate rights of the State for 2014-2018, it is proved that there is a trend of increasing the fiscal potential of public property. This indirectly demonstrates the efficiency of property management, which is the object of the rights of the State. The main factors influencing the amount of revenue received from the management of public property are defined; the main threats to the increase of efficiency of corporate management of the State property are disclosed. Prospect for further research in this direction is to bring the need to provide political support for the introduced reforms in the management of the State-owned enterprises to implement all the benefits from corporate management of the rights of the State, taking into account the best foreign experience and national traditions.

Published
2019
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15. Humanitarian Disciplines in the Conditions of Distance Learning at the Stage of Higher Education Transformation in Ukraine.

Author

Bohomaz, Kostiantyn, Sorokina, Liudmyla, Voronova, Zoia, Valuieva, Nataliia, and Kuzmenko, Natalia

Subjects
DISTANCE education, HUMANITIES education, TEACHING methods, TECHNOLOGICAL progress, COVID-19 pandemic, DIGITAL technology
Abstract

Humanities play a vital role in shaping intellectual, social, and emotional aspects in children. The COVID-19 pandemic and the rise of distance learning have transformed humanities education in Ukrainian universities. It is essential to examine the specific changes brought about by distance learning and their impact on humanities education in the country. The main research methods used were analysis, abstraction, and forecasting. The study reviewed the challenges faced by teachers and students in Ukraine regarding the introduction of digital technologies in education, particularly in the humanities. Furthermore, the role and possibilities of using technology in distance learning were described, as well as the importance of live communication for learning the humanities. The study also assesses the practice of distance learning in different countries. It was noted that countries with supranational organisations involved in the development of distance learning platforms have a more developed infrastructure and a wider range of available resources. Interactive teaching methods and digital educational materials are widely used in foreign institutions, promoting independent learning and improving access to knowledge. The study showed different approaches to distance teaching and describes how feasible their implementation was in the Ukrainian context. [ABSTRACT FROM AUTHOR]

Published
2024
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16. Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome

Author

Leiding, Jennifer W., primary, Vogel, Tiphanie P., additional, Santarlas, Valentine G.J., additional, Mhaskar, Rahul, additional, Smith, Madison R., additional, Carisey, Alexandre, additional, Vargas-Hernández, Alexander, additional, Silva-Carmona, Manuel, additional, Heeg, Maximilian, additional, Rensing-Ehl, Anne, additional, Neven, Bénédicte, additional, Hadjadj, Jérôme, additional, Hambleton, Sophie, additional, Ronan Leahy, Timothy, additional, Meesilpavikai, Kornvalee, additional, Cunningham-Rundles, Charlotte, additional, Dutmer, Cullen M., additional, Sharapova, Svetlana O., additional, Taskinen, Mervi, additional, Chua, Ignatius, additional, Hague, Rosie, additional, Klemann, Christian, additional, Kostyuchenko, Larysa, additional, Morio, Tomohiro, additional, Thatayatikom, Akaluck, additional, Ozen, Ahmet, additional, Scherbina, Anna, additional, Bauer, Cindy S., additional, Flanagan, Sarah E., additional, Gambineri, Eleonora, additional, Giovannini-Chami, Lisa, additional, Heimall, Jennifer, additional, Sullivan, Kathleen E., additional, Allenspach, Eric, additional, Romberg, Neil, additional, Deane, Sean G., additional, Prince, Benjamin T., additional, Rose, Melissa J., additional, Bohnsack, John, additional, Mousallem, Talal, additional, Jesudas, Rohith, additional, Santos Vilela, Maria Marluce Dos, additional, O’Sullivan, Michael, additional, Pachlopnik Schmid, Jana, additional, Průhová, Štěpánka, additional, Klocperk, Adam, additional, Rees, Matthew, additional, Su, Helen, additional, Bahna, Sami, additional, Baris, Safa, additional, Bartnikas, Lisa M., additional, Chang Berger, Amy, additional, Briggs, Tracy A., additional, Brothers, Shannon, additional, Bundy, Vanessa, additional, Chan, Alice Y., additional, Chandrakasan, Shanmuganathan, additional, Christiansen, Mette, additional, Cole, Theresa, additional, Cook, Matthew C., additional, Desai, Mukesh M., additional, Fischer, Ute, additional, Fulcher, David A., additional, Gallo, Silvanna, additional, Gauthier, Amelie, additional, Gennery, Andrew R., additional, Gonçalo Marques, José, additional, Gottrand, Frédéric, additional, Grimbacher, Bodo, additional, Grunebaum, Eyal, additional, Haapaniemi, Emma, additional, Hämäläinen, Sari, additional, Heiskanen, Kaarina, additional, Heiskanen-Kosma, Tarja, additional, Hoffman, Hal M., additional, Gonzalez-Granado, Luis Ignacio, additional, Guerrerio, Anthony L., additional, Kainulainen, Leena, additional, Kumar, Ashish, additional, Lawrence, Monica G., additional, Levin, Carina, additional, Martelius, Timi, additional, Neth, Olaf, additional, Olbrich, Peter, additional, Palma, Alejandro, additional, Patel, Niraj C., additional, Pozos, Tamara, additional, Preece, Kahn, additional, Lugo Reyes, Saúl Oswaldo, additional, Russell, Mark A., additional, Schejter, Yael, additional, Seroogy, Christine, additional, Sinclair, Jan, additional, Skevofilax, Effie, additional, Suan, Daniel, additional, Suez, Daniel, additional, Szabolcs, Paul, additional, Velasco, Helena, additional, Warnatz, Klaus, additional, Walkovich, Kelly, additional, Worth, Austen, additional, Seppänen, Mikko R.J., additional, Torgerson, Troy R., additional, Sogkas, Georgios, additional, Ehl, Stephan, additional, Tangye, Stuart G., additional, Cooper, Megan A., additional, Milner, Joshua D., additional, Forbes Satter, Lisa R., additional, Aleshkevich, Svetlana, additional, Allende, Luis M., additional, Atkinson, T. Prescott, additional, Atschekzei, Faranaz, additional, Aydemir, Sezin, additional, Aygunes, Utku, additional, Barlogis, Vincent, additional, Baumann, Ulrich, additional, Belko, John, additional, Bezrodnik, Liliana, additional, Biebl, Ariane, additional, Broderick, Lori, additional, Bunin, Nancy J., additional, Caldirola, Maria Soledad, additional, Castelle, Martin, additional, Celmeli, Fatih, additional, Charbonnier, Louis-Marie, additional, Chatila, Talal A., additional, Chellapandian, Deepak, additional, Cokugras, Haluk, additional, Conlon, Niall, additional, Cox, Fionnuala, additional, Crickx, Etienne, additional, Dalgic, Buket, additional, ASH Dalm, Virgil, additional, Danielian, Silvia, additional, Dominguez-Pinilla, Nerea, additional, Dujovny, Tal, additional, Ebbo, Mikael, additional, Eken, Ahmet, additional, Esty, Brittany, additional, Fabre, Alexandre, additional, Fischer, Alain, additional, Hannibal, Mark, additional, Huppert, Laura, additional, Ikeda, Marc D., additional, Jolles, Stephen, additional, Jolly, Kent W., additional, Jones, Neil, additional, Kanariou, Maria, additional, Karakoc-Aydiner, Elif, additional, Karamantziani, Theoni, additional, Kelaidi, Charikleia, additional, Keogan, Mary, additional, Pac Kisaarslan, Ayşenur, additional, Kiykim, Ayca, additional, Kotsonis, Kosmas, additional, Kuzmenko, Natalia, additional, Leroy, Sylvie, additional, Lianou, Dimitra, additional, Longhurst, Hilary, additional, Lorenz, Myriam Ricarda, additional, Maffucci, Patrick, additional, Manson, Ania, additional, Marchal, Sarah, additional, Malphettes, Marion, additional, Marega, Lia Furlaneto, additional, Mauracher, Andrea A., additional, Miller, Holly, additional, Mombourquette, Joy, additional, Morgan, Noel G., additional, Mukhina, Anna, additional, Nathalie, Aladjidi, additional, Nelken, Brigitte, additional, Nolan, David, additional, Norlin, Anna-Carin, additional, Oleastro, Matias, additional, Ozcan, Alper, additional, Pasquet, Marlene, additional, Pegler, José Roberto, additional, Picard, Capucine, additional, Polychronopoulou, Sophia, additional, Quartier, Pierre, additional, Quesada, Juan Francisco, additional, Ramakers, Jan, additional, Randall, Katrina L., additional, Rao, V. Koneti, additional, Remiker, Allison, additional, Resin, Geraldine, additional, Richmond, Peter, additional, Rieux-Laucat, Frederic, additional, Rodina, Yulia, additional, Rohrlich, Pierre, additional, Sachs, Johnathan, additional, Sakovich, Inga, additional, Santarlas, Christopher, additional, Sari, Sinan, additional, Sawicki, Gregory, additional, Schauer, Uwe, additional, Scheffler Mendoza, Selma C., additional, Schvetz, Oksana, additional, Schmidt, Reinhold Ernst, additional, Schwarz, Klaus, additional, Sediva, Anna, additional, Sinclair, Kyle, additional, Slatter, Mary, additional, Sleasman, John, additional, Stergiou, Katerina, additional, Suratannon, Narissara, additional, Tanita, Kay, additional, Thompson, Grace, additional, Travis, Stephen, additional, Trojan, Timothy, additional, Tsinti, Maria, additional, Unal, Ekrem, additional, Urdinez, Luciano, additional, Vazquez-Gomez, Felisa, additional, Villa, Mariana, additional, Weinrich, Michael, additional, Weiss, Mitchell J., additional, Wright, Benjamin, additional, Yilmaz, Ebru, additional, Zachova, Radana, additional, and Zhang, Yu, additional

Published
2023
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24. GPS Usage Analysis for Angular Orientation Practical Tasks Solving

Author

Dergachov, Kostiantyn, primary, Havrylenko, Olena, additional, Pavlikov, Vladimir, additional, Zhyla, Simeon, additional, Tserne, Eduard, additional, Volosyuk, Valerii, additional, Ruzhentsev, Nikolay, additional, Ostroumov, Ivan, additional, Averyanova, Yuliya, additional, Sushchenko, Olga, additional, Popov, Anatoly, additional, Shmatko, Oleksandr, additional, Solomentsev, Oleksandr, additional, Zaliskyi, Maksym, additional, Kuzmenko, Natalia, additional, Kuznetsov, Borys, additional, and Nikitina, Tatiana, additional

Published
2022
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26. Highly Luminescent Rb-Doped Cs 4 PbBr 6 Nanocrystals in Borogermanate Glass.

Author

Valiev, Damir, Kharisova, Rufina, Babkina, Anastasiia, Zyryanova, Ksenia, Kuzmenko, Natalia, Sgibnev, Yevgeniy, Shelaev, Artem, and Baryshev, Alexander V.

Subjects
NANOCRYSTALS, CRYSTAL glass, GLASS, RUBIDIUM, GLASS-ceramics, LUMINESCENCE, PEROVSKITE
Abstract

For the first time, the synthesis, luminescent and structural properties of stable perovskite-type (Cs1−xRbx)4PbBr6 (R = Cs, Rb) nanocrystals are shown. In the absence of rubidium, Cs4PbBr6 and CsPbBr3 perovskite crystals precipitate in the ZnO–Na2O–B2O3–GeO2 glass matrix. With ascending rubidium content, the precipitation of (Cs,Rb)4PbBr6 nanocrystals is replaced by the Rb4PbBr6 nanocrystals nucleation. Nucleated nanocrystals exhibit an intense green luminescence. With an increase of the rubidium content, the luminescence maximum shifts to the blue region, the luminescence quantum yield increases from 28 to 51%, and the average decay time increases from 2 to 8 ns. Several assumptions have been made about the nature of the green luminescence of perovskite-like Cs4PbBr6 and (Cs,Rb)4PbBr6 crystals in glasses. It is concluded that the most probable cause is the impurity inclusions of CsPbBr3 and (Cs,Rb)PbBr3 crystals. [ABSTRACT FROM AUTHOR]

Published
2023
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31. Structural and Luminescence Properties of (Gd 1−x Y x) 2 O 3 Powders Doped with Nd 3+ Ions for Temperature Measurements.

Author

Aseev, Vladimir, Babkina, Anastasiia, Evstropiev, Sergey, Kuzmenko, Natalia, Sevastianova, Irina, Prokuratov, Denis, and Khodasevich, Mikhail

Subjects
NEODYMIUM compounds, METAL ions, TEMPERATURE measurements, RARE earth oxides, PHOTOLUMINESCENCE
Abstract

Rare earth activated powders are widely regarded as promising candidates for optical thermometry due to their unique photoluminescence characteristics. The paper presents the structural and luminescent properties of crystalline powders of gadolinium and yttrium oxides (Gd1−xYx)2O3, doped with Nd3+ ions, synthesized by the liquid polymer-salt method. The addition of polyvinylpyrrolidone increases the homogeneity of the mixture and ensures high adhesion of the resulting powders. Scanning electron microscopy shows that powders are μm-sized aggregates, which consist of particles with several tens of nanometers in size. A smooth shift of the diffraction peaks of the powders occurs when Gd is replaced by Y without additional peaks. The successive decrease in the lattice constant of the powders from 10.816 to 10.607 Å confirms the existence of continuous solid solutions in the system. The Stark sublevels of the 4F3/2 → 4I9/2 fluorescent band are shifted to 4 nm when Gd is replaced by Y since the strength of the local field has a stronger effect on the inner F-shell of Nd ions in the case of Y. For thermometry, we chose the ratio of the fluorescence intensities between the Stark sublevels 4F3/2(2) → 4I9/2(2) and 4F3/2(1) → 4I9/2(2). The best obtained sensitivity is 0.22% °C−1 for Nd-doped GdYO3 powder in the range of 10–70 °C. This value of temperature sensitivity, together with radiation and excitation lying in the biological window, opens the possibility of using Nd3+-doped (Gd1−xYx)2O3 powders for real-time thermal probing of under tissue luminescence with sub-degree resolution. [ABSTRACT FROM AUTHOR]

Published
2022
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32. Primenenie distantsionnykh obrazovatel'nykh tekhnologii v sisteme dopolnitel'nogo obrazovaniia

Author

Ziganshina Elena Anatol'evna, Volobueva Tatiana Pavlovna, and Kuzmenko Natalia Alekseevna

Subjects
дистанционное образование, чат, вебинары, образовательные технологии, форум
Abstract

Статья посвящена актуальной проблеме организации и применения образовательных технологий в дополнительном образовании

Published
2021

34. Long-term prophylaxis therapy in patients with hereditary angioedema in Russia: resolution of the expert Council

Author

Bodnya, Olga S., primary, Demina, Darya V., additional, Kuzmenko, Natalia B., additional, Latysheva, Elena A., additional, Latysheva, Tatiana V., additional, Milichkina, Anzhelika M., additional, Pampura, Alexander N., additional, Sizyakina, Liudmila P., additional, Totolian, Areg A., additional, Uhanova, Olga P., additional, Fedotova, Natalia V., additional, and Fomina, Daria S., additional

Published
2021
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36. Hereditary angioedema. Clinical guidelines. (D84.1)

Author

Bliznetz, Elena A., primary, Viktorova, Ekaterina A., additional, Vishneva, Elena A., additional, Dzhobava, Eliso M., additional, Il'ina, Natalya I., additional, Kuzmenko, Natalia B., additional, Latysheva, Elena A., additional, Latysheva, Tatiana V., additional, Manto, Irina A., additional, Namazova-Baranova, Leyla S., additional, Nenasheva, Natalia M., additional, Pampura, Alexander N., additional, Polyakov, Aleksander V., additional, Selimzianova, Lilia R., additional, and Shcherbina, Anna Yu., additional

Published
2021
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38. Применение дистанционных образовательных технологий в системе дополнительного образования

Author

Зиганшина Елена Анатольевна, МАУ ДО «ЦДО «Успех», Ziganshina Elena Anatol'evna, MAUDO "Tsentr dopolnitel'nogo obrazovaniia "Uspekh", Волобуева Татьяна Павловна, Volobueva Tatiana Pavlovna, Кузьменко Наталья Алексеевна, Kuzmenko Natalia Alekseevna, Зиганшина Елена Анатольевна, МАУ ДО «ЦДО «Успех», Ziganshina Elena Anatol'evna, MAUDO "Tsentr dopolnitel'nogo obrazovaniia "Uspekh", Волобуева Татьяна Павловна, Volobueva Tatiana Pavlovna, Кузьменко Наталья Алексеевна, and Kuzmenko Natalia Alekseevna

Abstract

Статья посвящена актуальной проблеме организации и применения образовательных технологий в дополнительном образовании

Published
2021

39. Liquidation of national districts and village councils in the Ukrainian SSR in the 1930s: organizational and legal aspects

Author

Kuzmenko, Natalia

Subjects
Украинская ССР, национальные меньшинства, конституционные гарантии, национальные административно-территориальные единицы, национальные районы и сельсоветы, права национальных меньшинств, Ukrainian SSR, national minorities, constitutional guarantees, national administrative-territorial units, national districts and village councils, rights of national minorities, УДК 34(3/9), Українська РСР, національні меншин, конституційні гарантії, національні адміністративно-територіальні одиниці, національні райони та сільради, права національних меншин
Abstract

У статті досліджено організаційно-правові аспекти процесу ліквідації національних районів та сільрад в Українській РСР у 1930-х рр. Встановлено, що утворення у 1920-х – на початку 1930-х рр. національних адміністративно-територіальних одиниць сприяло залученню представників національних меншин до основних заходів радянського державотворення, зростанню їх соціально-економічного та національно-культурного рівня, забезпеченню реалізації конституційних прав національних меншин. З’ясовано, що на етапі утвердження тоталітарного режиму керівництво СРСР стало вбачати в існуванні національних районів та сільрад загрозу ізоляції населення від цілісно вибудованої радянської системи пропаганди та перешкоду здійсненню тотального контролю над суспільством. У новій Конституції СРСР 1936 р. та розробленій на її основі Конституції УРСР 1937 р. конституційно-правові гарантії існування національних районів та сільрад зникли, що в умовах радянської дійсності означало їх швидку подальшу ліквідацію. Визначено, що своєрідним каталізатором, який пришвидшив процес ліквідації національних адміністративно-територіальних одиниць стало запровадження у другій половині 1930-х рр. політики депортацій та масових політичних репресій щодо представників національних меншин. З’ясовано, що вимога щодо ліквідації національних районів та сільрад була ухвалена у грудні 1937 р. вищим партійним керівництвом СРСР. Доведено, що процес ліквідації національних районів та сільрад в Україні мав низку особливостей, зумовлених як розгортанням навесні 1938 р. пропагандистської кампанії по виборам першого складу депутатів Верховної Ради УРСР, так і подальшою організаційною підготовкою до проведення у січні 1939 р. повторного перепису населення СРСР, які призвели до тимчасового залишення у якості чинних адміністративно-територіальних одиниць національних районів та сільрад. Встановлено, що навесні 1939 р. на підставі відповідної постанови ЦК КП(б)У та постанови Президії Верховної Ради УРСР національні райони та сільради в Українській РСР припинили своє існування, В статье исследовано организационно-правовые аспекты процесса ликвидации национальных районов и сельсоветов в Украинской ССР в 1930-х гг. Установлено, что создание в 1920-х – начале 1930-х гг. национальных административно-территориальных единиц способствовало вовлечению представителей национальных меньшинств в основные мероприятия советского государственного строительства, росту их социально-экономического и национально-культурного уровня, обеспечению реализации конституционных прав национальных меньшинств. Выяснено, что на этапе установления тоталитарного режима руководство СССР стало видеть в существовании национальных районов и сельсоветов угрозу изоляции населения от целостно выстроенной советской пропагандистской системы и препятствие на пути осуществления тотального контроля над обществом. В новой Конституции СССР 1936 г. и разработанной на ее основе Конституции УССР 1937 г. исчезли конституционно-правовые гарантии существования национальных районов и сельсоветов, что в условиях советской действительности означало их быструю дальнейшую ликвидацию. Установлено, что своего рода катализатором, ускорившим процесс ликвидации национальных административно-территориальных единиц, стало введение во второй половине 30-х годов. политики депортаций и массовых политических репрессий против представителей национальных меньшинств. Выяснено, что требование о ликвидации национальных районов и сельсоветов было принято в декабре 1937 г. высшим партийным руководством СССР. Доказано, что процесс ликвидации национальных районов и сельсоветов в Украине имел ряд особенностей, определенных как развертыванием весной 1938 г. пропагандистской кампании по выборам первого состава депутатов Верховного Совета УССР, так и с дальнейшей организационной подготовкой к январской 1939 г. переписи населения СССР, что привело к временному оставлению в качестве действующих административно-территориальных единиц национальных районов и сельсоветов. Установлено, что весной 1939 г. на основании соответствующего постановления ЦК КП(б)У и постановления Президиума Верховного Совета УССР национальные районы и сельсоветы Украинской ССР прекратили свое существование, The article examines the organizational and legal aspects of the process of liquidation of national districts and village councils in the Ukrainian SSR in the 1930s. It was established, that the formation of national administrative-territorial units in the 1920s - early 1930s contributed to the involvement of representatives of national minorities in the main measures of Soviet state formation, the growth of their socio-economic and national-cultural level, ensuring the realization of constitutional rights of national minorities. It was found out, that at the stage of establishment of the totalitarian regime, the leadership of the USSR began to see the existence of national districts and village councils the threat of isolation of the population from the holistically built Soviet propaganda system and the impediment to the implementation of total control over society. In new Constitution of the USSR in 1936 and developed on its basis constitution of the USSR in 1937 constitutional and legal guarantees of existence of national districts and village councils disappeared, which in conditions of Soviet reality meant their rapid further elimination. It is determined, that a kind of catalyst that accelerated the process of liquidation of national administrative-territorial units was the introduction of the policy of deportations and mass political repression against representatives of national minorities in the second half of the 1930s. It is emphasized, that the requirement to eliminate national districts and village councils was adopted in December 1937 by the highest party leadership of the USSR. It was proved, that the process of liquidation of national districts and village councils in Ukraine had a number of features, due to both the deployment of the propaganda campaign for the election of the first composition of deputies of the Supreme Soviet of the USSR in the spring of 1938, and further organizational preparation for the January 1939 re-census of the population of the USSR, which led to the temporary leaving of national areas and village councils as the current administrative-territorial units. It was established, that national districts and village councils in the Ukrainian SSR ceased to exist in the spring of 1939 on the basis of the relevant resolution of the Central Committee of the Communist Party (b) and the resolution of the Presidium of the Supreme Soviet of the USSR

Published
2020

41. Специфика работы с одарёнными детьми по обучению художественной обработке кожи

Author

Mishenina Natalia Vladimirovna, Kuzmenko Natalia Alekseevna, and Borovets Oksana Sergeevna

Subjects
творческий потенциал, инновационные технологии, композиционные навыки, народное творчество, стандартное или творческое мышление
Abstract

Статья посвящена актуальной теме приобщения учащихся к народному творчеству – художественной обработке кожи.

Published
2020

42. Специфика работы с одарёнными детьми по обучению художественной обработке кожи

Author

Мишенина Наталья Владимировна, МАУ ДО «ЦДО «Успех», Mishenina Natalia Vladimirovna, MAUDO "Tsentr dopolnitel'nogo obrazovaniia "Uspekh", Кузьменко Наталья Алексеевна, Kuzmenko Natalia Alekseevna, Боровец Оксана Сергеевна, Borovets Oksana Sergeevna, Мишенина Наталья Владимировна, МАУ ДО «ЦДО «Успех», Mishenina Natalia Vladimirovna, MAUDO "Tsentr dopolnitel'nogo obrazovaniia "Uspekh", Кузьменко Наталья Алексеевна, Kuzmenko Natalia Alekseevna, Боровец Оксана Сергеевна, and Borovets Oksana Sergeevna

Abstract

Статья посвящена актуальной теме приобщения учащихся к народному творчеству – художественной обработке кожи.

Published
2020

44. Characterization of the clinical and immunologic phenotype and management of 157 individuals with 56 distinct heterozygous NFKB1 mutations

Author

Lorenzini, Tiziana, primary, Fliegauf, Manfred, additional, Klammer, Nils, additional, Frede, Natalie, additional, Proietti, Michele, additional, Bulashevska, Alla, additional, Camacho-Ordonez, Nadezhda, additional, Varjosalo, Markku, additional, Kinnunen, Matias, additional, de Vries, Esther, additional, van der Meer, Jos W.M., additional, Ameratunga, Rohan, additional, Roifman, Chaim M., additional, Schejter, Yael D., additional, Kobbe, Robin, additional, Hautala, Timo, additional, Atschekzei, Faranaz, additional, Schmidt, Reinhold E., additional, Schröder, Claudia, additional, Stepensky, Polina, additional, Shadur, Bella, additional, Pedroza, Luis A., additional, van der Flier, Michiel, additional, Martínez-Gallo, Mónica, additional, Gonzalez-Granado, Luis Ignacio, additional, Allende, Luis M., additional, Shcherbina, Anna, additional, Kuzmenko, Natalia, additional, Zakharova, Victoria, additional, Neves, João Farela, additional, Svec, Peter, additional, Fischer, Ute, additional, Ip, Winnie, additional, Bartsch, Oliver, additional, Barış, Safa, additional, Klein, Christoph, additional, Geha, Raif, additional, Chou, Janet, additional, Alosaimi, Mohammed, additional, Weintraub, Lauren, additional, Boztug, Kaan, additional, Hirschmugl, Tatjana, additional, Dos Santos Vilela, Maria Marluce, additional, Holzinger, Dirk, additional, Seidl, Maximilian, additional, Lougaris, Vassilios, additional, Plebani, Alessandro, additional, Alsina, Laia, additional, Piquer-Gibert, Monica, additional, Deyà-Martínez, Angela, additional, Slade, Charlotte A., additional, Aghamohammadi, Asghar, additional, Abolhassani, Hassan, additional, Hammarström, Lennart, additional, Kuismin, Outi, additional, Helminen, Merja, additional, Allen, Hana Lango, additional, Thaventhiran, James E., additional, Freeman, Alexandra F., additional, Cook, Matthew, additional, Bakhtiar, Shahrzad, additional, Christiansen, Mette, additional, Cunningham-Rundles, Charlotte, additional, Patel, Niraj C., additional, Rae, William, additional, Niehues, Tim, additional, Brauer, Nina, additional, Syrjänen, Jaana, additional, Seppänen, Mikko R.J., additional, Burns, Siobhan O., additional, Tuijnenburg, Paul, additional, Kuijpers, Taco W., additional, Warnatz, Klaus, additional, Grimbacher, Bodo, additional, Adhya, Zoe, additional, Alachkar, Hana, additional, Anantharachagan, Ariharan, additional, Antrobus, Richard, additional, Arumugakani, Gururaj, additional, Ashford, Sofie, additional, Astle, William J., additional, Attwood, Anthony, additional, Bacchelli, Chiara, additional, Batista, Joana, additional, Baxendale, Helen E., additional, Bethune, Claire, additional, Bibi, Shahnaz, additional, Bleda, Marta, additional, Boardman, Barbara, additional, Booth, Claire, additional, Bradley, John R., additional, Breen, Gerome, additional, Brown, Matthew, additional, Browning, Michael J., additional, Brownlie, Mary, additional, Buckland, Matthew S., additional, Burren, Oliver S., additional, Carss, Keren, additional, Chambers, John, additional, Chandra, Anita, additional, Brod, Naomi Clements, additional, Clifford, Hayley, additional, Cooper, Nichola, additional, Daugherty, Louise C., additional, Davies, E.G., additional, Davies, Sophie, additional, Davis, John, additional, Deacock, Sarah, additional, Deevi, Sri V.V., additional, Dempster, John, additional, Devlin, Lisa A., additional, Dewhurst, Eleanor F., additional, Downes, Kate, additional, Drewe, Elizabeth, additional, Duarte, Daniel, additional, Edgar, J. David M., additional, Edwards, Karen, additional, Egner, William, additional, El-Shanawany, Tariq, additional, Erwood, Marie, additional, Fletcher, Debra, additional, Fox, James, additional, Frary, Amy J., additional, Frontini, Mattia, additional, Furnell, Abigail, additional, Gaspar, H. Bobby, additional, Ghurye, Rohit, additional, Gilmour, Kimberly C., additional, Gleadall, Nicholas S., additional, Goddard, Sarah, additional, Gordins, Pavels, additional, Gräf, Stefan, additional, Grassi, Luigi, additional, Greene, Daniel, additional, Grigoriadou, Sofia, additional, Hackett, Scott, additional, Hague, Rosie, additional, Haimel, Matthias, additional, Harper, Lorraine, additional, Hayman, Grant, additional, Herwadkar, Archana, additional, Hu, Fengyuan, additional, Hughes, Stephen, additional, Huissoon, Aarnoud P., additional, James, Roger, additional, Jolles, Stephen, additional, Jolley, Jennifer, additional, Jones, Julie, additional, Karim, Yousuf, additional, Kasanicki, Mary A., additional, Kelleher, Peter, additional, Kempster, Carly, additional, Kiani, Sorena, additional, Kingston, Nathalie, additional, Klein, Nigel, additional, Kostadima, Myrto, additional, Kreuzhuber, Roman, additional, Kumararatne, Dinakantha, additional, Laffan, James, additional, Lear, Sara E., additional, Linger, Rachel, additional, Longhurst, Hilary, additional, Lorenzo, Lorena E., additional, Lyons, Paul A., additional, Maimaris, Jesmeen, additional, Manson, Ania, additional, Mapeta, Rutendo, additional, Martin, Jennifer, additional, McCarthy, Mark I., additional, McDermott, Elizabeth M., additional, McKinney, Harriet, additional, Meacham, Stuart, additional, Megy, Karyn, additional, Millar, Hazel, additional, Mistry, Anoop, additional, Morrisson, Valerie, additional, Murng, Sai H.K., additional, Nasir, Iman, additional, Nejentsev, Sergey, additional, Noorani, Sadia, additional, Oksenhendler, Eric, additional, Ouwehand, Willem H., additional, Papadia, Sofia, additional, Penkett, Christopher J., additional, Petersen, Romina, additional, Ponsford, Mark J., additional, Qasim, Waseem, additional, Quinn, Ellen, additional, Quinti, Isabella, additional, Raymond, F. Lucy, additional, Rayner-Matthews, Paula J., additional, Richter, Alex, additional, Samani, Nilesh, additional, Samarghitean, Crina, additional, Sanchis-Juan, Alba, additional, Sargur, Ravishankar B., additional, Savic, Sinisa, additional, Seneviratne, Suranjith L., additional, Sewell, W.A. Carrock, additional, Seyres, Denis, additional, Shackley, Fiona, additional, Shamardina, Olga, additional, Simeoni, Ilenia, additional, Simpson, Michael A., additional, Smith, Kenneth G.C., additional, Staines, Simon, additional, Staples, Emily, additional, Stark, Hannah, additional, Stauss, Hans, additional, Steele, Cathal L., additional, Stephens, Jonathan, additional, Stirrups, Kathleen E., additional, Thomas, David, additional, Thomas, Moira J., additional, Thomas, Patrick, additional, Thrasher, Adrian J., additional, Tilly, Tobias, additional, Titterton, Catherine, additional, Treadaway, Paul, additional, Tuna, Salih, additional, Turro, Ernest, additional, Urniaz, Rafal, additional, von Ziegenweidt, Julie, additional, Walker, Neil, additional, Watt, Christopher, additional, Welch, Steven B., additional, Whitehorn, Deborah, additional, Willcocks, Lisa, additional, Wood, Nicholas, additional, Wood, Yvette, additional, Workman, Sarita, additional, Worth, Austen, additional, Yates, Katherine, additional, Yeatman, Nigel, additional, Yong, Patrick F.K., additional, Young, Timothy, additional, Yu, Ping, additional, and Zlamalova, Eliska, additional

Published
2020
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45. The Clinical and Genetic Spectrum of 82 Patients With RAG Deficiency Including a c.256_257delAA Founder Variant in Slavic Countries

Author

Sharapova, Svetlana O., primary, Skomska-Pawliszak, Małgorzata, additional, Rodina, Yulia A., additional, Wolska-Kuśnierz, Beata, additional, Dabrowska-Leonik, Nel, additional, Mikołuć, Bozena, additional, Pashchenko, Olga E., additional, Pasic, Srdjan, additional, Freiberger, Tomáš, additional, Milota, Tomáš, additional, Formánková, Renata, additional, Szaflarska, Anna, additional, Siedlar, Maciej, additional, Avčin, Tadej, additional, Markelj, Gašper, additional, Ciznar, Peter, additional, Kalwak, Krzysztof, additional, Kołtan, Sylwia, additional, Jackowska, Teresa, additional, Drabko, Katarzyna, additional, Gagro, Alenka, additional, Pac, Małgorzata, additional, Naumova, Elissaveta, additional, Kandilarova, Snezhina, additional, Babol-Pokora, Katarzyna, additional, Varabyou, Dzmitry S., additional, Barendregt, Barbara H., additional, Raykina, Elena V., additional, Varlamova, Tatiana V., additional, Pavlova, Anna V., additional, Grombirikova, Hana, additional, Debeljak, Maruša, additional, Mersiyanova, Irina V., additional, Bondarenko, Anastasiia V., additional, Chernyshova, Liudmyla I., additional, Kostyuchenko, Larysa V., additional, Guseva, Marina N., additional, Rascon, Jelena, additional, Muleviciene, Audrone, additional, Preiksaitiene, Egle, additional, Geier, Christoph B., additional, Leiss-Piller, Alexander, additional, Yamazaki, Yasuhiro, additional, Kawai, Tomoki, additional, Walter, Jolan E., additional, Kondratenko, Irina V., additional, Šedivá, Anna, additional, van der Burg, Mirjam, additional, Kuzmenko, Natalia B., additional, Notarangelo, Luigi D., additional, Bernatowska, Ewa, additional, and Aleinikova, Olga V., additional

Published
2020
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48. Prevention of friends of the base in the aerobic sessions

Author

Kuzmenko, Natalia Viktorovna

Subjects
осанка, students, аэробика, фізичне виховання, physical exercises, 796.011.1, мышцы, студенты, аеробіка, aerobics, физическое воспитание, м’язи, постава, physical education, фізичні вправи, muscles, студенти, posture, физические упражнения
Abstract

У статті розглядаються засоби та методи профілактики порушень постави на заняттях аеробікою. Неправильна постава спостерігається, як правило, у фізично слабких студентів і тих, які мають негативні звички у повсякденному житті. Формування правильної постави, попередження виникнення дефектів – одне з найважливіших завдань фізичного виховання у вищих навчальних закладах. У формуванні постави значну роль відіграє сила м’язів, їх рівномірний розвиток та гармонійна робота. На навчальному відділенні з аеробіки виконуються вправи загальної фізичної підготовки та спеціальні вправи для формування правильної постави. In the article agitate resources and methods of preventive posture disorders in aerobics classes. Incorrect posture is observed, as a rule, in physically weak students and those, who have negative habits in everyday life. The formation of correct posture, the prevention of defects – one of the most important tasks of physical education in academy. In the formation of posture, the muscle strength plays an important role, their uniform development and harmonious work. At the training department of aerobics exercises of general physical training and special exercises for the formation of correct posture are carried out. В статье рассматриваются средства и методы профилактики нарушений осанки на занятиях аэробикой. Неправильная осанка наблюдается, как правило, в физически слабых студентов и тех, которые имеют негативные привычки в повседневной жизни. Формирование правильной осанки, предупреждение возникновения дефектов – одна из важнейших задач физического воспитания в высших учебных заведениях. В формировании осанки большую роль играет сила мышц, их равномерное развитие и гармоничная работа. На учебном отделении аэробики выполняются упражнения общей физической подготовки и специальные упражнения для формирования правильной осанки.

Published
2018

49. Fitness aerobic as a person for improving physical education of students in higher education

Author

Kuzmenko, Natalia Viktorovna

Subjects
high school, student, popularity, развитие, популярность, 378.4, health, физическое развитие, aerobics, physical development, вуз, fitness, здоровья, студентки, development, фитнес-аэробика
Abstract

Data of scientific and methodological literature, own observations and practical experience are analyzed. It is revealed that the content of the current program for physical education in higher education institutions is built on inefficient physical exercises and does not provide motivational attitudes to a healthy lifestyle, does not take into account the features of the physical and functional state of the student body. Recommendations are given on the modernization of the physical education system in the university, one of the directions of which is the introduction of innovative technologies in the educational process of university students, namely fitness aerobics. Анализируются данные научно-методической литературы, собственных наблюдений и практического опыта. Выявлено, что содержание действующей программы по физическому воспитанию в вузах построено на малоэффективных физических упражнениях и не обеспечивает мотивационных установок на здоровый стиль жизни, не учитывает особенности физического и функционального состояния организма студенток. Даются рекомендации по модернизации системы физического воспитания в вузе, одним из направлений которой является внедрение инновационных технологий в учебный процесс студентов вузов, а именно фитнес-аэробики.

Published
2018

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