Клинический случай синдрома Блоха — Сульцбергера

Purpose.To present a clinical case of Bloch Sulzberger syndrome. Material and methods. The examinations were performed to diagnose the disease: а visual examination of the skin, cytological analysis of the gallbladder fluid, general and biochemical blood tests, genetic research. Results.During a visual examination of the skin, a differential diagnosis was made with infectious dermatitis, toxic-allergic dermatitis, epidermolysis bullosa and linear IgA-dependent dermatosis in children. Crucial in the diagnosis belonged to a genetic study, after which a deletion of exons 410 of the IKBKG gene was detected, which confirmed Bloch Sulzberger syndrome. Conclusion.Newborns with vesicle-bullous rashes entering the neonatal pathology department and observed by neonatologists require a thorough examination, a mandatory consultation of a dermatologist inorder to determine further management tactics.Цель.Представить клинический случай синдрома БлохаСульцбергера. Материал и методы.Для диагностики заболевания были проведены осмотр кожи, цитологический анализ пузырной жидкости, общий и биохимический анализы крови, генетическое исследование. Результаты.При осмотре кожи был проведен дифференциальный диагноз с дерматитами, буллезным эпидермолизом и линеарным IgA-зависимым дерматозом у детей. Решающее значение в постановке диагноза принадлежало генетическому исследованию, после проведения которого была выявлена делеция экзонов 410 гена IKBKG, что подтвердило синдром БлохаСульцбергера. Заключение.Новорожденные с везикуло-буллезными высыпаниями, поступающие в отделение патологии новорожденных и наблюдаемые неонатологами, требуют тщательного обследования, обязательной консультации дерматолога с целью определения дальнейшей тактики ведения

Fragmentation and Multifragmentation of 10.6A GeV Gold Nuclei

We present the results of a study performed on the interactions of 10.6A GeV gold nuclei in nuclear emulsions. In a minimum bias sample of 1311 interac- tions, 5260 helium nuclei and 2622 heavy fragments were observed as Au projec- tile fragments. The experimental data are analyzed with particular emphasis of target separation interactions in emulsions and study of criticalexponents. Multiplicity distributions of the fast-moving projectile fragments are inves- tigated. Charged fragment moments, conditional moments as well as two and three -body asymmetries of the fast moving projectile particles are determined in terms of the total charge remaining bound in the multiply charged projectile fragments. Some differences in the average yields of helium nuclei and heavier fragments are observed, which may be attributed to a target effect. However, two and three-body asymmetries and conditional moments indicate that the breakup mechanism of the projectile seems to be independent of target mass. We looked for evidence of critical point observable in finite nuclei by study the resulting charged fragments distributions. We have obtained the values for the critical exponents gamma, beta and tau and compare our results with those at lower energy experiment (1.0A GeV data). The values suggest that a phase transition like behavior, is observed.Comment: latex, revtex, 28 pages, 12 figures, 3tables, submitted to Europysics Journal

The engaging leisure activities of an industrial town in the early 1950s

This article considers the main leisure activities of Magnitogorsk residents in the early 1950s based on their personal memories, local materials of the clerical sources, and periodicals. The analysis shows how the residents spent their free time and what emotions they had. On the one hand, the leisure options of that time (for example, going to cinemas, theaters, parks, circuses, libraries, concerts, sports sections, hobby clubs, public lectures, public readings of books, educational meetings arranged by the party, as well as organizing holidays and public events, amateur art shows, mass leisure out of town, resort holidays, etc.) were largely defined by the political regime and ideology. On the other hand, the urban community either tacitly rejected or changed those activities that turned out to be unpopular or boring. At the same time, one of the main trends supported by the Magnitogorsk residents was collectivism and publicity while engaging in a leisure activity. The latter two formed an integral part of the Soviet consciousness. Particular attention is paid to the problems faced by the city in the organization of leisure and recreational activities: the construction of cultural and entertainment venues failed to keep up with the growth of the city population; some residents were not properly educated about the value and importance of certain leisure types. The workers were tired because of their hard working shifts at the metallurgical plant and everyday life, thus many of them showed no interest in outdoor activities. It is concluded that the organization of leisure time in Magnitogorsk, which was a role model for the entire Soviet society, was essential in upholding the morale of its residents

Особенности обучения фразеологической системе на занятиях по русскому языку как иностранному

The object of research interest is the phraseological system of Russian. In the methodology of Russian as a foreign language, the question of including phraseological units in the content of education is not resolved. This is due to the lack of clear criteria for the selection of lexical material and the order of its inclusion in the learning process. The work presents an analysis of the most popular among teachers of Russian as a foreign textbooks and reference books; it is described and systematized the assignments for the study of phraseological units.Объектом исследовательского интереса является фразеологическая система русского языка. В методике русского языка как иностранного не решен вопрос включения в содержание обучения фразеологических единиц. Это связано с отсутствием четких критериев отбора лексического материала и порядка его включения в учебных процесс. В работе представлен анализ наиболее популярных среди преподавателей русского языка как иностранного учебных пособий и справочников; описаны и систематизированы задания по изучению фразеологических единиц

TEACHING IDIOMS IN THE RUSSIAN LESSONS AS A FOREIGN LANGUAGE

The article deals with the phraseological system of Russian in the context of teaching Russian as a foreign language. The object of attention is the technology of mastering idiomatic units by foreign students in the framework of preparatory faculties. Many methodologists believe that preparatory faculties do not prepare students for the perception and understanding of phraseological units. In the framework of this work, the methodology for presenting phraseological units to foreign students is analyzed in a number of textbooks on Russian as a foreign language

Investigating the Effect of Formation of Communicative and Pragmatic Competence in Phraseology in Educating Foreign Philological Students

This article deals with the analysis of the factors influencing the formation of communicative and pragmatic competence in the process of teaching foreign students of the Faculty of Philology, Department of Russian Phraseology. The authors prove that the system of training future teachers of Russian has no sections and courses aimed at the formation of communicative and pragmatic competence in Russian phraseology. The study of the assimilation of phraseological units was carried out in several stages. The first is related to the definition of the level of knowledge of the phraseological system of the Russian language. The second is the level of understanding of certain phraseological units by philology students. The third is the ability to express an evaluative attitude towards someone using phraseological units. The fourth is related to the ability to use phraseological units when evaluating something, any event, phenomena

Issues of Student Lexical Skills Development in English Classes of Secondary Professional Education with the Use of Neurolinguistic Programming in Distance Learning Environment

The article discusses the issue of development of lexical skills of secondary vocational education students in English classes with the use of elements of neurolinguistic programming (NLP) in distance learning environment. The authors make an attempt to solve the problems arising in building lexical skills in adolescent distance learning students (desire to be independent; fear of being ridiculous, desire to assert oneself; increased anxiety, negativism to criticism; trust only in those professors who enjoy authority among students; interest to problem solving, decreased motivation to learn). From the principles of NLP (the principle of subjective perception of reality; the principle of permanent change in human consciousness as a result of interaction with surrounding reality); elements of NLP (personality-oriented approach; creating a system of effective beliefs; taking into account the main channels for obtaining information; creating a system of effective beliefs, a clear learning route) and NLP techniques (determination of the type of student motivation; presentation of the “route of movement”, learning objectives, student assessment criteria and methods of promotion; determination of the student's main programming language, the use of “access keys”, “anchoring”; consideration of the psychophysical characteristics of students; selection of relevant examples; accounting for color gamut visual presentation of information; emphasis on the behavior of the teacher himself; creating a situation of increased emotional arousal; taking into account the student's modality at the stage of introducing new vocabulary, the energies of the three receive channels, use of imaging technology information; training based on compact reference signal), microstrategies of mastering the vocabulary of a foreign language (microstrategies to create the necessary emotional background; microstrategies aimed at memorizing visual and auditory images of a word and their correlation with the semantic component; combining the two previous microstrategies), the authors offer a set of exercises aimed at building lexical skills. The article also provides an analysis of individual existing teaching materials for students and proves the necessity for additional testing of English vocabulary based on them

The role of audio and video means in the training of foreign philologists concerning national vocabulary of Russian language

The article describes the linguistic didactic system of foreign student training studying philology at Russian universities in respect of Russian language national vocabulary. Russian screen versions of fairy tales are considered as a teaching resource, their educational value for foreign experts in Russian philology is described as the additional materials on RFL practical course. The proposed system is aimed at vocabulary expansion of students-philologists with the II-nd certification level of RFL proficiency (I, II university courses) in order to prepare students for the lectures on core subjects and for the solution of educational and communicative tasks

Universal values: Semantic-cognitive approach

The article deals with the theoretical aspects of semantic-cognitive approach concerning the study of universal values. It is proved that universal values are the component of a man's language picture of the world which provide the "entry" of a person in the ethnic culture. Being an invariant of the system of values, universal values are the code of linguistic culture, providing the knowledge about values and antivalues accumulated by society for succeeding generations