bluuebunny/crossref_metadata_2025_split huggingface dataset:
Alström Syndrome with Early Vision and Hearing Impairement
Authors: Beqiri-Jashari Ardiana , Janchevska Aleksandra , Ahmeti Irfan , Doksimovski Filip , Cipanovska Marija , Teov Bojan , Stefanovska Emilija Sukarova , Plaseska-Karanfilska Dijana , Gucev Zoran 
Published: July 2022

Abstract
Alström syndrome (ALMS) is an autosomal recessive disorder characterized by multiple organ involvement, including progressive cone-rod dystrophy, sensorineural hearing loss, childhood obesity, and type 2 diabetes mellitus. Pathogenic variants in the ALMS1 gene are the known cause for the occurrence of this devastating condition.
Here we report on a 12 year old boy referred to the University Clinic with early signs of impaired hearing and vision, obesity, and scoliosis. Central vision was first affected, followed by peripheral vision. In addition, his weight began increasing after the age of two years, reaching 78 kg at a height of 157 cm (BMI 31.64). No polydactyly was present. His mental development was normal in spite of his hearing and vision impairments. There was acanthosis nigricans on the neck. ECG and the cardiac ultrasound were normal. At the age of 12 years, his testicles are 12 ml and his pubertal status is P2 A2. OGTT revealed impaired glucose tolerance with elevated insulin concentrations 121ulU/mL (reference range 2,00-29,1 ulU/mL). Renal function was unaffected, liver functions were normal. Uric acid and lipids were within normal plasma concentrations. A Whole Exome Sequencing was performed and a homozygous ALMS1 pathogenic, frameshift gene variant (LRG_741t1(ALMS1):c.4156dup; p.Thr1386AsnfsTer15) was determined as the cause of the disease. Both parents were carriers for the variant. The absence of mental retardation and polydactyly differentiates Alström and Bardet-Biedle syndrome.
The Art of Dying: Making a Will in Old English and Its Sociolinguistic Context
Authors: Timofeeva Olga 
Published: July 2022

AbstractThis paper explores the potential of legal documents for the study of the sociology of Old English. It gives a rationale for the use of legal genres, or charters, and introduces research databases and tools that may elucidate the interconnections between practitioners of legal Old English and their linguistic practices. A series of short case studies on wills illustrates what legal genres tell us about the correlation between linguistic variation, supralocalisation, and change and such variables as archive and gender.
Comprehension processes on question-answering activities: A think-aloud study
Authors: Máñez Sáez Ignacio , Vidal-Abarca Eduardo , Magliano Joseph 
Published: July 2022

Introduction. Students often answer questions from available expository texts for assessment and learning purposes. These activities require readers to activate not only meaning-making processes (e.g., paraphrases or elaborations), but also metacognitive operations (e.g., monitoring readers’ own comprehension or self-regulating reading behaviors) in order to successfully use textual information to meet the task demands. The aim of the study was to explore the meaning-making processes readers activate while answering questions and using textual information at hand, and how they monitor and control the processing of these tasks.
Method. Forty eighth graders read two expository texts and answered ten multiple-choice comprehension questions per text. For each question, participants were forced to select the relevant pieces of textual information to provide the correct answer. Further, participants thought aloud in one of the texts, whereas they performed the task in silence in the other. This reading scenario served to collect valuable data on the reader’s question-answering process. The task was administered individually on a computer-based environment, which allowed recording the students’ reading behavior online.
Results. Results showed processing differences between the two recursive steps of question-answering. While reading the question readers mainly restated information and focused on monitoring the comprehension of the questions and their correspondence with the alternatives. However, they tended to paraphrase and assess textual relevance when searching the text. The study also reveals that think-aloud methodology affected these processes and outcomes differently.
Discussion and Conclusion. This study advances on our knowledge of cognitive and metacognitive processes involved in answering questions from an available text, a task extensively used to measure reading literacy skills as well as for learning purposes in academic settings. It shows how readers activate meaning-making processes while reading questions and searching the text for task-relevant information, and how they monitor the question-answering process. The study also reveals that think-aloud methodology may affect these processes differently. Apart from advancing our theoretical knowledge, our study also has important practical applications for the assessment of reading literacy skills.
Lithostratigraphy of the Bumbeni Complex and its associated subdivisions, South Africa
Authors: Ncume M. , Hicks N. , Hoyer L. , Bristow J.W. , Botha G.A. 
Published: July 2022

Abstract
The Bumbeni Complex represents an Early Cretaceous volcanic complex that developed at the southern termination of the Lebombo mountain range in northern KwaZulu-Natal, South Africa. The volcano-sedimentary complex intrudes and disconformably overlies the Lebombo Group of the Karoo Large Igneous Province. The volcano-stratigraphy is defined by an assemblage of volcanic and volcaniclastic rocks as well as late stage quartz-syenite intrusions. Based on current geological and geophysical studies the complex is considered to represent the remnants of a collapsed cauldron structure. The stratigraphic succession is intricate and laterally discontinuous, comprising mafic and felsic lava flows, welded and non-welded tuffs, ignimbrites, lahar deposits, rhyolite domes and dykes and coarse-grained syenitic rocks. Rhyolite dome formation and collapse, and associated plinian eruptions and column collapse, were largely responsible for the variety of volcaniclastic rocks, including classic air-fall tuff deposits. The complex forms part of a northeast trending volcanic basement lineament known as the Bumbeni Ridge defined by aeromagnetic data.
The petrogenesis and emplacement mechanism of layered ultramafic-mafic complexes of the Barberton Greenstone Belt: Insights from the Stolzburg Complex, South Africa
Authors: Tau M.M. , Bolhar R. , Wilson A.H. , Anhaeusser C.R. 
Published: July 2022

Abstract
Ultramaﬁc-maﬁc layered complexes are important but not-well studied components of Archaean granitoid-greenstone terranes. In the vicinity of the Barberton Greenstone Belt (BGB), at least 27 such complexes are intimately associated with the supracrustal succession. The petrogenesis of one of these layered bodies, the Stolzburg Complex (SC), is explored, together with its relationship to the surrounding Barberton volcanic succession.
Previous models for the origin of Barberton layered complexes proposed a variety of mechanisms, such as single chamber subvolcanic sills, ponded lavas, and alpine-type tectonites. In contrast, the present work suggests that emplacement mostly occurred as sheeted sills of crystal slurries into the country rocks. Unlike the subvolcanic sills model, whereby each complex grew through repetitive magma injection and differentiation in a single chamber, the preferred model regards the layered bodies as ‘stacks’ of discrete intrusions, where each magmatic unit represents a distinct sill. Through comparison of trace element geochemistry (i.e., trace element ratios and patterns), the Lower and Upper divisions of the SC are inferred to be petrogenetically related, but compositionally distinct from the enveloping Nelshoogte volcanic rocks. The trace element geochemistry of the Lower and Upper divisions of the complex is indistinguishable. While the SC ultramaﬁc rocks display an Al-undepleted character, Nelshoogte metavolcanics can be classiﬁed as Al-depleted komatiites and komatiitic basalts.
The industrial-grade hemp (Cannabis sativa L.) seed oil biodiesel application in a diesel engine: combustion, harmful pollutants, and performance characteristics
Authors: Yilbaşi Zeki , Yesilyurt Murat Kadir , Yaman Hayri , Arslan Mevlut 
Published: July 2022

The core focus of the present investigation is regarding biodiesel production from industrial hemp seed oil applying single-stage homogenous catalyzed transesterification process obtaining high yield of methyl ester. The engine tests were carried out on a single-cylinder, four-stroke, water-cooled, unmodified diesel engine operating with hemp seed oil methyl ester as well as its blends with conventional diesel fuel. The experimental findings of the test fuels were compared with those from diesel. The results pointed out that the performance and combustion behaviors of biodiesel fuels are just about in line with those of diesel fuel propensity. The specific fuel consumption for 5% biodiesel blend (0.291 kg/kW h), 10% biodiesel blend (0.305 kg/kW h), and 20% biodiesel blend (0.312 kg/kW h) blends at full load was closer to diesel (0.275 kg/kW h). In the meantime, the thermal efficiency for biodiesel was found to be at the range of 15.98–24.97% and it was slightly lower than that of diesel (18.10–29.85%) at the working loads. On the other hand, the harmful pollutant characteristics of carbon monoxide, hydrocarbon, and smoke opacity for biodiesel and its blends were observed to be lower in comparison with diesel during the trials. However, the oxides of nitrogen emissions for biodiesel were monitored to be as 6.85–15.40 g/kW h which was remarkably higher than that of diesel (4.71–8.63 g/kW h). Besides that, the combustion behaviors of biodiesel and its blends with diesel showed much the same followed those of diesel. Namely, the duration of ignition delay of biodiesel–diesel blends was shorter than that of diesel fuel because of the higher cetane number specification of the methyl ester. The highest gas pressures inside the cylinder as well as the rates of the heat release of biodiesel including test fuels are lower in contrast to the diesel due to the shorter ignition delay. It could be concluded that the utilization of biodiesel produced from industrial hemp seed oil in the diesel engine up to 20% (by vol.) will decrease the consumption of diesel and environmental pollution, especially in developing countries.
Fear of Cancer Recurrence 7 Scale Tamil Translation and Validation among Breast Cancer Survivors in India
Authors: Nandakumar Devi , Veeriah Surendran , Krishnamurthy Arvind , Veluswami Sridevi , Ananthi Balasubramanian 
Published: July 2022

Objectives:
Fear of cancer recurrence (FCR) is one of the most widely reported conditions among cancer survivors. The present study aims to translate and validate FCR7 scale into regional language tamil among breast cancer survivors (BCSv).

Material and Methods:
A cross-sectional study comprising a sample of 106 breast cancer survivors was carried out. FCR 7 scale, functional assessment of cancer therapy-B (FACT-B) and impact of event scale-R (IES-R) were used for establishing reliability and validity. Translation of the FCR7-T scale was done from English into Tamil following the international guidelines and a field study was performed.

Results:
The test-retest reliability was established for FCR 7 Tamil with a Cronbach’s alpha of 0.96 and ICC value of 0.910. On Spearman’s correlation, an inverse relationship was found between FCR7 and FACT-B (r = −0.259 and P = 0.01). The survivors with high FCR reported poorer quality of life.

Conclusion:
The Tamil version of the FCR7 tool is highly sensitive for measuring FCR.
My meetings with Nikolai Korndorf
Authors: Воронцов Ю.В. 
Published: July 2022

В настоящем материале Журнал публикует воспоминания профессора Ю. В. Воронцова о советском и российском композиторе Н. С. Корндорфе (1947 – 2001), который в 1972 – 1991 годах преподавал в Московской консерватории. В ходе рассказа профессор раскрывает интересные детали исторического контекста встреч со своим старшим современником, освещает примечательные подробности из собственной творческой биографии, а также затрагивает ряд непростых художественно-эстетических вопросов. Среди последних — влияние метатекста и интертекста на глубину художественного содержания произведения и на восприятие этого произведения. Кроме того, автор приводит данные из собственных метких аналитических наблюдений над стилем Корндорфа, пытаясь вскрыть особые механизмы художественного мышления композитора в условиях принципа интертекстуальности.
In this article the Journal publishes the memories by Professor Yu. V. Vorontsov about the Soviet and Russian composer N. S. Korndorf (1947 – 2001), who taught at the Moscow Conservatory in 1972 – 1991. During the story, the Professor reveals interesting details of the historical context of meetings with his elder contemporary, highlights the remarkable details of his own creative biography, and also touches upon a number of difficult artistic and aesthetic issues. Among the latter is the influence of metatext and intertext on the depth of the artistic content of the work and on the perception of this work. In addition, the author provides data from his own well-aimed analytical observations on the style of Korndorf, trying to reveal the special mechanisms of artistic thinking of the composer in terms of the principle of intertextuality.
On Bergiana of Theodor Adorno
Authors: Векслер Ю.С. 
Published: July 2022

Статья посвящена обзору «бергианы» Теодора Адорно, которая включает в себя обширный круг его статей и исследований о творчестве Альбана Берга, создававшихся в период 1925-1968 гг. Рассматриваются ключевые музыкально-эстетические идеи Адорно, в том числе «музыкальная физиогномика», задача которой в отыскании универсальных констант личности и творчества вне механического перенесения на музыку внемузыкального. В качестве таких констант анализируются понятия «тона» и «(незаметного) перехода» — последнее вынесено в заглавие монографии Адорно о Берге, творчество которого становится для Адорно абсолютным воплощением феномена перехода как в музыкально-историческом, так и в стилевом ракурсе.
The article is devoted to the review of «bergiana» of Theodor Adorno, which includes a wide range of his articles and studies of the work of Alban Berg, created in the period 1925 –1968. The author considers the main musical and aesthetic ideas of Adorno, including «musical physiognomy», whose task is to search for universal constants of personality and creativity outside the mechanical transmission of music to the music of non-musical. The terms «tone» and «(smallest) link» are analyzed as such constants. The latter was included in the title of Adorno's book about Berg, whose work for Adorno becomes the absolute embodiment of the link from the perspective of history of music and style.
Music criticism – yesterday, today… And will it be tomorrow?
Authors: Амрахова А.А. , Зенкин К.В. , Курышева Т.А. , Насонов Р.А. , Устинов А.А. 
Published: July 2022

Проблематика круглого стола обусловлена теми колоссальными изменениями в музыкальной критике, которые произошли в XX веке по сравнению с XIX веком. Лейтмотивом через все беседы проходят мысли о том, как изменились формы проявления исконно критических ипостасей: сиюминутности отзыва (которая сегодня не обязательна), установки на просветительство (которая перестала быть прерогативой только лишь критики), изменился сам язык, который теперь не чурается бытового модуса.Отдельный смысловой пласт бесед — научная критика, которую давно пора вывести из тени. Сегодня научная жизнь немыслима без огромного количества отзывов, рецензий, всякого уровня экспертных оценок — от отзывов оппонентов на защитах, до педагогической отчетности. Вся эта деятельность, дабы поднять ее статус, также нуждается в открытом обсуждении. В беседах приняли участие Т. А. Курышева, А. А. Устинов, К. В. Зенкин, Р. А. Насонов.
The problems of the round table are determined by the enormous changes in music criticism that happened in the XX century in comparison with the XIX century. The leitmotif going through all the conversations are thoughts of how manifestations of essentially critical forms have changed: immediate feedback (which today is not obligatory), enlightenment orientation (which has ceased to be the prerogative only of criticism), the language itself has changed which includes everyday-life modus nowadays. A separate semantic layer of conversations is scientific criticism, which it is high time to bring to light. Today, scientific life is unthinkable without a huge number of reviews, comments, all kinds of expert assessments – beginning with the reviews of opponents when dissertations are being supported, till pedagogical documentation. All these activities, in order to raise their status, also need an open discussion. T. A. Kurysheva, A. A. Ustinov, K. V. Zenkin, R. A. Nassonov took part in the talks.
On the Phenomenon of a Revolution in View of Jean Renoir (materials of the III International Congress of Russian Society on Music Theory)
Authors: Ровенко Е.В. 
Published: July 2022

Эстетико-философская концепция Жана Ренуара — режиссера, обладающего оригинальным видением как художественной реальности, так и реальности социокультурной, — важна для истории кинематографа, поскольку, во-первых, демонстрирует актуализацию французской ментальности в кино, а во-вторых, во многом объясняет мировоззренческую интенцию творчества Ренуара. В статье анализируются важнейшие аспекты данной концепции: проблема стиля как исторически эволюционирующего феномена; специфика иерархического устройства общества как отражение интеллектуально-психологической разности формирующих общество групп людей; особенности претворения национального в кинематографе. Несводимые к единому эстетическому знаменателю аспекты концепции Ренуара образуют уникальное мировоззренческое единство и обусловливают целостность художественного мира режиссера.
Jean Renoir had an original take on the artistic reality and sociocultural sphere. His aesthetic-philosophic conception is important for the history of cinema, firstly, because it demonstrates actualization of the French mentality in cinematograph, secondly, because it clarifies Renoir’s worldview intention to a great extent. In the article the most important aspects of Renoir’s conception are analyzed: the problem of a style as a phenomenon which develops in history; the specifics of social hierarchy as embodying of intellectual-psychological difference between groups of people which form a society; peculiar properties of incarnation of the specificity of the national in cinema. Though aspects of Renoir’s conception aren’t brought to a common denominator, they constitute the unique worldview unity and cause the entirety of the filmmaker’s artistic world.
INFLUENCE ANALYSIS OF EXTERNAL AND INTERNAL ENVIRONMENT ON THE STATE PROGRAM FORMATION
Authors: Черникин Олег Сергеевич 
Published: July 2022

В статье рассмотрено влияние основных элементов внутренней и внешней среды при формировании государственной программы (ГП). Целью исследования является разработка инструментов, позволяющих стратифицировать проблемы реализации ГП, а также нивелировать их влияние на реализацию программ. Научная новизна полученных результатов заключается в классификации элементов внутренней и внешней среды, установлении факторов, влияющих положительно и отрицательно на сферу реализации ГП посредством установления соответствующих проблем, в разработке алгоритма формирования ГП с учетом анализа внешней среды.
The article considers the impact of the main elements of the internal and external environment in establishing the main problems in the implementation of the state program. The purpose of the study is to develop tools to stratify the problems of the implementation of the state program, as well as to level their impact in the implementation of the program. The scientific novelty of the obtained results consists in the classification of elements of the internal and external environment, the establishment of factors influencing positively and negatively on the sphere of implementation of the state program, by establishing the relevant problems impeding the implementation of the state program, the development of an algorithm for the formation of the state program, taking into account the analysis of the external environment
METHODOLOGY OF INNOVATION ACTIVITY
Authors: Семенов Сергей Владимирович 
Published: July 2022

Любая деятельность в своей основе должна иметь методологию. Проработанная методология позволяет не только оптимально построить деятельность, но и заложить основы для ее автоматизированной поддержки. Целью данной статьи является рассмотрение базовых элементов методологии инновационной деятельности (ИД). В отечественной литературе эти вопросы проработаны слабо и системно нигде не рассматривались. Это и определяет новизну и актуальность данной работы. Методология ИД позволяет создавать эффективную методическую и программную поддержку формирования и оценки большого количества инновационных проектов. Базовые элементы методологии ИД могут использоваться для разработки нормативных и методических материалов на предприятиях, применяющих инновационные методы для повышения собственной конкурентоспособности и эффективности.
Any activity should be based on a methodology. The developed methodology allows not only to optimally build an activity, but also to lay the foundations for its automated support. In this article, the basic elements of the methodology of innovation activity are developed. In the literature, these issues have been worked out rather poorly, which does not allow creating effective methodological and programmatic support for the creation and evaluation of a large volume of innovative projects. The basic elements of the ID methodology can be used to develop regulatory and methodological materials at enterprises and organizations that use innovative methods to improve the competitiveness and efficiency of their activities.
ASSESSMENT OF THE «GREEN» ECONOMY IN THE EAEU MEMBER STATES UNDER THE CONDITIONS OF STATE REGULATION OF COMMODITY PRICES
Authors: Савельева Надежда Константиновна , Созинова Анастасия Андреевна 
Published: July 2022

В статье рассмотрены вопросы государственного регулирования цен на товары, возникающие в государствах-членах ЕАЭС, и последствия их наступления. Цель работы заключается в исследовании показателей «зеленой» экономики за 2013-2021 гг. на предмет обнаружения неоспоримости доказательства, что нарушение естественного механизма ценообразования может приводить к увеличению экологических издержек экономического роста. Научная новизна заключается в установлении авторами вывода при определении последствий государственного регулирования для «зеленой» экономики, в том числе в постпандемийный период, что отказ от государственного лимитирования цен на товарных рынках не только нецелесообразен, но и угрожает устойчивому развитию и «зеленой» экономике ЕАЭС.
The article considers the issues of state regulation of prices for goods arising in the EAEU state members and the consequences of their onset. The purpose of the work is to study the indicators of the “green” economy over 2013-2021. To find out the indisputable evidence that a violation of the natural pricing mechanism can lead to an increase in the environmental costs of economic growth. The scientific novelty consists in establishing by the authors the conclusion when determining the consequences of state regulation for the “green” economy, including in the post-pandemic period, that the rejection of state price caps in commodity markets is not only inappropriate, but also threatens sustainable development and the “green” economy of the EAEU.