bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Role of Behavioral Phenotyping in Establishing a Diagnosis of Pseudo-Angelman Syndrome
Authors: Arora Veronica , Takkar Aashita , Bhatia Sameer , Lall Meena , Kumar Praveen 
Published: May 2022

Background:
Behavioral phenotypes are observable patterns of behavior present in certain genetic syndromes that have distinctive social, linguistic, cognitive, and motor profiles. These may play an important role as pointers toward certain genetic disorders. The recognition of aberrant behavior is important for therapeutic targeting by behavioral modification strategies and medication. The repertoire of behavioral constellations is exhaustive, but common manifestations include aggression, self-injury, autistic features, and a happy demeanor comprising excessive smiling and outbursts of laughter without any preceding triggers.

Clinical Description:
We describe the approach that was used to establish diagnosis in a boy with a happy disposition, cognitive impairment, and seizures, the firstborn of a couple desiring genetic counseling for their second 7-week pregnancy. After deep phenotyping (identifying overt and concealed dysmorphism, assessment of vision, hearing, behavior, and cognition), a syndrome search was performed by a geneticist using suitable handles. The clinical phenotype of the proband was then matched with the generated list of disorders. The most likely diagnosis Angelman syndrome (AS) was excluded by negative specific genetic testing. Chromosomal microarray identified 2q23.1 microdeletion that is associated with pseudo-AS. Prenatal diagnosis at 16 weeks revealed an unaffected fetus.

Management:
There is no cure for this syndrome. Affected children benefit from symptomatic intervention provided by a multidisciplinary team including clinical geneticists, pediatricians, pediatric neurologists, developmental pediatricians, and various professional therapists.

Conclusions:
Behavioral phenotypes aid in establishing diagnosis in certain genetic disorders. A happy disposition coupled with intellectual disability should prompt the clinician to involve a geneticist in management, even if overt dysmorphism is not apparent.
Factor XIII Deficiency in a Mother-Baby Dyad
Authors: Thangaraj Shalini , Kadirvel Karthikeyan , Ramar Rathisharmila , Chandramohan Anandhi 
Published: May 2022

Background:
Factor XIII deficiency is a rare autosomal recessive coagulation disorder with varied presentations including prolonged bleeding from the umbilical cord stump, defective wound healing, recurrent miscarriages, or life-threatening intracranial hemorrhage.

Clinical Description:
A male baby was born at term gestation to a fourth gravida mother with a history of two abortions in the past. He was born out of a third-degree consanguineous marriage, with smooth perinatal transition, but developed multiple episodes of seizures associated with poor feeding and lethargy after 24 h of life.

Management:
On evaluation, septic screen, metabolic screen (serum electrolytes, calcium, and blood sugar), and coagulation assays were normal. Ultrasonogram revealed a hyperechoic lesion restricted to the left cerebral hemisphere, suggestive of an intraparenchymal hemorrhage. Magnetic resonance imaging brain showed left intraparenchymal hemorrhage with significant mass effect and midline shift. In view of intracranial bleed with normal coagulation assay and other causes being ruled out, factor XIII clot solubility assay was sent and found to have undetectable levels. Factor XIII levels of the mother were also found low (5.5%) though the levels in the father were normal. The baby was managed conservatively with supportive measures in the form of anticonvulsant and anti-edema measures. He recovered successfully and is under close follow-up.

Conclusion:
A high index of suspicion of factor XIII deficiency should be kept in any neonate presenting with intraparenchymal hemorrhage and recurrent abortions in the mother. This case is being reported to highlight factor XIII deficiency in recurrent pregnancy loss and neonatal intracranial bleeding. Prenatal screening for factor XIII deficiency in these circumstances will help in effective management of future pregnancies.
A level of empathy and spirituality among undergraduate medical students of a tertiary hospital in Telangana
Authors: Dhedhi Bhavesh Jayantilal , Gattavali Ramana , Nadukuru Gyan Nihal , Macharapu Raghuram , Mallepalli Pramod Kumar Reddy 
Published: May 2022

Introduction:
Patients' care with empathy has shown a higher clinical competence with great rapport. It leads to an accurate diagnosis with fewer medical errors. Patients tend to be more satisfied with improved outcomes both psychologically and pharmacologically. Empathy supports medical students to achieve capabilities essential for patient-centered care and in development of affective skill, manners, and personal as well as professional growth.

Aims and Objectives:
To assess the level of empathy among medical students and to assess the level of spiritual well-being and its relation with empathy.

Materials and Methods:
The cross-sectional study was carried out from January 2021 to March 2021 period. A total of 200 medical students were selected for the study, fifty from each year. Jefferson Scale of Physician Empathy Student version (JSPE-S), Toronto Empathy Questionnaire (TEQ), Spiritual Well-Being Scale (SWBS), and Demographic Questionnaire were used for the collection of data.

Results:
The mean JSPE-S score was 108.41 (14.19), mean TEQ score was 44.89 (6.26), and mean SWBS was 80.58 (18.89). By JSPE-S, the mean empathy score decreased from the 3rd year and was lower in the final year (P = 0.00002). By TEQ, the empathy score was higher in the 2nd year followed by 3rd and 1st and was lower in the final year (P = 0.002). Females had higher empathy than males (P < 0.002 for JSPE-S and P < 0.00001 for TEQ). There was a significant positive relationship between spiritual well-being score with mean JSPE-S (r = 0.4429, P = 0.0012) and TEQ score (r = 0.5777, P = 0.00001).

Conclusion:
Medical students had an average level of empathy and spiritual well-being. Clinical empathy decreased from the 3rd year and was lower in final-year students. Spiritual well-being had a positive significant relationship with empathy. There was a statistically significant association between mean empathy scores with demographic variables such as gender, parental education, habit of doing meditation, permanent residence area, and year of study.
A study of assessment of depression, anxiety, and stress among coronavirus disease 2019 patients at tertiary hospital
Authors: Baothman Abdul Rahman , Challuri Prashanth , Ramana Gattavali , Macharapu Raghuram , Reddy M. Pramod Kumar 
Published: May 2022

Introduction:
The coronavirus disease 2019 (COVID-19) is not only causing physical illness but has also been causing psychological impact on human beings. Most people are aware of physical complications the disease causes, but now, they are learning psychological impact of the disease.

Aim:
To estimate the level of depression, anxiety, and stress in general and with reference to age and sex among COVID-19 patients admitted at Mamata General Hospital, Khammam.

Materials and Methods:
This study is a cross-sectional study with sample of 100 participants who were admitted at Mamata General Hospital, Khammam, Telangana. The samples were drawn by using convenience sampling method. DASS-21 was the tool used in this study.

Study Design:
It is a cross-sectional study with a sample of 100 participants who were admitted at Mamata General Hospital, Khammam, Telangana. The samples were drawn by using convenience sampling method. Depression, anxiety, stress scale-21 was the tool used in this study.

Results:
From a total of 100 patients, about 64% of the patients had varying degree of depression, 70% of the patients had anxiety, and 40% of the patients had various levels of stress. Furthermore, females in this survey were found to have higher level of depression, anxiety, and stress scores as compared to males.

Conclusion:
Female patients require adequate psychiatric intervention in this COVID-19 pandemic to mitigate its psychological impact.
Review of ship behavior characteristics when operating at sea
Authors: SCURTU Ionut Cristian , PRICOP Mihail , TOMA Alecu , ATODIRESEI Dinu , POPESCU Emil 
Published: May 2022

During activity 1.1. included in Platmarisc project authors collected information for initial evaluation for ship behavior characteristics when operating at sea. Identification of loads and their influence on the behavior of marine intervention platforms is one of the main contributions of the article. As first step in modelling of the 3D ship we will present the 3D initial Model in ModelMaker and the initial loading case according equipment loading onboard.
Development of Pragmatic-Based English Learning Material for Front Office
Authors: Aryati Kadek Feni , Budiarsa Made , Suastra I Made , Putra Anak Agung Putu 
Published: May 2022

This study aimed at (1) describing the realization of the use of teaching materials in learning English speaking skills in the field of front office services, (2) developing learning materials for English speaking skills in the field of front office services, and (3) examining the effectiveness the learning materials for English speaking skills in the field of front office services with a pragmatic approach. This research was conducted using Research and Development (R&D) research methods and applying Borg and Gall (1983). Based on the results of the analysis, it was found that learning English for tourism and hospitality currently does not include linguistic elements in it. The books or modules available were limited. Based on this reality, this research develops two educational products, namely a pragmatic-based syllabus and module. The syllabus and modules have 12 learning topics that have been adapted to the use of English for hotel front office services. Learning materials for English speaking skills for front office services with a pragmatic approach are effective for use in organizing the teaching and learning process as evidenced by the t-test results, namely 0.001 (0.001 <0.005). In addition, the perceptions of students and lecturers also showed a positive response to the modules developed in this study.
The Victorian Literature Analysis of Ambition and Self-Improvement using Maslow's Theories on Dickens Message in Great Expectations
Authors: Cahyono Arif 
Published: May 2022

This research discusses the victorian literature analysis of ambition and self-improvement using Maslow's theories on Dickens's message in Great Expectations and the purpose of analyzing ambition and self-improvement in Dickens's Message Novel Great Expectations. The researcher used the analysis of industry and self-improvement in Dickens's Message in Great Expectations theory of Maslow. This research analyzes ambition and self-improvement in Dickens's Message in Great Expectations that occurred in Great Expectations. Analysis using
descriptive qualitative methods to identify, explain, and explore the data found in the novel. The researcher used a coding sheet as the instrument to find out the valid data. This research is conducted to answer the problems concerning the victorian literature analysis of ambition and self-improvement using Maslow's theories on Dickens's message in Great Expectations in the novel. The research, the victorian literature analysis of purpose and self-improvement using Maslow's ideas on Dickens message in Great Expectations concerning Maslow's theories moreover partitions human identity into three major components: Brain research is changed in its approach to diverse human characteristics, and like each other trait that characterizes people, aspiration can be clarified with various mental theories. On the one hand, ambition and self-improvement may be closely related to inspiration, mainly accomplishment inspiration, and may be characterized by Maslow's hypothesis of human motivation. The author found that there was ambition and self-improvement behaviour in pip where pip was the self of Charles Dickens.
COMPORTAMENTO DO CONSUMIDOR FRENTE AO CONSUMO SUSTENTÁVEL
Authors: ALVES RAFAEL CRISOSTOMO , RODRIGUES GABRIEL AUTUORI , GOMES RAFAEL CEDRO , DIAS RANYERE DE PAIVA 
Published: May 2022

Este artigo versa sobre o comportamento do consumidor frente a um consumo sustentável, bem como a forma que se estabelecem as relações de consumo no Brasil. Ao tratar o consumidor como o centro da análise, o documento traz uma perspectiva detalhada da mudança comportamental das pessoas e o foco dos estudos científicos sobre o tema. Nesse contexto, o artigo tem como objetivo, analisar o comportamento do consumidor frente aos produtos sustentáveis. O estudo é uma replicação do estudo de Gollo et al. (2015), sendo caracterizado como uma replicação do Tipo III e classifica-se como teórico-empírico de caráter descritivo-explicativo. Os procedimentos metodológicos utilizados foram o Quantitativo e o Levantamento do Tipo Survey. Com os resultados obtidos, se pode chegar à conclusão de que o consumidor não adota comportamentos uniformemente sustentáveis, pois o consumidor que não adota padrões de consumo sustentáveis, quando nunca ou quase nunca compra produtos que possam ser reutilizados, é o mesmo que se preocupa em separar o lixo para o devido descarte.
DETERMINAÇÃO DE UMA REGIÃO NO ESTADO DO PARANÁ: APLICAÇÃO DA TEORIA DA BASE DE EXPORTAÇÃO
Authors: Prestes Andréia Ferreira , Cattelan Renata , Moraes Marcelo Lopes de 
Published: May 2022

A Teoria da Base Exportadora (TBE) de Douglass North propõe que para uma região se desenvolver é necessário vincular sua estrutura produtiva a um produto de exportação. Isso fará com que haja crescimento econômico mediante efeito multiplicador que agirá sobre toda a economia. O objetivo deste artigo é, a partir da determinação de uma região no estado do Paraná, mediante os princípios de homogeneidade e contiguidade, aplicar a TBE, para os anos de 2010 e 2015, utilizando o Quociente Locacional, regressão simples e cálculo do multiplicador de empregos. Identificou-se uma região com 14 municípios, nomeada como região de “Marilon”, devido as duas principais cidades, Maringá e Londrina. Os resultados mostraram que as atividades básicas influenciam positivamente sobre as atividades não básicas e o multiplicador de emprego indicou que no ano de 2010 para cada emprego gerado na atividade básica 0,65 empregos foram criados nas atividades não básicas e em 2015 essa relação foi 1 emprego básico para 0,60 empregos não básicos.
Levels of bioactive endogenous lipids and health-related quality of life in Chronic Idiopathic Axonal Polyneuropathy
Authors: Lind Jonas , Stensson Niclas , Gerdle Björn , Ghafouri Nazdar 
Published: May 2022

Background: Although neuropathic pain is a significant problem in polyneuropathy, the underlying molecular mechanisms are poorly understood. The endogenous bioactive lipids 2-arachidonoyl-glycerol (2-AG), oleoylethanolamide (OEA), palmitoylethanolamide (PEA), and stearoylethanolamide (SEA) are known to influence pain and inflammation in the peripheral nervous system. The aim of this study was to explore the plasma levels of endocannabinoids and related lipids and health-related quality of life in patients with polyneuropathy with and without pain.
Methods: Patients (n = 48) with Chronic Idiopathic Axonal Neuropathy were included. Clinical data were retrieved from medical files. All patients filled out the SF-36 and EQ-5D questionnaires. In addition, blood samples were analyzed for 2-AG, OEA, PEA, and SEA.
Results: Neuropathic pain was reported in 21 of the patients. There were significantly lower levels of 2-AG in patients with neuropathic pain (P = 0.03), but there were no significant differences in OEA (P = 0.61), PEA (P = 0.95), or SEA (P = 0.97) levels. The patients reporting pain in the hands had significantly lower SEA levels, 10.0 versus 15.0 (P = 0.03). The levels of 2-AG were significantly higher among patients reporting paresthesia in their feet (80.1 vs. 56.3; P = 0.02). Levels of PEA, SEA, and 2-AG were decreased in patients with loss of vibration. PEA and SEA were decreased in patients with loss of pain and temperature, and SEA decreased in patients with loss of sense of touch. However, the differences in the levels of bioactive endogenous lipids were not statistically significant when corrected for multiple comparisons.
Conclusion: Alterations of 2-AG levels between polyneuropathy patients with and without neurogenic pain indicate that it could play an essential role. Further studies are warranted.
A comparative Use of Traditional and Multimedia Modes of Teaching Curriculum Studies in English
Authors: Mthethwa Patrick 
Published: May 2022

This study explored students’ preferences on the use of traditional versus multimedia modes of course delivery at the University of Eswatini. Since the advent of technology, the use of traditional and multimedia modes of teaching has received a lot of attention in research. Students’ preferences regarding either being taught in a traditional or technological way is one of the areas that has been investigated intensively, yielding different conclusions. This study was a cross-sectional survey. For data collection, the study used a five-point anchored Likert-scale. Forty-three (43) participants participated in the study. They completed a questionnaire that sought their preferences on the mode of teaching between two courses, CTE 319/519 and CTE320/520. CTE319/519 was taught using multimedia, and CTE 320/520 was taught using the traditional mode. To establish the trends in the students’ preferences, data were analyzed using the statistical package for social science (SPSS), mainly descriptive statistics. The mean, frequencies, modes, and standard deviation were the major domains for data interpretation. The results revealed that the students preferred the use of multimedia over the traditional mode of course delivery. However, when the data were further analyzed using the age-range, the older generation performed better than the younger generation in their preferences on the use of multimedia when teaching. Overall, the results have implications for the integration of multimedia technology when teaching English courses at tertiary.
Neoliberal Religion
Authors: Guest Mathew 
Published: May 2022

Mathew Guest explores neo-liberalism as an account of contemporary western society and considers what this means for our understanding of religion.
The rise of free market economics in western culture is associated with the celebration of competition and prioritization of consumer choices as cultural phenomena that profoundly influence human experience in all areas of life. However, several global political changes – including the election of Donald Trump as US president, ‘brexit’, and the rise of right-wing populism across continental Europe – point to a counter-response. This response emphasizes nativist forms of identity and the affirmation of narrow cultural or ethnic boundaries. Together they reflect a complex and seismic shift in assumptions about the role of the state and the future of social order within developed societies. A crisis in the status of the mass media and the rising prominence of social media add further elements of uncertainty into an already destabilized context.
This book is an accessible, topical discussion of a new set of tools and approaches to understanding contemporary religion and religious movements. In addition, Mathew Guest introduces a number of sociological and ethical questions that arise from considering the status of religion within a neo-liberal age.
Aesthetics of Care
Authors: Saito Yuriko 
Published: May 2022

From fussing over your personal appearance to digging in the garden, Yuriko Saito shows us how we can improve the quality of everyday life by sharpening our aesthetic sensibility.
Introducing us to the unseen assumptions and ideals guiding our daily actions and routines, Saito redirects our aesthetic focus back on objects, environments, and activities and away from the fine arts that have dominated Western discussions, opening the door to Chinese and Japanese ways of thinking about aesthetics.
Using examples and situations we all recognise, Saito identifies core questions lying at the heart of the subject, such as:
• How do you make informed, well-considered aesthetic judgements?
• Are aesthetic experiences always gained as a spectator contemplating an object?
• Can you gain aesthetic experience when you take part in an activity?
By revealing how paying aesthetic attention to day-to-day matters impacts everything from how we hang laundry or weigh up the environmental consequences of eating meat, this introduction teaches us how we can enrich our understanding of philosophy and everyday life when we expand our aesthetic experience.
Attack of the Monster Musical
Authors: Abraham Adam 
Published: May 2022

How many hit musicals are based on films that were shot in two days at a budget of $30,000? The answer is one: Little Shop of Horrors. Roger Corman’s monster movie opened in 1960, played the midnight circuit, and then disappeared from view. Two decades later, Little Shop of Horrors opened Off-Broadway and became a surprise success.
Attack of the Monster Musical: A Cultural History of Little Shop of Horrors chronicles this unlikely phenomenon. The Faustian tale of Seymour and his man-eating plant transcended its humble origins to become a global phenomenon, launching a popular film adaptation and productions all around the world. This timely and authoritative book looks at the creation of the musical and its place in the contemporary musical theatre canon. Examining its afterlives and wider cultural context, the book asks the question why this unlikely combination of blood, annihilation, and catchy tunes has resonated with audiences from the 1980s to the present.
At the core of this in-depth study is the collaboration between the show’s creators, Howard Ashman and Alan Menken. Told through archival research and eyewitness accounts, this is the first book to make extensive use of Ashman’s personal papers, offering a unique and inspiring study of one of musical theatre’s greatest talents.
New Philosophies of Film
Authors: Sinnerbrink Robert 
Published: May 2022

What can philosophy teach us about cinema? Can cinema transform how we understand philosophy? How should we describe the competing approaches to philosophizing on film? New Philosophies of Film answers these questions by offering a lucid introduction to the exciting developments and contentious debates within the philosophy of film. Mapping out the conceptual terrain, it examines both analytic and continental approaches to cinema and puts forward a pluralist film philosophy, grounded in practical examples from film, documentaries and television series.
Now thoroughly updated to showcase the most recent developments in the field, this 2nd edition features:
New chapters on phenomenology, cinematic ethics, philosophical documentary film and television as philosophy, incorporating feminist, socio-political, ethical and ecological approaches to cinemaContemporary case studies including Carol, Roma, Melancholia, two Derrida documentaries, and the Netflix series Black MirrorExpanded coverage of Gilles Deleuze and Stanley Cavell, two of the most influential philosophers of filmAn updated bibliography, filmography and reading lists, with links to online resources to support further study
Demonstrating how the film-philosophy encounter can open up new paths for thinking, New Philosophies of Film is an essential resource for putting interdisciplinary inquiry into practice.