bluuebunny/crossref_metadata_2025_split huggingface dataset:
A rare case of primary coenzyme Q10 deficiency due to COQ9 mutation
Authors: Olgac Asburce , Öztoprak Ülkühan , Kasapkara Çiğdem Seher , Kılıç Mustafa , Yüksel Deniz , Derinkuyu Emine Betül , Taşçı Yıldız Yasemin , Ceylaner Serdar , Ezgu Fatih Süheyl 
Published: December 2019

Abstract

Background
Coenzyme Q10 (CoQ10) serves as a shuttle for electrons from complexes I and II to complex III in the respiratory chain, and has important functions within the mitochondria. Primary CoQ10 deficiency is a mitochondrial disorder which has devastating effects, and which may be partially treated with exogenous CoQ10 supplementation.

Case presentation
A 9-month-old girl patient was referred to our clinic due to growth retardation, microcephaly and seizures. She was the third child of consanguineous parents (first-degree cousins) of Pakistani origin, born at 38 weeks gestation, weighing 2000 g after an uncomplicated pregnancy, and was hospitalized for 3 days due to respiratory distress. She had sustained clonic seizures when she was 4 months old. Physical examination showed microcephaly, truncal hypotonia and dysmorphic features. Metabolic tests were inconclusive. Abdominal ultrasonography revealed cystic appearance of the kidneys. Non-compaction of the left ventricle was detected in echocardiography. Cranial magnetic resonance imaging (MRI) showed hypoplasia of the cerebellar vermis and brain stem, corpus callosum agenesis, and cortical atrophy. A panel testing of 450 genes involved in inborn errors of metabolism (IEM) was performed that showed a novel frameshift c.384delG (Gly129Valfs*17) homozygous mutation in COQ9. A treatment of 5 mg/kg/day exogenous CoQ10 was started when she was 10 months old, and the dosage was increased to 50 mg/kg/day after the exact diagnosis. No objective neurological improvement could be observed after the adjustment of the drug dosage.

Conclusions
We report a case of CoQ10 deficiency due to a novel COQ9 gene mutation that adds clinical data from a newly diagnosed patient. Our case also outlines the importance of genetic panels used for specific diseases including IEM.
Explaining and tackling the informal economy: an evaluation of competing perspectives
Authors: Williams Colin C 
Published: December 2019

AbstractThis paper provides an evidence-based evaluation of the competing ways of explaining and tackling the informal economy. Conventionally, participants have been viewed as rational economic actors who engage in the informal economy when the benefits outweigh the costs, and thus participation is deterred by increasing the sanctions and/or risks of detection. Recently, however, an alternative social actor approach has emerged viewing participation to result from a lack of vertical trust (i.e., their norms, values and beliefs are not in symmetry with the laws and regulations) and horizontal trust (i.e., they believe many others are non-compliant). Reporting 2,000 face-to-face interviews conducted in Croatia in 2015, only a weak and partial association is found between participation in the informal economy and the perceived level of penalties and risks of detection, but a strong significant association with both the level of vertical and horizontal trust. Those who perceive a larger proportion of the population to be engaged in the informal economy, and those whose norms differ to the laws and regulations, display a significantly greater likelihood of participating in the informal economy. The theoretical and policy implications are then discussed.
Three-year-olds infer polite stance from intonation and facial cues
Authors: Hübscher Iris , Wagner Laura , Prieto Pilar 
Published: December 2019

AbstractDespite the evidence that infants are sensitive to facial cues and prosody for the detection of emotion, we have contradictory evidence regarding the use of these cues by older preschool and school children when inferring both emotional and politeness stance. This study assessed preschool aged children’s sensitivity to intonational and facial cues signalling a speaker’s polite stance in requestive speech acts with controlled lexical and contextual materials. Thirty-six 3-year-old American English-speaking children performed a forced-choice decision task which investigated whether children at this age use pitch and/or facial cues to infer a speaker’s affective stance in either audio-only, visual-only or audio-visual presentation modalities, when lexical cues are controlled for. Results showed that (a) children at three years can infer a speaker’s polite stance equally well in all three conditions (audio-only, visual-only and audio-visual) and thereby (b) unlike previous research, in the present task both intonation and facial cues are equally strong cues in children’s understanding of a speaker’s polite stance in requestive speech acts. The authors discuss especially the implications of this early use of intonation to detect politeness, relating it to other previous research on children’s ability to infer meaning from pitch.
Offering group mental health programs in a maximum security correctional facility
Authors: Ghelani Amar 
Published: December 2019

In the following article, the author discusses psychoeducational groups offered in a men’s maximum security prison by the Centre for Addiction and Mental Health’s (CAMH) Forensic Early Intervention Service (FEIS) in partnership with correctional services staff. The author shares observations and experiences facilitating mental health programs with clients in custody and explore related challenges, risks and opportunities. Outcomes and feedback from group participants are reviewed and recommendations for others interested in offering similar programs are presented.
Strength of Plantar- and Dorsiflexors Mediates Step Regularity During a High Cognitive Load Situation in a Cross-sectional Cohort of Older and Younger Adults
Authors: FallahTafti Farahnaz , Watson Kristen , Boron Julie Blaskewicz , Myers Sara A. , Schmid Kendra K. , Yentes Jennifer M. 
Published: December 2019

Background and Purpose:
Completing simultaneous tasks while standing or walking (ie, a high cognitive load situation [HCLS]) is inevitable in daily activities and can lead to interference in task performances. Age-related physical and cognitive changes may confound performance variability during HCLS in older and younger adults. Identification of these confounding effects may reveal therapy targets to maintain optimal physical function later in life. The aim of this study was to investigate the effect of increasing the difficulty levels of an additional motor task and restricting visual information, on gait parameters in younger and older adults while considering the effect of cognitive and physical covariates.

Methods:
Fifteen healthy younger and 14 healthy older adults were asked to complete assessments of cognitive function, balance, and strength. They were then asked to walk on a self-paced treadmill with or without carrying a plastic tray. Opaqueness of the tray (vision) and the presence of water in glasses placed on the tray (increasing task difficulty) were varied. Mean, standard deviation, and regularity (sample entropy) of step width and length were compared across conditions and groups using repeated-measures analyses of variance with and without covariate analysis. Only significantly correlated covariates of cognition, balance, and strength were entered into each model.

Results and Discussion:
Older adults had greater step width irregularity compared with younger adults across all conditions when controlling for concentric plantar- and dorsiflexion strength. A decline in strength may likely alter neuromuscular control of gait, specifically control of step width, which has been associated with fall risk in older adults. Adjusting for the same covariates revealed increased regularity of step length, as visual feedback from the feet was restricted. Specifically, step length was more regular while carrying an opaque tray compared with not carrying a tray. Visual restriction was a contributing factor, which led to more predictable gait kinematics, indicating the role of sensory information to enhance the adaptability during walking under HCLS.

Conclusion:
The knowledge of the regularity behavior of human movement can expand physical therapists' treatment approaches to promote further interactivity and coordination across body systems that model behavior of healthy young individuals. Targeting strength during therapy may provide additional benefits for gait performance under HCLS.
LA SIMPLIFICATION DES FABLES FRANÇAISES POUR LES LYCEENS
Authors: Safira Jihan Ayu , Widayanti Diah Vitri , Handayani Sri 
Published: December 2019

Le curriculum de français de l’année 2013 pour l’enseignement secondaire en Indonésie a évolué. Selon le curriculum 2013 KD 3.8, l’une des compétences visée actuelle est de connaître les fables françaises et de savoir les raconter en tenant compte les éléments linguistiques, la structure textuelle et la fonction sociale. Mais les fables françaises en version originale ne sont pas destinées pour les débutants puisque la plupart du vocabulaire et la grammaire utilisés sont pour le niveau intermédiaire ou avancé. Cet article vise à simplifier les fables françaises pour la matière de français au lycée en Indonésie. C’est une recherche et développement « R&D » adoptant cinq phases : potentiel et problèmes, collecte de données, conception de produit, validation de conception et révision de conception. La source utilisée est les douze livres de Jean de la Fontaine et nous en a pris une de chaque livre au hasard ou random sampling. La simplification recouvre trois éléments, ce sont : le lexique, la syntaxe et le texte en considérant également la structure de la fable. A part cela, pour aider la compréhension des apprenants, les fables simplifiées sont équipées des illustrations. Ce travail a passé les étapes de validation par l’expert et révision de conception.
The French curriculum of 2013 for secondary education in Indonesia has changed. One of the current skills is having knowledge about French fables and to retell them by considering linguistics elements, the textual structure ant the social function. But the French fables in original version on website or book story are not intended for beginners because most of the vocabulary and grammar used are for the intermediate or advanced level. This article aims to simplify the French fables for the lesson of French in senior high school in Indonesia. It is a research and development « R&D » adopting five phases: potential and problems, collecting the data, product design, validating the product and revising the product. The source used is the twelve books of Jean de la Fontaine and we took one from each book randomly or random sampling. The simplification covers three elements, these are: the lexicon, the syntax, and the text considering also the structure of the fable. In addition, to help learners’ comprehension, the simplified fables are equipped with illustrations. This work has passed the steps validation by the expert and design review.
LES FACTEURS INFLUENÇANT LA MOTIVATION DES ÉTUDIANTS DE CHOISIR LE PROGRAMME D’ÉTUDES DE PÉDAGOGIQUEDU FRANÇAIS À L’UNIVERSITÉ DE SEMARANG
Authors: Rahmawati Kartika Novi , Astuti Dwi , Purwani Neli 
Published: December 2019

La sélection du programme d'études par les étudiants est déterminée par leurs motivations, la motivation divise en deux types, ce sont la motivation interne et la motivation. L’objectif de cette recherche est de décrire la motivation des étudiants de choisir le programme d'éducation en français. Cette recherche est une recherche descriptive qualitative. La population de cette recherchesontles étudiants du premier semestre du programme d’études de l’enseignement du français à l’université de Semarang.La méthode de cette recherche est le questionnaire. Les questionnaires dans cette recherche utilisée la validité de la construction et pour savoir la fiabilité de l'instrument utilisé formule KR-21. Basé sur les résultats de l'analyse des données peut être conclu que les facteurs influençant la motivation des élèves à choisir un programme d'éducation en français qui est un facteur intrinsèque et un facteur extrinsèque. Les facteurs que beaucoup d'élèves choisissent pour choisir un programme d'éducation en langue française avec sous-variablessont un facteur intrinsèque.
Selecting the study programs by students is determined by the motivation of each individual, the motivation can be divided into internal motivation and external motivation. Based on this background, researcher wanted to examine what are the motivations of students in choosing French education program at the Semarang State University. The purpose of this research is to describe students’ motivation in choosing French education program.This research is a qualitative descriptive research. Subjects in this research are students of French education in the first semester. Method for collecting data used in this research was questionnaire. Questionnaires in this study used the construct validity and to measure the reliability of the instrument use KR-21 formula. Based on the results of data analysis can be concluded that factors influencing the motivation of students in choosing French language education program that is intrinsic factor and extrinsic factor. Factors that many students choose in choosing a French language education program is an intrinsic factor.
Victory in Tragic Ending: Analysis of Sophocles’ "Oedipus The King"
Authors: Aji Putra Madha Dwi , Ari Widayanti Maria Johana 
Published: December 2019

This study aims on revealing the concept of victory through the Tragical ending which reflected from Oedipus the King drama written by Sophocles. The objectives of this study are to explain How the concept of Victory in Tragic ending described in Sophocles’ Oedipus the King. This study is a qualitative study which applied Strauss’ Structuralism Theory and used discourse instrinsic elements of drama approach. The objects of this study include the material objects which are the drama “Oedipus the King” written by Sophocles as well as formal object which is the intrinsic elements of drama, such as dialogue, staging, characters, plot and themes which reflected from the dialogue in the drama. The data of this study is a qualitative data which is in the form of dialogues on the drama. From the data analysis, it was found that (1) intrinsic elements of drama analysis such as, dialogue, staging, character, plot and themes put a contribution. The contribution of the dialogue, staging, character, plot and themes is considered as a result of the supportive data which the writer used to analyze the main goal of this analysis using Strauss’ binary oppposition. (2) By the Strauss’ Binary Opposition, it appears that both of protagonist and antagonist of the story which this case between Oedipus and Teireisias shows their own figure. In the end, Teireisias who had a limited vision and the bad words when he talks to Oedipus, he has the knowledge behind his lackness and his knowledge is categorized as divine knowledge, since his every words he said was proven in the last of the story. (3) Concept of victory that the writer discovered after doing the binary opposition between Oedipus and Teireisias, then from the Oedipus’ oppositions the writer seeks the evidence that behind all of the Oedipus weakness againtsTeireisias, there still a concept of winning or victory. These evidences was obtained through the dialogues which occured in the tragic ending of Oedipus of the King Drama and shows that the tragic ending in Oedipus the King story writtten by Sophocles were symbolized as Victory.

Keywords: Intrinsic elements of drama; Binary Opposition; Structuralism
Self-Actualization in Main Character’s Life Journey in "The Bell Jar"
Authors: Nur Farid Muhammad Fadhli , Rosyidi Mohamad Ikhwan 
Published: December 2019

Self-actualization is term that is commonly used in psychology theories. The term can be simply translated as the full realization of someone’s potential or true self. This study was conducted to analyze the life journey of the main character of The Bell Jar in achieving herself-actualization. The purposes of this study are to describe the character of the main character in The Bell Jar and to describe the main character in achieving self-actualization and how it is portrayed in the novel. This study was done using a descriptive qualitative method and using Maslow's Hierarchy of Human Needs theory and New Criticism theory to explain the main character and her journey in achieving self-actualization in the form of words. The object of the study was the novel The Bell Jar and this study focused on Esther Greenwood as the main character. List of an observational sheet was used as the research instrument and the data was taken through library research. The analysis was conducted by interpreting Esther’s utterances and actions using New Criticism theory to describe Esther’s characterization and Maslow’s Hierarchy of Human Needs theory to describe Esther’s self-actualization. The study results showed that Esther’s self-actualization is the results of her being able to fulfill her previous stages both directly and indirectly before reaching self-actualization stage along her life journey.
Keywords: Characterization, Hierarchy of Human Needs, Life Journey, New Criticism, Self-actualization
Analysis of Actantial Model in Suzanne Collins’ "The Hunger Games"
Authors: Dzikriya Uliya 
Published: December 2019

This study aims to analyze plot structure in Collins’ The Hunger Games. There are several questions that researcher wants to find, what the elements of plot are, how do the elements of plot compose the plot structure of the story, and what kind of plot is applied in the novel. This study is a qualitative analysis by applying A.J. Greimas approach. The data were collected by reading, identifying, interpreting and analyzed using the approach and theories which used in this study by using actants. The result of this study were the elements of plot consist of beginning, problem of the story, rising action, climax, falling action, and resolution. To find out the main plot structure, this study classifies the function of each character into six actants, they are sender, receiver, subject, object, helper and opponent. Finally, the researcher concluded what kind of plot and how the ending of the novel is. The plot of the novel is dramatic or cronological plot because the story through in chronological order. In addition, the novel is closed plot because the problem of the story is solved.
Keywords: Actant, Greimas, Plot, Sructuralism
Translation Quality of Idiomatic Expressions in "Lenka Kripac’s" Song Lyrics From English into Indonesian
Authors: Admiati Admiati , Hartono Rudi 
Published: December 2019

This study aims to find out translation quality of idiomatic expressions in Lenka Kripac’s song lyric from English into Indonesian. The objectives of this study are to find out the accuracy, the acceptability, and the readability of the translation of idiomatic expression which are translated in Indonesian. The translation assessment proposed by Nababan (2012). This study was conducted a descriptive qualitative research. Descriptive research is a procedure of problem solving by describing the subject or the object of the study based on the visible facts. Qualitative research does not deal with testing of a theory or hypothesis, but it deals with understanding of theory. The data were obtained by content analysis and questionnaire. The data were obtained by handing out accuracy rating instruments to three expert respondents then acceptability and readability rating instruments to fifteen respondents. The result of this study showed that there 185 data and 86 idioms. The idioms which were classified into six categories by Seidl & McMordie’s theory. There is kind of idiomatic expressions; those are (1) Phrasal Verbs Idiom (2) Prepositional Phrase Idiom (3) Idiom with verbs as keywords (4) Idiom with nouns as keywords (5) Idiom with adjectives as a key (6) Idiomatic pairs. The mostly used idiomatic expression in these songs is a translation by Phrasal Verbs Idiom. Then, in term of accuracy, there (78,74 %) were accurate, (19,46%) were less accurate, and ( 1,8 %) were inaccurate. In the term of acceptability, there were (68,95%) were acceptable, (24,15%) was less acceptable, and (6,9 % ) were unacceptable. In the term of readability, there were (78,2 %) readable, (16,60%) were less readable, and (5,15%) were unreadable.
Keywords: acceptability, accuracy, idioms, readability, translation quality
Revealing The Meaning of Pilgrim’s Journey for Searching Salvation in Hermann Hesse’s "Siddhartha"
Authors: Shofiyani Yufi , Yulianto Henrikus Joko 
Published: December 2019

This study is the analysis of the character Siddhartha’s pilgrimage journey in the novel entitled Siddhartha. There are three objectives of this study. The first is to describe the conflict between human’s worldly desires and his spiritual quest in Hermann Hesse’s novel, Siddhartha. The second is to explain how Siddhartha’s conflict between his worldly affairs and spiritual journey in view of Gadamer’s hermeneutics. And, the third is to explain how Siddhartha’s worldly and spiritual duality as a revelation of human’s conflict in general. The analysis and the description of the data are provided to find the conclusion. The analysis of this study is using Gadamer’s Hermeneutics theory. The study shows that the main character Siddhartha is searching for salvation through the ordeal life. He learned many teachings from Hinduism, being ascetic Samana, learned Buddha’s teachings, being worldly slave as the courtesan adherent, and finally he finds salvation from the river. In his journey to find salvation, Siddhartha is difficult to find teachings can satisfy him. He got the internal conflict between his worldly and spiritual quest when he became courtesan’s adherent. However, all the inner conflicts that Siddhartha felt have big roles and led him to make the next decision in his life. In the end of Siddhartha’s journey, finally he found salvation. The river has succeeded to change Siddhartha by its voice and “listen” became the key why he gets salvation in his life. Besides, there is the fact that Siddhartha’s story modelled itself on the journey of Siddhartha Gautama the Buddha. Hesse’s Siddhartha was written based on the author’s travel experience in India. The conflict that Hesse portrayed in that story mostly faced by people. In the novel, Hesse characterized Siddhartha as an individual who later found his life wisdom in an act of listening to the voices of nature. Listening means everything for Siddhartha.
Keywords: Gadamer’s hermeneutics, internal conflict, pilgrim’s journey
Religión y razón pública
Authors: Díaz Jorge Aurelio , Rojas Claros Armando 
Published: December 2019

Se busca confrontar el llamado que hiciera Alwin Plantiga a los filósofos cristianos para que asuman los intereses de la comunidad a la que pertenecen, con el análisis que realiza Iván Garzón sobre el lugar de la religión en el ejercicio de la razón pública, con el propósito de examinar los cambios que se han producido, dentro del pensamiento político liberal, en lo que concierne al lugar de la religión en la organización de la sociedad. Esto le plantea a los creyentes nuevos retos para lograr, desde sus convicciones religiosas, participar activamente en la organización de sociedades que logren establecer formas de convivencia pacífica que respeten la creciente diversidad cultural de los ciudadanos.