Miracle-dz/UniFAR-FTUs huggingface dataset:
Adult-Onset Niemann-Pick Type C Disease: Clinical, Biochemical, and Genetic Study

Background: Niemann-Pick type C disease is an autosomal recessive neurometabolic disorder of unknown origin mapped to chromosome 18q11-12 in most of the studied families. In contrast to the sphingomyelin lipidoses, in Niemann-Pick type C disease, fibroblasts are impaired in intracellular homeostatic responses to exogenous lowdensity lipoprotein (LDL) cholesterol. Biochemical heterogeneity of the disorder in relation to abnormal LDL processing is associated with various clinical presentations, but adult-onset Niemann-Pick type C disease is rare and has not been comprehensively characterized. Objective: To describe clinical, biochemical, and genetic features of adult-onset Niemann-Pick type C disease in 3 siblings. Design and Setting: Case series in a tertiary care center. Patients: The 3 siblings manifested a variable combination of vertical supranuclear ophthalmoplegia, ataxia, and splenomegaly. Brain magnetic resonance imaging showed cerebellar atrophy; brainstem auditory evoked responses were unobtainable, and bone marrow examination disclosed typical foam cells. The patients were 20, 26, and 28 years old and belonged to a sibship of 13 born of consanguineous healthy parents. Methods: Esterification of exogenous LDL cholesterol in cultured skin fibroblasts and filipin staining for free intracellular cholesterol. Polymerase chain reaction—based DNA linkage study using AC microsatellite markers D18S40 , D18S44 , D18S480 , and D18S66 . Results: Fibroblasts of the 3 patients showed a 23% to 58% block in the induced cholesterol esterification after 4½ hours and a mild to moderate accumulation of free cholesterol. DNA study demonstrated linkage to the major 18q11-12 Niemann-Pick type C locus and identified unaffected carriers. Conclusions: These results confirm the diagnosis of the least biochemically affected Niemann-Pick type C phenotype in this family with adult-onset disease and support a correlation between the mild laboratory and clinical findings in this age group.
The False Dichotomy between Positive and Negative Affect in Game Play

Most of the time games make us happy, but sometimes they are frustrating or make us feel sad. They allow us to experience pleasure, success and joy, but they can also yield feelings of frustration, failure, or sorrow from darker themes. In games, we can experience the full range of emotions -- both positive and negative. While a positive experience is often the goal, there are many ways in which negative affect can enhance play. First, the almost masochistic experience of failure and frustration within play can lead to intense positive feelings when overcome. Second, negative emotional experiences, such as feeling uncomfortable, guilty, or sad can also provide additional emotional range that is valued by players. Third, a number of games have emerged in recent years that encourage players to think about difficult or challenging issues that are unlikely to engender positive emotions. The CHIPLAY 2015 False Dichotomy Workshop focuses on the range of valence in games and invites experts from across fields to contribute to our understanding of the interplay between positive and negative affect within play. The workshop goals are to investigate the interplay between positive and negative affect, identify gaps in our knowledge, determine future research directions, and build the community of people interested in the false dichotomy between positive and negative affect in games. The workshop will consist of a brief introduction game, followed by group brainstorming, small group interaction, and a closing plenary discussion.
On Generative Adversarial Network Based Synthetic Iris Presentation Attack And Its Detection

On Generative Adversarial Network Based Synthetic Iris Presentation Attack And Its Detection by Naman Kohli Human iris is considered a reliable and accurate modality for biometric recognition due to its unique texture information. Reliability and accuracy of iris biometric modality have prompted its large-scale deployment for critical applications such as border control and national identification projects. The extensive growth of iris recognition systems has raised apprehensions about the susceptibility of these systems to various presentation attacks. In this thesis, a novel iris presentation attack using deep learning based synthetically generated iris images is presented. Utilizing the generative capability of deep convolutional generative adversarial networks and iris quality metrics, a new framework, named as iDCGAN is proposed for creating realistic appearing synthetic iris images. In-depth analysis is performed using quality score distributions of real and synthetically generated iris images to understand the effectiveness of the proposed approach. We also demonstrate that synthetically generated iris images can be used to attack existing iris recognition systems. As synthetically generated iris images can be effectively deployed in iris presentation attacks, it is important to develop accurate iris presentation attack detection algorithms which can distinguish such synthetic iris images from real iris images. For this purpose, a novel structural and textural feature-based iris presentation attack detection framework (DESIST) is proposed. The key emphasis of DESIST is on developing a unified framework for detecting a medley of iris presentation attacks, including synthetic iris. Experimental evaluations showcase the efficacy of the proposed DESIST framework in detecting synthetic iris presentation attacks.
Twitter hashtags from ad hoc to calculated publics

As the use of Twitter has become more commonplace throughout many nations, its role in political discussion has also increased. This has been evident in contexts ranging from general political discussion through local, state, and national elections (such as in the 2010 Australian elections) to protests and other activist mobilisation (for example in the current uprisings in Tunisia, Egypt, and Yemen, as well as in the controversy around Wikileaks). Research into the use of Twitter in such political contexts has also developed rapidly, aided by substantial advancements in quantitative and qualitative methodologies for capturing, processing, analysing, and visualising Twitter updates by large groups of users. Recent work has especially highlighted the role of the Twitter hashtag – a short keyword, prefixed with the hash symbol ‘#’ – as a means of coordinating a distributed discussion between more or less large groups of users, who do not need to be connected through existing ‘follower’ networks. Twitter hashtags – such as ‘#ausvotes’ for the 2010 Australian elections, ‘#londonriots’ for the coordination of information and political debates around the recent unrest in London, or ‘#wikileaks’ for the controversy around Wikileaks thus aid the formation of ad hoc publics around specific themes and topics. They emerge from within the Twitter community – sometimes as a result of pre-planning or quickly reached consensus, sometimes through protracted debate about what the appropriate hashtag for an event or topic should be (which may also lead to the formation of competing publics using different hashtags). Drawing on innovative methodologies for the study of Twitter content, this paper examines the use of hashtags in political debate in the context of a number of major case studies.
Is CEM43 still a relevant thermal dose parameter for hyperthermia treatment monitoring

CEM43 has its roots in the direct cytotoxic effect of heat, whereby the amount of cell death depends on the temperature and exposure time. CEM43 is a normalising method to convert the various time-temperature exposures applied into an equivalent exposure time expressed as minutes at the reference temperature of 43 °C. The CEM43 concept also holds a number of weaknesses, however. When used to predict treatment efficacy of combined radiotherapy plus hyperthermia, CEM43 does not include the effect of sensitisation by enhanced oxygenation, variation in interval time and the effect of multiple fractions. Further, it does not include the effect of increased perfusion at the lower thermal dose - and the occurrence of stasis at the higher thermal dose. Overall, studies towards a thermal dose-effect relationship in radiotherapy plus hyperthermia present a diffuse message without a definitive conclusion. However, prospective studies and studies with large patient numbers did report significant thermal dose-effect relationships and provide a good reason to continue research in the CEM43 model. Such research would be best performed in homogeneous patient groups with a single pathology and a low variation in tumour size and heterogeneity. Further, high quality thermometry, strict treatment schedules with fixed intervals and preferably homogenous heating are important requirements to enhance the probability of detecting a thermal dose-effect relationship. The slowly growing availability of hybrid MR hyperthermia systems should be a strong stimulus to expand these studies with the inclusion of measuring thermal dose-dependent blood flow and oxygen changes in the tumour and normal tissues.
DESTINATION MARKETING STRATEGY IN BALI THROUGH OPTIMIZING THE POTENTIAL OF LOCAL PRODUCTS

This study was designed to study destination marketing strategy in Bali through optimizing the potential of local products. Seventy nine of hotel managers were interviewed based on cluster sampling method to gain their point of view. The results show that destination must build their images around unique attributes that provide them sustainable competitive advantage including its attraction which should be designed to meet the needs of the target market and should be served by local products. The results also show that hotel managers thought that foreign tourists always preferred imported products, meanwhile previous statistical results indicate that foreign tourists significantly look for local products. There is a need to encourage hotel managers to change their perception and attitude about local and imported products. In fact, hoteliers expressed willingness to use local products as long as these meet the quality standard. As tourism involves four types of activities, namely something to see, something to do, something to buy, something to learn, destination product development could be focused in the above activities through offering foreign tourist, such as to stay in hotels, homestays or villas owned by Balinese; to eat in restaurants owned by Balinese by choosing the authentic local foods that are using local meat, seafood and vegetables, exotic local fruits and beverages; and to buy products that are produced by the Balinese. By promoting vacation on the real Balinese atmosphere such as stay in accommodations owned by the Balinese supported by the authenticity of local Balinese foods, fruits and beverages, these will strengthen the local economy, so the benefit of tourism development can be more beneficial to the local Balinese. The results suggests that destination management related to improvement of service and hospitality are really important through improvement of human resource by giving training to their employees, educate consumers, and manage consumer expectations. Physical evidence can be easily associated with the product by the customer. This includes the atmosphere of the service operation and any tangible evidence used to market the product. Marketing through authenticity is precise approach for success in destination marketing of Bali tourism. Destination marketing strategy should then be focussed on promoting the physical evidences of the authenticity of the local products and excellent services offered to the visitors as well as participation of stakeholders in developing authenticity of local products at reasonable price which will differentiate the destination distinctively.
Pfadbedingungen in Abhängigkeitsgraphen und ihre Anwendung in der Softwaresicherheitstechnik

Diese Arbeit präsentiert eine neue Methode zur Sicherheitsanalyse von Software im Bereich der Manipulationsprüfung und der Einhaltung von Informationsflüssen zwischen verschiedenen Sicherheitsniveaus. Program-Slicing und Constraint-Solving sind eigenständige Verfahren, die sowohl zur Abhängigkeitsbestimmung als auch zur Berechnung arithmetischer Eigenschaften verwendet werden. Die erstmalige Kombination dieser beiden Verfahren mittels Pfadbedingungen liefert nicht nur binäre Abhängigkeitsinformationen wie Slicing, sondern exakte notwendige Bedingungen über die Informationsflüsse zwischen zwei Programmpunkten. Constraint-Solver können domänenspezifisch diese Bedingungen für Eingabevariablen auswerten und somit zeugenähnliche Variablenbelegungen ermitteln, unter denen die diagnostizierten Informationsflüsse tatsächlich stattfinden. Neben der Definition der Grundlagen von Abhängigkeitsgraphen und einfachen Pfadbedingungen werden neue Erweiterungen für kontextsensitive interprozedurale Pfadbedingungen gezeigt und die Integration von domänenspezifischen Verfahren für Arrayfelder und abstrakten Datentypen demonstriert. Der Schwerpunkt der Arbeit liegt in der Realisierung von Pfadbedingungen für echte Programme in echten Programmiersprachen. Hierfür werden Verfahren vorgeschlagen, realisiert und empirisch untersucht, wie Pfadbedingungen für große Programme skalieren. Die zum Einsatz kommenden Techniken sind u.a. Intervallanalyse und Binäre Entscheidungsgraphen, mit denen die generelle exponentielle Komplexität von Pfadbedingungen beherrschbar wird. Fallstudien für den Einsatz von Pfadbedingungen und die empirische Untersuchung mehrerer Verfahren zur Intervallanalyse zeigen, dass Pfadbedingungen für die praktische Programmanalyse und das Programmverstehen geeignet und empfehlenswert sind. Zur Veranschaulichung wird hierfür eine Manipulation sicherheitskritischer Berechnungen aufgedeckt und analysiert.
Adverse effects of organophosphorus pesticides on the liver: a brief summary of four decades of research

Abstract Organophosphorus pesticides (OPs) are widely used volatile pesticides that have harmful effects on the liver in acute and chronic exposures. This review article summarises and discusses a wide collection of studies published over the last 40 years reporting on the effects of OPs on the liver, in an attempt to propose general mechanisms of OP hepatotoxicity and possible treatment. Several key biological processes have been reported as involved in OP-induced hepatotoxicity such as disturbances in the antioxidant defence system, oxidative stress, apoptosis, and mitochondrial and microsomal metabolism. Most studies show that antioxidants can attenuate oxidative stress and the consequent changes in liver function. However, few studies have examined the relationship between OP structures and the severity and mechanism of their action. We hope that future in vitro, in vivo, and clinical trials will answer the remaining questions about the mechanisms of OP hepatotoxicity and its management.
Debiasing Advertising: Balancing Risk, Hope, and Social Welfare

This article explores the nuances of “debiasing through law,” a regulatory approach proposed by Christine Jolls and Cass Sunstein. Proponents have described advertising regulation as a form of debiasing through law. Debiasing approaches regulation through less paternalistic means using strategies that improve consumer decision making. Proponents believe that the improved decision making will gracefully lead to better choices. These debiasing strategies find some of their roots in addressing human cognitive biases and bounded rationality. Although debiasing through law purports to offer an attractive alternative to pure paternalism, this Article demonstrates that the net social welfare effects of this approach can prove difficult to anticipate. An examination of the recent tightening of the regulation of consumer endorsements in advertising illuminates the challenges and nuances of the debiasing approach. This Article attempts to answer a myriad of questions. Does removing the consumer endorsement tool from the marketing arsenal ensure that consumers will make better decisions? The truthful consumer endorsement can offer other consumers legitimate hope. When netted out, does taking hope off the market advance consumer welfare? Can regulators remove true information from the marketplace and expect to foster an improvement in social welfare? Can regulators preserve real choice while simultaneously improving decisions? Are regulators truly capable of harnessing cognitive and social science to engage in this sort of precision welfare engineering? Consumer endorsement regulation presents a live proxy for the analytical dissection of debiasing through law. Some scholars have expressed a keen skepticism toward this approach to regulation and toward the whole of behavioral law and economics. This Article presents a cautionary tale about the unpredictability of the net outcomes from deploying debiasing through law.
Wireless sensor networks with energy harvesting technologies: a game-theoretic approach to optimal energy management

Energy harvesting technologies are required for autonomous sensor networks for which using a power source from a fixed utility or manual battery recharging is infeasible. An energy harvesting device (e.g., a solar cell) converts different forms of environmental energy into electricity to be supplied to a sensor node. However, since it can produce energy only at a limited rate, energy saving mechanisms play an important role to reduce energy consumption in a sensor node. In this article we present an overview of the different energy harvesting technologies and the energy saving mechanisms for wireless sensor networks. The related research issues on energy efficiency for sensor networks using energy harvesting technology are then discussed. To this end, we present an optimal energy management policy for a solar-powered sensor node that uses a sleep and wakeup strategy for energy conservation. The problem of determining the sleep and wakeup probabilities is formulated as a bargaining game. The Nash equilibrium is used as the solution of this game.
Improved fault location computation using prony analysis for short duration fault

Estimating accurate phasor calculations and selection of best fault data window are the basic requirements of any fault location algorithm. This paper highlights the issues with conventional Fourier transform based filtering techniques to compute fundamental components. It also discusses the effect of RDFT on fault location especially in case of short duration faults. The paper introduces an improved fault location scheme based on Prony analysis. The comparison of RDFT filter and Prony analysis for the computation of fault location is also presented. Performance of the proposed scheme is showcased with the help of case studies and practical data.
Effectiveness of picture books for Italian instruction at Japanese universities

Abstract Recently, the use of children's picture books to teach English has been increasing in Japan. An advantage of these books is the high proportion of basic vocabulary they include. Can picture books also be useful for teaching Japanese students Italian and increasing their motivation? The present study analyses the effectiveness of employing a picture book for Italian instruction using a questionnaire survey administered to 39 Japanese learners of Italian at three Japanese universities. L'Acca in fuga by Gianni Rodari and Nicola Buiat was enjoyed by more than 92% of the participants. A total of 92.3% admitted that their degree of interest in the content was linked with their motivation to read children's literature in Italian. In addition, 58.9% admitted to experiencing a sense of fulfilment after reading the book, thus increasing their motivation to learn the language. As for grammatical aspects, 74.3% agreed that the book was useful for learning several types of past tense. Moreover, 69.2% and 79.5% agreed that the picture book effectively helped them understand the conditional and subjunctive moods. Based on these results, we conclude that picture books can facilitate significant adult learning. Japanese adult learners described enjoying the experience of reading Italian picture books at university, and found them to be useful for learning grammar, developing vocabulary and enhancing their intercultural understanding.
Isotope effects associated with the preparation and methylation of fatty acids by boron trifluoride in methanol for compound-specific stable hydrogen isotope analysis via gas chromatography/thermal conversion/isotope ratio mass spectrometry.

RATIONALE Compound-specific stable hydrogen isotope analysis of fatty acids is being used increasingly as a means of deriving information from a diverse range of materials of archaeological, geological and environmental interest. Preparative steps required prior to isotope ratio mass spectrometry (IRMS) analysis have the potential to alter determined δD values and hence must be accounted for if accurate δD values for target compounds are to be obtained. METHODS Myristic, palmitic, stearic, arachidic and behenic saturated fatty acids were derivatised to their respective fatty acid methyl esters (FAMEs), using 14% (w/v) boron trifluoride in methanol then analysed by gas chromatography/thermal conversion/IRMS (GC/TC/IRMS). FAMEs generated from fatty acid sodium salts of unknown δD values were then used to test a correction factor determined for this method of derivatisation. RESULTS Derivatisation was found to alter the hydrogen isotopic composition of FAMEs although this effect was reproducible and can be accounted for. The difference between the mean corrected and mean bulk δD values was always less than 6.7 ‰. Extraction of saturated fatty acids and acyl lipids from samples, subsequent hydrolysis, then separation on a solid-phase extraction cartridge, was found to alter the determined δD values by less than one standard deviation. CONCLUSIONS Overall, it has been shown that for natural abundance hydrogen isotope determinations, the isolation and derivatisation of extracted fatty acids alters the determined δD values only by a numerical increment comparable with the experimental error. This supports the use of the described analytical protocol as an effective means of determining fatty acid δD values by GC/TC/IRMS.