bluuebunny/crossref_metadata_2025_split huggingface dataset:
Non-dystrophic myotonias: clinical and mutation spectrum of 70 German patients
Authors: Vereb Noemi , Montagnese Federica , Gläser Dieter , Schoser Benedikt 
Published: December 2020

Abstract
Introduction
Non-dystrophic myotonias (NDM) are heterogeneous diseases caused by mutations in CLCN1 and SCN4A. The study aimed to describe the clinical and genetic spectrum of NDM in a large German cohort.

Methods
We retrospectively identified all patients with genetically confirmed NDM diagnosed in our center. The following data were analyzed: demographics, family history, muscular features, cardiac involvement, CK, EMG, genotype, other tested genes, treatment perceived efficacy.

Results
70 patients (age 40.2 years ± 14.9; 52.8% males) were included in our study (48 NDM-CLCN1, 22 NDM-SCN4A). The most frequent presenting symptoms were myotonia (NDM-CLCN1 83.3%, NDM-SCN4A 72.2%) and myalgia (NDM-CLCN1 57.4%, NDM-SCN4A 52.6%). Besides a more prominent facial involvement in NDM-SCN4A and cold-sensitivity in NDM-CLCN1, no other significant differences were observed between groups. Cardiac arrhythmia or conduction defects were documented in sixNDM-CLCN1 patients (three of them requiring a pacemaker) and one patient with NDM-SCN4A. CK was normal in 40% of patients. Myotonic runs in EMG were detected in 89.1% of CLCN1 and 78.9% of SCN4A. 50% of NDM-CLCN1 patients had the classic c.2680C>T (p.Arg894*) mutation. 12 new genetic variants are reported. About 50% of patients were not taking any anti-myotonic drug at the last follow-up. The anti-myotonic drugs with the best patient’s perceived efficacy were mexiletine and lamotrigine.

Conclusion
This study highlights the relevant clinical overlap between NDM-CLCN1 and NDM-SCN4A patients and warrants the use of early and broad genetic investigation for the precise identification of the NDM subtype. Besides the clinical and genetic heterogeneity, the limited response to current anti-myotonic drugs constitutes a continuing challenge.
Mapping the neural circuitry of predator fear in the nonhuman primate
Authors: Montardy Quentin , Kwan William C. , Mundinano Inaki C. , Fox Dylan M. , Wang Liping , Gross Cornelius T. , Bourne James A. 
Published: December 2020

AbstractIn rodents, innate and learned fear of predators depends on the medial hypothalamic defensive system, a conserved brain network that lies downstream of the amygdala and promotes avoidance via projections to the periaqueductal gray. Whether this network is involved in primate fear remains unknown. To address this, we provoked flight responses to a predator (moving snake) in the marmoset monkey under laboratory conditions. We combined c-Fos immunolabeling and anterograde/retrograde tracing to map the functional connectivity of the ventromedial hypothalamus, a core node in the medial hypothalamic defensive system. Our findings demonstrate that the ventromedial hypothalamus is recruited by predator exposure in primates and that anatomical connectivity of the rodent and primate medial hypothalamic defensive system are highly conserved.
The common understanding of simplification approaches in published LCA studies—a review and mapping
Authors: Gradin Katja Tasala , Björklund Anna 
Published: December 2020

Abstract
Purpose
This paper aims to investigate the common understanding of the variety of simplifications in LCA, by reviewing what simplification approaches are described in LCA and propose how these simplifications can be categorised. Such an overview can give guidance to researchers/practitioners as to how they should document simplifications and explain their implications to decision-makers.

Methods
The basis for this study is a systematic literature review of simplification approaches in LCA, including both previously published overviews of categories of LCA simplification approaches and LCA case studies using different simplification approaches. The PRISMA statement protocol (Moher et al. 2009) was used to minimise the risk of bias, increase scientific validity and provide guidelines for conducting the review.

Results and discussion
In all, ten categories of simplification were identified in the literature. Initially, six simplification approaches were identified based on previous categories. However, not all approaches found in case studies fit into these six previously published categories; these were therefore examined and grouped with regard to what was simplified and how, and four additional categories were identified.
The identified simplification categories were mapped and explained in terms of their role in the different stages of the LCA framework. Our results support the idea that simplifications in LCA are most often motivated by a lack of data. Most simplifications target the inventory analysis step, with an aim to reduce the inventory analysis effort.

Conclusions and recommendations
There is a need for a common simplification terminology and reporting standard. As this study shows, the categories of simplification from early studies remain relevant despite the development in LCA over the years, but additional categories are needed to cover the different types of simplification being applied in LCAs. What this study also highlights is despite recommendations that have been around for decades, there is still a lack of clear documentation of simplification with consistent terminology. One way, to ensure more transparent documentation of simplified studies and to improve the ability to interpret them and compare results, could be to include the development of a communication standard, with clear terminology as well as investigation of the applicability of different simplification approaches for different product systems and application areas. Due to the wide variety of purposes, scenarios and products assessed, it is impossible to devise a one-size-fits-all approach for simplifications. LCA practitioners need to describe, explain and evaluate the simplifications used.
Fantasy Football (Soccer) Playing and Internet Addiction Among Online Fantasy Football Participants: A Descriptive Survey Study
Authors: Columb David , Griffiths Mark D. , O’Gara Colin 
Published: December 2020

AbstractThe aim of the present study was to ascertain the levels of possible internet addiction within fantasy football (FF) (soccer) participants and the characteristics of the participants within this group. An online survey of questions regarding characteristics of regular FF participants and consumption of FF-related content was posted on FF internet forums (Reddit and Boards.ie). Self-selecting participants (N = 684) completed the survey containing questions on FF (time spent during weekdays/weekend on FF, gambling on FF, devices used to access FF), internet use (time spent on internet on weekdays/weekends) and an internet addiction screening questionnaire (Chen Internet Addiction Scale). Subgroup analysis was performed on each variable by nationality (Irish, UK and worldwide). Of the 684 participants, 17.5% (diagnostic) and 24.9% (screening) participants met criteria for internet addiction, above the expected level in the general population. The most frequent time spent on FF during weekdays was 30–60 min per day (32.2%) and 1–2 h per day on weekends (29.1%). Over half of participants (50.6%) gambled on FF with the majority (61.3%) gambling once per year and 74.3% of participants gambling less than €50 per year on FF. Avid FF participants demonstrated an increased likelihood of internet addiction compared prevalence rates of previous epidemiological studies among different cohorts. This may be due to FF itself and the increased consumption of FF-related content. Further large-scale nationally representative studies are required to compare regular and casual participants of FF in relation to possible internet addiction.
EVALOG
Authors: Prümper Jochen , Schneeberg Tom , Prümper Alina Marie 
Published: December 2020

Zusammenfassung
Hintergrund
In Kleinstbetrieben werden Arbeitsplatzevaluierungen/Gefährdungsbeurteilungen – insbesondere zur psychischen Belastung – seltener umgesetzt als in größeren Betrieben.

Ziel der Arbeit
Mit EVALOG existiert seit jüngstem ein Verfahren zur Evaluierung psychischer Belastung in Kleinstbetrieben. EVALOG beruht auf dem KFZA (Kurzfragebogen zur Arbeitsanalyse), der im Rahmen der Gefährdungsbeurteilung psychischer Belastung als Standardinstrument gilt. Der vorliegende Beitrag geht der Frage nach, ob EVALOG die Gütekriterien der Gebrauchstauglichkeit gem. EN ISO 10075‑3 (2004) erfüllt und wie die Verständlichkeit, Handhabbarkeit, Teilnahmemotivation und emotionale Reaktion aus Sicht von Evaluierenden und Beschäftigten beurteilt wird.

Material und Methoden
Die Studie wurde durch standardisierte Fragebögen im Feld erhoben sowie mithilfe deskriptiver sowie inferenzstatistischer Verfahren ausgewertet. Es nahmen 19 Evaluierer/-innen und 27 Mitarbeiter/-innen aus 19 österreichischen Kleinstbetrieben teil.

Ergebnisse
Der EVALOG wurde eine hohe Gebrauchstauglichkeit attestiert, die Informationen zur Evaluierung waren eingängig und gut verständlich und die einzelnen Schritte der Evaluierung einfach umzusetzen. Zudem waren die Teilnehmer/-innen der Studie hoch motiviert den Evaluierungsdialog durchzuführen und fühlten sich nach dem Dialog positiv gestimmt.

Diskussion
Mit EVALOG liegt ein wissenschaftliches fundiertes, standardisiertes, leitfadenorientiertes ökonomisches, dialogorientiertes und anwendungsfreundliches Verfahren zur Evaluierung psychischer Belastung vor, welches von Kleinstbetrieben eigenständig zum Einsatz gebracht werden kann.
The economics of the democratic deficit: The effect of IMF programs on inequality
Authors: Lang Valentin 
Published: December 2020

AbstractDoes the International Monetary Fund (IMF) increase inequality? To answer this question, this article introduces a new empirical strategy for determining the effects of IMF programs that exploits the heterogeneous effect of IMF liquidity on loan allocation based on a difference-in-differences logic. The results show that IMF programs increase income inequality. An analysis of decile-specific income data shows that this effect is driven by absolute income losses for the poor and not by income gains for the rich. The effect persists for up to 5 years, and is stronger for IMF programs in democracies, and when policy conditions, particularly those that demand social-spending cuts and labor-market reforms, are more extensive. These results suggest that IMF programs can constrain government responsiveness to domestic distributional preferences.
A leverage points perspective on social networks to understand sustainability transformations: evidence from Southern Transylvania
Authors: Lam David P. M. , Martín-López Berta , Horcea-Milcu Andra I. , Lang Daniel J. 
Published: December 2020

AbstractSustainability transformations research increasingly recognizes the importance of local actors and their networks to foster fundamental societal change. Local actors have different types of relations between each other (e.g., sharing material resources, giving advice) through which they jointly intervene in different system characteristics. We conducted social network analyses of 32 non-governmental organizations (NGOs) who drive initiatives to foster sustainability in Southern Transylvania, Romania. In so doing, we applied a leverage points perspective by differentiating between relations according to the system characteristic they address, such as the parameters, feedbacks, design and intent of the system. Additionally, we tested for differences of centrality metrics (i.e., weighted degree, betweenness, eigenvector) from NGOs that conduct different actions (i.e., amplification processes) to increase the impact of their sustainability initiatives. Our results reveal several NGOs that have central positions in their networks for intervening in both shallower (i.e., parameters and feedbacks) and deeper (i.e., design and intent of a system) system characteristics. We also identified NGOs that are only central for intervening in specific system characteristics. In addition, we found that specific groups of amplification processes (i.e.,amplifying withinandout) are associated with the NGOs’ positions in the parameters, feedbacks, and design networks. We conclude that the leverage points perspective in social network analysis has the potential to identify key actors and shed light on the attributes of local actors for intervening in shallower and deeper system characteristics to foster sustainability transformations.
Measuring and modelling of process forces during tapping using single tooth analogy process
Authors: Geßner F. , Weigold M. , Abele E. 
Published: December 2020

AbstractFor machining internal threads, tapping is a commonly used process. However, due to the complex geometry of the tapping tool, each tooth has a unique geometry resulting in individual forces. Since the forces act synchronously during the process, they partly compensate each other. However, since resulting forces in tapping can cause undesired deflection of the tool which can lead to threads that are not true to gauge or tool breakage, the knowledge of the forces is crucial. To predict the occurring forces on each tooth, different modelling approaches can be used. An approach based on the chip load-cutting force relationship is the mechanistic modelling. Therefore, a suitable force model is of central importance. An empirical force model can be established using an analogy process. Within this work a single tooth analogy process is presented to measure the forces of each tooth separately. By means of a geometrical analysis of the real tool, the chip sizes, such as the cross-section area of the undeformed chip are calculated. Merging the measured process forces from the analogy process and the actual chip sizes, an empirical force model is set up using multivariate regression. The model is validated by implementing it in an existing framework and comparing the results to experimental data.
Clinicians’ Perceptions of the Appropriateness of Neurocritical Care for Patients with Spontaneous Intracerebral Hemorrhage (ICH): A Qualitative Study
Authors: Mc Lernon Siobhan , Werring David , Terry Louise 
Published: December 2020

Abstract
Background and Objective
Clinicians working in intensive care frequently report perceptions of inappropriate care (PIC) situations. Intracerebral haemorrhage (ICH) is associated with high rates of mortality and morbidity. Prognosticating after ICH is complex and may be influenced by clinicians’ subjective impressions and biases, which may, in turn, influence decision making regarding the level of care provided. The aim of this study was to qualitatively explore perceptions of neurocritical care in relation to the expected functional outcome for ICH patients.

Design
Qualitative study using semi-structured interviews with neurocritical care doctors and nurses.

Setting
Neurocritical care (NCC) department in a UK neuroscience tertiary referral center.

Subjects
Eleven neurocritical care nurses, five consultant neurointensivists, two stroke physicians, three neurosurgeons.

Intervention
None.

Measurements and Main Results
We conducted 21 semi-structured interviews and identified five key themes: (1) prognostic uncertainty (2) subjectivity of good versus poor outcome (3) perceived inappropriate care (PIC) situations (including for frail elderly patients) (4) challenging nature of decision-making (5) clinician distress.

Conclusions
Caring for severely affected ICH patients in need of neurocritical care is challenging, particularly with frail elderly patients. Awareness of the challenges could facilitate interventions to improve decision-making for this group of stroke patients and their families, as well as measures to reduce the distress on clinicians who care for this patient group. Our findings highlight the need for effective interdisciplinary shared decision making involving the family, taking into account patients’ previously expressed values and preferences and incorporating these into bespoke care planning.
Research on head cooling of high-speed aircraft by liquid nitrogen
Authors: Xiong H.X. , Yi S.H. , Ding H.L. , Jin L. , Huo J.J. 
Published: December 2020

AbstractIn the development process of high-speed aircraft, the head of the aircraft is subject to high temperatures and high speed flows, supporting the maximum heat flow and thus requiring a reliable cooling system. A new type of head cooling system is proposed herein. An internal flow channel model of the heat transfer in a ball head made from high-temperature alloy steel is constructed, then an experimental platform is built to carry out relevant experiments on the performance of this cooling system. Firstly, the influence of different experimental conditions on the cooling efficiency of the ball head is studied. For given liquid-nitrogen supply pressure, a higher heating heat flux density on the outer surface of the ball head corresponds to higher cooling efficiency. Then, the vaporisation effect under different experimental conditions is evaluated using temperature sensors at the inlet and outlet of the ball head heat exchange channel in combination with images of the visualised glass tube. It is found that liquid nitrogen can vaporise completely when flowing through the heat exchange channel. The characteristics of the heating effect and liquid nitrogen injection for the ball head were evaluated using an infrared camera. Finally, under different experimental conditions of liquid-nitrogen supply pressure, it is found that liquid nitrogen can vaporise completely in each case, and the total temperature of the vaporised nitrogen is about 300K. It can thus be collected as a secondary gas source.
Continuity of Lyapunov exponents for non-uniformly fiber-bunched cocycles
Authors: FREIJO CATALINA , MARIN KARINA 
Published: December 2020

AbstractWe provide conditions that imply the continuity of the Lyapunov exponents for non-uniformly fiber-bunched cocycles in

$SL(2,\mathbb {R})$
. The main theorem is an extension of the result of Backes, Brown and Butler and gives a partial answer to a conjecture of Marcelo Viana.
The capability of the Australian Square Kilometre Array Pathfinder to detect prompt radio bursts from neutron star mergers
Authors: Wang Ziteng , Murphy Tara , Kaplan David L. , Bannister Keith W. , Dobie Dougal 
Published: December 2020

AbstractWe discuss observational strategies to detect prompt bursts associated with gravitational wave (GW) events using the Australian Square Kilometre Array Pathfinder (ASKAP). Many theoretical models of binary neutron stars mergers predict that bright, prompt radio emission would accompany the merger. The detection of such prompt emission would greatly improve our knowledge of the physical conditions, environment, and location of the merger. However, searches for prompt emission are complicated by the relatively poor localisation for GW events, with the 90% credible region reaching hundreds or even thousands of square degrees. Operating in fly’s eye mode, the ASKAP field of view can reach$\sim1\,000$deg$^2$at$\sim$$888\,{\rm MHz}$. This potentially allows observers to cover most of the 90% credible region quickly enough to detect prompt emission. We use skymaps for GW170817 and GW190814 from LIGO/Virgo’s third observing run to simulate the probability of detecting prompt emission for GW events in the upcoming fourth observing run. With only alerts released after merger, we find it difficult to slew the telescope sufficiently quickly as to capture any prompt emission. However, with the addition of alerts releasedbeforemerger by negative-latency pipelines, we find that it should be possible to search for nearby, bright prompt fast radio burst-like emission from GW events. Nonetheless, the rates are low: we would expect to observe$\sim$0.012 events during the fourth observing run, assuming that the prompt emission is emitted microseconds around the merger.
The Effects of China's Development Projects on Political Accountability
Authors: Ping Szu-Ning , Wang Yi-Ting , Chang Wen-Yang 
Published: December 2020

AbstractThe literature on the relationship between foreign aid and institutions has found that the effects of aid vary across different donor characteristics and delivery mechanisms. This article focuses on China's resource-related development projects, which have been considered controversial due to the relative lack of conditionality. By distinguishing between vertical and horizontal dimensions of political accountability, the study finds that China's resource-related projects are particularly detrimental to the accountability of recipient countries' horizontal (legislative and judicial) institutions. These projects are often delivered to resource-rich countries, in the form of packaging access to resources and infrastructure construction, to improve China's own energy access. Local officials may be tempted to weaken horizontal institutions so that the projects can be implemented quickly. Nevertheless, these projects have little effect on vertical accountability, as China has less intention and capacity to fundamentally restrain electoral competition in recipient countries.
Influence of women’s empowerment indices on the utilization of skilled maternity care: evidence from rural Nigeria
Authors: Ntoimo Lorretta Favour C. , Okonofua Friday E. , Aikpitanyi Josephine , Yaya Sanni , Johnson Ermel , Sombie Issiaka , Aina Olabisi , Imongan Wilson 
Published: December 2020

AbstractThere is increasing evidence that women with the ability to exercise control over their sexual and reproductive lives have greater access to prompt prevention and treatment of maternal health disorders, resulting in a concomitant reduction in maternal morbidity and mortality. This study assessed the association between indices of women’s empowerment and utilization of skilled antenatal, intrapartum and postnatal maternity care in two rural Local Government Areas in Edo State, Nigeria. Data were taken from a household survey conducted in July and August 2017, and the study sample comprised 1245 ever-married women currently in a union who had given birth in the 5 years preceding the survey. A Gender Roles Framework guided the selection of independent women’s empowerment variables. Using hierarchical logistic regression, the likelihood of receiving all three levels of skilled maternal health care service (antenatal, intrapartum and postnatal) by women’s empowerment variables, grouped into resource, decision-making and influencer domains following the model of Anderson and Neuman, was assessed. Of the resource domain variables, respondent’s education and respondent’s participation in payment for their own health care positively predicted their use of all three levels of skilled maternal care, whereas their ownership of land negatively predicted this. Two decision-making domain variables were significantly associated with respondent’s use of all three levels of service: those who made decisions alone about major household purchases were twice as likely to use all three levels of services than when decisions were made by their partners or others, while respondent making decisions alone about what food to cook each day was a negative predictor. Of the influencer variables, religion and a large spousal education gap were strong positive factors, whereas living in a consensual union rather than being legally married was a negative factor. Although health system factors are important, interventions geared towards changing gender norms that constrain women’s empowerment are critical to achieving maternal health-related development goals in Nigeria. A composite strategy that targets all women’s empowerment indices is recommended, as Nigeria strives towards achieving SDG-3.