bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and genetic features of Chinese adult patients with tumour necrosis factor receptor-associated periodic fever syndrome
Authors: Zhao Mengzhu , Luo Yi , Wu Di , Yang Yunjiao , Sun Yang , Wang Rongrong , Shen Min 
Published: October 2019

Abstract

Objectives
TNF receptor-associated periodic fever syndrome (TRAPS) is an autosomal dominant systemic autoinflammatory disease caused by mutations of TNF receptor superfamily member 1 A (TNFRSF1A) gene. TRAPS has hardly been reported in the Chinese population. We aimed to characterize the clinical and genetic features of Chinese adult patients with TRAPS.

Methods
Nine adult patients (≥16 years) were diagnosed during April 2015 to June 2019, at the Department of Rheumatology, Peking Union Medical College Hospital. Clinical and genetic features of these patients were evaluated and compared with those from Japan and Europe.

Results
The median age of disease onset was 3 (0.5–38.5) years old, and adult-onset was observed in two (22.2%) patients. The median time of diagnosis delay was 16.5 (1.5–50.5) years. One patient had a family history of TRAPS. The frequent symptoms were fever (nine, 100%), rash (seven, 77.8%), arthralgia/arthritis (five, 55.6%) and abdominal pain (five, 55.6%). Only two (22.2%) patients had periorbital oedema. Nine TNFRSF1A gene variants were detected, including C58R, G65E, F89L, C99G, V202G, V202D, c.769-23T&gt;C, S290I and c.*64T&gt;C. Rash was more frequently seen in Chinese than in Japanese and European patients, while chest pain and amyloidosis occurred less frequently.

Conclusion
This is the first and largest case series of TRAPS in Chinese adult patients. Two novel TNFRSF1A variants, S290I and V202G, have been identified. The different clinical manifestations of our patients compared with those from Japan and Europe might be related to their TNFRSF1A variants.
Identifying an accurate self‐reported screening tool for alcohol use disorder: evidence from a Swiss, male population‐based assessment
Authors: Baggio Stéphanie , Trächsel Bastien , Rousson Valentin , Rothen Stéphane , Studer Joseph , Marmet Simon , Heller Patrick , Sporkert Frank , Daeppen Jean‐Bernard , Gmel Gerhard , Iglesias Katia 
Published: October 2019

AbstractBackground and aimsShort screenings for alcohol use disorder (AUD) are crucial for public health purposes, but current self‐reported measures have several pitfalls and may be unreliable. The main aim of our study was to provide empirical evidence on the psychometric performance of self‐reports currently used. Our research questions were: compared with a gold standard clinical interview, how accurate are (1) self‐reported AUD, (2) self‐reported alcohol use over time and (3) biomarkers of alcohol use among Swiss men? Finally, we aimed to identify an alternative screening tool.DesignA single‐center study with a cross‐sectional design and a stratified sample selection.SettingLausanne University Hospital (Switzerland) from October 2017 to June 2018.ParticipantsWe selected participants from the French‐speaking participants of the ongoing Cohort Study on Substance Use and Risk Factors (n = 233). The sample included young men aged on average 27.0 years.MeasurementsWe used the Diagnostic Interview for Genetic Studies as the gold standard for DSM‐5 AUD. The self‐reported measures included 11 criteria for AUD, nine alcohol‐related consequences, and previous 12 months’ alcohol use. We also assessed biomarkers of chronic excessive drinking (ethyl glucuronide and phosphatidylethanol).FindingsNone of the self‐reported measures/biomarkers taken alone displayed both sensitivity and specificity close to 100% with respect to the gold standard (e.g. self‐reported AUD: sensitivity = 92.3%, specificity = 45.8%). The best model combined eight self‐reported criteria of AUD and four alcohol‐related consequences. Using a cut‐off of three, this screening tool yielded acceptable sensitivity (83.3%) and specificity (78.7%).ConclusionsNeither self‐reported alcohol use disorder nor heavy alcohol use appear to be adequate to screen for alcohol use disorder among young men from the Swiss population. The best screening alternative for alcohol use disorder among young Swiss men appears to be a combination of eight symptoms of alcohol use disorder and four alcohol‐related consequences.
Experimental manipulations of behavioral economic demand for addictive commodities: a meta‐analysis
Authors: Acuff Samuel F. , Amlung Michael , Dennhardt Ashley A. , MacKillop James , Murphy James G. 
Published: October 2019

AbstractBackground and AimsReinforcing value, an index of motivation for a drug, is commonly measured using behavioral economic purchase tasks. State‐oriented purchase tasks are sensitive to phasic manipulations, but with heterogeneous methods and findings. The aim of this meta‐analysis was to characterize the literature examining manipulations of reinforcing value, as measured by purchase tasks and multiple‐choice procedures, to inform etiological models and treatment approachesMethodsA random‐effects meta‐analysis of published findings in peer‐reviewed articles. Following the Preferred Reporting Items for Systematic Reviews and Meta‐Analysis (PRISMA) protocol, studies were gathered through searches in PsycINFO and PubMed/MEDLINE (published 22 May 2018). Searches returned 34 unique studies (aggregate sample n = 2402; average sample size = 68.94) yielding 126 effect sizes. Measurements included change (i.e. Cohen's d) in six behavioral economic indices (intensity, breakpoint, Omax, Pmax, elasticity, cross‐over point) in relation to six experimental manipulations (cue exposure, stress/negative affect, reinforcer magnitude, pharmacotherapy, behavioral interventions, opportunity cost).ResultsCue exposure (d range = 0.25–0.44, all Ps < 0.05) and reinforcer magnitude [d = 0.60; 95% confidence interval (CI) = 0.18, 1.01; P < 0.005] manipulations resulted in significant increases in behavioral economic demand across studies. Stress/negative affect manipulations also resulted in a small, significant increase in Omax (d = 0.18; 95% CI = 0.01, 0.34; P = 0.03); all other effect sizes for negative affect/stress were non‐significant, albeit similar in size (d range = 0.14–0.18). In contrast, pharmacotherapy (d range = −0.37 to −0.49; Ps < 0.04), behavioral intervention (d = −0.36 to −1.13) and external contingency (d = −1.42; CI = −2.30, −0.54; P = 0.002) manipulations resulted in a significant decrease in intensity. Moderators (substance type) explained some of the heterogeneity in findings across meta‐analyses.ConclusionsIn behavioral economic studies, purchase tasks and multiple‐choice procedures appear to provide indices that are sensitive to manipulations found to influence motivation to consume addictive substances in field experiments.
The association of the optimal bolus of dexmedetomidine with its favourable haemodynamic outcomes in adult surgical patients under general anaesthesia
Authors: Wang Cheng‐yu , Chen Fang , Wu Junzheng , Fu Shu‐ying , Xu Xi‐mou , Chen Jia , Jiang Yi‐fei , Lian Qingquan , Liu Hua‐cheng 
Published: October 2019

AimsDexmedetomidine is highly specific α2‐adrenoceptor agonist. A single bolus of dexmedetomidine can achieve clinical therapeutic effect. Therefore, it is essential to know the safety margin between the clinical effectiveness dosages of dexmedetomidine and its side effect.MethodsA total of 42 patients who underwent elective thyroidectomy were enrolled in this study. Dexmedetomidine was given as a single bolus injection 30 min towards the end of surgery. The up‐and‐down sequential schedule was used in this study. The starting dose of dexmedetomidine was set at 0.1 μg/kg in the first patient and the next patient would then receive a dose of dexmedetomidine decremented by 0.05 μg/kg if the prior patient's baseline heart rate (HR) had a decrease of ≥20% and/or mean arterial blood pressure (MAP) increase or decrease of ≥20%, otherwise, the following patient would receive an incremental 0.05 μg/kg dose of dexmedetomidine. The analytic techniques of linear, linear‐logarithmic, exponential regressions and centred isotonic regression were used to determine the ED50 of dexmedetomidine and the residual standard errors were calculated for the comparison of goodness of fit among the different models.ResultsThe median (interquartile range [range]) lowest HR was 57 beats/min (53–63.3[46–76]) with an average HR decrease of 8.0 beats/min (5–13 [4 to 23]). The median (interquartile range [range]) highest MAP was 98 mmHg (91.8–105 [83–126]) with a MAP increase of 10.0 mmHg (6.8–18.0 [2–24]). The ED50 (95% confidence interval) from 4 different statistical approaches (linear, linear‐logarithmic, exponential regressions and centred isotonic regression) were 0.262 μg/kg (0.243, 0.306), 0.252 μg/kg (0.238, 0.307), 0.283 μg/kg (0.238, 0.307), and 0.278 μg/kg, respectively. Among the 4 models, the exponential regression had the least residual standard error (0.03618).ConclusionThe ED50 derived from 4 statistical models for an intravenous bolus of dexmedetomidine without significant haemodynamic effects was distributed in a narrow range of 0.252–0.283 μg/kg, and the exponential regression was the model to best match the study data.
Using GRADE methodology to assess innovation of new medicinal products in Italy
Authors: Fortinguerra Filomena , Tafuri Giovanni , Trotta Francesco , Addis Antonio 
Published: October 2019

AimIn April 2017 the Italian Medicine Agency (AIFA) developed new criteria to grant any new medicinal product with an innovative designation. The aim of this study is to describe this new model and how it works.MethodsA retrospective descriptive analysis was performed on the results of the assessment process of innovativeness of new medicinal products (or therapeutic indications) based on the AIFA's new innovation criteria (therapeutic need, added therapeutic value and quality of clinical evidence through GRADE methodology) carried out between April 2017 and February 2019 and made publicly available on the AIFA website starting from January 2018.ResultsA total of 37 full reports (22 for oncological indications) explaining the rationale for the AIFA's decision is made publicly available on the agency's website. A total of 12 therapeutic indications (5 oncological) were evaluated as fully innovative, 13 indications (11 oncological) were evaluated as conditionally innovative, while 12 indications (6 oncological) as non‐innovative.ConclusionThe new AIFA innovation criteria resulted in a much more flexible and transparent model to define and assess what constitutes a therapeutic innovation. In particular, the choice of AIFA to use the GRADE methodology to evaluate the quality of clinical evidence within a process of drug innovativeness assessment is essential for the early identification of the discrepancy between the need for patients of a rapid access to innovative therapies and the available clinical data needed to make decisions on drug innovativeness.
Raltegravir pharmacokinetics before and during treatment with ombitasvir, paritaprevir/ritonavir plus dasabuvir in adults with human immunodeficiency virus‐1 and hepatitis C virus coinfection: AIDS Clinical Trials Group sub‐study A5334s
Authors: Venuto Charles S. , Cramer Yoninah S. , Rosenkranz Susan L. , Sulkowski Mark , Wyles David L. , Cohen Daniel E. , Schmidt Jeffrey , Alston‐Smith Beverly L. , Morse Gene D. 
Published: October 2019

AimsAIDS Clinical Trials Group study A5334s evaluated the pharmacokinetics of raltegravir before and during combined administration of ombitasvir, paritaprevir/ritonavir, plus dasabuvir (OBV/PTV/r + DSV) and weight‐based ribavirin in human immunodeficiency virus (HIV) and hepatitis C virus (HCV) coinfected adults. The pharmacokinetics of OBV/PTV/r + DSV during raltegravir coadministration were also characterized.MethodsAdults living with HIV/HCV coinfection receiving steady‐state raltegravir (400 mg twice daily) with 2 nucleos(t)ide analogues were enrolled. Pharmacokinetics of raltegravir were assessed prior to HCV therapy, and 4 weeks later following initiation of OBV/PTV/r (25/150/100 mg) once daily + DSV (250 mg) twice daily. Geometric mean ratios (GMRs) and 90% confidence intervals (CIs) were used to compare the following: raltegravir pharmacokinetics with HCV therapy (week 4) vs before HCV therapy (week 0); OBV/PTV/r and DSV pharmacokinetics vs historical healthy controls; raltegravir pharmacokinetics at week 0 vs historical control adults living with HIV.ResultsEight of 11 participants had decreased raltegravir exposures after initiation of HCV therapy. The GMRs (90% CI) for maximum concentration and area under the concentration–time curve of raltegravir with vs without HCV therapy were 0.68 (0.38–1.19) and 0.82 (0.58–1.17), respectively. Comparing OBV/PTV/r pharmacokinetics in healthy controls, A5334s study participants demonstrated generally lower maximum concentration and area under the concentration–time curve values by 41–82% and 4–73%, respectively. Raltegravir exposures tended to be higher in A5334s study participants compared to adults living with HIV.ConclusionsThe majority of participants' plasma raltegravir exposures were lower after initiation of HCV therapy in coinfected adults; however, confidence intervals were wide.
The Opioid Hydra: Understanding Overdose Mortality Epidemics and Syndemics Across the Rural‐Urban Continuum
Authors: Peters David J. , Monnat Shannon M. , Hochstetler Andrew L. , Berg Mark T. 
Published: October 2019

AbstractThe rapid increase of fatal opioid overdoses over the past two decades is a major U.S. public health problem, especially in non‐metropolitan communities. The crisis has transitioned from pharmaceuticals to illicit synthetic opioids and street mixtures, especially in urban areas. Using latent profile analysis, we classify n = 3,079 counties into distinct classes using CDC fatal overdose rates for specific opioids in 2002–2004, 2008–2012, and 2014–2016. We identify three distinct epidemics (prescription opioids, heroin, and prescription‐synthetic opioid mixtures) and one syndemic involving all opioids. We find that prescription‐related epidemic counties, whether rural or urban, have been “left behind” the rest of the nation. These communities are less populated and more remote, older and mostly white, have a history of drug abuse, and are former farm and factory communities that have been in decline since the 1990s. Overdoses in these places exemplify the “deaths of despair” narrative. By contrast, heroin and opioid syndemic counties tend to be more urban, connected to interstates, ethnically diverse, and in general more economically secure. The urban opioid crisis follows the path of previous drug epidemics, affecting a disadvantaged subpopulation that has been left behind rather than the entire community. County data on opioid epidemic class membership are provided.
The Rise of the Jöüngars Based on Primary Oyirod Sources
Authors: Taupier Richard 
Published: October 2019

Abstract
This paper draws on primary Oyirod and Mongol sources concerning the rise of the seventeenth-century Jöüngars. It relies on eighteenth- and nineteenth-century Mongolian historical texts to identify the 1638 creation of an Oyirod Jöüngar [Left Wing] and Baroun Gar [Right Wing]. This origin of the Jöüngars differs substantially from historical accounts that project existence of a Jöüngar to an earlier time. From Sarayin Gerel [Moon Light] and other sources we learn that the creation of the Jöüngar Khanate was sudden and even unlikely after the division of Baatar Khong Tayiji’s people among his sons. The Jöüngars were so weakened by this division that the Dalai Lama gave the title of Chechen Khaan to the Khoshoud Ochirtu Tayiji in 1666. The Yeke Caaji [Great Code] describes Oyirod political organisation in 1640. Sarayin Gerel also provides details of the reign of Galdan Boshugtu from 1672 until 1692.
The Ruler, the Wrestler, and the Archer
Authors: Delaplace Grégory 
Published: October 2019

Abstract
This paper is an attempt to understand dwelling in Mongolia as the cultivated balancing of three interconnected virtues, prominently exhibited by some particular characters and exemplary people, yet actually to varying degrees expected from anybody else. These virtues are skilfulness (being mergen), force (hiimor’) and power or diplomacy (erh); they are best embodied by archers, wrestlers and rulers, respectively. Drawing on three ethnographic vignettes featuring a troubled diviner, an unlucky young man and a confused anthropologist, this paper highlights how different kinds of people strive to dwell well in post-socialist Mongolia, associating elements that compose the world they live in and checking the conditions in which they might impose themselves in it.
Dark Tent and Light Tent
Authors: Stépanoff Charles 
Published: October 2019

Abstract
Humans have a unique ability to coordinate their imaginations and together explore virtual spaces. Shamanic traditions have cultivated this ability and developed powerful techniques to share mental travels. This article discusses two basic types of shamanic seance spread among indigenous peoples in North Asia and partly in North America and explores the relational and sensory-cognitive contrasts between these ritual techniques. One is carried out in the dark and the audience is more focused on hearing, while in the other the tent is light and watching the shaman’s act is a central part of the participants’ experience. This article describes the geographical distribution of these rituals and analyses the different ways in which they divide cognitive labour.
Powerful Deity or National Geopark?: the Pilgrimage to A-myes-rma-chen in 2014/2015, Transformations of Modernisation and State Secularism, and Environmental Change
Authors: Sehnalova Anna 
Published: October 2019

Abstract
The paper focuses on one of the most sacred mountains of Tibet, A-myes-rma-chen, located in east Tibet (contemporary mGo-log Prefecture, Qinghai Province, People’s Republic of China). It deals mainly with two topics: the ongoing vivid revitalisation of the cult of the mountain and its deity since the Cultural Revolution, and how this interacts with the current changes at the site due to state-planned modernisation and development within the ‘Great Development of the West’ (Xibu da kaifa) strategy extensively implemented since the beginning of the twenty-first century. Particular attention is paid to the recent great circumambulation pilgrimage to A-myes-rma-chen, performed once every 12 years in a Horse Year, which took place in 2014/15, in the Horse Year 2143 of the Tibetan calendar. The article shows the present form of the pilgrimage, its reflection of and accustomisation to these changes, and the resulting quick transformation of the institution of pilgrimage. Pilgrims’ and local people’s understandings and views, alterations and modifications of their behaviour and pilgrimage practice, as well as actual reactions, are discussed. The article argues that the site of A-myes-rma-chen is currently being reinterpreted by the state in a secularised, commodifying and territorialising discourse in order to incorporate the area more closely, both politically and culturally. A-myes-rma-chen thus represents a space contested by different cultural and interest groups.
Il nucleo di manoscritti arabi provenienti da San Martino delle Scale e conservati presso la Biblioteca Centrale della Regione Siciliana
Authors: Sciortino Maria Grazia 
Published: October 2019

Abstract
Among the manuscripts located into the Central Library of the Sicilian Region “Alberto Bombace” in Palermo there is a corpus of Arabic manuscripts coming from the Benedictine Monastery of San Martino delle Scale, as testified by the shelf-mark S.M., indicating that they used to belong to the Library of San Martino delle Scale’s Monastery. Through an in-depth philological and historical investigation, the author will try to give a general description of the documents providing a reconstruction of their arrival to San Martino delle Scale first and then to the Central Library of the Sicilian Region.
Sufism and the Muslim Brotherhood: Ḥasan al-Bannā’s wird and the Transformation of Sufi Traditions in Modern Islamic Activism
Authors: Elsässer Sebastian 
Published: October 2019

Abstract
Scholarship on the Muslim Brotherhood has often commented on its initial connection with Sufism. However, the question of how and why Sufism matters in the fully established Muslim Brotherhood movement has so far gone unanswered. This article gives a detailed account of the Sufi elements adopted into Muslim Brotherhood activism by its founder, Ḥasan al-Bannā (1906-1949). It analyses their function, especially their important role in the education of recruits as they become members of a hierarchical organisation based on the ‘family system’. Among the Sufi practices and ideas relevant in the Muslim Brotherhood tradition are the heart-bond (rābiṭah) prayer and self-examination, as well as concepts such as the spiritual path and ‘friendship of God’. Their incorporation into an Islamic project focused on activism and politics radically altered the meaning of these Sufi elements. Beyond al-Bannā and Egypt, the article follows this development within the Muslim Brotherhood movement across the Arab world from the 1950s until the present day.
The Kurdish Emirate of Brādōst, 1510-1609
Authors: Dehqan Mustafa , Genç Vural 
Published: October 2019

Abstract
The Brādōst Kurdish emirate, ruling over Rawāndiz and adjoining areas including parts of Urmīya, is one of the numerous Kurdish ruling families of Kurdistan, which succumbed to the conquering Ottoman and Safavid arms in the 16th-century. While Ardalān, Ḥakkārī, Chamīšgazak, and many other Kurdish emirates were yielded to the several recent studies, Brādōst remained a neglected Kurdish emirate. By analyzing written documents produced during the 16th-century — from both Ottoman and Safavid sources — a better understanding can be had of what political interactions were possible at this emirate in Ottoman-Safavid frontier history. This paper critically contributes to scholarly discussions of 16th-century political history of Brādōst and Ottoman-Safavid borderlands.