bluuebunny/crossref_metadata_2025_split huggingface dataset:
Parliamentary Democracy and International Treaties
Authors: Schütze Robert 
Published: October 2017

AbstractClassic constitutional thought considered the power to conclude international treaties to fall within the executive's exclusive domain. But this nineteenth‐century logic hardly convinces in the twenty‐first century. For the function of international treaties has dramatically shifted from the military to the regulatory domain; and in the wake of this ‘new internationalism’, the traditional divide between ‘internal’ and ‘external’ affairs has increasingly disappeared. Has this enlarged scope of the treaty power also triggered a transformation of its nature; and in particular: has the rise of ‘legislative’ international treaties been compensated by a greater role accorded to parliaments? This article explores this question comparatively by looking at the constitutional law of the United States and the European Union. Within the United States, international treaties were traditionally concluded under a procedure that excluded the House of Representatives; yet with the rise of the Congressional‐Executive‐Agreement in the twentieth century, this democratic deficit has been partially remedied. We find a similar evolution within the context of the European Union, where an increasing ‘parliamentarisation’ of the treaty power has taken place. Yet a democratic deficit here nevertheless also continues to exist.
Democracy under Siege of Authoritarian Liberalism: Remarks on the Crisis of National and Transnational Republicanism in Europe
Authors: Brunkhorst Hauke 
Published: October 2017

AbstractModern republicanism must be distinguished sharply from ancient republicanism and modern liberalism. Modern republicanism is strongly restricted in Europe due to the constitutional hegemony of ordo‐ and neoliberalism. Therefore, Europe is running out of democratic alternatives on both, the national and the transnational level. This is a challenge for the entire Western legal tradition. During a lasting period of multiple crises the new prevalence of private over public law became evident, and as a result democracy became façade democracy. However, at the same time technocratic incrementalism no longer is a solution because it destroys the legitimatory basis of European. But is the legitimation crisis a last chance – or is it already the catastrophe?
Prenatal development in greater mouse‐eared bat, Myotis myotis (Borkhausen, 1797) (Chiroptera, Vespertilionidae)
Authors: Paksuz E. P. , Hayretdağ S. , Olgun K. 
Published: October 2017

SummaryOrder Chiroptera is the second largest mammal group after rodents. An understanding of the development of the bats, which is a very special mammal group in terms of their lifestyles, morphology and their ability to fly, is very important because most of the adult anatomical differences characterizing species occur during organogenesis. In this study, developmental stages were determined for Myotis myotis species based on external morphological characteristics from embryos obtained from wild‐caught pregnant females. The developmental stages of M. myotis were comparable with those of other bat species.
Differential relations of grandiose narcissism and vulnerable narcissism to emotion dysregulation: Self‐esteem matters
Authors: Zhang Hui , Luo Yun , Zhao Yimeng , Zhang Runzhu , Wang Zhenhong 
Published: October 2017

The present study aims to explore the effects of grandiose and vulnerable narcissism on emotion dysregulation and examine the mediating role of self‐esteem on these associations. Undergraduates (N = 426) completed self‐report measurements on grandiose narcissism, vulnerable narcissism, self‐esteem and emotion dysregulation. Correlation analyses indicated that grandiose narcissism was negatively correlated with emotion dysregulation, while vulnerable narcissism was positively correlated with emotion dysregulation. Moreover, mediational analyses revealed that self‐esteem fully mediated the association between grandiose narcissism and emotion dysregulation, and partially mediated the association between vulnerable narcissism and emotion dysregulation. The present study highlights the importance of self‐esteem and deepens the understanding of the associations between the two forms of narcissism and emotion dysregulation.
Pre‐operative variables including fitness associated with complications after oesophagectomy
Authors: Sinclair R. C. F. , Phillips A. W. , Navidi M. , Griffin S. M. , Snowden C. P. 
Published: October 2017

SummaryOesophagectomy is a technically‐demanding operation associated with a high level of morbidity. We analysed the association of pre‐operative variables, including those from cardiopulmonary exercise testing, with complications (logistic regression) and survival and length of stay (Cox regression) after scheduled transthoracic oesophagectomy in 273 adults, in isolation and on multivariate testing (maximum Akaike information criterion). On multivariate analysis, any postoperative complication was associated with ventilatory equivalents for carbon dioxide, odds ratio (95%CI) 1.088 (1.02–1.17), p = 0.018. Cardiorespiratory complications were associated with FEV1 and pre‐operative background survival (in an analogous group without cancer), odds ratios (95%CI) 0.55 (0.37–0.80), p = 0.002 and 0.89 (0.82–0.96), p = 0.004, respectively. Survival was associated with the ratio of expected‐to‐observed ventilatory equivalents for carbon dioxide and predicted postoperative survival, hazard ratios (95%CI) 0.17 (0.03–0.91), p = 0.039 and 0.96 (0.90–1.01), p = 0.076. Length of hospital stay was associated with FVC, hazard ratio (95%CI) 1.38 (1.17–1.63), p < 0.0001.
“We will not perish; we’re going to keep flourishing”: Race, Food Access, and Geographies of Self‐Reliance
Authors: Reese Ashanté M. 
Published: October 2017

AbstractDrawing from 18 months of ethnographic fieldwork in Washington, DC, this article outlines geographies of self‐reliance; a theoretical framework for understanding black food geographies that are embedded in histories of self‐reliance as a response to structural inequalities. Using a community garden as a case study, I argue that the garden functions as a site for addressing several manifestations of structural violence: racist and classist depictions of low‐income and working class people, joblessness, gentrification, and youth underdevelopment. Drawing on self‐reliance ideologies as well as collective and personal histories, the residents exhibit a form of agency that demonstrates unwavering hope in the sustainability of their shared community. Through this analysis, I show that self‐reliance functions as a mechanism through which residents navigate spatial inequalities.
Cost analysis of open radical cystectomy versus robot‐assisted radical cystectomy
Authors: Bansal Sukhchain S. , Dogra Tara , Smith Peter W. , Amran Maisarah , Auluck Ishna , Bhambra Maninder , Sura Manraj S. , Rowe Edward , Koupparis Anthony 
Published: October 2017

ObjectivesTo perform a cost analysis comparing the cost of robot‐assisted radical cystectomy (RARC) with open RC (ORC) in a UK tertiary referral centre and to identify the key cost drivers.Patients and MethodsData on hospital length of stay (LOS), operative time (OT), transfusion rate, and volume and complication rate were obtained from a prospectively updated institutional database for patients undergoing RARC or ORC. A cost decision tree model was created. Sensitivity analysis was performed to find key drivers of overall cost and to find breakeven points with ORC. Monte Carlo analysis was performed to quantify the variability in the dataset.ResultsOne RARC procedure costs £12 449.87, or £12 106.12 if the robot was donated via charitable funds. In comparison, one ORC procedure costs £10 474.54. RARC is 18.9% more expensive than ORC. The key cost drivers were OT, LOS, and the number of cases performed per annum.ConclusionHigh ongoing equipment costs remain a large barrier to the cost of RARC falling. However, minimal improvements in patient quality of life would be required to offset this difference.
GBF1 and Arf1 function in vesicular trafficking, lipid homoeostasis and organelle dynamics
Authors: Kaczmarek Beata , Verbavatz Jean‐Marc , Jackson Catherine L. 
Published: October 2017

AbstractThe ADP‐ribosylation factor (Arf) small G proteins act as molecular switches to coordinate multiple downstream pathways that regulate membrane dynamics. Their activation is spatially and temporally controlled by the guanine nucleotide exchange factors (GEFs). Members of the evolutionarily conserved GBF/Gea family of Arf GEFs are well known for their roles in formation of coat protein complex I (COPI) vesicles, essential for maintaining the structure and function of the Golgi apparatus. However, studies over the past 10 years have found new functions for these GEFs, along with their substrate Arf1, in lipid droplet metabolism, clathrin‐independent endocytosis, signalling at the plasma membrane, mitochondrial dynamics and transport along microtubules. Here, we describe these different functions, focussing in particular on the emerging theme of GFB1 and Arf1 regulation of organelle movement on microtubules.
‘The challenges of sharing information when a young person is experiencing severe emotional difficulties’: implications for schools and CAMHS
Authors: Hart Tania , O'Reilly Michelle 
Published: October 2017

BackgroundSupporting the education of children and young people with complex emotional mental health difficulties requires schools to have knowledge of their needs. Exchanging information about less visible mental health difficulties is, however, known to be complex. Exploring the perceptions of young people experiencing problems can explicate some of this complexity and identify solutions. Yet their views are rarely given credence in this context.MethodsThe findings were derived from a broader qualitative study exploring the school experiences of young people, aged 14–16 years, identified by CAMHS as having severe emotional difficulties. Their parents’ and teachers’ perceptions were also explored. Data were collected via semistructured interviews and analysed using thematic analysis.ResultsFindings demonstrated that young people experiencing emotional difficulties need to feel safe about exchanging private information pertaining to their mental health. Teachers having a basic knowledge of mental health promoted their safety as this ensured confidentiality. Participants reported that CAMHS practitioners needed to be more proactive regarding the practicalities of exchanging information.ConclusionsArguably, teachers need to have basic knowledge of mental health and schools need clearer mental health confidentiality guidance. CAMHS also have responsibility in identifying more information exchange mechanisms and young service users and parents can play a part in this.
Expression level of CXCL7 in peripheral blood cells is a potential biomarker for the diagnosis of renal cell carcinoma
Authors: Kinouchi Toshiro , Uemura Motohide , Wang Cong , Ishizuya Yu , Yamamoto Yoshiyuki , Hayashi Takuji , Matsuzaki Kyosuke , Nakata Wataru , Yoshida Takahiro , Jingushi Kentaro , Kawashima Atsunari , Ujike Takeshi , Nagahara Akira , Fujita Kazutoshi , Imamura Ryoichi , Ueda Yuko , Kitae Kaori , Tsujikawa Kazutake , Nonomura Norio 
Published: October 2017

There are no blood biomarkers for the diagnosis of renal cell carcinoma (RCC) in routine clinical use. We focused on the gene expression profile of peripheral blood cells obtained from RCC patients to discover novel biomarkers for RCC diagnosis. Using microarray analysis and quantitative verification, CXCL7 was shown to be significantly upregulated in the peripheral blood cells of RCC patients. Importantly, aberrant CXCL7 expression was confirmed even in peripheral blood cells obtained from early stage (pT1a) RCC patients, and the expression level of CXCL7 in peripheral blood cells was a potential independent biomarker for the diagnosis of RCC by receiver operating characteristic curve analysis (sensitivity, 70.0%; specificity, 64.0%; area under the curve = 0.722; multiple logistic regression analysis: odds ratio, 1.07; 95% confidence interval, 1.03–1.11; P = 0.0004). Moreover, CXCL7 expression in peripheral blood cells significantly decreased after resection of the primary tumor. CXCL7 is more highly expressed in PBMCs than in neutrophils from both healthy controls and RCC patients. Interestingly, CXCL7 expression in PBMCs from healthy volunteers was significantly elevated following coculture with RCC cells compared to those cocultured with normal cells as a control. These results suggest that aberrant CXCL7 expression in peripheral blood cells is induced by RCC cells and may serve as a novel biomarker in the diagnosis of RCC.
Whole genome microarray expression analysis in blood identifies pathways linked to signs and symptoms of a patient with hypercalprotectinaemia and hyperzincaemia
Authors: Isaksson H S , Farkas S A , Müller P , Gustafsson D , Nilsson T K 
Published: October 2017

Summary
A child, 2 years with the ‘hypercalprotectinaemia with hyperzincaemia’ clinical syndrome, presented with atypical symptoms and signs, notably persistent fever of approximately 38°C, thrombocythaemia of &gt; 700 × 109/l and a predominance of persistent intestinal symptoms. In an effort to find a cure by identifying the dysregulated pathways we analysed whole-genome mRNA expression by the Affymetrix HG U133 Plus 2·0 array in blood on three occasions 3–5 months apart. Major up-regulation was demonstrated for the Janus kinase/signal transducer and activators of transcription (JAK/STAT) pathway including, in particular, CD177, S100A8, S100A9 and S100A12, accounting for the thrombocytosis; a large number of interleukins, their receptors and activators, accounting for the febrile apathic state; and the high mobility group box 1 (HMBG1) gene, possibly accounting for part of the intestinal symptoms. These results show that gene expression array technology may assist the clinician in the diagnostic work-up of individual patients with suspected syndromal states of unknown origin, and the expression data can guide the selection of optimal treatment directed at the identified target pathways.
B cell tolerance to epidermal ribonuclear-associated neo-autoantigenin vivo
Authors: Degn S E , Alicot E , Carroll M C 
Published: October 2017

SummaryDefining how self-antigens are perceived by the immune system is pivotal to understand how tolerance is maintained under homeostatic conditions. Clinically relevant, natural autoantigens targeted by autoantibodies, in e.g. systemic lupus erythematosus (SLE), commonly have an intrinsic ability to engage not only the B cell receptor (BCR), but also a co-stimulatory pathway in B cells, such as the Toll-like receptor (TLR)-7 pathway. Here we developed a novel mouse model displaying inducible expression of a fluorescent epidermal neo-autoantigen carrying an OT-II T cell epitope, B cell antigen and associated ribonucleic acids capable of stimulating TLR-7. The neo-autoantigen was expressed in skin, but did not drain in intact form into draining lymph nodes, even after ultraviolet B (UVB)-stimulated induction of apoptosis in the basal layer. Adoptively transferred autoreactive B cells were excluded follicularly and perished at the T–B border in the spleen, preventing their recirculation and encounter with antigen peripherally. This transitional check-point was bypassed by crossing the reporter to a BCR knock-in line on a C4-deficient background. Adoptively transferred OT-II T cells homed rapidly into cutaneous lymph nodes and up-regulated CD69. Surprisingly, however, tolerance was not broken, as the T cells subsequently down-regulated activation markers and contracted. Our results highlight how sequestration of intracellular and peripheral antigen, the transitional B cell tolerance check-point and T cell regulation co-operate to maintain immunological tolerance in vivo.
Direct immunofluorescence testing in vasculitis—A single institution experience with Henoch‐Schönlein purpura
Authors: Feasel Patrick , Billings Steven D. , Bergfeld Wilma F. , Piliang Melissa P. , Fernandez Anthony P. , Ko Jennifer S. 
Published: October 2017

BackgroundDirect immunofluorescence (DIF) panels (IgG, IgA, IgM, C3 and fibrinogen) are ordered for clinically suspected vasculitis, with frequently negative results.MethodsCases submitted for DIF and histology (2010‐2014) with “vasculitis” in the clinical data were examined, and the electronic medical record reviewed for clinical suspicion of Henoch‐Schönlein purpura (HSP). Peri/intravascular IgA was considered positive, other reactants non‐specific and no immunoreactivity negative.ResultsVasculitis was the given indication for 20% (258/1318) of DIF studies. HSP was clinically suspected in 36% (95/258). In this setting, leukocytoclastic vasculitis (LCV) was common (66%, 63/95) and DIF was positive in 43% (27/63). One hundred percentage of DIF+ had LCV+. In cases without HSP suspicion, 26% (42/163) were LCV+ and <1% DIF+. Of the 258 cases, LCV+ greatly enriched for DIF+ (105/258 LCV+ with 28/105 [27%] DIF+), captured 100% of HSP and included cases with non‐specific DIF/etiologic findings. In LCV cases, DIF positivity was not seen, HSP was not diagnosed and non‐specific DIF findings were common.ConclusionsLCV is an H&E‐based histopathologic diagnosis that can have positive, negative and non‐specific DIF results that are rarely contributory except in the setting of HSP, where DIF is best utilized with IgA as the sole immunoreactant. H&E‐based triage of DIF orders is recommended.
Quality of life and comorbidities associated with Dravet syndrome severity: a multinational cohort survey
Authors: Lagae Lieven , Brambilla Isabella , Mingorance Ana , Gibson Eddie , Battersby Alysia 
Published: October 2017

AimTo test the hypothesis that higher seizure burden in Dravet syndrome is associated with increased comorbidities and lower quality of life (QoL) in a large cohort of patients with Dravet syndrome and their caregivers in Europe.MethodAn extensive survey of caregivers of patients with Dravet syndrome on experiences of diagnosis, seizure burden, management, social and financial impact, and health services use was administered online in 10 languages.ResultsThe survey received 584 unique responses from caregivers of paediatric (83%) and adult (17%) patients with Dravet syndrome (aged <1–48y). Despite broadly following current treatment guidance, less than 10% of patients were seizure free in the previous 3 months. Nearly all (99.6%) patients aged 5 years or older experienced at least one or more motor, speech, learning, or behavioural impairment. High seizure frequency was related to more reports of emergency treatment, comorbidities, and a lower QoL (as measured by the standardized instrument EQ‐5D‐5L). If not diagnosed at the first instance, the majority (83%) of adults, but less than 20% of 6‐ to 11‐year‐olds were diagnosed after 4 or more years.InterpretationPatients with Dravet syndrome with the highest current seizure frequency suffer from more comorbidities and have a lower QoL. Therefore, more effective antiepileptic treatments are needed.What this paper adds

The survey captured about 15% of all patients with Dravet syndrome in Europe.

Less than 10% of patients had current seizure freedom.

Patients with a high current seizure burden have more comorbidities and lower quality of life.
Broad clinical high‐risk mental state (CHARMS): Methodology of a cohort study validating criteria for pluripotent risk
Authors: Hartmann Jessica A. , Nelson Barnaby , Spooner Rachael , Paul Amminger G. , Chanen Andrew , Davey Christopher G. , McHugh Meredith , Ratheesh Aswin , Treen Devi , Yuen Hok Pan , McGorry Patrick D. 
Published: October 2017

AimThe development of the ultra‐high risk (UHR) criteria for psychosis created a new paradigm for the prevention research in psychiatry. Since (1) prevention research faces the challenge of achieving adequate statistical power when focusing on single low‐incidence syndromes and (2) early clinical phenotypes are overlapping and non‐specific, this study broadens the UHR state beyond psychosis as an outcome. The CHARMS (clinical high at‐risk mental state) study aims to prospectively validate a set of trans‐diagnostic criteria to identify help‐seeking young people at risk of developing a range of serious mental illnesses.MethodsThis paper describes the methodology of the CHARMS study, which involves applying the CHARMS criteria to a cohort of help‐seeking young people aged 12 to 25 attending youth mental health services in Melbourne. New referrals meeting the CHARMS criteria are allocated to the CHARMS+ group; referrals not meeting CHARMS threshold are allocated to CHARMS‐ group (control group); referrals meeting criteria for a full‐threshold disorder are excluded. Transition status and clinical and functional outcomes are re‐assessed at 6 and 12 months.ConclusionsThis study will be the first to introduce and validate clinical criteria to identify a broader at‐risk patient population, which may facilitate young people's access to clinical services and early treatment by reducing the reliance on “caseness” defined according to current diagnostic categories being required for service entry. These criteria may introduce a new, trans‐diagnostic approach for understanding risk factors and pathogenic mechanisms that drive the onset of severe mental illness and the next generation of preventive intervention trials.
Rates and predictors of disengagement of patients with first‐episode psychosis from the early intervention service for sychosis service (EASY) covering 15 to 64 years of age in Hong Kong
Authors: Lau Ka Wa , Chan Sherry K.W. , Hui Christy L.M. , Lee Edwin H.M. , Chang Wing Chung , Chong Catherine Shiu‐yin , Lo William Tak‐lam , Chen Eric Y.H. 
Published: October 2017

AimThis study aims at assessing the rate and predictors of disengagement of patients with first‐episode psychosis (FEP) from the early intervention (EI) service in Hong Kong (EASY) that covers age 15 to 64.MethodsAll FEP patients aged 15 to 64 years who were newly registered with the EASY programme of Kowloon West Psychiatric Unit from January to December 2012 were included. Data on socio‐demographic, clinical characteristics and disengagement over 3 years of the EI service were obtained retrospectively through systematic clinical record review. Predictors of 3 different types of disengagement and their rate of occurrence were identified: complete disengagement (type I), disengaged and re‐engaged through hospitalization (type II) and re‐engaged as outpatient (type III).ResultsAmong the 277 patients included for analysis, 36 patients (13%) had type I disengagement, 17.2% (N = 21) were of age 15 to 25 and 9.7% (N = 15) were of age 25 to 64. Type II and type III disengagements were 4.3% and 13.4%, respectively. Early‐stage poor drug compliance significantly predicted type I and type II disengagements. History of substance use, suicidal attempts and poor drug compliance predicted type III disengagement. Younger patients had significant earlier disengagement (χ2 = 5.01, df = 1, P = .025).ConclusionResults of the current study highlighted the different patterns of disengagement for different age groups and the importance of identifying the high‐risk group at early stage of the illness. With the expansion of EI service for wider age group, studies of the differential needs of patient with different ages would be important to guide the future service development.