The Gilles de la Tourette Syndrome-Quality of Life Scale for children and adolescents (C&A-GTS-QOL):development and validation of the Italian version

BACKGROUND: Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients' health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410-1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS. METHODS: A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6-12 year-olds and a self-report questionnaire for 13-18 year-olds. RESULTS: The C&A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach's alpha > 0.7) and validity was supported by interscale correlations (range 0.4-0.7), principal-component factor analysis and correlations with other rating scales and clinical variables. CONCLUSIONS: The present version of the C&A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity. © 2013 - IOS Press and the authors. All rights reserved.

The relationship between transformational leadership and innovative behaviour in a healthcare context:a team learning versus a cohesion perspective

The purpose of this study was to explore whether the relationship between transformational leadership and innovative behaviour is explained via the mediating role of team learning, or whether instead team cohesion mediates this relationship. Using survey data from 158 professionals within 21 teams in the Dutch healthcare context, we tested by means of hierarchical regression analyses: (a) the relationship between transformational leadership and innovative behaviour; (b) whether team learning or cohesion mediates this relationship; and (c) the relationship between team learning and cohesion, in relation to transformational leadership. Results showed that transformational leadership is positively related to innovative behaviour and that both cohesion and team learning mediate this relationship, with team learning being the strongest mediator. Addressing a neglected area, our study provides evidence to show that managers who enhance team learning are likely to maximise employees' scope for engaging in innovative behaviours. © 2012 Inderscience Enterprises Ltd.

Celiac disease-specific TG2-targeted autoantibodies inhibit angiogenesis ex vivo and in vivo in mice by interfering with endothelial cell dynamics

A characteristic feature of celiac disease is the presence of circulating autoantibodies targeted against transglutaminase 2 (TG2), reputed to have a function in angiogenesis. In this study we investigated whether TG2-specific autoantibodies derived from celiac patients inhibit angiogenesis in both ex vivo and in vivo models and sought to clarify the mechanism behind this phenomenon. We used the ex vivo murine aorta-ring and the in vivo mouse matrigel-plug assays to address aforementioned issues. We found angiogenesis to be impaired as a result of celiac disease antibody supplementation in both systems. Our results also showed the dynamics of endothelial cells was affected in the presence of celiac antibodies. In the in vivo angiogenesis assays, the vessels formed were able to transport blood despite impairment of functionality after treatment with celiac autoantibodies, as revealed by positron emission tomography. We conclude that celiac autoantibodies inhibit angiogenesis ex vivo and in vivo and impair vascular functionality. Our data suggest that the anti-angiogenic mechanism of the celiac disease-specific autoantibodies involves extracellular TG2 and inhibited endothelial cell mobility. © 2013 Kalliokoski et al.

The Role of Aquaporin 3 (AQP3) in Breast Cancer

The increasing prevalence of breast cancer (BC) in different parts of the world, particularly in the UK, highlights the importance of research into the aetiology and pathology of the disease. BC is the most common malignancy affecting women worldwide. Aquaporins (AQPs) are membrane protein channels that regulate cellular water flow. Recently, studies have demonstrated that expression of AQP3 is up-regulated in cancerous breast tissue. The present study examines the role of AQP3 in BC cell biology. Examination of clinical cases of BC showed higher AQP3 gene and protein expression in cancer tissues compared to healthy border tissues. In distinct clinicopathological groups however there were no differences observed with regards to AQP3 expression, suggesting that AQP3 expression may not be a predictor of lymph node infiltration or tumour grade. shRNA technology was used to knockdown gene expression of AQP3 in the invasive MDA-MB-231 BC cellular model. Cellular proliferation, migration, invasion, adhesion and response to the 5- fluorouracil (5-FU) based chemotherapy treatment were investigated in parental and knockdown cell line. AQP3 knockdown cells showed reduction in cellular proliferation, migration, invasion and increase in cell sensitivity to 5-FU compared with wild type (WT) or scrambled control (SC) cells. The effects of AQP3 knockdown on cellular glycolytic ability and ATP cellular content were quantified. Indirect glucose uptake was also measured by quantifying reconditioned media. AQP3 knockdown cells showed significantly lower levels of glucose uptake as compared to WT or SC. However there was no difference in the glycolytic ability and ATP content of the cells suggesting AQP3 has no role in cancer cell energetics. These data collectively suggest AQP3 expression is associated with the BC disease clinically and plays a role in multiple important aspects of BC pathophysiology, thus AQP3 represents a novel target for therapeutic intervention.

Planning the neighbourhood economy:land-use plans and the economic potential of urban residential neighbourhoods in the Netherlands

This article investigates the relationship between zoning by-laws, as put forward in governmental land-use plans and the viability of urban residential neighbourhood economies. The Dutch planning tradition has long been characterized by strict separation of functions and top-down planning. We argue that profound changes in social and economic structures make land-use planning practices less suitable for the current policy formula of "mixed urban milieus". Although the residential neighbourhood might not be the location of large firms, it definitely attracts small ones, and facilitates starting businesses whose presence (and potential growth) can be beneficial to the city as a whole. We present a typology of spatial patterns of neighbourhood economies based on land-use plans and describe whether these are related to the distinctive economic development of the neighbourhood over the period 1999-2007. © 2013 Taylor & Francis.

Buborékok és legendák. Válságok és válságmagyarázatok - a tulipánmánia és a Déltengeri Társaság, I. rész (Bubbles and myths, crises and explanations: tulip mania and the South Sea bubble. I.)

A 2007 óta kibontakozó válság újból divatba hozta a korai buborékok és válságok témáját. A jelenlegi válság nyomán újraéledtek a makroökónómiai viták, kétségessé vált a "nagy mérséklődés" és az újklasszikus szintézis érvényessége. Az 1634-1637 közötti holland tulipánmánia - mint az első buborék - sokféle értelmezése ismeretes: a tömeghisztéria kitörésétől a hatékony pénzügyi piacok korai példáján át a kulturális sokkig. Áttekintve ezeket, a cikk visszavezeti a tulipánmánia közismert anekdotikus leírásait az eredeti forrásokig, és megmutatja, milyen módon és céllal használták fel ezeket a közgazdászok és történészek saját elemzéseikben. / === / The accounts of early bubbles and crises are becoming fashionable again in economic discourse during the recent downturn. The article, having looked at the revival of macroeconomic debate provoked by the failure of current theory, sums up various interpretations of the Dutch tulip mania of 1634-7. These range from an outburst of popular madness, through an early example of an efficient financial market, to an instance of culture shock. Some well-known anecdotes about tulip mania are traced back to their original sources, and the article explores the various patterns and intentions in the use economists and historians have made of them.

Aspects of the transplantation, storage and maintenance of corals (Montastraea Faveolata, Acropora Cervicornis and A. Palmata)

The purpose of this study is to explore aspects of coral transplantation for restoration. Montastraea faveolata cores of 2.54 and 5.0 cm were stored in aquaria, on an array and on the substrate. Survival on the array and substrate were 100% for 12 and 11 months respectively. Branches of Acropora cervicornis had 75.0 % survival on the substrate and 91.7% on the array. Disease caused mortality for the A. cervicornis and the 2.54 cm cores in the aquaria but not for the 5.0 cm cores. Growth was significantly higher for A. cervicornis and A. palmata branches stored on an array than in an open seawater system. The storage type affected growth patterns of both species. M. faveolata fed three times/week increased in surface area significantly more than those fed once and twice/week. Corals fed once per week significantly increased their polyp density. Corals had intermittent respiration while under sub-aerial conditions.

International expert opinion on the management of infection caused by azole-resistant Aspergillus fumigatus

Acknowledgements We thank Gilead Sciences Europe Ltd., UK for financially supporting logistical aspects of this study. The logistics of the meeting were handled by Congress Care, Den Bosch, The Netherlands. The sponsor was not involved in the selection of the participants or procedures, or in the discussion, data collection, analysis or writing of the manuscript. The medical writer was financially supported by the Dutch Society for Medical Mycology and the Department of Medical Microbiology, Radboud University Medical Centre, Nijmegen, The Netherlands.

Retinal microvascular dysfunction occurs similarly in Alzheimer's disease and primary open angle glaucoma patients

Purpose: The exact nature of the relationship between Alzheimer’s disease (AD) and primary open angle glaucoma (POAG) is still the subject of debate. One factor attributed to the aetiology of both conditions is vascular dysfunction. This study aimed to investigate the similarities and differences in retinal microvascular function between mild AD patients, early stage POAG patients and healthy controls Methods: Retinal vessel reactivity to flickering light was assessed in 10 AD, 19 POAG and 22 healthy age matched control patients by means of dynamic retinal vessel analysis (DVA, IMEDOS, GmbH, Jena, Germany) according to an established protocol. All patients additionally underwent BP measurements and blood analysis for glucose and lipid metabolism markers Results: AD and POAG patients demonstrated comparable alterations in retinal artery reactivity, in the form of an increased arterial reaction time (RT) to flicker light on the final flicker cycle (p=0.014), which was not replicated in the healthy age and cardiovascular risk matched controls (p>0.05). Furthermore, the sequential changes in RT on progressing from flicker one to flicker three were found to differ between healthy controls and the two disease groups (p=0.001) Conclusions: AD and POAG patients demonstrate comparable signs of vascular dysfunction in their retinal arteries at the early stages of their disease process. These comparable signs may reflect similarities in the pathophysiological processes that occur in the development of both conditions

The Political Economy of Climate Adaptation and Environmental Health: The Case of Ethiopia

The environment affects our health, livelihoods, and the social and political institutions within which we interact. Indeed, nearly a quarter of the global disease burden is attributed to environmental factors, and many of these factors are exacerbated by global climate change. Thus, the central research question of this dissertation is: How do people cope with and adapt to uncertainty, complexity, and change of environmental and health conditions? Specifically, I ask how institutional factors, risk aversion, and behaviors affect environmental health outcomes. I further assess the role of social capital in climate adaptation, and specifically compare individual and collective adaptation. I then analyze how policy develops accounting for both adaptation to the effects of climate and mitigation of climate-changing emissions. In order to empirically test the relationships between these variables at multiple levels, I combine multiple methods, including semi-structured interviews, surveys, and field experiments, along with health and water quality data. This dissertation uses the case of Ethiopia, Africa’s second-most populous nation, which has a large rural population and is considered very vulnerable to climate change. My fieldwork included interviews and institutional data collection at the national level, and a three-year study (2012-2014) of approximately 400 households in 20 villages in the Ethiopian Rift Valley. I evaluate the theoretical relationships between households, communities, and government in the process of adaptation to environmental stresses. Through my analyses, I demonstrate that water source choice varies by individual risk aversion and institutional context, which ultimately has implications for environmental health outcomes. I show that qualitative measures of trust predict cooperation in adaptation, consistent with social capital theory, but that measures of trust are negatively related with private adaptation by the individual. Finally, I describe how Ethiopia had some unique characteristics, significantly reinforced by international actors, that led to the development of an extensive climate policy, and yet with some challenges remaining for implementation. These results suggest a potential for adaptation through the interactions among individuals, communities, and government in the search for transformative processes when confronting environmental threats and climate change.

Diversity and television in Argentina: the case of the Audiovisual Technology Hub Programme

This paper describes and analyses the Audiovisual Technology Hub Programme (Programa Polos Audiovisuales Tecnológicos - PPAT), which has been implemented in Argentina between 2010 and 2015 as part of the public policy of former administration of Cristina Fernández de Kirchner. The main goal was to promote a television industry that reflects the cultural diversity of Argentina by dividing the national territory in nine into nine audiovisual technology hubs, where national public universities acted as centres that gathered a range of regional stakeholders. Considering the 18 TV seasons that were produced for television between 2013 and 2014, the text analyses the diversity of sources and genres / subgenres and its restricted marketing. The article closes with a brief set of conclusions about this initiative.

Measuring the Diversity and the Integration of Film Production in Mercosur: A Social Network Analysis

Based on an original and comprehensive database of all feature fiction films produced in Mercosur between 2004 and 2012, the paper analyses whether the Mercosur film industry has evolved towards an integrated and culturally more diverse market. It provides a summary of policy opportunities in terms of integration and diversity, emphasizing the limiter role played by regional policies. It then shows that although the Mercosur film industry remains rather disintegrated, it tends to become more integrated and culturally more diverse. From a methodological point of view, the combination of Social Network Analysis and the Stirling Model opens up interesting research tracks to analyse creative industries in terms of their market integration and their cultural diversity.

Intraclonal heterogeneity is a critical early event in the development of myeloma and precedes the development of clinical symptoms.

The mechanisms involved in the progression from monoclonal gammopathy of undetermined significance (MGUS) and smoldering myeloma (SMM) to malignant multiple myeloma (MM) and plasma cell leukemia (PCL) are poorly understood but believed to involve the sequential acquisition of genetic hits. We performed exome and whole-genome sequencing on a series of MGUS (n=4), high-risk (HR)SMM (n=4), MM (n=26) and PCL (n=2) samples, including four cases who transformed from HR-SMM to MM, to determine the genetic factors that drive progression of disease. The pattern and number of non-synonymous mutations show that the MGUS disease stage is less genetically complex than MM, and HR-SMM is similar to presenting MM. Intraclonal heterogeneity is present at all stages and using cases of HR-SMM, which transformed to MM, we show that intraclonal heterogeneity is a typical feature of the disease. At the HR-SMM stage of disease, the majority of the genetic changes necessary to give rise to MM are already present. These data suggest that clonal progression is the key feature of transformation of HR-SMM to MM and as such the invasive clinically predominant clone typical of MM is already present at the SMM stage and would be amenable to therapeutic intervention at that stage.

Validation of the self-regulated online learning questionnaire

Abstract The number of students engaged in Massive Open Online Courses (MOOCs) is increasing rapidly. Due to the autonomy of students in this type of education, students in MOOCs are required to regulate their learning to a greater extent than students in traditional, face-to-face education. However, there is no questionnaire available suited for this online context that measures all aspects of self-regulated learning (SRL). In this study, such a questionnaire is developed based on existing SRL questionnaires. This is the self-regulated online learning ques- tionnaire. Exploratory factor analysis (EFA) on the first dataset led to a set of scales differing from those theoretically defined beforehand. Confirmatory factor analysis (CFA) was conducted on a second dataset to compare the fit of the theoretical model and the exploratively obtained model. The exploratively obtained model provided much better fit to the data than the theoretical model. All models under investigation provided better fit when excluding the task strategies scale and when merging the scales measuring metacognitive activities. From the results of the EFA and the CFA it can be concluded that further development of the questionnaire is necessary.

Functional Magnetic Resonance Imaging Neurofeedback and Motor training for Parkinson’s Disease: Randomised Trial

Objective: Real-time functional magnetic resonance imaging (rt-fMRI) neurofeedback (NF) uses feedback of the patient’s own brain activity to self-regulate brain networks which in turn could lead to a change in behaviour and clinical symptoms. The objective was to determine the effect of neurofeedback and motor training and motor training (MOT) alone on motor and non-motor functions in Parkinson’s disease (PD) in a 10-week small Phase I randomised controlled trial. Methods: 30 patients with PD (Hoehn & Yahr I-III) and no significant comorbidity took part in the trial with random allocation to two groups. Group 1 (NF: 15 patients) received rt-fMRI-NF with motor training. Group 2 (MOT: 15 patients) received motor training alone. The primary outcome measure was the Movement Disorder Society – Unified Parkinson’s Disease Rating Scale-Motor scale (MDS-UPDRS-MS), administered pre- and post-intervention ‘off-medication’. The secondary outcome measures were the ‘on-medication’ MDS-UPDRS, the Parkinson’s disease Questionnaire-39, and quantitative motor assessments after 4 and 10 weeks. Results: Patients in the NF group were able to upregulate activity in the supplementary motor area by using motor imagery. They improved by an average of 4.5 points on the MDS-UPDRS-MS in the ‘off-medication’ state (95% confidence interval: -2.5 to -6.6), whereas the MOT group improved only by 1.9 points (95% confidence interval +3.2 to -6.8). However, the improvement did not differ significantly between the groups. No adverse events were reported in either group. Interpretation: This Phase I study suggests that NF combined with motor training is safe and improves motor symptoms immediately after treatment, but larger trials are needed to explore its superiority over active control conditions. Clinical Trial website : Unique Identifier: NCT01867827 URL: https://clinicaltrials.gov/ct2/show/NCT01867827?term=NCT01867827&rank=1