Guidance on Dravet Syndrome from Infant to Adult Care: Road Map for Treatment Planning in Europe

Dravet syndrome (DS) is a severe, rare and complex developmental and epileptic encephalopathy affecting 1 in 16'000 live births and characterized by a drug resistant epilepsy, cognitive, psychomotor and language impairment, as well as behavioral disorders. Evidence suggests that optimal treatment of seizures in DS may improve outcomes, even though neurodevelopmental impairments are the likely result of both the underlying genetic variant and the epilepsy. We present an updated guideline for DS diagnosis and treatment, taking into consideration care of the adult patient and non-pharmaceutical therapeutic options for this disease. This up-to-date guideline, which is based on an extensive review of the literature and culminates with a new treatment algorithm for DS, is a European consensus developed through a survey involving 29 European clinical experts in DS. Theis guideline will serve professionals in their clinical practice and, as a consequence, will benefit DS patients and their families

DESARROLLO CONCEPTUAL DE LA PROMOCIÓN DE LA SALUD EN LOS LUGARES DE TRABAJO

Introduction: Health promotion, from an integrated, intersection and interdisciplinary viewpoint, has been slowly developed in the workplace. The promotion of health in workplaces (PSHW) as a strategy is oriented towards contributing to the integrated attention to workers, involving all determining factors to optimize the health and safety conditions in the workplace involving workers, directors and the organization as a whole. Due to the complexity posed by structural processes, such as globalization and its effects on the working processes and life of people at current times, this strategy becomes a challenge to be implemented in several different labor settings. Objectives: To analyze the bibliography and documentary references in relation to health promotion in workplaces. Methodology: By means of a bibliographic revision, in scientific databases and sites related to the field of interest, the conceptual development of health in workplaces will be analyzed. Descriptors used for searching were the following: Promotion of health, work, and work environment. Results: The bibliographic search showed that the promotion of health in the workplace is a potentially integrating strategy, generating policies and actions of the organization leading to the enhancement of working conditions, as well as all other factors implied in workers‟ health status. Conclusions: Organizations nowadays should assure a balance between the compliance with their objectives and the needs of the health of workers, through an efficient PSHW program, with the objective of continuously improving their quality of life, which can be affected by changes in the labor setting as the result of new technologies and the nature of work. The application of strategies is limited, according to the revision made.Introducción: La promoción de la salud, desde su perspectiva integral, intersectorial e interdisciplinaria, se viene desarrollando tímidamente en los entornos del trabajo. La promoción de la salud en los lugares de trabajo-PSLT, como estrategia, se orienta a contribuir en la atención al trabajador de forma integral e involucrando todos los factores determinantes en la optimización de las condiciones de la salud y seguridad en los lugares de trabajo, implicando a los trabajadores, directivos y a la organización de manera integral. Ante la complejidad que involucra procesos estructurales como la globalización y sus efectos en los procesos de trabajo y vida de las personas en la actualidad, esta estrategia se convierte en un desafío por desarrollar en los diversos escenarios laborales. Objetivos: Analizar la producción bibliográfica y documental relacionada con la promoción de la salud en los lugares de trabajo. Metodología: Mediante revisión bibliográfica, en bases de datos científicas y en sites relacionados con el área, se analizará el desarrollo conceptual de la promoción de la salud en los lugares de trabajo. Los descriptores utilizados para la búsqueda fueron: promoción de la salud, trabajo, ambiente de trabajo. Resultados: La búsqueda bibliográfica mostró que la promoción de la salud en los lugares de trabajo es una estrategia potencial integradora, generadora de políticas y acciones de la organización, encaminadas a mejorar las condiciones de trabajo, y demás factores que intervienen en la salud de los trabajadores. Conclusiones: las organizaciones en la actualidad, deben garantizar un equilibrio entre el cumplimiento de sus objetivos y necesidades de salud de los trabajadores, mediante un programa eficaz de PSLT, con el objetivo de mejorar continuamente su calidad de vida, la cual se puede ver afectada por los cambios del entorno laboral debido a las nuevas tecnologías y la naturaleza del trabajo. La aplicación de la estrategia es limitada, de acuerdo a la revisión realizada

A smog chamber comparison of a microfluidic derivatisation measurement of gas-phase glyoxal and methylglyoxal with other analytical techniques

A microfluidic lab-on-a-chip derivatisation technique has been developed to measure part per billion (ppbV) mixing ratios of gaseous glyoxal (GLY) and methylglyoxal (MGLY), and the method is compared with other techniques in a smog chamber experiment. The method uses-(2, 3, 4, 5, 6-pentafluorobenzyl) hydroxylamine (PFBHA) as a derivatisation reagent and a microfabricated planar glass micro-reactor comprising an inlet, gas and fluid splitting and combining channels, mixing junctions, and a heated capillary reaction microchannel. The enhanced phase contact area-to-volume ratio and the high heat transfer rate in the micro-reactor resulted in a fast and highly efficient derivatisation reaction, generating an effluent stream ready for direct introduction to a gas chromatograph-mass spectrometer (GC-MS). A linear response for GLY was observed over a calibration range 0.7 to 400 ppbV, and for MGLY of 1.2 to 300 ppbV, when derivatised under optimal reaction conditions. The analytical performance shows good accuracy (6.6% for GLY and 7.5% for MGLY), suitable precision (<12.0%) with method detection limits (MDLs) of 75 pptV for GLY and 185 pptV for MGLY, with a time resolution of 30 min. These MDLs are below or close to typical concentrations of these compounds observed in ambient air. The feasibility of the technique was assessed by applying the methodology to quantify α-dicarbonyls formed during the photo-oxidation of isoprene in the EUPHORE chamber. Good correlations were found between microfluidic measurements and Fourier Transform InfraRed spectroscopy (FTIR) with a correlation coefficient (2) of 0.84, Broadband Cavity Enhanced Absorption Spectroscopy (BBCEAS) (2 Combining double low line 0.75), solid phase micro extraction (SPME) (2 Combining double low line 0.89), and a photochemical chamber box modelling calculation (2 Combining double low line 0.79) for GLY measurements. For MGLY measurements, the microfluidic technique showed good agreement with BBCEAS (2 Combining double low line 0.87), SPME (2 Combining double low line 0.76), and the modeling simulation (2 Combining double low line 0.83), FTIR (2 Combining double low line 0.72) but displayed a discrepancy with Proton-Transfer Reaction Time-of-Flight Mass Spectrometry (PTR-ToF-MS) with 2 value of 0.39

The histone deacetylase inhibitor valproic acid attenuates phospholipase Cγ2 and IgE-mediated mast cell activation.

Mast cell activation through the high-affinity IgE receptor (FcεRI) plays a central role in allergic reactions. FcεRI-mediated activation triggers multiple signaling pathways leading to degranulation and synthesis of different inflammatory mediators. IgE-mediated mast cell activation can be modulated by different molecules, including several drugs. Herein, we investigated the immunomodulatory activity of the histone deacetylase inhibitor valproic acid (VPA) on IgE-mediated mast cell activation. To this end, bone marrow-derived mast cells (BMMC) were sensitized with IgE and treated with VPA followed by FcεRI cross-linking. The results indicated that VPA reduced mast cell IgE-dependent degranulation and cytokine release. VPA also induced a significant reduction in the cell surface expression of FcεRI and CD117, but not other mast cell surface molecules. Interestingly, VPA treatment inhibited the phosphorylation of PLCγ2, a key signaling molecule involved in IgE-mediated degranulation and cytokine secretion. However, VPA did not affect the phosphorylation of other key components of the FcεRI signaling pathway, such as Syk, Akt, ERK1/2, or p38. Altogether, our data demonstrate that VPA affects PLCγ2 phosphorylation, which in turn decreases IgE-mediated mast cell activation. These results suggest that VPA might be a key modulator of allergic reactions and might be a promising therapeutic candidate

Atomically dispersed Pt-N-4 sites as efficient and selective electrocatalysts for the chlorine evolution reaction

Chlorine evolution reaction (CER) is a critical anode reaction in chlor-alkali electrolysis. Although precious metal-based mixed metal oxides (MMOs) have been widely used as CER catalysts, they suffer from the concomitant generation of oxygen during the CER. Herein, we demonstrate that atomically dispersed Pt-N-4 sites doped on a carbon nanotube (Pt-1/CNT) can catalyse the CER with excellent activity and selectivity. The Pt-1/CNT catalyst shows superior CER activity to a Pt nanoparticle-based catalyst and a commercial Ru/Ir-based MMO catalyst. Notably, Pt-1/CNT exhibits near 100% CER selectivity even in acidic media, with low Cl- concentrations (0.1M), as well as in neutral media, whereas the MMO catalyst shows substantially lower CER selectivity. In situ electrochemical X-ray absorption spectroscopy reveals the direct adsorption of Cl- on Pt-N-4 sites during the CER. Density functional theory calculations suggest the PtN4C12 site as the most plausible active site structure for the CER

Childhood and adult tuberculosis in a rural hospital in Southeast Ethiopia: a ten-year retrospective study

<p>Abstract</p> <p>Background</p> <p>Many DOTS experiences in developing countries have been reported. However, experience in a rural hospital and information on the differences between children and adults are limited. We described the epidemiology and treatment outcome of adult and childhood tuberculosis (TB) cases, and identified risk factors associated with defaulting and dying during TB treatment in a rural hospital over a 10-year period (1998 to 2007).</p> <p>Methods</p> <p>Retrospective data collection using TB registers and treatment cards in a rural private mission hospital. Information was collected on number of cases, type of TB and treatment outcomes using standardised definitions.</p> <p>Results</p> <p>2225 patients were registered, 46.3% of whom were children. A total of 646 patients had smear-positive pulmonary TB (PTB), [132 (20.4%) children]; 816 had smear-negative PTB [556 (68.2%) children], and 763 extra-PTB (EPTB) [341 (44.8%) children]. The percentage of treatment defaulters was higher in paediatric (13.9%) than in adult patients (9.3%) (p = 0.001). The default rate declined from 16.8% to 3.5%, and was independently positively associated with TB meningitis (AOR: 2.8; 95% CI: 1.2-6.6) and negatively associated with smear-positive PTB (AOR: 0.6; 95% CI: 0.4-0.8). The mortality rate was 5.3% and the greatest mortality was associated with adult TB (AOR: 1.7; 95% CI: 1.1-2.5), TB meningitis (AOR: 3.6; 95% CI:1.2-10.9), and HIV infection (AOR: 4.3; 95% CI: 1.9-9.4). Decreased mortality was associated with TB lymphadenitis (AOR: 0.24; 95% CI: 0.11-0.57).</p> <p>Conclusion</p> <p>(1) The registration of TB cases can be useful to understand the epidemiology of TB in local health facilities. (2) The defaulter and mortality rate of childhood TB is different to that of adult TB. (3) The rate of defaulting from treatment has declined over time.</p

Stranded research? Leading finance journals are silent on climate change

Finance research has shaped the modern financial system, influencing investors and market participants directly through research findings and indirectly through teaching and training programmes. Climate change presents major risks to the global financial system as well as new opportunities for investors. Is climate finance an important topic in finance research? We systematically analyse the content of 20,725 articles published in the leading 21 finance journals between January 1998 and June 2015. We find that only 12 articles (0.06%) are related in some way to climate finance. The three elite finance journals (Journal of Finance, Journal of Financial Economics and Review of Financial Studies) did not publish a single article related to climate finance over the 17.5-year period. We repeat our analysis across a sample of 29 elite business journals spanning accounting, economics, management, marketing and operations research, as well as finance. We find a similar dearth of published climate finance research. We consider four possible explanations for this failure of top finance and business journals to engage with climate finance as a research topic. These include methodological constraints and editorial policies. We conclude by arguing why it is critical for climate-related research to be given greater attention and prominence in finance journals