Novel therapeutic approaches to familial HLH (emapalumab in FHL)

Primary Hemophagocytic lymphohistiocytosis (pHLH) is a rare, life-threatening, hyperinflammatory disorder, characterized by uncontrolled activation of the immune system. Mutations affecting several genes coding for proteins involved in the cytotoxicity machinery of both natural killer (NK) and T cells have been found to be responsible for the development of pHLH. So far, front-line treatment, established on the results of large international trials, is based on the use of glucocorticoids, etoposide ± cyclosporine, followed by allogeneic hematopoietic stem cell transplantation (HSCT), the sole curative treatment for the genetic forms of the disease. However, despite major efforts to improve the outcome of pHLH, many patients still experience unfavorable outcomes, as well as severe toxicities; moreover, treatment-refractory or relapsing disease is a major challenge for pediatricians/hematologists. In this article, we review the epidemiology, etiology and pathophysiology of pHLH, with a particular focus on different cytokines at the origin of the disease. The central role of interferon-γ (IFNγ) in the development and maintenance of hyperinflammation is analyzed. The value of emapalumab, a novel IFNγ-neutralizing monoclonal antibody is discussed. Available data support the use of emapalumab for treatment of pHLH patients with refractory, recurrent or progressive disease, or intolerance to conventional therapy, recently, leading to FDA approval of the drug for these indications. Additional data are needed to define the role of emapalumab in front-line treatment or in combination with other drugs

Low Energy STEM of Multi-Layers and Dopant Profiles

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Are French Individual Investors reluctant to realize their losses?

We analyze the presence of the disposition effect for 90 244 French individual investors based on a large brokerage account database between 1999 and 2006. Main results show that a) French investors demonstrate a strong preference for realizing their winning stocks rather than their losing ones (disposition effect).b) the behavioral bias is not eliminated for sophisticated individual investors (higher trading activity or international diversification) c) more originally, based on French account specificities, we demonstrate that the change of “fiscal account type” does not imply a change in investors’ behavior (at an individual level of the disposition effect).

Efficacy, Safety, and Timing of Anticoagulant Thromboprophylaxis for the Prevention of Venous Thromboembolism in Patients With Acute Spinal Cord Injury: A Systematic Review

Study Design: Systematic review. Objectives: The objective of this study was to answer 5 key questions: What is the comparative effectiveness and safety of (1a) anticoagulant thromboprophylaxis compared to no prophylaxis, placebo, or another anticoagulant strategy for preventing deep vein thrombosis (DVT) and pulmonary embolism (PE) after acute spinal cord injury (SCI)? (1b) Mechanical prophylaxis strategies alone or in combination with other strategies for preventing DVT and PE after acute SCI? (1c) Prophylactic inferior vena cava filter insertion alone or in combination with other strategies for preventing DVT and PE after acute SCI? (2) What is the optimal timing to initiate and/or discontinue anticoagulant, mechanical, and/or prophylactic inferior vena cava filter following acute SCI? (3) What is the cost-effectiveness of these treatment options? Methods: A systematic literature search was conducted to identify studies published through February 28, 2015. We sought randomized controlled trials evaluating efficacy and safety of antithrombotic strategies. Strength of evidence was evaluated using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. Results: Nine studies satisfied inclusion criteria. We found a trend toward lower risk of DVT in patients treated with enoxaparin. There were no significant differences in rates of DVT, PE, bleeding, and mortality between patients treated with different types of low-molecular-weight heparin or between low-molecular-weight heparin and unfractionated heparin. Combined anticoagulant and mechanical prophylaxis initiated within 72 hours of SCI resulted in lower risk of DVT than treatment commenced after 72 hours of injury. Conclusion: Prophylactic treatments can be used to lower the risk of venous thromboembolic events in patients with acute SCI, without significant increase in risk of bleeding and mortality and should be initiated within 72 hours. © 2017, © The Author(s) 2017

Towards the development of cascaded surface plasmon resonance POF sensors exploiting gold films and synthetic recognition elements for detection of contaminants in transformer oil

The possibility of developing a multichannel optical chemical sensor, based on molecularly imprinted polymers (MIPs) and surface plasmon resonance (SPR) in a D-shaped multimode plastic optical fiber (POF), is presented by two cascaded SPR-POF-MIP sensors with different thicknesses of the gold layer. The low cost, the high selectivity and sensitivity of the SPR-POF-MIP platforms and the simple and modular scheme of the optical interrogation layout make this system a potentially suitable on-line multi-diagnostic tool. As a proof of principle, the possibility of simultaneous determination of two important analytes, dibenzyl disulfide (DBDS) and furfural (2-FAL), in power transformer oil was investigated. Their presence gives useful indication of underway corrosive or ageing processes in power transformers, respectively. Preliminarily, the dependence of the performance of the D-shaped optical platform on the gold film thickness has been studied, comparing two platforms with 30 nm and 60 nm thick gold layers. It has been found that the resonance wavelengths are different on platforms with gold layer of different thickness, furthermore when MIPs are present on the gold as receptors, the performances of the platforms are similar in the two considered sensors. Keywords: Cascaded multianalyte detection, Surface plasmon resonance, Dibenzyl disulfide, Furfural (furan-2-carbaldehyde), Molecularly imprinted polymers, Plastic optical fiber

Određivanje etilnog etera u krvi primjenom plinske kromatografije

Les auteurs décrivent une technique de chromatographie gazeuse pour I ‘identification et la détermination quantitative de I\u27éther éthylique dans le sang, frais ou putréfié. En considérant Jas preuves que l\u27on a faites on a constaté une séparation aisée de l\u27éther éthylique de l\u27alcool éthylique et des produits dûs à la putréfaction. A\u27 propos de ces derniers on n\u27a pas rencontré dans les échantillons examinés la présence d\u27alcools supérieurs (butylique, propylique, amylique) signalée pair d\u27autres auteurs.Vršena su određivanja etilnog etera u uzorcima trule krvi primjenom plinske kromatografije u slučajevima kad su klasične metode rada dale prevelike pogreške. Opisana je upotrebljena aparatura i metodika rada. Slika 1. prikazuje kromatogram etera u zraku. Slika 2. daje kromatograme različite koncentracije etera. Slika 3. prikazuje kromatogram za uzorak krvi pokusne životinje koja je tretirana letalnom dozom etilnog etera. Slika 4. je kromatogram trule krvi uz dodatak etilnog etera, a bez tragova etilnog alkohola. Slika 5. dobivena je na isti način, ali uz prisutnost etilnog alkohola. Ukratko se pokazuje značenje plinske kromatografije, naročito za sudsko medicinsku identifikaciju tvari kojih klasična analiza nije jednostavna

Glycine Receptor Antibody-Associated Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM) During SARS-CoV-2 Infection: a Video-Case Report

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Application of the Maximum Power Extrapolation Procedure for Human Exposure Assessment to 5G Millimeter Waves: Challenges and Possible Solutions

This paper describes an investigation on the application of the Maximum Power Extrapolation (MPE) technique on a fully operational Fixed Wireless Access (FWA) FR2-band 5G gNB. The data was acquired in [27.1-27.3] GHz band using a network scanner over nearly 10 minutes periods to allow a statistical analysis and an accurate estimation of the role of each contribution to the total uncertainty, including the fading affecting the 5G FR2 reference signal. The results show that the level of the electromagnetic field is well below the limits imposed by Italian legislation. However the goal of the paper is more fundamental, and shows an approach that can be used to identify the critical elements of the measurement set-up, suggesting where to concentrate efforts to improve the measurement procedure. In particular, the uncertainty budget highlights three contributions, (i.e. estimation of the traffic beam level, of the probe response and of the 5G FR2 reference signal) that deserve further investigations