Seismic risk assessment of a new RC-framed skin technology for integrated retrofitting interventions on existing buildings

A RC-framed-skin technology for the integrated seismic and thermal retrofitting interventions on existing buildings, recently proposed by some of the authors, is thoroughly investigated. By means of numerical analyses, its effectiveness and suitability within the framework of seismic risk class assessment is proved. The system is composed of a RC-framed structure with an external reinforced plaster layer that does not offer a structural contribution to the capacity of the system in ultimate conditions, but which can be effective by increasing the lateral stiffness in serviceability conditions. The system is realized from the outside of the existing building so guaranteeing limited invasiveness of the intervention and preventing the interruption of the building use by their occupants. An existing RC building, representative of a typical example of the Italian building stock, is analyzed as a case study, and its seismic risk class upgrade, obtained by the proposed strengthening intervention, is assessed by non-linear static analysis. The numerical models are developed within the OpenSees framework. The Expected Annual Loss (EAL) parameter, together with the Life Safety Index (LS-I), are chosen as synthetic measures that include both aspects related to Ultimate Limit state (ULS) and Serviceability Limit State (SLS) conditions. The risk class accounting for or disregarding the contribution of the external reinforced plaster are finally compared

The Use of a Scenario-Based Nominal Group Technique to Assess P/CVE Programs: Development and Pilot Testing of a Toolkit

Preventing and countering violent extremism (P/CVE) requires coordination among multiple agencies, stakeholders and systems. The complexity of this task (compounded by the variety of P/CVE programming around the world) creates a  challenge for those hoping to develop these initiatives. The purpose of this project was to develop a replicable process and corresponding toolkit to engage multiple stakeholders in consensus building around the efficacy and improvement of nascent, developing or mature systems-level P/CVE programs. As a method, we adapted the process of nominal group technique (NGT), a structured-brainstorming tool that provides an orderly procedure for obtaining qualitative and ranked information from heterogenous participant pools. The technique we developed is based on a case-study approach (“scenario”) which we then tested in three countries (USA, Sweden, and North Macedonia) with existing P/CVE initiatives at different stages of development. We conducted scenario-based NGT sessions in each location and then systematically analyzed the results using iterative qualitative coding based on a common framework. Results were analyzed to achieve consensus on the most common system-level challenges and system-level functions, necessary to overcome those challenges, in each location. Practitioners in each local jurisdiction were then able to utilize the results derived from the NGT for their own purposes, such as advocacy to policy makers, strategic regional P/CVE planning, and ongoing stakeholder engagement. Acknowledgments: This project was funded by the NATO Science for Peace and Security Programme under the award entitled "Evaluation Support for CVE at the Local Level" SPS.MYPG5556, the Swedish Contingency Agency (MSB), and the U.S. Department of Homeland Security (DHS), Science and Technology Directorate (Cooperative Agreement Number: 2015-ST-108-FRG005). The content of this manuscript as well as the views and discussions expressed are solely those of the authors and do not necessarily represent the official views of any of the above institutions, nor does mention of trade names, commercial practices, or organizations imply endorsement by the U.S. government

Congenital amegakaryocytic thrombocytopenia: clinical and biological consequences of five novel mutations

BACKGROUND AND OBJECTIVES: Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare, autosomal recessive disorder induced by mutations of the gene coding for thrombopoietin (TPO) receptor (c-MPL). Patients initially present with isolated thrombocytopenia that subsequently progresses into pancytopenia. Although the mechanisms leading to aplasia are unknown, the age of onset has been reported to depend on the severity of the c-MPL functional defect. To improve our knowledge in this field, we studied clinical and biological features of five new patients. DESIGN AND METHODS: We diagnosed five CAMT patients, identified c-MPL mutations, including five novel alterations and investigated relationships between mutations and their clinical-biological consequences. RESULTS: In all cases, platelet c-MPL and bone marrow colonies were reduced, while serum TPO levels were elevated. We also documented that the percentage of bone marrow cells expressing tumor necrosis factor-a and interferon-g was increased during pancytopenia as compared to in controls, suggesting that, as in other bone marrow failure diseases, these inhibitory cytokines contributed to the pancytopenia. Contrary to previously published data, we found no evidence of correlations between different types of mutations and the clinical course. INTERPRETATION AND CONCLUSIONS: These results suggest that therapies, such as hematopoietic stem cell transplantation, which are potentially curative although associated with a risk of treatment-related mortality, should not be postponed even in those CAMT patients whose c-MPL mutations might predict residual activity of the TPO receptor

Comparative Analysis of CT/CVE Policies: USA, Canada, United Kingdom, Sweden, and North Macedonia

The article record may be found at https://www.hsaj.org/articles/19378We conducted a comparative analysis of five countries’ CT/CVE policies—USA, Canada, United Kingdom, Sweden and North Macedonia—to identify common values and themes.Sponsored the U. S. Department of Homeland Security’s National Preparedness Directorate, FEMA, CHDS is part of the Naval Postgraduate School (NPS)

Evaluation of energy metabolism and calcium homeostasis in cells affected by Shwachman-Diamond syndrome

Isomorphic mutation of the SBDS gene causes Shwachman-Diamond syndrome (SDS). SDS is a rare genetic bone marrow failure and cancer predisposition syndrome. SDS cells have ribosome biogenesis and their protein synthesis altered, which are two high-energy consuming cellular processes. The reported changes in reactive oxygen species production, endoplasmic reticulum stress response and reduced mitochondrial functionality suggest an energy production defect in SDS cells. In our work, we have demonstrated that SDS cells display a Complex IV activity impairment, which causes an oxidative phosphorylation metabolism defect, with a consequent decrease in ATP production. These data were confirmed by an increased glycolytic rate, which compensated for the energetic stress. Moreover, the signalling pathways involved in glycolysis activation also appeared more activated; i.e. we reported AMP-activated protein kinase hyper-phosphorylation. Notably, we also observed an increase in a mammalian target of rapamycin phosphorylation and high intracellular calcium concentration levels ([Ca2+]i), which probably represent new biochemical equilibrium modulation in SDS cells. Finally, the SDS cell response to leucine (Leu) was investigated, suggesting its possible use as a therapeutic adjuvant to be tested in clinical trials