1,710 research outputs found

    Thermal behaviour of siliceous faujasite: further structural interpretation of negative thermal expansion

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    The high-temperature behaviour of siliceous faujasite (Si-Y) was investigated by in situ synchrotron Xray powder diffraction from room temperature up to 1123 K. This porous phase is remarkably stable when heated, and no phase transitions or changes in symmetry are observed. A marked negative thermal expansion (NTE), already reported in literature for a heating range from 25 to 573 K, was confirmed up to about 923 K. Above this temperature a positive thermal expansion was observed. Si-Y’s thermal behaviour was interpreted on the basis of the transverse thermal vibrations of the oxygen atoms involved in the T–O–T linkages and a series of other structural features characterizing the faujasite structure, namely the T–T distances between adjacent tetrahedral sites, the thickness of the double 6-membered rings, and the ditrigonal index of the 6-membered rings. Moreover, the thermal behaviour of several other anhydrous porous materials with NTE behaviour is discussed and compared to that of Si-Y

    New spatial mechanisms for the kinematic analysis of the tibiotalar joint

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    In virtually unloaded conditions, the tibiotalar (ankle) joint behaves as a single degree-of-freedom system, and two fibres within the calcaneal-fibular and tibio-calcaneal ligaments remain nearly isometric throughout the flexion arc. A relevant theoretical model also showed that three articular surfaces and two ligaments act together as a mechanism to control the passive kinematics. Two equivalent spatial parallel mechanisms were formulated, with ligament fibres assumed isometric and articulating surfaces assumed rigid, either as three sphere-plane contacts, or as a single spherical pair. Predicted and measured motion in three specimens compared fairly well. Important enhancement of this previous work is here presented, with more accurate experimental data, more anatomical model surfaces, and a more robust mathematical model

    Experimental behaviour of a three-stage metal hydride hydrogen compressor

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    A three-stage metal hydride hydrogen compressor (MHHC) system based in AB2-type alloys has been set-up. Every stage can be considered as a Sieverts-type apparatus. The MHHC system can work in the pressure and temperature ranges comprised from vacuum to 250 bar and from RT to 200C, respectively. An efficient thermal management system was set up for the operational ranges of temperature designed. It dumps temperature shifts due to hydrogen expansion during stage coupling and hydrogen absorption/desorption in the alloys. Each reactor consists of a single and thin stainless-steel tube to maximize heat transfer. They are filled with similar amount of AB2 alloy. The MHHC system was able to produce a compression ratio (CR) as high as of 84.7 for inlet and outlet hydrogen pressures of 1.44 and 122 bar for a temperature span of 23 to 120C

    El hidrógeno como vector energético: Mucho hecho pero casi todo por hacer

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    Los autores desarrollan, muy brevemente, los aspectos esenciales de lo que globalmente se denomina “Sistema Energético Solar-Hidrógeno” o a veces “Economía del hidrógeno”. El hidrógeno, obtenido por descomposición del agua mediante energías primarias renovables (solar, eólica…) se convierte en transportador de energía (vector energético) y en combustible limpio. Se discute la conveniencia del uso del hidrógeno en el contexto energético actual, así como los tres pilares fundamentales del uso del hidrógeno como vector energético: su producción usando fuentes renovables, su acumulación mediante diferentes métodos y, finalmente, su combustió

    Gut microbiome in pediatric acute leukemia: From predisposition to cure

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    The gut microbiome (GM) has emerged as a key factor in the genesis and progression of many diseases. The intestinal bacterial composition also influences treatment-related side effects and even the efficacy of oncological therapies. Acute leukemia (AL) is the most common cancer among children and the most frequent cause of cancer-related death during childhood. Outcomes have improved considerably over the past 4 decades, with the current long-term survival for acute lymphoblastic leukemia being ∼90%. However, several acute toxicities and long-term sequelae are associated with the multimodal therapy protocols applied in these patients. Specific GM configurations could contribute to the multistep developmental hypothesis for leukemogenesis. Moreover, GM alterations occur during the AL therapeutic course and are associated with treatment-related complications, especially during hematopoietic stem cell transplantation. The GM perturbation could last even after the removal of microbiome-modifying factors, like antibiotics, chemotherapeutic drugs, or alloimmune reactions, contributing to several health-related issues in AL survivors. The purpose of this article is to provide a comprehensive review of the chronological changes of GM in children with AL, from predisposition to cure. The underpinning biological processes and the potential interventions to modulate the GM toward a potentially health-promoting configuration are also highlighted

    Synthesis, optical band gap and thermoelectric properties of Sr1+xTiS3-y chalcogenide perovskites

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    Inorganic chalcogenide perovskites are semiconductors with attractive optoelectronic properties, which make them of interest in different fields, such as energy harvesting. Some of these compounds have been poorly investigated to date. For instance, very few works deal with the synthesis and characterization of Sr1+xTiS3. Here we present a novel synthesis procedure to obtain Sr1+xTiS3-y powders. Moreover, we show for the first time an experimental characterization of some fundamental properties of this compound that may be relevant for many potential applications. First, we demonstrate that this perovskite shows very high thermal stability (up to 700◦C in air and up to 1200◦C in Ar atmosphere). Next, we experimentally determine its optical band gap (about 0.97 eV) corresponding to a direct allowed transition, in agreement with previous predictions. Finally, we demonstrate a tuneable Seebeck coefficient (changing from n-type to p-type behaviour) by changing the amount of sulfur vacanciesThis research has been funded by Spanish MICINN under RTI2018-099794-B-I00 gran

    Role of CBL Mutations in Cancer and Non-Malignant Phenotype

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    CBL plays a key role in different cell pathways, mainly related to cancer onset and progres-sion, hematopoietic development and T cell receptor regulation. Somatic CBL mutations have been reported in a variety of malignancies, ranging from acute myeloid leukemia to lung cancer. Growing evidence have defined the clinical spectrum of germline CBL mutations configuring the so-called CBL syndrome; a cancer-predisposing condition that also includes multisystemic involvement char-acterized by variable phenotypic expression and expressivity. This review provides a comprehensive overview of the molecular mechanisms in which CBL exerts its function and describes the clinical manifestation of CBL mutations in humans

    Scintillation of Ar/CF4_4 mixtures: glass-THGEM characterization with 1% CF4_4 at 1-1.5 bar

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    Argon gas doped with 1% wavelength-shifter (CF4_4) has been employed in an optical time projection chamber (OTPC) to image cosmic radiation. We present results obtained during the system commissioning, performed with two stacked glass-THGEMs and an EMCCD camera at 1 bar. Preliminary estimates indicate that the combined optical gain was of the order of 106^6 (ph/e), producing sharp and high-contrast raw images without resorting to any filtering or post-processing. A first assessment of the impact of pressurization showed no change in the attainable gains when operating at 1.5 barComment: Added reference with DOI, fixed error with axis label in plo

    Gata2 related conditions and predisposition to pediatric myelodysplastic syndromes

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    Myelodysplastic syndromes (MDS) are hematopoietic disorders rare in childhood, often occurring in patients with inherited bone marrow failure syndromes or germinal predisposition syndromes. Among the latter, one of the most frequent involves the gene GATA binding protein 2 (GATA2), coding for a transcriptional regulator of hematopoiesis. The genetic lesion as well as the clinical phenotype are extremely variable; many patients present hematological malignancies, especially MDS with the possibility to evolve into acute myeloid leukemia. Variable immune dysfunction, especially resulting in B-and NK-cell lymphopenia, lead to severe infections, including generalized warts and mycobacterial infection. Defects of alveolar macrophages lead to pulmonary alveolar proteinosis through inadequate clearance of surfactant proteins. Currently, there are no clear guidelines for the monitoring and treatment of patients with GATA2 mutations. In patients with MDS, the only curative treatment is allogeneic hematopoietic stem cell transplantation (HSCT) that restores normal hematopoiesis preventing the progression to acute myeloid leukemia and clears long-standing infections. However, to date, the donor type, conditioning regimen, and the optimal time to proceed to HSCT, as well as the level of chimerism needed to reverse the phenotype, remain unclear highlighting the need for consensus guidelines
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