New Insights into the Pathogenesis of Systemic Mastocytosis

Mastocytosis is a type of myeloid neoplasm characterized by the clonal, neoplastic proliferation of morphologically and immunophenotypically abnormal mast cells that infiltrate one or more organ systems. Systemic mastocytosis (SM) is a more aggressive variant of mastocytosis with extracutaneous involvement, which might be associated with multi-organ dysfunction or failure and shortened survival. Over 80% of patients with SM carry the KIT D816V mutation. However, the KIT D816V mutation serves as a weak oncogene and appears to be a late event in the pathogenesis of mastocytosis. The management of SM is highly individualized and was largely palliative for patients without a targeted form of therapy in past decades. Targeted therapy with midostaurin, a multiple kinase inhibitor that inhibits KIT, has demonstrated efficacy in patients with advanced SM. This led to the recent approval of midostaurin by the United States Food and Drug Administration and European Medicines Agency. However, the overall survival of patients treated with midostaurin remains unsatisfactory. The identification of genetic and epigenetic alterations and understanding their interactions and the molecular mechanisms involved in mastocytosis is necessary to develop rationally targeted therapeutic strategies. This review briefly summarizes recent developments in the understanding of SM pathogenesis and potential treatment strategies for patients with SM.     

The structure,vacancy characteristics,and magnetic properties of GdMn1-xCrxO3 ceramics

To investigate the evolution of the structural and enhanced magnetic properties of GdMnO₃ systems induced by the substitution of Mn with Cr, polycrystalline GdMn_1-xCr_xO₃ samples were synthesized via solid-state reactions. XRD characterization shows that all GdMn_1-xCr_xO₃ compounds with single-phase structures crystallize well and that Cr³⁺ ions entering the lattice sites of GdMnO₃ induce structural distortion. SEM results indicate that the grain size of the synthesized samples (a few microns) decreases as the Cr substitution concentration increases. Positron annihilation lifetime spectroscopy reveals that vacancy-type defects occur in GdMn_1-xCr_xO₃ ceramics and that the vacancy size and concentration clearly change with the Cr content. The temperature and field dependence of the magnetization curves show that Cr substitution significantly influences the magnetic ordering of the gadolinium sublattice, improving the weak ferromagnetic transition temperature and magnetization of GdMn_1-xCr_xO₃. The enhanced magnetization of GdMn_1-xCr_xO₃ is closely related to the vacancy defect concentration.     

High energy storage and ultrafast discharge in NaNbO3-based lead-free dielectric capacitors via a relaxor strategy

Dielectric capacitors with decent energy storage and fast charge-discharge performances are essential in advanced pulsed power systems. In this study, novel ceramics (1-x)NaNbO₃-xBi(Ni_2/3Nb_1/3)O₃(xBNN, x = 0.05, 0.1, 0.15 and 0.20) with high energy storage capability, large power density and ultrafast discharge speed were designed and prepared. The impedance analysis proves that the introducing an appropriate amount of Bi(Ni_0·5Nb_0.5)O₃ boosts the insulation ability, thus obtaining a high breakdown strength (E_b) of 440 kV/cm in xBNN ceramics. A high energy storage density (W_total) of 4.09 J/cm³, recoverable energy storage density (W_rec) of 3.31 J/cm³, and efficiency (η) of 80.9% were attained in the 0.15BNN ceramics. Furthermore, frequency and temperature stability (fluctuations of W_rec ≤ 0.4% over 5–100 Hz and W_rec ≤ 12.3% over 20–120 °C) were also observed. The 0.15BNN ceramics exhibited a large power density (19 MW/cm³) and ultrafast discharge time (~37 ns) over the range of ambient temperature to 120 °C. These enhanced performances may be attributed to the improved breakdown strength and relaxor behavior through the incorporation of BNN. In conclusion, these findings indicate that 0.15BNN ceramics may serve as promising materials for pulsed power systems.     

Ensemble relation network with multi-level measure

Fine-grained few-shot learning is a difficult task in image classification. The reason is that the discriminative features of fine-grained images are often located in local areas of the image, while most of the existing few-shotlearning image classification methods only use top-level features and adopt a single measure. In that way, the localfeatures of the sample cannot be learned well. In response to this problem, ensemble relation network with multi-level measure (ERN-MM) is proposed in this paper. It adds the relation modules in the shallow feature space tocompare the similarity between the samples in the local features, and finally integrates the similarity scores from thefeature spaces to assign the label of the query samples. So the proposed method ERN-MM can use local details andglobal information of different grains. Experimental results on different fine-grained datasets show that the proposedmethod achieves good classification performance and also proves its rationality.     

Effect of PyC interface phase on the cyclic ablation resistance and flexural properties of two-dimensional Cf/HfC composites

Composites based on hafnium carbide and reinforced with continuous naked carbon fiber with and without PyC interface were prepared at low temperature by precursor infiltration and pyrolysis and chemical vapor deposition method. The microstructure, mechanical property, cyclic ablation and fiber bundle push-in tests of the composites were investigated. The results show that after three times ablation cycles, the bending strength of samples without PyC interface decreased by 63.6 %; the bending strength of samples with PyC interface only decreased by 37.8 %. The force displacement curve of the samples with PyC interface presented a well pseudoplastic deformation state. The mechanical behavior difference of two kinds of composites was due to crucial function of PyC interface phase including protection of fiber and weakening of fiber/matrix interface.     

Optical and electrical properties of ITO film on flexible fluorphlogopite substrate

Indium Tin Oxide (ITO) films were prepared, at room temperature, on a fluorphlogopite substrate using magnetron sputtering technology. At various temperatures of 500 °C, 600 °C, 700 °C, 800 °C, and 900 °C, the samples were (had) annealed for 2 h (a 2-h duration). The results showed improvement in the crystalline performance of ITO film at selected annealing temperatures, with a significant reduction in resistivity at 800 °C. The lowest resistivity is 4.08 × 10^?4 Ω-cm, which is nearly an order of magnitude lower than the unannealed sample. All samples have an average light transmittance above 85% in the visible light range (400–800 nm), and with increasing annealing temperature, the average light transmittance tends to decrease. Besides, at the sensitive wavelength of 550 nm, the light transmittance is as high as 93.74%. The sheet resistance testing of the sample was through the number of bending times, which revealed that with the increase of the number of bending, the sheet resistance increases. However, after 1200 bending times, the change rate of the sheet resistance remains below 5%. Thus, the ITO film prepared on the flexible fluorphlogopite substrate revealed excellent optical and electrical properties, good flexibility, and improved stability after high-temperature annealing, which guarantees successful application in flexible electronic devices.     

Influence of surfactants on rheological behaviors of polyacrylonitrile/dimethyl sulfoxide/silicon blending polymer solutions

KH550, KH560, CTAB, and F127 were adopted to modify silicon (Si) to improve the dispersity and stability of Si in the polyacrylonitrile/dimethyl sulfoxide (PAN/DMSO) polymer solutions. The influence of surfactants on rheological behaviors of PAN/DMSO/Si blending polymer solutions was investigated by an advanced solution and melt rotation rheometer. The homogeneity and stability were also studied. The results showed that the surfactants could change the viscosity dependence of blending polymer solutions on shear rate, temperature and storage time by increase the steric hindrance of Si. Among the four solutions, PAN/DMSO/Si blending polymer solution with F127 exhibited the lowest viscosity, activation energy and the smallest structural viscosity index and exhibited the trend close to the Newtonian fluids. Moreover, PAN/DMSO/Si blending polymer solution with F127 exhibited the best dispersity and stability, indicating its best physical properties and machinability.     

面向老龄化社会的产品服务系统设计赋能

目的 针对面向老龄化社会的产品及产品服务系统设计,将赋能的设计理念和价值引入其中,探索设计结果提升老年人能动性和参与性的赋能品质,为设计师开展设计赋能实践提供参考.方法 以设计教学中的学生设计方案作为研究材料,邀请设计研究者参与工作坊,对设计结果的赋能属性进行分析,并通过聚类获得类别化的设计属性,产生了能描述赋能品质的属性词汇表.结果 总结了包含5组形容词组的设计赋能品质集,这一集合包含"顺应性"和"激励性"两个面向.结论 研究结果为设计师进行老龄化设计提供了知识参考,有助于提升设计师的赋能意识.揭示了设计赋能充满矛盾性的品质,提出面向老龄化的设计赋能需要在"顺应"和"激励"之间找到平衡.     

Solution-Synthesized Multifunctional Janus Nanotree Microswimmer

Synthetic active matters are perfect model systems for non-equilibrium thermodynamics and of great potential for novel biomedical and environmental applications. However, most applications are limited by the complicated and low-yield preparation, while a scalable synthesis for highly functional microswimmers is highly desired. In this paper, an all-solution synthesis method is developed where the gold-loaded titania-silica nanotree can be produced as a multi-functional self-propulsion microswimmer. By applying light, heat, and electric field, the Janus nanotree demonstrated multi-mode self-propulsion, including photochemical self-electrophoresis by UV and visible light radiation, thermophoresis by near-infrared light radiation, and induced-charge electrophoresis under AC electric field. Due to the scalable synthesis, the Janus nanotree is further demonstrated as a high-efficiency, low-cost, active adsorbent for water decontamination, where the toxic mercury ions can be reclaimed with enhanced efficiency.     

Effect of wind condition on unintended hydrogen release in a hydrogen refueling station

Ambient condition, especially the wind condition, is an important factor to determine the behavior of hydrogen diffusion during hydrogen release. However, only few studies aim at the quantitative study of the hydrogen diffusion in a wind-exist condition. And very little researches aiming at the variable wind condition have been done. In this paper, the hydrogen diffusion in different wind condition which including the constant wind velocity and the variable wind velocity is investigated numerically. When considering the variable wind velocity, the UDF (user defined function) is compiled. Characteristics of the FGC (flammable gas cloud) and the HMF (hydrogen mass fraction) are analyzed in different wind condition and comparisons are made with the no-wind condition. Results indicate that the constant wind velocity and the variable wind velocity have totally different effect for the determination of hydrogen diffusion. Comparisons between the constant wind velocity and the variable wind velocity indicate that the variable wind velocity may cause a more dangerous situation since there has a larger FGC volume. More importantly, the wind condition has a non-negligible effect when considering the HMF along the radial direction. As the wind velocity increases, the distribution of the HMF along the radial direction is not Gaussian anymore when the distance between the release hole and the observation line exceeds to a critical value. This work can be a supplement of the research on the hydrogen release and diffusion and a valuable reference for the researchers.