18 research outputs found

    Sialidosis type 1 without cherry-red spots: a case report and literature review

    No full text
    Background Sialidosis is a rare disorder caused by mutations in the NEU1 gene located on chromosome 6p21.3, constituting a group of autosomal recessive diseases. Enzyme activity analysis, electron microscopy examination and genetic testing are reliable methods for diagnosis. Despite previous reports on the disease, its rarity means that its clinical manifestations and prognosis still warrant attention due to the limited amount of information available.Methods We report a case of a 40-year-old woman who was admitted to our hospital for worsening dysarthria of 16 years duration and facial and limb twitching that had been present for 2 years. Genetic testing was undertaken.Results Genetic testing confirmed type I sialidosis, the first reported instance of this disease in the Hainan Free Trade Port in China. The patient did not have the typical cherry-red spot in the fundus. Despite aggressive treatment, she died of status epilepticus 2 months later. This result indicates that the disease has a poor prognosis.Discussion Cherry-red spots in the fundus are characteristic features of type I sialidosis and it has been referred to as the cherry-red spot myoclonus syndrome. We hypothesise that environmental factors may also play a significant role. Overemphasis on the presence of cherry-red spots may mislead clinicians and delay diagnosis. Furthermore, patients presenting with isolated myoclonus should undergo visual evoked potential and somatosensory evoked potential tests, as well as genetic testing to confirm or rule out sialidosis

    A Hybrid Search Algorithm for Midterm Optimal Scheduling of Thermal Power Plants

    Get PDF
    A hybrid search algorithm consisting of three stages is presented to solve the midterm schedule for thermal power plants (MTSFTPP) problem, where the primary objective is to achieve equal accumulated operating hours of installed capacity (EAOHIC) for all thermal power plants during the selected period. First, feasible spaces are produced and narrowed based on constraints on the number of units and power load factors. Second, an initial feasible solution is obtained by a heuristic method that considers operating times and boundary conditions. Finally, the progressive optimality algorithm (POA), which we refer to as the vertical search algorithm (VSA), is used to solve the MTSFTPP problem. A method for avoiding convergence to a local minimum, called the lateral search algorithm (LSA), is presented. The LSA provides an updated solution that is used as a new feasible starting point for the next search in the VSA. The combination of the LSA and the VSA is referred to as the hybrid search algorithm (HSA), which is simple and converges quickly to the global minimum. The results of two case studies show that the algorithm is very effective in solving the MTSFTPP problem accurately and in real time

    Plasma-assisted low-temperature oxidation of n-butane: A synchrotron photoionization mass spectrometry and kinetic modeling study

    No full text
    The chemical kinetics of plasma-assisted low-temperature oxidation of n-butane (n-C4H10, 340 K, 30 Torr) is investigated by synchrotron vacuum ultraviolet photoionization mass spectrometry (SVUV-PIMS) and kinetic modeling. Species measurements are conducted in a flow reactor activated by a nanosecond discharge using SVUV-PIMS. Detailed species identification and quantification are reported in the present work. Fuel-specific oxygenated species including 2-butenal (2-C3H5CHO), methyl vinyl ketone (CH3COC2H3), butanone (CH3COC2H5), butanal (n-C3H7CHO), 2-methyloxetane (-CH(CH3)CH2CH2O-), 1,2-epoxybutane (-CH(C2H5)CH2O-), tetrahydrofuran (-CH2CH2CH2CH2O-), 1-/2-butanol (1-/2-C4H9OH), and butyl hydroperoxide (C4H9O2H) are detected. A kinetic model for n-C4H10 plasma is developed, which well predicts the mole fractions of most species observed in experiments. Kinetic modeling reveals that electron-impact reactions of n-C4H10 are the main sources for various C1-C4 alkenes, alkynes, and radicals. Therefore, these reactions have a significant effect on the fuel consumption pathways and species pool formation. Reactions involving alkyl peroxy (RO2), especially the self- and cross-reactions of RO2, are essential for RO2 depletion and the formation of alcohols, ketones, and aldehydes. On the other hand, the misprediction of the species detected in the system, such as C4 cyclic ethers, ethyl formate (HCOOC2H5), and acrolein (C2H3CHO), indicates that their formation pathways in the current mechanism are incompletely described. Further experimental and numerical research on these subjects is desired for the development of the kinetic models

    Wafer-scale epitaxial growth of the thickness-controllable van der Waals ferromagnet CrTe2 for reliable magnetic memory applications

    No full text
    To harness the intriguing properties of two-dimensional van der Waals (vdW) ferromagnets (FMs) for versatile applications, the key challenge lies in the reliable material synthesis for scalable device production. Here, we demonstrate the epitaxial growth of single-crystalline 1T-CrTe2 thin films on 2-inch sapphire substrates. Benefiting from the uniform surface energy of the dangling bond-free Al2O3(0001) surface, the layer-by-layer vdW growth mode is observed right from the initial growth stage, which warrants precise control of the sample thickness and atomically smooth surface morphology across the entire wafer. Moreover, the presence of the Coulomb interaction at the CrTe2/Al2O3 interface serves as an effective tuning parameter to tailor the anomalous Hall response, and the structural optimization of the CrTe2-based spin-orbit torque device leads to a substantial switching power reduction by 54%. Our results may lay out a general framework for the design of energy-efficient spintronics based on configurable vdW FMs
    corecore