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Background: Bernard–Soulier syndrome (BSS) is a rare inherited macrothrombocytopenia, usually autosomal recessive, which is characterized by prolonged bleeding, thrombocytopenia, and abnormally large platelets. Methods: For more than 6 years, we misdiagnosed a patient with BSS without an obvious bleeding tendency as having idiopathic thrombocytopenia purpura (ITP), prior to obtaining a genetic analysis. On admission, routine hematology showed a platelet count of 30 × 109/L and mean platelet volume (MPV) of 14.0 fL. Results: Whole-exome sequencing revealed two likely pathogenic heterozygous mutations (c.95_101del and c.1012del) in GP1BA. Flow cytometry analysis of platelet membrane glycoproteins indicated that the expression of GP1b was 0.28% of the normal level. Platelet aggregation tests indicated that platelet aggregation was inhibited by ristocetin- (1.7%), ADP- (14.5%), and arachidonic acid- (5.6%) induced platelet aggregation. A literature review identified reports on 53 mutations in the GP1BA gene in 253 patients, 29 mutations in the GP1BB gene in 90 patients, and 32 mutations in the GP9 gene in 114 patients. Conclusion: This case report describes two novel gene mutation sites that have not been reported previously, enriching understanding of the GP1BA mutation spectrum. [ABSTRACT FROM AUTHOR]

Wrinkling is a typical failure mode of sandwich structures with thin skins, where the bending deformation of face skins occurs simultaneously in conjunction with transverse stretching deformation of core layer. To analyze the three-dimensional (3D) deformation of wrinkling, a model is required to possess a capability describing completely different deformations at each ply. Therefore, by utilizing the Chebyshev polynomial at each layer of sandwich panels, this paper focuses on proposing a higher-order model to individually illustrate deformations of the skins and the core. By means of the proposed model, a refined triangular element has been constructed. By analyzing the stability of a sandwich plate without stiffeners, the finite-element formulation has been verified by comparing it to the quasi-3D elasticity solutions. In addition, it is anticipated that wrinkling failure can be effectively restrained by designing the stiffener layout. To this end, the influence of stiffener layouts on wrinkling behaviors of sandwich plates has been investigated in detail, and it is found that the capability of resisting the wrinkling deformation can be obviously improved by designing reasonable stiffener layouts. Moreover, the translation between buckling and wrinkling behaviors of sandwich panels with different stiffeners has been also explored, which can help to better understand wrinkling deformation mechanism. In summary, the proposed model can be used to design a reasonable arrangement of stiffeners to resist the wrinkling failure of sandwich panels. [ABSTRACT FROM AUTHOR]

Objective and Background: Sinusoidal obstruction syndrome (SOS) is a life‐threatening complication in hematopoietic stem cell transplantation (HSCT) patients. However, the related risk factors in pediatric and young adult HSCT recipients remain unclear. Thus, we conducted this meta‐analysis to identify potential risk factors for SOS in children and young adults undergoing HSCT. Method: We acquired related articles through searching PubMed, EMBASE, and the Cochrane Library up to May 31, 2024. We calculated odds ratios (ORs) and corresponding 95% confidence intervals (CIs) to identify potential risk factors. Results: A total of 12 studies with 7644 HSCT recipients were included. Bone marrow transplantation (OR = 1.35, 95% CI: 1.03–1.77, I2 = 0%), busulfan (BU) (OR = 3.63, 95% CI: 1.78–7.38, I2 = 70%), and fludarabine (FLU) (OR = 1.55, 95% CI: 1.09–2.21, I2 = 16%) were risk factors for SOS after HSCT in children and young adults. Conclusion: Bone marrow transplantation and the use of BU or FLU might be risk factors for SOS after HSCT in children and young adults. [ABSTRACT FROM AUTHOR]

Facing the problems of central single point failure and malicious tampering in the centralized storage of electric energy data, a trusted storage mechanism including identity authentication, sensor data upload, IPFS storage, blockchain upload and access verification was proposed by building the inter satellite storage consortium blockchain (ISCB) of electric energy data based on the decentralized, tamper proof and highly expandable characteristics of blockchain. In view of the characteristics of electric energy data, such as wide source and large capacity, the blockchain technology was combined with the inter planetary file system (IPFS), and the data hash and query attributes returned by IPFS were saved on the chain, so as to effectively solve the problems of storage expansion and data tampering identification of electric energy data blockchain.

The natural sunlight‐driven conversion of CO2 into valuable C2+ products is urgently being pursued at ambient pressure, yet it poses a substantial challenge. Herein, a bifunctional Cu‐promoted CoFe alloy catalyst is designed for the natural light‐driven CO2 hydrogenation into light olefins. Under a weak solar‐irradiation intensity of 0.45 kW m−2 (0.45 sun), the optimal catalyst exhibits excellent activity and selectivity, with an impressive 73.7% selectivity for C2+ hydrocarbons and an outstanding 56.5% selectivity for C2‐4 olefins, which is the best catalyst for C2+ hydrocarbons photosynthesis from natural sunlight to date. The bifunctional design of the catalyst combines the advantages of both metallic Cu and CoFe alloy components, providing a synergistic effect that enhances the CO2 hydrogenation performance. The Cu promoter plays a crucial role in enhancing the adsorption of CO2 and hydrogen spillover, while the CoFe alloy provides a stable coupling site of the C1 intermediate for promoting the C2‐4 olefins. This study provides new insights into the design of bifunctional catalysts for CO2 hydrogenation and opens up new possibilities for sustainable production of light olefins from renewable resources. [ABSTRACT FROM AUTHOR]

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AbstractSandwich structures are susceptible to wrinkling when subject to in-plane compressive loads, which threatens structural safety. To accurately investigate the failure behavior, the following work is to be required. Firstly, the wrinkling behavior of sandwich plates is a typical three-dimensional (3D) issue. To consider a 3D effect, an enhanced higher-order finite element formulation including transverse normal strain has been proposed for the first time. Utilizing the proposed finite element formulation, a refined quadrilateral element model has been constructed. Secondly, the buckling and wrinkling behaviors of square sandwich plates have been investigated. The performance of the proposed model has been evaluated by comparing it with the quasi-3D solutions, the results obtained from the 3D finite element method (3D-FEM), and other models. The differences between the buckling and wrinkling failure behaviors have been carefully explained. Thirdly, the effects of boundary conditions on buckling and wrinkling deformation have been explored in detail. By utilizing the proposed model, the translation mechanism between the buckling and wrinkling deformations of sandwich plates under different boundary conditions has been revealed, which can help other investigators to well understand the buckling and wrinkling behaviors of sandwich plates. It can be shown that the wrinkling behaviors of thick sandwich plates occur earlier than the buckling behaviors when the thickness ratio of core to face panel is more than 35. Besides, it also can be shown that the buckling and wrinkling loads decrease gradually with the relaxation of the boundary restriction. [ABSTRACT FROM AUTHOR]