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1.
The germline carrier of the BRCA1 pathogenic mutation has been well proven to confer an increased risk of breast and ovarian cancer. Despite BRCA1 biallelic pathogenic mutations being extremely rare, they have been reported to be embryonically lethal or to cause Fanconi anemia (FA). Here we describe a patient who was a 48-year-old female identified with biallelic pathogenic mutations of the BRCA1 gene, with no or very subtle FA-features. She was diagnosed with ovarian cancer and breast cancer at the ages of 43 and 44 and had a strong family history of breast and gynecological cancers.  相似文献   
2.
周慧 《机械管理开发》2021,36(8):131-132
盘形制动器属于提升系统内的一重要部件,为确保提升机盘形制动器的稳定可靠运行,基于同家梁矿矿井提升机盘形制动器应用现状,分析了现存的主要问题,提出了采用智能监测盘形制动器来替代原盘形制动器的策略.通过在该新型盘形制动器内装设内置制动正压力传感器,解决了老旧盘形制动器无法准确测量内部碟簧力的问题,取得了较好的应用效果.  相似文献   
3.
Aromatic and functional polymers with processibility derived from biobased starting materials are prerequisite considering sustainable society. Poly(2,5-benzimidazole)s are rigid-rod polymers to show ultrahigh thermal stability such as flame retardance, while usually suffer from poor solubility. Here, poly(benzimidazole-co-amide)s are synthesized from two biobased monomers, 3,4-diaminobenzoic acid and a semirigid comonomer, 4-aminohydrocinnamic acid. The copolymers with an amide composition of 80 mol% and higher are soluble in widely used polar solvents to fabricate the films keeping high flame retardance, which is comparable with popular high-performance polymers such as aromatic polyimides, polyetheretherketone, polyphenylene sulfide, etc.  相似文献   
4.

The presence of Mn(II) in water exceeding the permitted concentration limits declared by the World Health Organization (WHO) influences individuals, animals, and the ecosystem negatively. Therefore, there is a necessity for an efficient material to eliminate this potentially toxic element from wastewater. We herein focused on the adsorptive removal of Mn(II) ions from polluted aqueous media using natural Egyptian glauconite clay (G) and its nanocomposites with modified chitosan (CS). We applied modified chitosan with glutaraldehyde (GL), ethylenediaminetetraacetic acid (EDTA), sodium dodecyl sulfate (SDS), and cetyltrimethyl ammonium bromide (CTAB). The utilized nanocomposites were referred to as GL-CS/G, EDTA-GL-CS/G, SDS-CS/G, and CTAB-CS/G, respectively. The point of zero charge values of the materials were estimated. The adsorption properties of the G clay and its nanocomposites toward the removal of Mn(II) ions from polluted aqueous media as well as the adsorption mechanism were explored using a batch technique. The glauconite (G) and its nanocomposites: GL-CS/G, CTAB-CS/G, EDTA-GL-CS/G, and SDS-CS/G, exhibited maximum adsorption capacity values of 3.60, 24.0, 26.0, 27.0, and 27.9 mg g?1, respectively. The adsorption results fitted well the Langmuir isotherm and pseudo-second-order kinetic models. The estimated thermodynamic parameters: ΔH° (from 1.03 to 5.55 kJ/mol) and ΔG° (from ? 14.5 to ? 18.8 kJ/mol), indicated that Mn(II) ion adsorption process was endothermic, spontaneous, and physisorption controlled. Furthermore, the obtained adsorption results are encouraging and revealing a great potentiality for using the modified adsorbents as accessible adsorbents for Mn(II) ion removal from polluted aqueous solutions, depending on their reusability, high stability, and good adsorption capacities.

Graphic Abstract
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5.
Ce:Y3Al5O12 transparent ceramics (TCs) with appropriate emission light proportion and high thermal stability are significant to construct white light emitting diode devices with excellent chromaticity parameters. In this work, strategies of controlling crystal-field splitting around Ce3+ ion and doping orange-red emitting ion, were adopted to fabricate Ce:(Y,Tb)3(Al,Mn)5O12 TCs via vacuum sintering technique. Notably, 85.4 % of the room-temperature luminescence intensity of the TC was retained at 150 °C, and the color rendering index was as high as 79.8. Furthermore, a 12 nm red shift and a 16.2 % increase of full width at half maximum were achieved owing to the synergistic effects of Tb3+ and Mn2+ ions. By combining TCs with a 460 nm blue chip, a warm white light with a low correlated color temperature of 4155 K was acquired. Meanwhile, the action mechanism of Tb3+ ion and the energy transfer between Ce3+ and Mn2+ ions were verified in prepared TCs.  相似文献   
6.
Recently, circularly polarized organic light-emitting diodes (CP-OLEDs) fabricated with thermally activated delayed fluorescence (TADF) emitters are developed rapidly. However, most devices are fabricated by vacuum deposition technology, and developing efficient solution-processed CP-OLEDs, especially nondoped devices, is still a challenge. Herein, a pair of triptycene-based enantiomers, (S,S)-/(R,R)-TpAc-TRZ, are synthesized. The novel chiral triptycene scaffold of enantiomers avoids their intermolecular π–π stacking, which is conducive to their aggregation-induced emission characteristics and high photoluminescence quantum yield of 85% in the solid state. Moreover, the triptycene-based enantiomers exhibit efficient TADF activities with a small singlet-triplet energy gap (ΔEST) of 0.03 eV and delayed fluorescence lifetime of 1.1 µs, as well as intense circularly polarized luminescence with dissymmetry factors (|gPL|) of about 1.9 × 10−3. The solution-processed nondoped CP-OLEDs based on (S,S)-/(R,R)-TpAc-TRZ not only display obvious circularly polarized electroluminescence signals with gEL values of +1.5 × 10−3 and −2.0 × 10−3, respectively, but also achieve high efficiencies with external quantum, current, and power efficiency up to 25.5%, 88.6 cd A−1, and 95.9 lm W−1, respectively.  相似文献   
7.
8.
Alumina platelets were arranged horizontally in submicron alumina particles by shear force in the flow of slurries during casting. The obtained alumina green bodies with platelets were pressureless-sintered in vacuum, producing ceramics with thoroughly oriented grains and high transmittance. The effects of sintering parameters on the densification, microstructure evolution, and orientation degree of alumina ceramics were investigated and discussed. The results showed that the densification, grain size, orientation degree, and in-line transmittance were increased with increasing sintering temperature. The enhancement of orientation degree was mainly coherent with grain growth. The grain-oriented samples exhibited a much higher in-line transmittance (at 600 nm) of 61 % than that of the grain random sample (29 %). Moreover, the transmission remained a high level in the ultraviolet range (<300 nm).  相似文献   
9.
Reliable joints of Ti3SiC2 ceramic and TC11 alloy were diffusion bonded with a 50 μm thick Cu interlayer. The typical interfacial structure of the diffusion boned joint, which was dependent on the interdiffusion and chemical reactions between Al, Si and Ti atoms from the base materials and Cu interlayer, was TC11/α-Ti + β-Ti + Ti2Cu + TiCu/Ti5Si4 + TiSiCu/Cu(s, s)/Ti3SiC2. The influence of bonding temperature and time on the interfacial structure and mechanical properties of Ti3SiC2/Cu/TC11 joint was analyzed. With the increase of bonding temperature and time, the joint shear strength was gradually increased due to enhanced atomic diffusion. However, the thickness of Ti5Si4 and TiSiCu layers with high microhardness increased for a long holding time, resulting in the reduction of bonding strength. The maximum shear strength of 251 ± 6 MPa was obtained for the joint diffusion bonded at 850 °C for 60 min, and fracture primarily occurred at the diffusion layer adjacent to the Ti3SiC2 substrate. This work provided an economical and convenient solution for broadening the engineering application of Ti3SiC2 ceramic.  相似文献   
10.
Laminated Si3N4/SiCw ceramics were successfully prepared by tape casting and hot-pressing. Its mechanical properties were measured and the impact resistance was discussed. The toughness of the laminated Si3N4/SiCw ceramics was 13.5 MPa m1/2, which was almost 1.6 times that of Si3N4/SiCw composite ceramics, namely 8.5 MPa m1/2. Moreover, the indentation strength of laminated Si3N4/SiCw ceramics was not sensitive to increasing indentation loads and exhibited a rising R-curve behaviour, indicating that the laminated Si3N4/SiCw ceramics had excellent impact resistance. The improved toughness and impact resistance of laminated Si3N4/SiCw ceramics was attributed to the residual stress caused by a thermal expansion coefficient mismatch between the different layers, resulting in crack deflection and bridging of SiC whiskers in the interface layer, thus consuming a large amount of fracture work.  相似文献   
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