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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.

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
  相似文献   
3.
The direct-synthesis of conductive PbS quantum dot (QD) ink is facile, scalable, and low-cost, boosting the future commercialization of optoelectronics based on colloidal QDs. However, manipulating the QD matrix structures still is a challenge, which limits the corresponding QD solar cell performance. Here, for the first time a coordination-engineering strategy to finely adjust the matrix thickness around the QDs is presented, in which halogen salts are introduced into the reaction to convert the excessive insulating lead iodide into soluble iodoplumbate species. As a result, the obtained QD film exhibits shrunk insulating shells, leading to higher charge carrier transport and superior surface passivation compared to the control devices. A significantly improved power-conversion efficiency from 10.52% to 12.12% can be achieved after the matrix engineering. Therefore, the work shows high significance in promoting the practical application of directly synthesized PbS QD inks in large-area low-cost optoelectronic devices.  相似文献   
4.
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   
5.
Novel lead-free (1-x)Ba0·9Ca0·1Ti0·9Zr0·1O3-xSrNb2O6 ceramics were synthesized via a two-step high energy ball milling process. The evolution of microstructural properties, phase transformation, and energy storage characteristics was comprehensively investigated to assess the applicability of material in multi-layered ceramic capacitors. The substitution of SrNb2O6 (SNO) in Ba0·9Ca0·1Ti0·9Zr0·1O3 (BTCZ) has resulted in substantial improvement in materials density along with a small increase in the grain size of the synthesized ceramic. A thorough microstructural investigation indicates an excellent dispersibility and compatibility between BTCZ and SNO phases. With an increase in SNO substitution, a transition from typical ferroelectric to relaxor ferroelectric has been observed, which has led to a significantly slimmer ferroelectric loop along with frequency dispersive dielectric properties. The optimized composition (i.e., x = 0.10) exhibits an ultra-high recoverable energy density of 2.68 J/cm3 along with a moderately high energy efficiency of 83.4%. Further, SNO substituted samples have also shown an enhancement in breakdown strength. The improvement in energy storage performance and breakdown strength of SNO substituted BTCZ composites are mainly attributed to relatively homogeneous grain morphology, optimum grain size, microstructural density, and improved grain boundary interface.  相似文献   
6.
Laser aided additive manufacturing(LAAM)was used to fabricate bulk Fe49.5Mn30Co10Cr10C0.5 interstitial multicomponent alloy using pre-alloyed powder.The room temperature yield strength(σy),ultimate tensile strength(σUTS)and elongation(εUST)were 645 MPa,917 MPa and 27.0%respectively.The as-built sample consisted of equiaxed and dendritic cellular structures formed by elemental segregation.These cellular structures together with oxide particle inclusions were deemed to strengthen the material.The other contributing components include dislocation strengthening,friction stress and grain bound-ary strengthening.The high εUTS was attributed to dislocation motion and activation of both twinning and transformation-induced plasticity(TWIP and TRIP).Tensile tests performed at-40℃and-130℃demonstrated superior tensile strength of 1041 MPa and 1267 MPa respectively.However,almost no twinning was observed in the fractured sample tested at-40℃and-130℃.Instead,higher fraction of strain-induced hexagonal close-packed(HCP)ε phase transformation of 21.2%were observed for fractured sample tested at-40℃,compared with 6.3%in fractured room temperature sample.  相似文献   
7.
The uniform refinement mechanisms and methods of deformed mixed and coarse grains inside a solution-treatment Ni-based superalloy during two-stage annealing treatment have been investigated.The two-stage heat treatment experiments include an aging annealing treatment(AT)and a subsequent recrystallization annealing treatment(RT).The object of AT is to precipitate some δ phases and consume part of storage energy to inhibit the grain growth during RT,while the RT is to refine mixed and coarse grains by recrystallization.It can be found that the recrystallization grains will quickly grow up to a large size when the AT time is too low or the RT temperature is too high,while the deformed coarse grains cannot be eliminated when the AT time is too long or the RT temperature is too low.In addition,the mixed microstructure composed of some abnormal coarse recrystallization grains(ACRGs)and a large number of fine grains can be observed in the annealed specimen when the AT time is 3 h and RT tem-perature is 980℃.The phenomenon attributes to the uneven distribution of δ phase resulted from the heterogeneous deformation energy when the AT time is too short.In the regions with a large number of δ phases,the recrystallization nucleation rate is promoted and the growth of grains is limited,which results in fine grains.However,in the regions with few δ phases,the recrystallization grains around grain boundaries can easily grow up,and the new recrystallization nucleus is difficult to form inside grain,which leads to ACRGs.Thus,in order to obtain uniform and fine annealed microstructure,it is a prereq-uisite to precipitate even-distributed δ phase by choosing a suitable AT time,such as 12 h.Moreover,a relative high RT temperature is also needed to promote the recrystallization nucleation around δ phase.The optimal annealing parameters range for uniformly refining mixed crystal can be summarized as:900℃×12 h+990℃×(40-60 min)and 900℃×12 h+1000℃×(10-15 min).  相似文献   
8.
Bone related diseases have caused serious threats to human health owing to their complexity and specificity. Fortunately, owing to the unique 3D network structure with high aqueous content and functional properties, emerging hydrogels are regarded as one of the most promising candidates for bone tissue engineering, such as repairing cartilage injury, skull defect, and arthritis. Herein, various design strategies and synthesis methods (e.g., 3D-printing technology and nanoparticle composite strategy) are introduced to prepare implanted hydrogel scaffolds with tunable mechanical strength, favorable biocompatibility, and excellent bioactivity for applying in bone regeneration. Injectable hydrogels based on biocompatible materials (e.g., collagen, hyaluronic acid, chitosan, polyethylene glycol, etc.) possess many advantages in minimally invasive surgery, including adjustable physicochemical properties, filling irregular shapes of defect sites, and on-demand release drugs or growth factors in response to different stimuli (e.g., pH, temperature, redox, enzyme, light, magnetic, etc.). In addition, drug delivery systems based on micro/nanogels are discussed, and its numerous promising designs used in the application of bone diseases (e.g., rheumatoid arthritis, osteoarthritis, cartilage defect) are also briefed in this review. Particularly, several key factors of hydrogel scaffolds (e.g., mechanical property, pore size, and release behavior of active factors) that can induce bone tissue regeneration are also summarized in this review. It is anticipated that advanced approaches and innovative ideas of bioactive hydrogels will be exploited in the clinical field and increase the life quality of patients with the bone injury.  相似文献   
9.
Various products, including foods and pharmaceuticals, are sensitive to temperature fluctuations. Thus, temperature monitoring during production, transportation, and storage is critical. Facile indicators are required to monitor temperature conditions via color changes in real time. This study aimed to prepare and apply thiol-functionalized covalent organic frameworks (COFs) as a novel indicator for monitoring thermal history and temperature abuse. The COFs underwent obvious color changes from bright yellow to purple after exposure to different temperatures for varying durations. The reaction kinetics are analyzed under isothermal conditions, which reveal that the order of reaction rates is k−20°C < k4°C < k20°C < k35°C < k55°C. The activation energy (Ea) of the COFs is calculated using the Arrhenius equation as 50.71 kJ moL−1. The COFs are capable of sensitive color changes and offer a broad temperature tracking range, thereby demonstrating their application potential for the monitoring of temperature and time exposure history during production, transportation, and storage. This excellent performance thermal history indicator also shows promise for expanding the application field of COFs.  相似文献   
10.
Lithium metal anodes (LMAs) are promising for next-generation batteries but have poor compatibility with the widely used carbonate-based electrolytes, which is a major reason for their severe dendrite growth and low Coulombic efficiency (CE). A nitrate additive to the electrolyte is an effective solution, but its low solubility in carbonates is a problem that can be solved using a crown ether, as reported. A rubidium nitrate additive coordinated with 18-crown-6 crown ether stabilizes the LMA in a carbonate electrolyte. The coordination promotes the dissolution of NO3 ions and helps form a dense solid electrolyte interface that is Li3N-rich which guides uniform Li deposition. In addition, the Rb (18-crown-6)+ complexes are adsorbed on the dendrite tips, shielding them from Li deposition on the dendrite tips. A high CE of 97.1% is achieved with a capacity of 1 mAh cm−2 in a half cell, much higher than when using the additive-free electrolyte (92.2%). Such an additive is very compatible with a nickel-rich ternary cathode at a high voltage, and the assembled full battery with a cathode material loading up to 10 mg cm−2 shows an average CE of 99.8% over 200 cycles, indicating a potential for practical use.  相似文献   
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