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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.
基于现有热成形零件冷弯性能不足、氢脆敏感性强等共性技术问题,在22MnB5钢的基础上采用Nb、V微合金化的设计思路,对试验钢的显微组织、淬透性和极限尖冷弯性能进行研究。结果表明:微合金化前后22MnB5热成形钢的显微组织均为全马氏体,但是微合金化后的试验钢组织更为细小;Nb-V微合金化能有效提高22MnB5热成形钢的淬透性,其淬硬层深度达到13~14 mm,并且试验钢的极限尖冷弯角达到58°~72°,进一步拓宽了热冲压成形的工艺窗口。综合分析试验钢微观组织、淬透性能及极限尖冷弯性能的检测结果,添加0.04%Nb和0.04%V实现复合微合金化,提升材料的综合强塑性,满足了高冷弯性能汽车零部件用材需求。  相似文献   
3.
对航天用紧固件TC4钛合金棒材进行固溶时效处理,对棒材不同位置进行显微组织观察、硬度和室温拉伸性能检测。结果表明:TC4钛合金棒材经固溶时效后表面至心部的组织与性能受冷却速度的影响呈现显著差异。固溶时效后的显微组织由稳定的等轴α相、弥散的马氏体α′相和亚稳定β相组成,试样端面上因冷却速度相差不大,次生α相的形态和含量没有明显差异;中部截面上边部至心部的次生α相含量逐渐增多,同时次生α相片层厚度逐渐增大并趋于等轴化。端面上不同位置显微硬度值没有明显差异,但中部截面上由边部至心部的显微硬度值呈总体降低趋势,且中部截面上边部的显微硬度值与端面相差不大。试样心部因固溶过程中冷却缓慢,整体试样的室温拉伸性能明显低于去除心部的试样。  相似文献   
4.
The aim of this study was to determine the influence of severe plastic deformation processing and the changes in microstructure resulting therefrom on the corrosion resistance of an Al–Mg–Si alloy. The alloy was processed using incremental equal channel angular pressing, which caused a reduction in grain size from 15 to 0.9 µm. The grain refinement was accompanied by an increase in the number of grain boundaries and dislocations, and by changes in grain orientation. However, there was no change in the size and number of intermetallic particles, which presumably resulted in a constant number of galvanic couplings. Electrochemical experiments revealed only slight differences between the samples before and after processing. Higher potential transients/oscillations upon immersion and increased corrosion currents in the vicinity of corrosion potential point to slightly higher reactivity of the most refined material. This indicates that intermetallic particles are the most crucial microstructural elements in terms of corrosion resistance. Their impact exceeds that of grain boundaries, in particular, at the stage of corrosion initiation. The development of corrosion attack is controlled more by the microstructure of the matrix as the grain refinement resulted in a less pronounced corrosion attack in comparison with the coarse-grained sample.  相似文献   
5.
为解决非对称断面钢轨镦粗时的波浪变形问题,设计了局部镦粗成套装置,利用已有的锻造生产线,有效地控制了非对称断面钢轨镦粗时产生的失稳和轨肢波浪变形,减少了模具更换次数;同时,设计的镦粗测量和显示装置,可精确控制镦粗量,保证镦粗成形质量稳定。经实物验证,采用本装置及工艺,单根镦粗时间由原工艺的60 min降低至8 min,效率提高了7倍以上;镦粗钢轨一次合格率达100%,较原工艺合格率提高了3倍以上。利用已有的锻造生产线,减少了设备投入,降低了投资成本,在工艺布局上可以减少镦粗工序的生产区域,优化了车间的工艺布局。最后,通过实物验证了方案可行性,并确定了工艺参数,可用于指导实际生产。  相似文献   
6.
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   
7.
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   
8.
During any non-LOCA event, the average temperature of the reactor cooling system (RCS) should be decreased to 215.6℃within 36 hours in AP1000 nuclear power plant. However, this goal cannot be achieved because the rate of condensate return was far lower than that expected. Through the analysis and verification of the dome condensation drop test, the path of condensation loss was determined. Therefore, a series of the design modifications to the polar crane girder, the internal stiffener and the condensation return gutter were carried out. Lastly, the safety shutdown temperature evaluation showed that the AP1000 plant could reduce the RCS average temperature to 215.6℃ within 34.6 hours after the loss of normal feedwater and the loss of off-site power concurrently.  相似文献   
9.
To benefit from recent advances in modeling and computational algorithms,as well as the availability of new covariance data,sensitivity and uncertainty analyses are needed to quantify the impact of uncertain sources on the design parameters of small prismatic high-temperature gas-cooled reactors(HTGRs).In particular,the contribution of nuclear data to the keff uncertainty is an important part of the uncertainty analysis of small-sized HTGR physical calculations.In this study,a small-sized HTGR designed by China Nuclear Power Engineering Co.,Ltd.was selected for keff uncertainty analysis during full lifetime burnup calculations.Models of the cold zero power(CZP)condition and full lifetime burnup process were constructed using the Reactor Monte Carlo Code RMC for neutron transport calculation,depletion calculation,and sensitivity and uncertainty analysis.For the sensitivity analysis,the Contribution-Linked eigenvalue sensitivity/Uncertainty estimation via Track length importance Characterization(CLUTCH)method was applied to obtain sensitive infor-mation,and the"sandwich"method was used to quantify the keff uncertainty.We also compared the keff uncertainties to other typical reactors.Our results show that 235U is the largest contributor to keff uncertainty for both the CZP and depletion conditions,while the contribution of 239Pu is not very significant because of the design of low discharge burnup.It is worth noting that the radioactive capture reaction of 28Si significantly contributes to the keff uncer-tainty owing to its specific fuel design.However,the keff uncertainty during the full lifetime depletion process was relatively stable,only increasing by 1.12%owing to the low discharge burnup design of small-sized HTGRs.These numerical results are beneficial for neutronics design and core parameters optimization in further uncertainty prop-agation and quantification study for small-sized HTGR.  相似文献   
10.
针对瓦斯抽采监控系统中气体流量计上游管道内气体流速分布不均匀问题,提出一种多组去心叶片交错分布流动调整器优化结构。以叶片组数和相邻两叶片组的交错角度为优化参数,建立4种调整器结构优化模型。利用CFD数值模拟技术分析不同雷诺数时各调整器下游的流场特性,对比调整器下游6个位置处流体的径向速度与充分发育流体的相差程度。结果表明,4组叶片交错布置11.25°时调整器整流效果相对较好,可将调整器下游所需直管长度缩短50%,为瓦斯流量测量减小空间成本、提高测量准确性提供理论参考。  相似文献   
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