Sortilin-related receptor 1(SORL1)is a critical gene associated with late-onset Alzheimer’s disease.SORL1 contributes to the development and progression of this neurodegenerative condition by affecting the transport ...Sortilin-related receptor 1(SORL1)is a critical gene associated with late-onset Alzheimer’s disease.SORL1 contributes to the development and progression of this neurodegenerative condition by affecting the transport and metabolism of intracellularβ-amyloid precursor protein.To better understand the underlying mechanisms of SORL1 in the pathogenesis of late-onset Alzheimer s disease,in this study,we established a mouse model of SorI1 gene knockout using cluste red regularly inters paced short palindro mic repeats-associated protein 9 technology.We found that Sorl1-knocko ut mice displayed deficits in learning and memory.Furthermore,the expression of brain-derived neurotrophic factor was significantly downregulated in the hippocampus and co rtex,and amyloidβ-protein deposits were observed in the brains of 5orl1-knockout mice.In vitro,hippocampal neuronal cell synapses from homozygous Sorl1-knockout mice were impaired.The expression of synaptic proteins,including Drebrin and NR2B,was significantly reduced,and also their colocalization.Additionally,by knocking out the Sorl1 gene in N2a cells,we found that expression of the N-methyl-D-aspartate receptor,NR2B,and cyclic adenosine monophosphate-response element binding protein was also inhibited.These findings suggest that SORL1 participates in the pathogenesis of late-onset Alzheimer s disease by regulating the N-methyl-D-aspartate receptor NR2B/cyclic adenosine monophosphate-response element binding protein signaling axis.展开更多
In machining processes,chatter vibrations are always regarded as one of the major limitations for production quality and efficiency.Accurate and timely monitoring of chatter is helpful to maintain stable machining ope...In machining processes,chatter vibrations are always regarded as one of the major limitations for production quality and efficiency.Accurate and timely monitoring of chatter is helpful to maintain stable machining operations.At present,most chatter monitoring methods are based on the energy level at specified chatter frequencies or frequency bands.However,the spectral features of chatter could change during machining operations due to complexity and time-varying dynamics of the physical machining process.The purpose of this paper is to investigate the time-varying chatter features in turning of thin-walled tubular workpieces from the perspective of entropy.The airborne acoustics was selected as the source of information for machining condition monitoring.First,corresponding to the distinguishing surface topographies relevant to machining conditions,the features of the sound signal emitted during turning of the thin-walled cylindrical workpieces were extracted using the spectral analysis and wavelet packet transform,respectively.It was shown that the dominant vibration frequency as well as the energy distribution could shift with the transition of the machining status.After that,two relative entropy indicators based on the spectrum and the wavelet packet energy were constructed to identify chattering events in turning of the thin-walled tubes.The experimental results demonstrate that the proposed indicators could accurately reflect the transition of machining conditions with high sensitivity and robustness in comparison with the traditional FFT-based methods.The achievement of this study lays the foundations of the online chatter monitoring and control technique for turning of the thin-walled tubular workpieces.展开更多
目的比较晚期肝细胞癌一线系统治疗药物的有效性和安全性,供临床实践作参考。方法网络检索PubMed、Cochrane Library、Embase和Web of Science等文献数据库,检索时限均为建库至2021年6月,根据预设的纳入标准筛选文献进行网络荟萃分析。...目的比较晚期肝细胞癌一线系统治疗药物的有效性和安全性,供临床实践作参考。方法网络检索PubMed、Cochrane Library、Embase和Web of Science等文献数据库,检索时限均为建库至2021年6月,根据预设的纳入标准筛选文献进行网络荟萃分析。结果共纳入12项随机对照研究,合计8058例患者。网络荟萃分析结果显示,相较于索拉非尼,信迪利单抗联合贝伐珠单抗生物类似物(IBI305)、阿替利珠单抗联合贝伐珠单抗和多纳非尼均降低了患者的死亡风险(HR=0.57,95%CI:0.43~0.75;HR=0.58,95%CI:0.42~0.80;HR=0.83,95%CI:0.70~0.99),该3种治疗方式有最高的P-score值,分别为0.956、0.948和0.764;在有或无“大血管侵犯/肝外转移”的两组患者亚组中,3种治疗方案的排名类似;安全性方面,除安慰剂之外,多纳非尼因不良事件终止研究治疗、减量研究药物和暂停研究治疗的P-score值均位居首位。结论晚期肝细胞癌一线系统治疗药物中,信迪利单抗联合贝伐珠单抗生物类似物、阿替利珠单抗联合贝伐珠单抗和多纳非尼单药治疗均可显著改善晚期肝细胞癌患者的总生存时间;而多纳非尼的安全性和耐受性更佳。展开更多
Compared with the traditional pyrometallurgical process, copper bioleaching has distinctive advantages of high efficiency and lower cost, enabling efficiently extracts of valuable metal resources from copper sulfides....Compared with the traditional pyrometallurgical process, copper bioleaching has distinctive advantages of high efficiency and lower cost, enabling efficiently extracts of valuable metal resources from copper sulfides. Moreover, during long-term industrial applications of bioleaching, many regulatory enhancements and technological methods are used to accelerate the interfacial reactions. With advances in microbial genetic and sequencing technologies, bacterial communities and their mechanisms in bioleaching systems have been revealed gradually. The bacterial proliferation and dissolution of sulfide ores by a bacterial community depends on the pH, temperature, oxygen, reaction product regulation, additives, and passivation substances, among other factors. The internal relationship among the influencing factors and the succession of microorganism diversity are discussed and reviewed in this paper. This paper is intended to provide a good reference for studies related to enhanced bioleaching.展开更多
To solve the problem of low permeability and lower extraction rates of high-mud ores, a surfactant was added as a penetrant to the pregnant leaching solution during column leaching tests. On the basis of the theories ...To solve the problem of low permeability and lower extraction rates of high-mud ores, a surfactant was added as a penetrant to the pregnant leaching solution during column leaching tests. On the basis of the theories of physical chemistry and seepage flow mechanics, the mechanism by which seepage is enhanced under the effects of the surfactant was analyzed. The results show that the action modes of the surfactant were divided into four aspects: changing the wettability of the ore, reducing the viscosity of the leaching solution, adsorbing onto the surface of ore, and enhancing the permeability effect. The findings of column leaching tests demonstrated that permeability was substantially improved by the surfactant. In the later period of leaching, the permeability coefficient was two times higher than that of the control group. Meanwhile, the ore extraction rate increased by approximately 10%. During the leaching process, the surface tension of the solution did not substantially change, and that of the solution with surfactant increased slightly. The kinetics analysis of ore column leaching illustrated that the leaching processes were controlled by both internal diffusion(principal factor) and chemical reaction.展开更多
AIM：To assess the safety,efficacy,predictability and stability of implantable collamer lens（ICL） for residual refractive error after corneal refractive surgery.METHODS： This study evaluated 19 eyes of 12 patients ...AIM：To assess the safety,efficacy,predictability and stability of implantable collamer lens（ICL） for residual refractive error after corneal refractive surgery.METHODS： This study evaluated 19 eyes of 12 patients who underwent ICL implantation after corneal refractive surgeries.They were followed up for 1y to 5y of uncorrected distance visual acuity（UDVA）,corrected distance visual acuity（CDVA）,manifest refractive error,flat and steep K value,axial length,intraocular pressure,corneal endothelial cell density,adverse events after ICL surgery.RESULTS： The mean follow-up period was 39.05 ±19.22 mo（range,1-5y）.Spherical equivalent refractive error changed from-7.45±3.02 D preoperatively to-0.85±1.10 D 1wk to 1mo after ICL implantation,with the safety and efficacy indices being 1.12 and 1.15,respectively.A total of 52.63% of eyes were within ±0.5 D of the predicted spherical equivalents,73.68% were within ±1.0 D.A trend of mild regression towards myopia with axial elongation after 5y was observed.One eye with mild anterior capsule opacity and retinal detachment 1y after surgery were observed.CONCLUSION： ICL implantation is safe and effective for the correction of residual refractive error after corneal refractive surgeries,especially in moderate to high residual myopia.展开更多
BACKGROUND Geleophysic dysplasia(GD)presents the characterized clinical manifestations of acromelic dysplasia,including extremely short stature,short limbs,small hands and feet,stubby fingers and toes,joint stiffness ...BACKGROUND Geleophysic dysplasia(GD)presents the characterized clinical manifestations of acromelic dysplasia,including extremely short stature,short limbs,small hands and feet,stubby fingers and toes,joint stiffness and others.It is clinically distinct from the other acromelic dysplasia in terms of symptoms such as cardiac valvular abnormalities,progressive hepatomegaly and tracheal stenosis.CASE SUMMARY We report on a Chinese 9-year-old girl with GD with the c.5243G>T(p.C1748F)mutation in FBN1(fibrillin 1,OMIM 134797).She was born in Guangxi Zhuang Autonomous Region of China.The patient presented with typical clinical features of GD and recurrent respiratory tract infections over 6 years.Laboratory studies and chest computed tomography(CT)scan indicated bronchopneumonia.Her echocardiography revealed mild mitral valve thickening with regurgitation.Laryngopharyngeal CT and electronic bronchoscopy revealed severe glottic stenosis.Echocardiography examination displayed mild mitral valve thickening and regurgitation.Ophthalmic examination did not reveal myopia or lens dislocation.Treated with ceftriaxone sodium and methylprednisolone sodium succinate for injection as well as methylprednisolone orally,patient’s symptoms had improved.CONCLUSION GD is a rare genetic condition that can cause life-threatening cardiovascular and respiratory problems.This study also found that the identified genotype of GD could be related to different clinical phenotypes.展开更多
The chemical binder is one of the critical factors affecting ore agglomeration behavior and leaching efficiency.In this study,we in-vestigated the effect of the type of binder and mass fraction of the H_(2)SO_(4)solut...The chemical binder is one of the critical factors affecting ore agglomeration behavior and leaching efficiency.In this study,we in-vestigated the effect of the type of binder and mass fraction of the H_(2)SO_(4)solution used on the curing,soaking,and leaching behavior of ag-glomerations.The results revealed that Portland cement(3CaO·SiO_(2),2CaO·SiO_(2),and 3CaO·Al_(2)O_(3))was the optimal binder for obtaining a well-shaped,stable agglomeration structure.A higher extraction rate was achieved when using Portland cement than that obtained using sodi-um silicate,gypsum,or acid-proof cement.An excessive geometric mean size is not conducive to obtaining well-shaped agglomerations and desirable porosity.Using computed tomography(CT)and MATLAB,the porosity of two-dimensional CT images in sample concentrations L1-L3 was observed to increase at least 4.5vol%after acid leaching.Ore agglomerations began to be heavily destroyed and even to disinteg-rate when the sulfuric acid solution concentration was higher than 30 g/L,which was caused by the excessive accumulation of reaction products and residuals.展开更多
In our published letter,[1]we have identified a minor error in Figs.2 and 3.Instead,we have 2111 events in these two plots with 646,249,382,and 834 events in Run 9(20.0 ton·day),Run 10(19.4 ton·day),Run 11–...In our published letter,[1]we have identified a minor error in Figs.2 and 3.Instead,we have 2111 events in these two plots with 646,249,382,and 834 events in Run 9(20.0 ton·day),Run 10(19.4 ton·day),Run 11–1(24.2 ton·day),and Run 11–2(37.1 ton·day).The mistake is due to an incorrect application of a small energy non-linearity(known as the BLS non-linearity)in making plots,but has no impact to the final results.Figures 2 and 3 are now updated in this note.展开更多
Single crystal silicon carbide(SiC)is widely used for optoelectronics applications.Due to the anisotropic characteristics of single crystal materials,the C face and Si face of single crystal SiC have different physica...Single crystal silicon carbide(SiC)is widely used for optoelectronics applications.Due to the anisotropic characteristics of single crystal materials,the C face and Si face of single crystal SiC have different physical properties,which may fit for particular application purposes.This paper presents an investigation of the material removal and associated subsurface defects in a set of scratching tests on the C face and Si face of 4H-SiC and 6H-SiC materials using molecular dynamics simulations.The investigation reveals that the sample material deformation consists of plastic,amorphous transformations and dislocation slips that may be prone to brittle split.The results showed that the material removal at the C face is more effective with less amorphous deformation than that at the Si face.Such a phenomenon in scratching relates to the dislocations on the basal plane(0001)of the SiC crystal.Subsurface defects were reduced by applying scratching cut depths equal to integer multiples of a half molecular lattice thickness,which formed a foundation for selecting machining control parameters for the best surface quality.展开更多
Effects of residues produced by agricultural wastes fermentation(AWF)on low grade copper sulfide ores bioleaching,copper recovery,and microbial community were investigated.The results indicated that adding appropriate...Effects of residues produced by agricultural wastes fermentation(AWF)on low grade copper sulfide ores bioleaching,copper recovery,and microbial community were investigated.The results indicated that adding appropriate bulk of AWF made contributions to low grade copper sulfide ores bioleaching,which may be mainly realized through reducing the passivation layer formed by Fe3+hydrolysis.Improved copper recovery(78.35%)and bacteria concentration(9.56×10^(7)cells·mL^(−1))were yielded in the presence of 5 g·L^(−1)AWF.The result of 16S rDNA analysis demonstrated that microbial community was differentiated by adding AWF.Bacteria proportion,such as Acidithiobacillus ferrooxidans,Moraxella osloensis,and Lactobacillus acetotolerans changed distinctly.Great difference between samples was showed according to beta diversity index,and the maximum value reached 0.375.Acidithiobacillus ferrooxidans accounted for the highest proportion throughout the bioleaching process,and that of sample in the presence of 5 g·L^(−) AWF reached 28.63%.The results should show reference to application of agricultural wastes and low grade copper sulfide ores.展开更多
The conflict between poverty and the resulting over-exploitation of natural resources on the one hand, and ecological restoration and sustainable development on the other hand, in the southwest China karst region was ...The conflict between poverty and the resulting over-exploitation of natural resources on the one hand, and ecological restoration and sustainable development on the other hand, in the southwest China karst region was studied. In this region, the karst forest (a mixed evergreen and deciduous broad-leaved forest) is rapidly degrading due to over-exploitation (sloping farming). We suggest that an Ecological Compensation (EC) model should be established with: financial institutions, local people, and a third part as an intermediate link. The process would continue for 20 years. As a case study we used Bangui town (3800 families) in the upper reaches of Pearl River. The per capita income of residents was used as the benchmark. The compensation would start with 80%, and decrease to 20% over a period of 20 years. Infrastructure investment would decrease from 20% of the total person’s compensation to 5% as the farmers increasingly use alternative income sources. The EC includes compensation for individual, infrastructure, and environmental investments. The total EC for Bangui would be 305,064 × 104 yuan during the 20 years.展开更多
In this paper, a Multi Modal Reasoning (MMR) method integrated with probabilistic reasoning is proposed for the diagnosis support module of the open eHealth platform. MMR is based on both Rule Based Reasoning (RBR) an...In this paper, a Multi Modal Reasoning (MMR) method integrated with probabilistic reasoning is proposed for the diagnosis support module of the open eHealth platform. MMR is based on both Rule Based Reasoning (RBR) and Case Based Reasoning (CBR). It is not only applied to the identification of diseases and syndromes based on medical guidelines, but also deals with exceptional cases and individual therapies in order to improve diagnostic accuracy. Moreover, a new rule expression frame is introduced to deal with uncertainty, which can represent and process vague, imprecise, and incomplete information. Furthermore, this system is capable of updating the attributes of rules and inducing rules with a small data sample. Keywords Information management - uncertainty - decision making Jia Tian received her BSc degree from China University of Mining and Technology (CUMT), Beijing, China in 2002. She is currently pursuing her PhD at the University of Nottingham, United Kingdom. Her research interests include expert system, artificial intelligence and data mining, especially Medical Decision Support System, Case Based Reasoning, Rule Based Reasoning and Bayesian Networks.Xun Chen obtained his B. Eng. from Fuzhou University. He received his M. Sc. from Zhejiang University and his Ph. D from Liverpool John Moores University. He has been a visiting professor to Fuzhou University since 2001. He specialises in advanced manufacturing technology including application of computer science, mechatronics and artificial intelligence to broad engineering application. He has published more than 100 research papers. Dr Chen is a founder member of the International Committee of Abrasive Technology. Before his employment at Nottingham, Dr Chen was a lecturer of Mechanical Engineering at the University of Dundee. Prior to that, he was a research fellow, a Royal Society Royal Fellow at Liverpool John Moores University and a lecturer at Fuzhou University.Sheng-Ping Dong graduated from China University of Traditional Chinese Medicine, Beijing, China. She is an attending doctor of Traditional Chinese Medicine with many years clinical experience in Exhibition Road Hospital, Beijing, China.展开更多
基金supported by the Community Development Office of Hunan Provincial Science and Technology DepartmentChina,Nos.2020SK53613(to DH),21JJ31006(to DH)the Fundamental Research Funds of Central South University,Nos.CX20220375(to TX),2023zzts215(to MZ)。
文摘Sortilin-related receptor 1(SORL1)is a critical gene associated with late-onset Alzheimer’s disease.SORL1 contributes to the development and progression of this neurodegenerative condition by affecting the transport and metabolism of intracellularβ-amyloid precursor protein.To better understand the underlying mechanisms of SORL1 in the pathogenesis of late-onset Alzheimer s disease,in this study,we established a mouse model of SorI1 gene knockout using cluste red regularly inters paced short palindro mic repeats-associated protein 9 technology.We found that Sorl1-knocko ut mice displayed deficits in learning and memory.Furthermore,the expression of brain-derived neurotrophic factor was significantly downregulated in the hippocampus and co rtex,and amyloidβ-protein deposits were observed in the brains of 5orl1-knockout mice.In vitro,hippocampal neuronal cell synapses from homozygous Sorl1-knockout mice were impaired.The expression of synaptic proteins,including Drebrin and NR2B,was significantly reduced,and also their colocalization.Additionally,by knocking out the Sorl1 gene in N2a cells,we found that expression of the N-methyl-D-aspartate receptor,NR2B,and cyclic adenosine monophosphate-response element binding protein was also inhibited.These findings suggest that SORL1 participates in the pathogenesis of late-onset Alzheimer s disease by regulating the N-methyl-D-aspartate receptor NR2B/cyclic adenosine monophosphate-response element binding protein signaling axis.
基金The financial support of National Natural Science Foundation of China(Grant Nos.52175108,51805352)is gratefully acknowledgedWe also would like to acknowledge the Key Research and Development Project of Shanxi Province(Grant No.202102010101009).
文摘In machining processes,chatter vibrations are always regarded as one of the major limitations for production quality and efficiency.Accurate and timely monitoring of chatter is helpful to maintain stable machining operations.At present,most chatter monitoring methods are based on the energy level at specified chatter frequencies or frequency bands.However,the spectral features of chatter could change during machining operations due to complexity and time-varying dynamics of the physical machining process.The purpose of this paper is to investigate the time-varying chatter features in turning of thin-walled tubular workpieces from the perspective of entropy.The airborne acoustics was selected as the source of information for machining condition monitoring.First,corresponding to the distinguishing surface topographies relevant to machining conditions,the features of the sound signal emitted during turning of the thin-walled cylindrical workpieces were extracted using the spectral analysis and wavelet packet transform,respectively.It was shown that the dominant vibration frequency as well as the energy distribution could shift with the transition of the machining status.After that,two relative entropy indicators based on the spectrum and the wavelet packet energy were constructed to identify chattering events in turning of the thin-walled tubes.The experimental results demonstrate that the proposed indicators could accurately reflect the transition of machining conditions with high sensitivity and robustness in comparison with the traditional FFT-based methods.The achievement of this study lays the foundations of the online chatter monitoring and control technique for turning of the thin-walled tubular workpieces.
文摘目的比较晚期肝细胞癌一线系统治疗药物的有效性和安全性,供临床实践作参考。方法网络检索PubMed、Cochrane Library、Embase和Web of Science等文献数据库,检索时限均为建库至2021年6月,根据预设的纳入标准筛选文献进行网络荟萃分析。结果共纳入12项随机对照研究,合计8058例患者。网络荟萃分析结果显示,相较于索拉非尼,信迪利单抗联合贝伐珠单抗生物类似物(IBI305)、阿替利珠单抗联合贝伐珠单抗和多纳非尼均降低了患者的死亡风险(HR=0.57,95%CI:0.43~0.75;HR=0.58,95%CI:0.42~0.80;HR=0.83,95%CI:0.70~0.99),该3种治疗方式有最高的P-score值,分别为0.956、0.948和0.764;在有或无“大血管侵犯/肝外转移”的两组患者亚组中,3种治疗方案的排名类似;安全性方面,除安慰剂之外,多纳非尼因不良事件终止研究治疗、减量研究药物和暂停研究治疗的P-score值均位居首位。结论晚期肝细胞癌一线系统治疗药物中,信迪利单抗联合贝伐珠单抗生物类似物、阿替利珠单抗联合贝伐珠单抗和多纳非尼单药治疗均可显著改善晚期肝细胞癌患者的总生存时间;而多纳非尼的安全性和耐受性更佳。
基金financially supported by the National Key Research and Development Program of China (No. 2016YFC0600704)the National Science Fund for Excellent Young Scholars of China (No. 51722401)the Key Program of National Natural Science Foundation of China (No. 51734001)
文摘Compared with the traditional pyrometallurgical process, copper bioleaching has distinctive advantages of high efficiency and lower cost, enabling efficiently extracts of valuable metal resources from copper sulfides. Moreover, during long-term industrial applications of bioleaching, many regulatory enhancements and technological methods are used to accelerate the interfacial reactions. With advances in microbial genetic and sequencing technologies, bacterial communities and their mechanisms in bioleaching systems have been revealed gradually. The bacterial proliferation and dissolution of sulfide ores by a bacterial community depends on the pH, temperature, oxygen, reaction product regulation, additives, and passivation substances, among other factors. The internal relationship among the influencing factors and the succession of microorganism diversity are discussed and reviewed in this paper. This paper is intended to provide a good reference for studies related to enhanced bioleaching.
基金financially supported by the National Natural Science Foundation of China (No. 51604138)the Open Fund of the Key Laboratory of Ministry of Education of China for Efficient Mining and Safety of Metal Mines (No. ustbmslab201806)
文摘To solve the problem of low permeability and lower extraction rates of high-mud ores, a surfactant was added as a penetrant to the pregnant leaching solution during column leaching tests. On the basis of the theories of physical chemistry and seepage flow mechanics, the mechanism by which seepage is enhanced under the effects of the surfactant was analyzed. The results show that the action modes of the surfactant were divided into four aspects: changing the wettability of the ore, reducing the viscosity of the leaching solution, adsorbing onto the surface of ore, and enhancing the permeability effect. The findings of column leaching tests demonstrated that permeability was substantially improved by the surfactant. In the later period of leaching, the permeability coefficient was two times higher than that of the control group. Meanwhile, the ore extraction rate increased by approximately 10%. During the leaching process, the surface tension of the solution did not substantially change, and that of the solution with surfactant increased slightly. The kinetics analysis of ore column leaching illustrated that the leaching processes were controlled by both internal diffusion(principal factor) and chemical reaction.
基金Supported by the Committee of Science and Technology of Shanghai,China(No.09411962100)the Health and Family Planning Committee of Pudong New District of Shanghai,China(No.PW2014D-1)
文摘AIM：To assess the safety,efficacy,predictability and stability of implantable collamer lens（ICL） for residual refractive error after corneal refractive surgery.METHODS： This study evaluated 19 eyes of 12 patients who underwent ICL implantation after corneal refractive surgeries.They were followed up for 1y to 5y of uncorrected distance visual acuity（UDVA）,corrected distance visual acuity（CDVA）,manifest refractive error,flat and steep K value,axial length,intraocular pressure,corneal endothelial cell density,adverse events after ICL surgery.RESULTS： The mean follow-up period was 39.05 ±19.22 mo（range,1-5y）.Spherical equivalent refractive error changed from-7.45±3.02 D preoperatively to-0.85±1.10 D 1wk to 1mo after ICL implantation,with the safety and efficacy indices being 1.12 and 1.15,respectively.A total of 52.63% of eyes were within ±0.5 D of the predicted spherical equivalents,73.68% were within ±1.0 D.A trend of mild regression towards myopia with axial elongation after 5y was observed.One eye with mild anterior capsule opacity and retinal detachment 1y after surgery were observed.CONCLUSION： ICL implantation is safe and effective for the correction of residual refractive error after corneal refractive surgeries,especially in moderate to high residual myopia.
文摘BACKGROUND Geleophysic dysplasia(GD)presents the characterized clinical manifestations of acromelic dysplasia,including extremely short stature,short limbs,small hands and feet,stubby fingers and toes,joint stiffness and others.It is clinically distinct from the other acromelic dysplasia in terms of symptoms such as cardiac valvular abnormalities,progressive hepatomegaly and tracheal stenosis.CASE SUMMARY We report on a Chinese 9-year-old girl with GD with the c.5243G>T(p.C1748F)mutation in FBN1(fibrillin 1,OMIM 134797).She was born in Guangxi Zhuang Autonomous Region of China.The patient presented with typical clinical features of GD and recurrent respiratory tract infections over 6 years.Laboratory studies and chest computed tomography(CT)scan indicated bronchopneumonia.Her echocardiography revealed mild mitral valve thickening with regurgitation.Laryngopharyngeal CT and electronic bronchoscopy revealed severe glottic stenosis.Echocardiography examination displayed mild mitral valve thickening and regurgitation.Ophthalmic examination did not reveal myopia or lens dislocation.Treated with ceftriaxone sodium and methylprednisolone sodium succinate for injection as well as methylprednisolone orally,patient’s symptoms had improved.CONCLUSION GD is a rare genetic condition that can cause life-threatening cardiovascular and respiratory problems.This study also found that the identified genotype of GD could be related to different clinical phenotypes.
基金This work was financially supported by the National Nat-ural Science Foundation for Excellent Youth of China(No.51722401)the State Key Research Development Program of China(No.2016YFC0600704)the Key Program of Na-tional Natural Science Foundation of China(No.51734001).
文摘The chemical binder is one of the critical factors affecting ore agglomeration behavior and leaching efficiency.In this study,we in-vestigated the effect of the type of binder and mass fraction of the H_(2)SO_(4)solution used on the curing,soaking,and leaching behavior of ag-glomerations.The results revealed that Portland cement(3CaO·SiO_(2),2CaO·SiO_(2),and 3CaO·Al_(2)O_(3))was the optimal binder for obtaining a well-shaped,stable agglomeration structure.A higher extraction rate was achieved when using Portland cement than that obtained using sodi-um silicate,gypsum,or acid-proof cement.An excessive geometric mean size is not conducive to obtaining well-shaped agglomerations and desirable porosity.Using computed tomography(CT)and MATLAB,the porosity of two-dimensional CT images in sample concentrations L1-L3 was observed to increase at least 4.5vol%after acid leaching.Ore agglomerations began to be heavily destroyed and even to disinteg-rate when the sulfuric acid solution concentration was higher than 30 g/L,which was caused by the excessive accumulation of reaction products and residuals.
文摘In our published letter,[1]we have identified a minor error in Figs.2 and 3.Instead,we have 2111 events in these two plots with 646,249,382,and 834 events in Run 9(20.0 ton·day),Run 10(19.4 ton·day),Run 11–1(24.2 ton·day),and Run 11–2(37.1 ton·day).The mistake is due to an incorrect application of a small energy non-linearity(known as the BLS non-linearity)in making plots,but has no impact to the final results.Figures 2 and 3 are now updated in this note.
基金financial support from National Natural Science Foundation of China(Grant No.51835004 and 51575197)Huaqiao University International Cultivation Program for Outstanding Postgraduates and Subsidized Projec for Postgraduates’Innovative Fund in Scientific Research of Huaqiao University(No.18011080010)。
文摘Single crystal silicon carbide(SiC)is widely used for optoelectronics applications.Due to the anisotropic characteristics of single crystal materials,the C face and Si face of single crystal SiC have different physical properties,which may fit for particular application purposes.This paper presents an investigation of the material removal and associated subsurface defects in a set of scratching tests on the C face and Si face of 4H-SiC and 6H-SiC materials using molecular dynamics simulations.The investigation reveals that the sample material deformation consists of plastic,amorphous transformations and dislocation slips that may be prone to brittle split.The results showed that the material removal at the C face is more effective with less amorphous deformation than that at the Si face.Such a phenomenon in scratching relates to the dislocations on the basal plane(0001)of the SiC crystal.Subsurface defects were reduced by applying scratching cut depths equal to integer multiples of a half molecular lattice thickness,which formed a foundation for selecting machining control parameters for the best surface quality.
基金financially supported by the Key Program of National Natural Science Foundation of China (Nos. 52034001 and 51734001)the Innovation Team in Key Fields of Ministry of Science and Technology of the People’s Republic of China (No. 2018RA400)+2 种基金the 111 Project (No. B20041)the Fundamental Research Funds for the Central Universities (No. FRF-TP-18-003C1)China Scholarship Council (No. 202006460037)
文摘Effects of residues produced by agricultural wastes fermentation(AWF)on low grade copper sulfide ores bioleaching,copper recovery,and microbial community were investigated.The results indicated that adding appropriate bulk of AWF made contributions to low grade copper sulfide ores bioleaching,which may be mainly realized through reducing the passivation layer formed by Fe3+hydrolysis.Improved copper recovery(78.35%)and bacteria concentration(9.56×10^(7)cells·mL^(−1))were yielded in the presence of 5 g·L^(−1)AWF.The result of 16S rDNA analysis demonstrated that microbial community was differentiated by adding AWF.Bacteria proportion,such as Acidithiobacillus ferrooxidans,Moraxella osloensis,and Lactobacillus acetotolerans changed distinctly.Great difference between samples was showed according to beta diversity index,and the maximum value reached 0.375.Acidithiobacillus ferrooxidans accounted for the highest proportion throughout the bioleaching process,and that of sample in the presence of 5 g·L^(−) AWF reached 28.63%.The results should show reference to application of agricultural wastes and low grade copper sulfide ores.
文摘The conflict between poverty and the resulting over-exploitation of natural resources on the one hand, and ecological restoration and sustainable development on the other hand, in the southwest China karst region was studied. In this region, the karst forest (a mixed evergreen and deciduous broad-leaved forest) is rapidly degrading due to over-exploitation (sloping farming). We suggest that an Ecological Compensation (EC) model should be established with: financial institutions, local people, and a third part as an intermediate link. The process would continue for 20 years. As a case study we used Bangui town (3800 families) in the upper reaches of Pearl River. The per capita income of residents was used as the benchmark. The compensation would start with 80%, and decrease to 20% over a period of 20 years. Infrastructure investment would decrease from 20% of the total person’s compensation to 5% as the farmers increasingly use alternative income sources. The EC includes compensation for individual, infrastructure, and environmental investments. The total EC for Bangui would be 305,064 × 104 yuan during the 20 years.
文摘In this paper, a Multi Modal Reasoning (MMR) method integrated with probabilistic reasoning is proposed for the diagnosis support module of the open eHealth platform. MMR is based on both Rule Based Reasoning (RBR) and Case Based Reasoning (CBR). It is not only applied to the identification of diseases and syndromes based on medical guidelines, but also deals with exceptional cases and individual therapies in order to improve diagnostic accuracy. Moreover, a new rule expression frame is introduced to deal with uncertainty, which can represent and process vague, imprecise, and incomplete information. Furthermore, this system is capable of updating the attributes of rules and inducing rules with a small data sample. Keywords Information management - uncertainty - decision making Jia Tian received her BSc degree from China University of Mining and Technology (CUMT), Beijing, China in 2002. She is currently pursuing her PhD at the University of Nottingham, United Kingdom. Her research interests include expert system, artificial intelligence and data mining, especially Medical Decision Support System, Case Based Reasoning, Rule Based Reasoning and Bayesian Networks.Xun Chen obtained his B. Eng. from Fuzhou University. He received his M. Sc. from Zhejiang University and his Ph. D from Liverpool John Moores University. He has been a visiting professor to Fuzhou University since 2001. He specialises in advanced manufacturing technology including application of computer science, mechatronics and artificial intelligence to broad engineering application. He has published more than 100 research papers. Dr Chen is a founder member of the International Committee of Abrasive Technology. Before his employment at Nottingham, Dr Chen was a lecturer of Mechanical Engineering at the University of Dundee. Prior to that, he was a research fellow, a Royal Society Royal Fellow at Liverpool John Moores University and a lecturer at Fuzhou University.Sheng-Ping Dong graduated from China University of Traditional Chinese Medicine, Beijing, China. She is an attending doctor of Traditional Chinese Medicine with many years clinical experience in Exhibition Road Hospital, Beijing, China.
