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Clinical Analysis and Mental Health Survey of Hemophilia Carriers:a Cross-sectional Study

Current Medical Science 2024年第2期44卷 435-440页

作者： Wen WANG Li-juan JIANG Dong-yan CUI Ai ZHANG Xiong WANG Ai-guo LIU qun HU Department of Pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China Department of Laboratory Medicine Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China

Objective:Hemophilia carriers(HCs),who are heterozygous for mutations in the clotting factor VIII/clotting factor IX gene(F8 or F9),may have a wide range of clotting factor levels,from very low,similar to afflicted males,to the upper limit of normal,and may experience mental health *** purpose of this study was to provide genetic information on mothers of hemophilia patients and to understand the clotting factor activity and phenotype of ***,we aimed to investigate the mental health status of HCs in ***:A total of 127 hemophilia mothers,including 93 hemophilia A(HA)mothers and 34 hemophilia B(HB)mothers,were enrolled in this *** distance PCR,multiplex PCR,and Sanger sequencing were used to analyze mutations in F8 or *** factor activity was detected by a one-stage clotting *** Symptom Checklist 90(SCL-90,China/Mandarin version)was given to HCs at the same time to assess their mental ***:A total of 90.6%of hemophilia mothers were diagnosed genetically as carriers,with inversion in intron 22 and missense mutations being the most common mutation types in HA and HB carriers,*** median clotting factor level in carriers was 0.74 IU/mL(ranging from 0.09 to 1.74 IU/mL)compared with 1.49 IU/mL(ranging from 0.93 to 1.89 IU/mL)in noncarriers,of which 14.3%of HCs had clotting factor levels of 0.40 IU/mL or below.A total of 53.8%(7/13)of HA carriers with low clotting factor levels(less than 0.50 IU/mL)had a history of bleeding,while none of the HB carriers displayed a bleeding *** total mean score and the global severity index of the SCL-90 for surveyed HCs were 171.00(±60.37)and 1.78(±0.59),respectively.A total of 67.7%of the respondents had psychological symptoms,with obsessive-compulsive disorder being the most prevalent and *** pooled estimates of all nine factors were significantly higher than those in the general population(P<0.05).Conclusions:The detection rate of gene mutations

关键词： hemophilia A hemophilia B mutation carrier mental health

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Double-confined nanoheterostructure Sb/Sb_(2)S_(3)@Ti_(3)C_(2)T_(x)@C toward ultra-stable Li-/Na-ion batteries

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Rare Metals 2024年第5期43卷 2067-2079页

作者： Dan Wang qun Ma Huan He Zhi-Yuan Wang Run-Guo Zheng Hong-Yu Sun yan-Guo Liu Chun-Li Liu School of Materials Science and Engineering Northeastern UniversityShenyang110819China School of Resources and Materials Northeastern University at QinhuangdaoQinhuangdao 066004China Key Laboratory of Dielectric and Electrolyte Functional Material Hebei Province Qinhuangdao 066004China Department of Physics and Oxide Research Center Hankuk University of Foreign StudiesYongin 17035Korea

Antimony-based materials with high capacities and moderate potentials are promising anodes for lithium-/-sodium-ion ***,their tremendous volume expansion and inferior conductivity lead to poor structural stability and sluggish reaction ***,a doubleconfined nanoheterostructure Sb/Sb_(2)S_(3)@Ti_(3)C_(2)T_(x)@C has been fabricated through a solvothermal method followed by low-temperature heat *** dual protection of“MXene”and“carbon”can better accommodate the volume expansion of Sb/Sb_(2)S_(3).The strong covalent bond(Ti-S,Ti-O-Sb,C-O-Sb)can firmly integrate Sb-based material with Ti_(3)C_(2)T_(x)and carbon,which significantly improves the structure *** addition,the carbon layer can restrain the oxidation of MXenes,and the nano-Sb/Sb_(2)S_(3)can facilitate electron/ion transport and suppress the restacking of *** heterogeneous interface between Sb and Sb_(2)S_(3)can further promote interfacial charge *** MXene-Sb/Sb_(2)S_(3)@C-1 with the optimal Sb content shows high specific capacities,comparable rate properties and ultra-stable cycling performances(250 m Ah·g^(-1)after 2500 cycles at 1 A·g^(-1)for sodium-ion batteries).Ex situ X-ray diffractometer(XRD)test reveals the storage mechanism including the conversion and alloying process of MXene-Sb/Sb_(2)S_(3)@*** voltammetry(CV)test results demonstrate that the pseudocapacitance behavior is dominant in MXene-Sb/Sb_(2)S_(3)@C-1,especially at large *** design paves the way for exploring high-performance alloy-based/conversion-type anode for energy storage devices.

关键词： Sb/Sb_(2)S_(3) MXene Carbon Anode Lithium-ion batteries Sodium-ion batteries

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Nickel-catalyzed carbonylative four-component 1,4-dicarbofunctionalization of 1,3-enynes

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Science China Chemistry 2024年第6期67卷 2029-2038页

作者： yan Zhao Qi-chao Shan Hong Xin Pin Gao Xu yang Xin-Hua Duan Li-Na Guo Department of Chemistry School of ChemistryXi’an Key Laboratory of Sustainable Energy Material Chemistry and Engineering Research Center of Energy StorageMaterials and DevicesMinistry of EducationXi’an Jiaotong UniversityXi’an710049China School of Electrical Engineering Xi’an Jiaotong UniversityXi’an710049China

An efficient Ni-catalyzed four-component 1,4-carbocarbonylation of 1,3-enynes with activated alkyl halides and arylboronic acids under atmospheric pressure of CO is *** tuning the electronic and steric effects of alkyl radicals,both electronrich and electron-deficient 1,3-enynes were compatible with this *** protocol features mild conditions,broad substrate scope,excellent functional group compatibility and facile gram-scale synthesis,providing a practical approach to the quaternary carbon center-containing allenyl *** study revealed that the acyl-NiIIspecies plays an important role in both the coupling and the alkyl radical generation processes.

关键词： Ni catalysis four-component 1 4-carbocarbonylation 1 3-enynes

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Effects of Lactobacillus paracasei N1115 on gut microbial imbalance and liver function in patients with hepatitis B-related cirrhosis

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World Journal of Gastroenterology 2024年第11期30卷 1556-1571页

作者： yan-chao Hu Xiang-Chun Ding Hui-Juan Liu Wan-Long Ma Xue-yan Feng Li-Na Ma Department of Infectious Disease General Hospital of Ningxia Medical UniversityYinchuan 750004Ningxia Hui Autonomous RegionChina Infectious Disease Clinical Research Center of Ningxia Yinchuan 750004Ningxia Hui Autonomous RegionChina

BACKGROUND Hepatitis B cirrhosis(HBC)is a chronic disease characterized by irreversible diffuse liver damage and aggravated by intestinal microbial imbalance and metabolic *** the relationship between certain single probiotics and HBC has been explored,the impact of the complex ready-to-eat Lactobacillus paracasei N1115(LP N1115)supplement on patients with HBC has not been *** To compare the changes in the microbiota,inflammatory factor levels,and liver function before and after probiotic treatment in HBC *** This study included 160 HBC patients diagnosed at the General Hospital of Ningxia Medical University between October 2018 and December *** were randomly divided into an intervention group that received LP N1115 supplementation and routine treatment and a control group that received routine treatment *** samples were collected at the onset and conclusion of the 12-wk intervention *** structure of the intestinal microbiota and the levels of serological indicators,such as liver function and inflammatory factors,were *** Following LP N1115 intervention,the intestinal microbial diversity significantly increased in the intervention group(P<0.05),and the structure of the intestinal microbiota was characterized by an increase in the proportions of probiotic microbes and a reduction in harmful ***,the intervention group demonstrated notable improvements in liver function indices and significantly lower levels of inflammatory factors(P<0.05).CONCLUSION LP N1115 is a promising treatment for ameliorating intestinal microbial imbalance in HBC patients by modulating the structure of the intestinal microbiota,improving liver function,and reducing inflammatory factor levels.

关键词： Hepatitis B cirrhosis N1115 ready-to-eat lactobacillus Inflammation Liver function Lachnospiraceae incertae sedis Probiotic

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Mismatch analysis of all-fiber coherent beam combiners based on the self-imaging effect

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High Power Laser Science and Engineering 2024年第2期12卷 1-15页

作者： Yuefang yan Yu Liu Haoyu Zhang Yuwei Li chao Guo Qiang Shu Wenhui Huang Feng Jing Rumao Tao Laser Fusion Research Center China Academy of Engineering PhysicsMianyangChina Key Laboratory of Particle&Radiation Imaging(Tsinghua University) Ministry of EducationBeijingChina

All-fiber coherent beam combiners based on the self-imaging effect can achieve a near-perfect single laser beam,which can provide a promising way to overcome the power limitation of a single-fiber *** of the key points is combining efficiency,which is determined by various mismatches during fabrication.A theoretical model has been built,and the mismatch error is analyzed numerically for the first *** mismatch errors have been numerically studied with the beam quality and combining efficiency being chosen as the evaluation *** tolerance of each mismatch error for causing 1%loss is calculated to guide the design of the beam *** simulation results are consistent with the experimental results,which show that the mismatch error of the square-core fiber is the main cause of the efficiency *** results can provide useful guidance for the fabrication of all-fiber coherent beam combiners.

关键词： all-fiber coherent beam combination mismatch analysis self-imaging

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Plutonium utilization in a small modular molten-salt reactor based on a batch fuel reprocessing scheme

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Nuclear Science and Techniques 2024年第4期35卷 15-28页

作者： Xue-chao Zhao Rui yan Gui-Feng Zhu Ya-Fen Liu Jian Guo Xiang-Zhou Cai yang Zou Shanghai Institute of Applied Physics Chinese Academy of SciencesShanghai 201800China CAS Innovative Academies in TMSR Energy System Chinese Academy of SciencesShanghai 201800China University of Chinese Academy of Sciences Beijing 100049China

A molten salt reactor(MSR)has outstanding features considering the application of thorium fuel,inherent safety,sustainability,and resistance to ***,fissile material^(233)U is significantly rare at the current stage,thus it is difficult for MSR to achieve a pure thorium-uranium fuel ***,using plutonium or enriched uranium as the initial fuel for MSR is more *** this study,we aim to verify the feasibility of a small modular MSR that utilizes plutonium as the starting fuel(SM-MSR-Pu),and highlight its advantages and ***,the structural design and fuel management scheme of the SM-MSR-Pu were ***,the neutronic characteristics,such as the graphite-irradiation lifetime,burn-up performance,and coefficient of temperature reactivity were calculated to analyze the physical characteristics of the *** results indicate that plutonium is a feasible and advantageous starting fuel for a SM-MSR;however,there are certain shortcomings that need to be *** a 250 MWth SM-MSR-Pu,approximately 288.64 kg^(233)U of plutonium with a purity of greater than 90% is produced while 978.00 kg is burned every ten *** temperature reactivity coefficient decreases from -4.0 to -6.5 pcm K^(-1) over the 50-year operating time,which ensures a long-term safe ***,the amount of plutonium and accumulation of minor actinides(MAs)would increase as the burn-up time increases,and the annual production and purity of^(233)U will *** achieve an optimal burn-up performance,setting the entire operation time to 30 years is ***,more than 3600 kg of plutonium eventually accumulate in the *** research is required to effectively utilize this accumulated plutonium.

关键词： Molten salt fuel Plutonium utilization ^(233)U TRUs mole fraction Temperature feedback coefficient

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Establishment of an Iron Deficiency Model by Iron Depletion in Pregnant Rats

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Biomedical and Environmental Sciences 2024年第2期37卷 210-215页

作者： CHEN Xi HAN chao ZHAO Jin Peng SHEN Shi WANG Li Yuan REN Shou WANG Tong Lei MA yan XU Ze chao HUO Jun Sheng Key Laboratory of Trace Element Nutrition National Health Commission of the People’s Republic of ChinaNational Institute for Nutrition and HealthChinese Center for Disease Control and PreventionBeijing 100050China

Anemia,a global public health problem,has significant adverse consequences on the cognitive development of children and the work capacity of adults;affecting social and economic ***,roughly 43%of children under five years of age,38%of pregnant women,and 29%of nonpregnant women had *** during pregnancy significantly increases the risk of low birth weight and preterm birth.A 10 g/L increase in hemoglobin has been estimated to decrease the risk of maternal and perinatal mortality by 29%and 28%.

关键词： mortality pregnancy anemia

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Data-driven mapping-relationship mining between hardness and mechanical properties of dual-phase titanium alloys via random forest and statistical analysis

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Rare Metals 2024年第2期43卷 829-841页

作者： Hai-chao Gong qun-Bo Fan Hong-Mei Zhang Xing-Wang Cheng Wen-Qiang Xie Kai Chen Lin yang Jun-Jie Zhang Bing-Qiang Wei Shun Xu School of Materials Science and Engineering Beijing Institute of TechnologyBeijing100081China National Key Laboratory of Science and Technology on Materials Under Shock and Impact Beijing100081China Beijing Institute of Technology Chongqing Innovation Center Chongqing401135China Department of Mechanical and Materials Engineering University of Nebraska-LincolnLincolnNE68588USA

In order to accelerate the research on the property optimization of titanium alloy based on high-throughput methods,it is necessary to reveal the relationship between hardness and other mechanical properties which is still *** this work,taking Ti20C alloy as research object,almost all the microstructure of dual-phase titanium alloys were covered by traversing over 100 heat treatment ***,massive experiments including microstructure characterization and performance test were conducted,obtaining 51,590 pieces of microstructure data and 3591 pieces of mechanical property ***,based on large-scale data-driven technology,the quantitative mapping relationship between hardness and other mechanical properties was deeply *** results of random forest models showed that the correlation between hardness(H)and Charpy impact energy(A_(k))(or elongation,A)was hardly dependent on the microstructure types,while the relationship between H and tensile strength(R_(m))(or yield strength,R_(p0.2))was highly dependent on microstructure ***,combined with statistical analysis,it was found that the relationship between H and Ak(or A)were negatively ***,the relationship between H and strength was positively linear for equiaxed microstructure,and strength was linked to d^(−1/2)(d,equivalent circle diameter)ofα-grains in the form of classical Hall–Petch formula;but for other microstructures,the relationships were ***,the above rules were nearly the same in the rolling direction and transverse ***,a"four-quadrant partition map"between H and R_(p0.2)/R_(m) was established as a versatile material-screening tool,which can provide guidance for on-demand selection of titanium alloys.

关键词： Dual-phase titanium alloy Data-driven Hardness Mapping relationship

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Quasi-in-situ Observation and SKPFM Studies on Phosphate Protective Film and Surface Micro-Galvanic Corrosion in Biological Mg-3Zn-xNd Alloys

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Acta Metallurgica Sinica(English Letters) 2024年第4期37卷 648-664页

作者： Zhaochen Yu Kaixuan Feng Shuyun Deng yang Chen Hong yan Honggun Song chao Luo Zhi Hu School of Advanced Manufacturing Nanchang UniversityNanchang330031China Institute for Advanced Study Nanchang UniversityNanchang330031China

The phosphate protective film and micro-galvanic corrosion of biological Mg-3Zn-xNd (x = 0, 0.6, 1.2) alloys were investigated by scanning and transmission electron microscopy, quasi-in-situ observation, scanning Kelvin probe force microscopy (SKPFM) and electrochemical tests. The results revealed the Mg-Zn-Nd phases formed in Mg-3Zn alloy contained with Nd. Adding Nd resulted in a significant decline in the cracks of the phosphate protective film and micro-galvanic corrosion of alloys, which were recorded by quasi-in-situ observation. In addition, the Volta potential difference of Mg-Zn-Nd/α-Mg (~ 188 mV) was lower than MgZn/α-Mg (~ 419 mV) and Zn-rich/α-Mg (~ 260 mV), and the corrosion rates of alloys markedly decreased after the addition of 0.6 wt% Nd. The improvement in corrosion resistance of Nd-containing alloys was mainly attributed to the following: (i) the addition of Nd reduced the Volta potential difference (second phases/α-Mg);(ii) the phosphate protective film containing Nd_(2)O_(3) deposited on the surface of the alloys, effectively preventing the penetration of harmful anions.

关键词： Magnesium alloys Quasi-in-situ observation Phosphate protective film Scanning Kelvin probe force microscopy(SKPFM) Micro-galvanic corrosion

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New recessive compound heterozygous variants of RP1L1 in RP1L1 maculopathy

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International Journal of Ophthalmology(English edition) 2024年第1期17卷 107-112页

作者： Wen-chao Cao Qing-Shan Chen Run Gan Tao Huang Xiao-He yan The Second Clinical Medical College Jinan UniversityShenzhen 518040Guangdong ProvinceChina Shenzhen Eye Hospital Jinan UniversityShenzhen Eye InstituteShenzhen 518040Guangdong ProvinceChin

AIM:To identify a maculopathy patient caused by new recessive compound heterozygous variants in ***:Comprehensive retinal morphological and functional examinations were evaluated for the patient with RP1L1 *** sequence capture array technique was used to screen potential pathologic *** chain reaction and Sanger sequencing were used to confirm the screening ***:Fundus examination showed round macular lesions appeared in both *** coherence tomography showed that the inner segment/outer segment continuity was disorganized and disruptive in the left eye,but it was uneven and slightly elevated in the right *** autofluorescence showed patchy hyper-autofluorescence in the *** field examination indicates central defects in both ***(ERG)and multifocal ERG showed no obvious *** fluorescein angiography in the macula showed obviously irregular hyper-fluorescence in the right eye and slightly hyper-fluorescence in the left *** found that the proband carried a missense variant(c.1972C>T)and a deletion variant(c.4717_4718del)of RP1L1,which were originated from the parents and formed compound heterozygous *** variants are likely pathogenic according to the ACMG *** imaging,ERG and detailed medical history are important diagnostic tools for differentiating between acquired and inherited retinal ***:A maculopathy case with detailed retinal phenotype and new recessive compound heterozygous variants of RP1L1 is identified in a Chinese family,which expands the understanding of phenotype and genotype in RP1L1 maculopathy.

关键词： maculopathy recessive compound heterozygous variants RP1L1

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