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Clinical and molecular characterization of 10 Chinese children with congenital adrenal hyperplasia due to 11beta‑hydroxylase deficiency

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World Journal of Pediatrics 2024年第4期20卷 422-433页

作者： Wen‑Li Lu Xiao‑Yu Ma Jiao Zhang Jun‑Qi Wang Ting‑Ting Zhang Lei ye Yuan Xiao Zhi‑Ya Dong Wei Wang Shou‑Yue Sun Chuan‑Yin Li Rong‑Gui Hu guang Ning Li‑Dan Zhang Department of Pediatrics Ruijin HospitalShanghai Jiao Tong University School of MedicineNo.197Ruijin 2nd RdHuangpu DistrictShanghai 200025China Department of Pediatric Genetic and Metabolic Endocrinology West China Second University HospitalSichuan UniversityNo.20Section 3Renmin South RoadSichuan 610041China Department of Endocrine and Metabolic Diseases Shanghai Clinical Center for Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Cancer Center School of MedicineShanghai Tenth People’s HospitalTongji UniversityYanchang Zhong Lu 301St RdJing’an DistrictShanghai 200031China

Background The clinical manifestations of nonclassical 11beta-hydroxylase deficiency are very similar to those of nonclassical 21-hydroxylase *** this study,we investigated the relationship between the clinical and molecular features of congenital adrenal hyperplasia caused by 11beta-hydroxylase deficiency and reviewed the related literature,which are expected to provide assistance for the clinical diagnosis and analysis of congenital adrenal *** Clinical data for 10 Chinese patients diagnosed with congenital adrenal hyperplasia in our hospital from 2018 to 2022 were retrospectively *** examined the effects of gene mutations on protease activity and constructed threedimensional structure prediction models of *** We describe 10 patients with 11beta-hydroxylase gene mutations(n=5,46,XY;n=5,46,XX),with 10 novel mutations were *** patients received treatment at an early stage,with an average age of 2.08±1.66 years,whereas male patients received treatment significantly later,at an average age of 9.77±3.62 *** most common CYP11B1 pathogenic variant in the Chinese population was found to be c.1360C>*** mutations lead to spatial conformational changes that affect protein *** Our study found that there was no significant correlation between each specific mutation and the severity of clinical *** patients with the same gene pathogenic variant may have mild or severe clinical *** correlation between genotype and phenotype needs further ***-dimensional protein simulations may provide additional support for the physiopathological mechanism of genetic mutations.

关键词： 11β-hydroxylase deficiency CYP11B1 Enzymatic activity Phenotype-genotype correlation Threedimensional protein simulations

Enhancing pediatric asthma management through multifaceted health education

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World Journal of Clinical Cases 2024年第19期12卷 3708-3716页

作者： guang-Qun Lin Zu-Qiong ye En-Lian Song Yu-Nan Lin Department of Pediatrics First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina Day Surgery Ward First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina Anesthesia&Operation Center First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina

BACKGROUND Pediatric asthma is a significant public health issue that impacts the quality of life of children *** management approaches focus on symptom control and medication adherence but often overlook the comprehensive educational needs of patients and their families.A multifaceted health education approach may offer a more holistic strategy in managing pediatric asthma,especially in outpatient *** To evaluate the efficacy of a comprehensive health education strategy in improving disease management,medication adherence,and quality of life among children with asthma in outpatient *** In total,100 pediatric patients with severe asthma were enrolled from January 2021 to November 2022 and randomly allocated to a control group(n=50)or an observation group(n=50).The control group received standard nursing care,including basic nursing interventions and health education upon *** contrast,the observation group was exposed to a broad spectrum of health education methodologies,including internet-based hospital systems,social media channels,one-on-one verbal education,informational brochures,slide present ations,telephone check-ins,animated videos,and illustrated health education *** on asthma management knowledge,symptom control,quality of life[***’s Respiratory Questionnaire(SGRQ)],treatment adherence,and nursing satisfaction were collected and *** The scores of the observation group in knowledge areas,such as medication,home care,disease understanding,symptom management,prevention strategies,and nutritional guidance,were significantly higher than those of the control group(P<0.05).In addition,the observation group exhibited greater symptom control,improved quality of life based on their SGRQ scores,and higher treatment adherence post-intervention(P<0.05).Nursing satisfaction was also rated higher in the observation group across all evaluated areas(P<0.05).CONCLUSION Implementing a diversified hea

关键词： Multifaceted health education mode Pediatrics Outpatient service Bronchial asthma

Using choroidal thickness to detect myopic macular degeneration

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International Journal of Ophthalmology(English edition) 2024年第2期17卷 317-323页

作者： Ran Liu Meng Xuan De-Cai Wang Ou Xiao Xin-xing Guo Jian Zhang Wei Wang Monica Jong Padmaja Sankaridurg Kyoko Ohno-Matsui Qiu-Xia Yin Ming-guang He Zhi-Xi Li State Key Laboratory of Ophthalmology Zhongshan Ophthalmic CenterSun Yat-sen UniversityGuangdong Provincial Key Laboratory of Ophthalmology and Visual ScienceGuangdong Provincial Clinical Research Center for Ocular DiseasesGuangzhou 510060Guangdong ProvinceChina New England College of Optometry BostonMassachusetts 02115USA Wilmer Eye Institute Johns Hopkins UniversityBaltimoreMD 21231USA Brien Holden Vision Institute Sydney 2052Australia School of Optometry and Vision Science University of New South WalesSydney 2052Australia Department of Ophthalmology and Visual Science Tokyo Medical and Dental UniversityTokyo 113-8510Japan School of Optometry the Hong Kong Polytechnic UniversityHong Kong SARChina Research Centre for SHARP Vision the Hong Kong Polytechnic UniversityHong Kong SARChina Center for Eye and Vision Research(CEVR) 17W Hong Kong Science ParkHong Kong SARChina

AIM:To explore the usage of choroidal thickness measured by swept-source optical coherence tomography(SS-OCT)to detect myopic macular degeneration(MMD)in high myopic ***:Participants with bilateral high myopia(≤−6 diopters)were recruited from a subset of the guangzhou Zhongshan Ophthalmic Center-Brien Holden Vision Institute High Myopia Cohort ***-OCT was performed to determine the choroidal thickness,and myopic maculopathy was graded by the International Meta-Analysis for Pathologic Myopia(META-PM)Classiﬁ*** of MMD was defined as META-PM category 2 or ***:A total of 568 right eyes were included for *** with MMD(n=106,18.7%)were found to have older age,longer axial lengths(AL),higher myopic spherical equivalents(SE),and reduced choroidal thickness in each Early Treatment Diabetic Retinopathy Study(ETDRS)grid sector(P<0.001).The area under the receiver operating characteristic(ROC)curves(AUC)for subfoveal choroidal thickness(0.907)was greater than that of the model,including age,AL,and SE at 0.6249,0.8208,and 0.8205,*** choroidal thickness of the inner and outer nasal sectors was the most accurate indicator of MMD(AUC of 0.928 and 0.923,respectively).An outer nasal sector choroidal thickness of less than 74μm demonstrated the highest odds of predicting MMD(OR=33.8).CONCLUSION:Choroidal thickness detects the presence of MMD with high agreement,particularly of the inner and outer nasal sectors of the posterior pole,which appears to be a biometric parameter more precise than age,AL,or SE.

关键词： high myopia choroidal thickness myopic macular degeneration swept-source optical coherence tomography

A shape-reconfigurable,light and magnetic dual-responsive shape-memory micropillar array chip for droplet manipulation

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Chinese Chemical Letters 2024年第1期35卷 456-460页

作者： Wen-Qi ye Wen-Xin Fu Xiao-Peng Liu Chun-guang Yang Zhang-Run Xu Research Center for Analytical Sciences Northeastern UniversityShenyang 110819China

Droplet manipulation on an open surface has great potential in chemical analysis and biomedicine ***,most of the reported platforms designed for the manipulation of water droplets cannot thoroughly solve the problem of droplet ***,we report a shape-reconfigurable micropillar array chip for the manipulation of water droplets,oil droplets and water-in-oil ***-in-oil droplets provide an enclosed space for water droplets,preventing the evaporation in an open *** coated on the surface of the chip effectively reduces the droplet movement *** micropillar array chip has light and magnetic dual-response due to the Fe3O4 nanoparticles and the reduced iron powder mixed in the shape-memory *** micropillars irradiated by a near-infrared laser bend under the magnetic force,while the unirradiated micropillars still keep their original *** the absence of a magnetic field,when the micropillars in a temporary shape are irradiated by the near-infrared laser to the transition temperature,the micropillars return to their initial *** this process,the surface morphology gradient caused by the deformation of the micropillars and the surface tension gradient caused by the temperature change jointly produce the driving force of droplet movement.

关键词： Microfluidics Droplet manipulation Micropillar array Shape memory polymer Magnetic response Light response

Regeneration of photovoltaic industry silicon waste toward high-performance lithium-ion battery anode

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Rare Metals 2024年第10期43卷 4948-4960页

作者： Kai Wang Xiao-Bin Zhong Yue-Xian Song Yao-Hui Zhang Yan-Gang Zhang Xiao-Gang You Pu-guang Ji Kurbanov Mirtemir Shodievich Umedjon Khalilov Gong-Kai Wang Xin Zhang xing-Liang Yao Feng Li Jun-Fei Liang Hua Wang School of Energy and Power Engineering North University of China Zhongyuan Critical Metals Laboratory Zhengzhou University Tianjin Key Laboratory of Materials Laminating Fabrication and Interface Control Technology School of Material Science and EngineeringHebei University of Technology Arifov Institute of Ion-Plasma and Laser Technologies Academy of Sciences of the Republic of Uzbekistan New Uzbekistan University Arifov Institute of Ion-Plasma and Laser Technologies Academy of Sciences of Uzbekistan University of Antwerp School of Chemistry Beihang University

The diamond-wire sawing silicon waste（DWSSW） from the photovoltaic industry has been widely considered as a low-cost raw material for lithium-ion battery silicon-based electrode,but the effect mechanism of impurities presents in DWSSW on lithium storage performance is still not well understood;meanwhile,it is urgent to develop a strategy for changing DWSSW particles into high-performance electrode *** this work,the occurrence state of impurities presents in DWSSW was carefully analyzed using in situ Ar ion etching technology Then,the novel Si@C@SiOx@PAl-NDC composite was designed through in situ encapsulation *** obtained Si@C@SiOx@PAl-NDC electrode shows a high first capacity of 2343.4 mAh·g-1with an initial Coulombic efficiency （ICE） of 84.4%under current density of 1.0 A·g-1,and can deliver an impressive capacity of 984.9 mAh·g-1after 200 *** numerical simulation modeling calculations,the increase in proportion of Si4+/Si0and Si3+/Si0valence states in SiOxlayer leads to a decrease in von Mises stress,which ultimately improves the cycling structural ***,the porous 2D-3D aluminum/nitrogen （Al/N） co-doped carbon layer and nanowires on SiOxlayer can provide abundant active sites for lithium storage due to its developed hierarchical pores structure,which facilitates ion transport What is more,the performance of Si@C@SiOx@PAl-NDC//LiFePO4full cell shows its great potential in practical application.

关键词： Aluminum/nitrogen co-doped In situ encapsulation Lithium-ion batteries Porous carbon nanowires Sawing silicon waste

Improvement and security analysis of multi-ring discrete modulation continuous variable quantum secret sharing scheme

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Chinese Physics B 2024年第7期33卷 189-197页

作者：姜欢窈聂敏杨光孙爱晶张美玲裴昌幸 School of Communications and Information Engineering&School of Artificial Intelligence Xi'an University of Posts and TelecommunicationsXi'an 710121China State Key Laboratory of Integrated Service Networks Xidian UniversityXi'an 710071China

In order to avoid the complexity of Gaussian modulation and the problem that the traditional point-to-point communication DM-CVQKD protocol cannot meet the demand for multi-user key sharing at the same time, we propose a multi-ring discrete modulation continuous variable quantum key sharing scheme(MR-DM-CVQSS). In this paper, we primarily compare single-ring and multi-ring M-symbol amplitude and phase-shift keying modulations. We analyze their asymptotic key rates against collective attacks and consider the security key rates under finite-size effects. Leveraging the characteristics of discrete modulation, we improve the quantum secret sharing scheme. Non-dealer participants only require simple phase shifters to complete quantum secret sharing. We also provide the general design of the MR-DM-CVQSS *** conduct a comprehensive analysis of the improved protocol's performance, confirming that the enhancement through multi-ring M-PSK allows for longer-distance quantum key distribution. Additionally, it reduces the deployment complexity of the system, thereby increasing the practical value.

关键词： discrete modulation continuous variable quantum secret sharing scheme

A discussion on the anomalous threshold enhancement of J/ψ–ψ(3770)couplings and X(6900)peak

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Chinese Physics C 2024年第4期48卷 1-5页

作者：陆业陈畅秦广友郑汉青 Institute of Particle Physics and Key Laboratory of Quark and Lepton Physics(MOE) Central China Normal UniversityWuhan 430079China Department of Physics Peking UniversityBeijing 100871China College of Physics Sichuan UniversityChengdu 610065China

The attractive interaction between J/ψandψ(3770)has to be strong enough if X(6900)is of the molecule *** argue that sinceψ(3770)decays predominantly into a DD^(¯)pair,the interactions between J/ψandψ(3770)may be significantly enhanced owing to the three point DD^(¯)loop *** enhancement originates from the anomalous threshold located at t=−1.288 GeV^(2),whose effect propagates into the s-channel partial wave amplitude in the vicinity of √s≃6.94 *** effect may be helpful in the formation of the X(6900)peak.

关键词： exotic hadrons hadron molecule X(6900)

Aurora B promotes the CENP-T–CENP-W interaction to guide accurate chromosome segregation in mitosis

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Journal of Molecular Cell Biology 2024年第2期16卷 11-23页

作者： Wei Liu Zhen Dou Chunyue Wang Gangyin Zhao Fengge Wu Chunli Wang Felix Aikhionbare Mingliang ye Divine Mensah Sedzro Zhenye Yang Chuanhai Fu Zhikai Wang Xinjiao Gao Xuebiao Yao Xiaoyu Song xing Liu MOE Key Laboratory for Cellular Dynamics Center for Advanced Interdisciplinary Science and Biomedicine of IHMUniversity of Science and Technology of China School of Life SciencesHefei 230027China Keck Center for Cellular Dynamics and Organoids Plasticity AtlantaGA 30310USA Anhui Key Laboratory for Cellular Dynamics and Chemical Biology Hefei National Research Center for Interdisciplinary Sciences at the MicroscaleHefei 230026China National Chromatographic Research and Analysis Center Chinese Academy of SciencesDalian 116023China

Accurate chromosome segregation in mitosis depends on kinetochores that connect centromeric chromatin to spindle *** are captured by individual microtubules via a kinetochore constitutive centromere-associated network(CCAN)during chromosome *** contains 16 subunits,including CENP-W and ***,the molecular recognition and mitotic regulation of the CCAN assembly remain ***,we revealed that CENP-W binds to the histone fold domain and an uncharacterized N-terminal region of *** B phosphorylates CENP-W at threonine 60,which enhances the interaction between CENP-W and CENP-T to ensure robust metaphase chromosome alignment and accurate chromosome segregation in *** findings delineate a conserved signaling cascade that integrates protein phosphorylation with CCAN integrity for the maintenance of genomic stability.

关键词： mitosis kinetochore Aurora B CENP-T CENP-W phosphorylation

Vibration reduction effect of the shielding pile based on seismic wave attenuation in shallow underground spaces

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Journal of Earth Science 2024年

作者： Qiankuan Wang Shili Qiu Aiguo xing Xueyong Xu ye Zhou Yao Cheng Haoshan Zhang Bo Wu Yiwei Liu Mingzhe Zhang Yu Zhuang State Key Laboratory of Ocean Engineering， Shanghai Jiao Tong University State Key Laboratory of Geomechanics and Geotechnical Engineering， Institute of Rock and Soil Mechanics， Chinese Academy of Sciences North Information Control Research Academy Group CO.， LTD Faculty of Engineering， China University of Geosciences

Seismic hazards threaten underground structures and subsequently affect surface buildings， making effective vibration reduction measures crucial in shallow underground spaces. This paper proposes a multi-layer shielding pile based on the seismic wave attenuation theory of refraction dispersion and diffraction loss as a new vibration reduction method. By using the dynamic wave interaction between the unique multi-layer structure of the shielding pile and the seismic wave， a shielding area with significant attenuation of the ground motion is formed to achieve the purpose of vibration reduction. The spectral element method (SEM) with high-order source time function and convolution perfect matching layer (PML) is used to simulate the numerical models of shielding piles with different parameters (including background models and excavation models)， and the characteristics of ground motion and site effect near shielding piles in various models are analyzed. The study reveals the control effect and regulation of the material， size， and shape of the shielding pile on its vibration reduction effect， and determines the shielding pile parameters with the best vibration reduction effect. Shielding piles enriches the vibration reduction measures for structures in shallow underground spaces， and provides a new method for preventing seismic hazards under dynamic load.

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