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Novel compound heterozygous mutations in the hemojuvelin gene in a juvenile hemochromatosis patient:A case report

World Journal of Clinical Cases 2024年第19期12卷 3961-3970页

作者： Ling-Ding Xie Xiao-Mu Kong Jing-Xia Shen Tai-Ling Wang Jing Ma yun-fen zhang Xiao-Ping Chen Department of Endocrinology China-Japan Friendship HospitalBeijing 100029China Department of Tumor Radiotherapy and Chemotherapy Qian’an People’s HospitalQian’an 064400Hebei ProvinceChina Department of Pathology China-Japan Friendship HospitalBeijing 100029China Department of Endocrinology Aksu First People's HospitalAksu Region 843000Xinjiang Uygur Autonomous RegionChina Department of Metabolic Diseases Qian’an Traditional Chinese Medical HospitalQian’an 064400Hebei ProvinceChina

BACKGROUND Juvenile hemochromatosis(JH)is an early-onset,rare autosomal recessive disorder of iron overload observed worldwide that leads to damage in multiple *** mutations in the hemojuvelin(HJV)gene are the major cause of *** SUMMARY A 34-year-old male Chinese patient presented with liver fibrosis,diabetes,hypogonadotropic hypogonadism,hypophysis hypothyroidism,and skin *** test revealed a markedly elevated serum ferritin level of 4329μg/L and a transferrin saturation rate of 95.4%.Targeted exome sequencing and Sanger sequencing revealed that the proband had a novel mutation c.863G>A(p.R288Q)in the HJV gene which was transmitted from his father,and two known mutations,c.18G>C(p.Q6H)and c.962_963delGCinsAA(p.C321*)in cis,which were inherited from his *** p.R288W mutation was previously reported to be pathogenic for hemochromatosis,which strongly supported the pathogenicity of p.R288Q reported for the first time in this *** 72 wk of intensive phlebotomy therapy,the patient achieved a reduction in serum ferritin to 160.5μg/*** patient's clinical symptoms demonstrated a notable *** This study highlights the importance of screening for hemochromatosis in patients with diabetes and hypogonadotropic *** also suggests that long-term active phlebotomy could efficiently improve the prognosis in severe JH.

关键词： Juvenile hemochromatosis Liver cirrhosis Diabetes Iron overload Phlebotomy Case report

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一种语音课堂考勤系统的设计与实现

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韶关学院学报 2016年第12期37卷 10-14页

作者：杨云海章芬芬韶关学院信息科学与工程学院广东韶关512005

课堂考勤工作是教师必须开展的一项日常教学管理工作,传统的考勤方式、登记办法和统计方式都存在费时费力、效率不高的问题.使用信息技术开发一种语音课堂考勤系统辅助教师进行课堂考勤以及记录、统计等工作,可在一定程度上减轻教师在... 详细信息

课堂考勤工作是教师必须开展的一项日常教学管理工作,传统的考勤方式、登记办法和统计方式都存在费时费力、效率不高的问题.使用信息技术开发一种语音课堂考勤系统辅助教师进行课堂考勤以及记录、统计等工作,可在一定程度上减轻教师在课堂考勤和考勤统计方面的工作量.

关键词：课堂考勤语音数据库

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Structural characterization of a 2-O-acetylglucomannan from Dendrobium officinale stem

Structural characterization of a 2-O-acetylglucomannan from ...

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首届长三角科技论坛——长三角生物医药发展论坛

作者： yun-fen Hua~a Ming zhang~b Cheng-xin Fu~(a,*) zhang-hui Chen~b ~aLab of Plant Systematic Evolution and Biodiversity,College of life Science,Zhejiang University ~bInstitute of biotechnology,College of life Science,Zhejiang University,Hangzhou 3113012,China

A heteropolysaccharide,obtained from an aqueous extract of dried stem of Dendrobiumofficinale Kimura et Migo by anion-exchange chromatography and gel-permeationchromatography,was investigated by chemical techniques and NMR spectroscopy,and isdemonstrated to be a 2-O-acetyl glucomannan,composed of mannose,glucose and arabinose in40.2:8.4:1 molar *** has a backbone of(1?4)-linked β-D-mannopyranosyl residues andβ-D-glucopyranosyl residues,with branches at O-6 consisting of terminal and(1?3)-linked Manp,(1?3)-linked Glcp,and a small proportion of arabinofuranosyl residues at the terminal *** acetyl groups are substituted at O-2 of (1?4)-linked Manp and *** main repeating unitof the polysaccharide is reported.

关键词： Dendrobium officinale Kimura et Migo Polysaccharide Glucomannan Structure

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Advances in Graphene‑Based Electrode for Triboelectric Nanogenerator

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Nano-Micro Letters 2025年第1期17卷 378-403页

作者： Bin Xie Yuanhui Guo yun Chen Hao zhang Jiawei Xiao Maoxiang Hou Huilong Liu Li Ma Xin Chen Chingping Wong State Key Laboratory of Precision Electronic Manufacturing Technology and Equipment Guangdong University of TechnologyGuangzhou 510006People’s Republic of China School of Electromechanical Engineering Guangdong University of TechnologyGuangzhou 510006People’s Republic of China School of Materials Science and Engineering Georgia Institute of TechnologyAtlantaGA 30332USA

With the continuous development of wearable electronics,wireless sensor networks and other micro-electronic devices,there is an increasingly urgent need for miniature,flexible and efficient nanopower generation *** nanogenerator(TENG)technology can convert small mechanical energy into electricity,which is expected to address this *** the core component of TENG,the choice of electrode materials significantly affects its *** metal electrode materials often suffer from problems such as durability,which limits the further application of ***,as a novel electrode material,shows excellent prospects for application in TENG owing to its unique structure and excellent electrical *** review systematically summarizes the recent research progress and application prospects of TENGs based on graphene *** precision processing methods of graphene electrodes are introduced,and the applications of graphene electrode-based TENGs in various scenarios as well as the enhancement of graphene electrodes for TENG performance are *** addition,the future development of graphene electrode-based TENGs is also prospectively discussed,aiming to promote the continuous advancement of graphene electrode-based TENGs.

关键词： Triboelectric nanogenerator Precision processing Graphene electrode Self-powered sensor

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Evaluation of the Left Ventricular Remodeling in Patients with Myocardial Infarction after Revascularization with Intravenous Real-time Myocardial Contrast Echocardiography

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Journal of Huazhong University of Science and Technology(Medical Sciences) 2008年第3期28卷 287-290页

作者：毕小军邓又斌申屠伟慧熊莉张芸余芬黄润青 Department of Medical Ultrasound Tongji HospitalTongji Medical CollegeHuazhong University of Science and Technology

In order to evaluate the left ventricular remodeling in patients with myocardial infarction after revascularization with intravenous real-time myocardial contrast echocardiography （RT-MCE）, intravenous RT-MCE was performed on 20 patients with myocardial infarction before coronary revascularization. Follow-up echocardiography was performed 3 months after coronary revascularization. Segmental wall motion was assessed using 18-segment LV model and classified as normal, hypokinesis, akinesis and dyskinesis. Myocardial perfusion was assessed by visual interpretation and divided into 3 conditions： homogeneous opacification=l; partial or reduced opaciflcation or subendocardial contrast defect=2; constrast defect=3. Myocardial perfusion score index （MPSI） was calculated by dividing the total sum of contrast score by the total number of segments with abnormal wall motion. Twenty patients were classified into 2 groups according to the MPSI： MPSI≤I.5 as good myocardial perfusion, MPSI〉1.5 as poor myocardial perfusion. To assess the left ventricular remodeling, the following comparisons were carried out：（1） Comparisons of left ventricular ejection fraction （LVEF）, left ventricular end-systolic volume （LVESV） and left ventricular end-diastolic volume （LVEDV） before and 3 months after revascularization in two groups;（2） Comparisons of LVEF, LVESV and LVEDV pre-revascularization between two groups and comparisons of these 3 months post-revascularization between two groups; （3） Comparisons of the differences in LVEF, LVESV and LVEDV between 3 months post-and pre-revascularization （ALVEF, ALVESV and ALVEDV） between two groups; （4） The linear regression analysis between ALVEF, ALVESV, ALVEDV and MPSI. The results showed that the LVEF obtained 3 months after revascularization in patients with MPSI〉1.5 was obviously lower than that in those with MPSI〈1.5. The LVEDV obtained 3 months post-revascularization in patients with MPSI〉1.5 was obviously larger than that in those with MPSI≤1.5

关键词： real-time myocardial contrast echocardiography myocardial infarction left ventricularremodeling revascularization

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Transition of One Mode-Phase at the Crisis and Onset of Spatiotemporal Chaos

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Chinese Physics Letters 2001年第2期18卷 178-180页

作者： HE Kai-fen zhang Hai-yun Key Laboratory in University for Radiation Beam Technology and Materials Modification Beijing Normal UniversityBeijing 100875 Institute of Low Energy Nuclear Physics Beijing Normal UniversityBeijing 100875 Beijing Radiation Center Beijing 100875

In our previous work[***.80(1998)696]we investigated a crisis-induced transition from temporal chaos to spatiotemporal chaos(STC)due to a collision between the unstable orbit of the carrier saddle steady wave(SSW)and the attractor of its perturbation wave(PW).In this letter,we find that at the crisis one PW mode-phase experiences a state *** variations of PW mode-phases are governed by the linear dispersion as well as two competitive effects arising from the system nonlinearity:(1)interaction between the SSW and the PW(SP);(2)self-nonlinearity of the PW(PP).With the increasing importance of system nonlinearity we find that before the crisis for all PW modes the SP effects dominate and the mode-phases vibrate chaotically;while after the crisis for one PW mode the PP effect surpasses that of SP,in which case the phase transits to a state of combination of chaotic whirling and vibrating,which is different from the case of weak *** the meantime,the PW partial wave is free from the trapping of its carrier,and can be driven or damped chaotically depending on the phase difference relative to the carrier SSW,causing the STC motion after the crisis.

关键词： nonlinearity chaotic chaos

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青藏高原农田生态系统碳平衡

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土壤学报 2002年第5期39卷 636-642页

作者：刘允芬欧阳华张宪洲钟华平罗天祥中国科学院地理科学与资源研究所北京100101

为探讨青藏高原农田生态系统碳平衡规律及其影响因子 ,并揭示其对全球含碳温室气体源、汇变化的贡献与响应 ,1 998～ 1 999连续 2年在拉萨农业生态站农田用静态箱法和生物量取样法开展土壤—植被界面CO2 吸收与排放的试验研究。农作物... 详细信息

为探讨青藏高原农田生态系统碳平衡规律及其影响因子 ,并揭示其对全球含碳温室气体源、汇变化的贡献与响应 ,1 998～ 1 999连续 2年在拉萨农业生态站农田用静态箱法和生物量取样法开展土壤—植被界面CO2 吸收与排放的试验研究。农作物生长季 ,以冬小麦为例 ,生长前期 (从播种到拔节 )土壤碳排放量高于植被碳固定 ,生长后期 (灌浆到成熟 )则是植被碳固定高于土壤碳排放量 ,从全年来看。

关键词：青藏高原农田生态系统土壤碳排放植被碳固定碳平衡

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5-羟甲基糠醛在CuPd(111)上的加氢反应机理研究

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原子与分子物理学报 2025年第4期42卷 53-57页

作者：张兴云周广丽石云山东临沂大学化学化工学院临沂276000

采用密度泛函理论,利用包含零点能校正的PBE-D3方法研究了5-羟甲基糠醛在双金属催化剂CuPd(111)上表面的加氢还原反应,考虑了两种反应路径,第一种反应路径是侧链上的醛基氧首先发生加氢反应生成F-CHOH中间体,然后F-CHOH中间体继续加氢生... 详细信息

采用密度泛函理论,利用包含零点能校正的PBE-D3方法研究了5-羟甲基糠醛在双金属催化剂CuPd(111)上表面的加氢还原反应,考虑了两种反应路径,第一种反应路径是侧链上的醛基氧首先发生加氢反应生成F-CHOH中间体,然后F-CHOH中间体继续加氢生成2,5-二羟甲基呋喃;第二种反应路径是侧链上的醛基碳先加氢原还原生成F-CH_(2)O中间体,生成的F-CH_(2)O进一步加氢还原生成2,5-二羟甲基呋喃.计算了两种竞争反应路径的活化能垒和反应热,结果表明,5-羟甲基糠醛在CuPd(111)面加氢还原生成2,5-二羟甲基呋喃的最佳反应路径为:F-CHO→F-CHOH→F-CH_(2)OH.

关键词： 5-羟甲基糠醛活化能垒反应热

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基于空间特点的老旧居民小区同功能区域重划分研究

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山西建筑 2025年第1期51卷 38-40,54页

作者：张芸安顺市棚户区改造服务中心贵州安顺561099

老旧小区的居住环境可能因年久失修而变得陈旧和不安全,重划分研究有助于改善小区的绿化、照明、交通等基础设施,提升居民的居住舒适度和安全感。为此,该研究分析了老旧居民小区空间特点,基于此,以空气质量、噪声水平、交通便利性、绿... 详细信息

老旧小区的居住环境可能因年久失修而变得陈旧和不安全,重划分研究有助于改善小区的绿化、照明、交通等基础设施,提升居民的居住舒适度和安全感。为此,该研究分析了老旧居民小区空间特点,基于此,以空气质量、噪声水平、交通便利性、绿化覆盖率为目标函数,结合目标权重,构建老旧居民小区同功能区域重划分模型。引入免疫机制改进免疫粒子群算法,求解该模型。实例分析结果显示:研究方法应用后同功能区域利用率明显升高。该方法能够充分利用小区现有的空间资源,通过合理布局和功能整合,提高空间利用率,减少资源浪费。

关键词：空间特点老旧居民小区同功能区域改进免疫粒子群算法重划分

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Comprehensive recovery of lead, zinc, and iron from hazardous jarosite residues using direct reduction followed by magnetic separation

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International Journal of Minerals,Metallurgy and Materials 2018年第2期25卷 123-130页

作者： Ya-yun Wang Hui-fen Yang Bo Jiang Rong-long Song Wei-hao zhang Key Laboratory for High-Efficient Mining and Safety of Metal Mines of Ministry of Education University of Science and Technology Beijing School of Civil and Resource Engineering University of Science and Technology Beijing

Lead, zinc, and iron were recovered from jarosite residues using direct reduction followed by magnetic separation. The influence of the coal dosage, reduction temperature, and reduction time on the volatilization rates of lead and zinc and the metallization rate of iron were investigated. The results show that the volatilization rates of lead and zinc were 96.97% and 99.89%, respectively, and the iron metallization rate was 91.97% under the optimal reduction roasting conditions of a coal dosage of 25.0 wt% and reduction roasting at 1250°C for 60 min. The magnetic concentrate with an iron content of 90.59 wt% and an iron recovery rate of 50.87% was obtained under the optimum conditions in which 96.56% of the reduction product particles were smaller than 37 μm and the magnetic field strength was 24 k A/m. Therefore, the results of this study demonstrate that recovering valuable metals such as lead, zinc, and iron from jarosite residues is feasible using the developed approach.

关键词： jarosite residues recovery direct reduction magnetic separation valuable metals

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