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Clinical features of Huntington disease in 243 Chinese patients

Neural Regeneration Research 2010年第2期5卷 102-107页

作者： Xiaoyan Guo Shushan Zhang jean-marc Burgunder Huifang Shang Department of Neurology West China Hospital Sichuan University Chengdu 610041 Sichuan Province China Department of Neurology Inselspital BernUniversity CH-3010 Bern Switzerland

OBJECTIVE： To thoroughly explore the clinical characteristics of Huntington disease in China. METHODS： A computer-based online search of China National Knowledge Infrastructure was performed to review case reports concerning Huntington disease published between 1980 and 2008; the clinical characteristics were analyzed. RESULTS： A total of 80 studies involving 243 patients （142 males and 101 females） were collected, 82.0% of which were from provinces of North China. In addition, 97.1% of the cases had a family history of Huntington disease, and paternal inheritance （64.6%） was greater than maternal inheritance （35.4%）. Moreover, onset age was significantly less than from maternal inheritance. The mean onset age of Huntington disease was （35.2 ± 11.5） years, mean age of death was （45.6 ± 13.5） years, and the mean course of disease from onset to death was （11.6 ± 5.6） years. Onset characterized by involuntary movement accounted for 47.7%, including 66.4% in the entire body, 16.4% in the upper limbs, and 14.7% in the head and face. Psychiatric symptoms accounted for 18.1%, and disturbed intelligence accounted for 2.1%. With disease progression, 99.5% of patients exhibited involuntary movement, 69.8% displayed cognitive impairment, and 39.2% suffered from psychiatric symptoms. In addition, 38.7% of patients were complicated by dysarthria, dysphagia, and cough after drinking. A total of 70.8% of patients exhibited an abnormal electroencephalogram, 18.8% had mild abnormalities in the cerebrospinal fluid, and 70.1% displayed brain atrophy and lateral ventriculomegaly on CT or MRI. A total of 88.9% of patients scored ≤ 23 in the Mini-Mental State Examination （MMSE）. Of the reported patients, only 22 underwent/T15 gene testing with positive results. CONCLUSION： Huntington disease is more frequently detected in males than females, and the majority has a family history. The disease has high incidence in Northern China, in particular with paternal inheritance. In addition, the d

关键词： Huntington disease clinical characteristics literature evaluation neurodegenerative disease

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Concomitant autoimmune and genetic pancreatitis leads to severe inflammatory conditions

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World Journal of Gastroenterology 2008年第16期14卷 2596-2598页

作者： jean Louis Frossard jean marc Dumonceau Catherine Pastor Laurent Spahr Antoine Hadengue Division de Gastroentérologie et Hépatologie Geneva University Hospital

Chronic pancreatitis characterized by an early onset should be extensively investigated including the search for a mutation of the PRSS1, SPINK-1 or CFTR genes and potential features of autoimmune pancreatitis. We here describe a case of chronic pancreatitis with an onset at a very young age in which a mutation of the PRSS1 and several features of autoimmune pancreatitis were identified.

关键词： Chronic pancreatitis Genetics Autoimmunepancreatltis SPINK-1 CFTR PRSS1

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57Fe M?ssbauer spectrometry: A powerful technique to analyze the magnetic and phase characteristics in RE–Fe–B permanent magnets

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Chinese Physics B 2021年第1期30卷 92-105页

作者：赵利忠张雪峰严密刘仲武 jean-marc Greneche Institute of Advanced Magnetic Materials College of Materials & Environmental Engineering Hangzhou Dianzi University School of Materials Science and Engineering South China University of Technology State Key Laboratory of Silicon Materials School of Materials Science and EngineeringKey Laboratory of Novel Materials for Information Technology of Zhejiang Province Zhejiang University Institut des Molécules et Matériaux du Mans CNRS UMR-6283 Le Mans Université

This review summarizes the recent advances on the application ofFe M?ssbauer spectrometry to study the magnetic and phase characteristics of Nd–Fe–B-based permanent magnets. First of all, the hyperfine structures of the CeFeB,(Ce,Nd)FeB and MMFeB phases are well-defined by using the model based on the Wigner-Seitz analysis of the crystal structure. The results show that the isomer shift δ and the quadrupole splitting ?Eof those 2:14:1 phases show minor changes with the Nd content, while the hyperfine field Bhfincreases monotonically with increasing Nd content and its value is influenced by the element segregation and phase separation in the 2:14:1 phase. Then, the hyperfine structures of the low fraction secondary phases are determined by theFe M?ssbauer spectrometry due to its high sensitivity. On this basis,the content, magnetic behavior, and magnetization of the REFephase, the amorphous grain boundary(GB) phase, and the amorphous worm-like phase, as well as their effects on the magnetic properties, are systematically studied.

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Usefulness of staging systems and prognostic scores for hepatocellular carcinoma treatments

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World Journal of Hepatology 2016年第17期8卷 703-715页

作者： Xavier Adhoute Guillaume Penaranda jean Luc Raoul Patrice Le Treut Emilie Bollon jean Hardwigsen Paul Castellani Hervé Perrier marc Bourlière Department of Hepato-Gastroenterology Hopital Saint-Joseph Alpha Bio Laboratory Department of Hepato-Gastroenterology and Digestive Oncology Institut Paoli-Calmette Department of Hepatobiliary Surgery Centre Hospitalo-Universitaire Timone

Therapeutic management of hepatocellular carcinoma(HCC) is quite complex owing to the underlying cirrhosis and portal vein hypertension. Different scores or classification systems based on liver function and tumoral stages have been published in the recent years. If none of them is currently "universally" recognized, the Barcelona Clinic Liver Cancer(BCLC) staging system has become the reference classification system in Western countries. Based on a robust treatment algorithm associated with stage stratification, it relies on a high level of evidence. However, BCLC stage B and C HCC include a broad spectrum of tumors but are only matched with a single therapeutic option. Some experts have thus suggested to extend the indications for surgery or for transarterial chemoembolization. In clinical practice, many patients are already treated beyond the scope of recommendations. Additional alternative prognostic scores that could be applied to any therapeutic modality have been recently proposed. They could represent complementary tools to the BCLC staging system and improve the stratification of HCC patients enrolled in clinical trials, as illustrated by the NIACE score. Prospective studies are needed to compare these scores and refine their role in the decision making process.

关键词： Scoring system Hepatocellular carcinoma Barcelona Clinic Liver Cancer staging system NIACE Transarterial chemoembolization

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特质情绪智力及线上学习收获感对外语课堂无聊的预测作用

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外语与外语教学 2020年第5期 33-44,148,149页

作者：李成陈 jean-marc DEWAELE 华中科技大学外国语学院湖北武汉430074 伦敦大学伯贝克学院英国伦敦WC1B5DT

基于Pekrun(2006)的控制—价值理论,本研究探讨了特质情绪智力及线上学习收获感对外语课堂无聊的预测作用。348名我国大学生参与了线上问卷调查,使用SPSS19.0进行了描述性统计、独立样本t检验以及多元回归分析。结果表明:学生的情绪智... 详细信息

基于Pekrun(2006)的控制—价值理论,本研究探讨了特质情绪智力及线上学习收获感对外语课堂无聊的预测作用。348名我国大学生参与了线上问卷调查,使用SPSS19.0进行了描述性统计、独立样本t检验以及多元回归分析。结果表明:学生的情绪智力水平中等,在线上英语课程中的收获感一般,无聊水平较低,但显著高于面授水平。情绪智力和网课收获感共同负向预测无聊情绪,后者的预测作用(中到高等程度)远高于前者(小到中等程度)。研究结果不仅证实了控制—价值理论在网络外语教学情境中的适用性,还为如何优化线上外语教学及提高学习者的情绪健康提供一定启示。

关键词：外语课堂无聊特质情绪智力学习收获感网络学习《外语无聊量表》

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A review of processing of Cu/C base plate composites for interfacial control and improved properties

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International Journal of Extreme Manufacturing 2020年第1期2卷 25-45页

作者： jean-François Silvain jean-marc Heintz Amélie Veillere Loic Constantin Yong Feng Lu Univ.Bordeaux CNRSBordeaux INPICMCBUMR 5026F-33600 PessacFrance Department of Electrical and Computer Engineering University of Nebraska-LincolnLincolnNE68588United States of America

The increase in both power and packing densities in power electronic devices has led to an increase in the market demand for effective heat-dissipating materials with a high thermal conductivity and thermal expansion coefficient compatible with chip materials while still ensuring the reliability of the power *** matrix composites,especially copper matrix composites,containing carbon fibers,carbon nanofibers,or diamond are considered very promising as the next generation of thermalmanagement materials in power electronic *** composites exhibit enhanced thermal properties,as compared to pure copper,combined with lower *** paper presents powder metallurgy and hot uniaxial pressing fabrication techniques for copper/carbon composite materials which promise to be efficient heat-dissipation materials for power electronic *** analyses clearly indicate that interfacial treatments are required in these composites to achieve high thermal and thermomechanical *** of interfaces(through a novel reinforcement surface treatment,the addition of a carbide-forming element inside the copper powders,and processing methods),when selected carefully and processed properly,will form the right chemical/mechanical bonding between copper and carbon,enhancing all of the desired thermal and thermomechanical properties while minimizing the deleterious *** paper outlines a variety of methods and interfacial materials that achieve these goals.

关键词： metal matrix composite physical properties interface/interphase copper carbon reinforcement

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Localized magnetization reversal processes in cobalt nanorods with different aspect ratios

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Nano Research 2015年第7期8卷 2231-2241页

作者： marc Pousthomis Evangelia Anagnostopoulou Ioannis Panagiotopoulos Rym Boubekri Weiqing Fang Frederic Ott Kahina Ait Atmane jean-Yves Piquemal Lise-Marie Lacroix Guillaume Viau Universite de Toulouse LPCNO UMR 5215 INSA CNRS UPS 135 av de Rangueil 31077 Toulouse Cedex 4 France Laboratoire Leon Brillouin CEA/CNRS UMR 12 IRAMIS CEA-Saclay 91191 Gifsur Yvette France Department of Materials Science and Engineering University ofloannina Ioannina 45110 Greece Universite Paris Diderot Sorbonne Paris Cite ITODYS CNRS UMR 7086 15 rueJ.-A de Baif 75205 Paris Cedex 13 France

We present results of the synthesis of cobalt nanorods using the polyol process and the mechanism of magnetization reversal. We show that the nucleation step is significantly dependent on the nature of the ruthenium chloride used as the nucleating agent. This allows varying the diameter and aspect ratio of the cobalt nanorods independently. Co nanorods with aspect ratio, mean diameter, and mean length in the ranges ARm =3-16, Din= 7-25 nm, and Lm=30-300 nm, respectively, were produced using this method. X-ray diffraction and electron microscopy showed that a strong discrepancy between the structural coherence and morphological aspect ratio can exist because of stacking faults. The coercivity of assemblies of different nanorods was systematically measured, and the highest values were obtained for the smallest diameter and the largest structural coherence length. Micromagnetic simulations were performed to account for the dependence of the coercive field on the diameter. An important observation is that simple coherent magnetization rotation models do not apply to these magnetic nano-objects. Even for very small diameters （Dm = 5-10 nm） well below the theoretical coherent diameter Dcoh（CO）= 24 nm, we observed inhomogeneous reversal modes dominated by nucleation at the rod edges or at structural defects such as stacking faults. We conclude that, in order to produce high-coercivity materials based on nanowires, moderate aspect ratios of 5-10 are sufficient for providing a structural coherence similar to the morphological aspect ratio. Thus, the first priority should be to avoid the formation of stacking faults within the Co nanowires.

关键词： nanorod nanowire permanent magnets micromagnetic calculations shape anisoteopy

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Wafer-level vapor cells filled with laser-actuated hermetic seals for integrated atomic devices

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Microsystems & Nanoengineering 2022年第6期8卷 225-235页

作者： Vincent Maurice Clément Carlé Shervin Keshavarzi Ravinder Chutani Samuel Queste Ludovic Gauthier-Manuel jean-marc Cote Rémy Vicarini Moustafa Abdel Hafiz Rodolphe Boudot Nicolas Passilly IEMN-Institut d’Electronique de Microélectronique et de Nanotechnologie UMR8520 CNRSUniversitéLilleCentrale LilleF-59000 LilleFrance FEMTO-ST Institute UMR6174 CNRSUniversitéBourgogne Franche-ComtéF-25030 BesançonFrance

Atomic devices such as atomic clocks and optically-pumped magnetometers rely on the interrogation of atoms contained in a cell whose inner content has to meet high standards of purity and ***-blowing techniques and craftsmanship have evolved over many decades to achieve such standards in macroscopic vapor *** the emergence of chip-scale atomic devices,the need for miniaturization and mass fabrication has led to the adoption of microfabrication techniques to make millimeter-scale vapor ***,many shortcomings remain and no process has been able to match the quality and versatility of glass-blown ***,we introduce a novel approach to structure,fill and seal microfabricated vapor cells inspired from the century-old approach of glass-blowing,through opening and closing single-use zero-leak microfabricated *** valves are actuated exclusively by laser,and operate in the same way as the“make-seals”and“break-seals”found in the filling apparatus of traditional *** structures are employed to fill cesium vapor cells at the *** make-seal structure consists of a glass membrane that can be locally heated and deflected to seal a *** break-seal is obtained by breaching a silicon wall between *** new approach allows adapting processes previously restricted to glass-blown *** can also be extended to vacuum microelectronics and vacuum-packaging of micro-electro-mechanical systems(MEMS)devices.

关键词： filled vacuum heated

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Grain boundary construction and properties enhancement for hot deformed(Ce,La,Y)-Fe-B magnet by a two-step diffusion process

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Journal of Materials Science & Technology 2023年第34期165卷 253-261页

作者： Xuefeng Liao Weiwei Zeng Lizhong Zhao Qing Zhou Jiayi He Wei Li Xiangyi Liu Hongya Yu Xiaolian Liu Haoyang Jia jean-marc Greneche Xuefeng Zhang Zhongwu Liu School of Materials Science and Engineering South China University of TechnologyGuangzhou 510640China Institute of Advanced Magnetic Materials College of Materials&Environmental EngineeringHangzhou Dianzi UniversityHangzhou 310018China Guangdong Provincial Key Laboratory of Rare Earth Development and Application Institute of Resources Utilization and Rare Earth DevelopmentGuangdong Academy of SciencesGuangzhou 510640China School of Materials Science and Engineering Nanchang Hangkong UniversityNanchang 330063China Institut des Molécules et Matériaux du Mans IMMM CNRS UMR-6283 Le Mans UniversitéAvenue MessiaenLe Mans F-72085Cedex 9France

The rare earth-iron-boron magnets based on high abundance rare earths(REs)show potential for costeffective permanent magnets but their hard magnetic properties have to be greatly *** grain boundary diffusion process(GBDP)is known as an effective way to improve the coercivity of Nd-Fe-B magnets,however,the conventional diffusion method faces a challenge for Ce-based magnets since there is no enough continuous GB layer as the diffusion ***,a two-step(Nd-Cu doping followed by Nd-Cu diffusion)GBDP was introduced for hot deformed(Ce,La,Y)-Fe-B magnet,and the excellent magnetic properties ofμ0Hc=0.63 T,μ0Mr=0.68 T,and(BH)max=72.4 kJ/m^(3)were *** Nd-Cu doping helps the formation of RE-rich GB layer,and then it acts as the diffusion channel for increasing the ef-ficiency of the subsequent Nd-Cu diffusion and results in the increased volume fraction of continuously distributed GB phase,whose paramagnetism was verified by 57Fe Mössbauer *** paramagnetic GB phases help to form the discontinuous domain walls,as observed by Lorentz transmission electron microscopy,and break the magnetic exchange coupling of RE2Fe14B *** thus contributes to the coercivity enhancement of the hot deformed magnet with two-step diffusion,which is further proved by micromagnetic *** study proposes a potential technique to prepare anisotropic hot deformed(Ce,La,Y)-Fe-B magnet with high cost-performance.

关键词： High abundance rare earth Hot deformed RE-Fe-B magnets Grain boundary diffusion process Magnetic properties Grain boundary phase

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Methylome and transcriptome data integration reveals potential roles of DNA methylation and candidate biomarkers of cow Streptococcus uberis subclinical mastitis

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Journal of Animal Science and Biotechnology 2023年第2期14卷 593-613页

作者： Mengqi Wang Nathalie Bissonnette Mario Laterriere Pier‑Luc Dudemaine David Gagne jean‑Philippe Roy Xin Zhao marc‑Andre Sirard Eveline M.Ibeagha‑Awemu Sherbrooke Research and Development Centre Agriculture and Agri-Food CanadaSherbrookeQuebecCanada Department of Animal Science Laval UniversityQuebecQuebecCanada Quebec Research and Development Centre Agriculture and Agri-Food CanadaQuebecQuebecCanada Department of Clinical Sciences Universite de MontrealSt‑HyacintheQuebecCanada Department of Animal Science McGill UniversitySte‑Anne‑De‑BellevueQuebecCanada.

Background:Mastitis caused by different pathogens including Streptococcus uberis(***)is responsible for huge economic losses to the dairy *** order to investigate the potential genetic and epigenetic regulatory mecha‑nisms of subclinical mastitis due to ***,the DNA methylome(whole genome DNA methylation sequencing)and transcriptome(RNA sequencing)of milk somatic cells from cows with naturally occurring *** subclinical mastitis and healthy control cows(n=3/group)were ***:Globally,the DNA methylation levels of CpG sites were low in the promoters and first exons but high in inner exons and *** DNA methylation levels at the promoter,first exon and first intron regions were nega‑tively correlated with the expression level of genes at a whole‑genome‑wide *** general,DNA methylation level was lower in ***‑positive group(SUG)than in the control group(CTG).A total of 174,342 differentially methylated cytosines(DMCs)(FDR<0.05)were identified between SUG and CTG,including 132,237,7412 and 34,693 DMCs in the context of CpG,CHG and CHH(H=A or T or C),***,101,612 methylation haplotype blocks(MHBs)were identified,including 451 MHBs that were significantly different(dMHB)between the two groups.A total of 2130 differentially expressed(DE)genes(1378 with up‑regulated and 752 with down‑regulated expression)were found in *** of methylome and transcriptome data with MethGET program revealed 1623 genes with signifi‑cant changes in their methylation levels and/or gene expression changes(MetGDE genes,MethGET P‑value<0.001).Functional enrichment of genes harboring≥15 DMCs,DE genes and MetGDE genes suggest significant involvement of DNA methylation changes in the regulation of the host immune response to *** infection,especially cytokine ***,discriminant correlation analysis with DIABLO method identified 26 candidate biomarkers,including 6 DE genes,15 CpG‑DMCs and 5 dMHBs that discrimina

关键词： Discriminant biomarkers Gene expression Genome‑wide DNA methylation pattern Immune processes and pathways Methylation haplotype block Milk somatic cell Streptococcus uberis Subclinical mastitis

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