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Characteristics of patients with Wilson disease in the United States: An insurance claims database study

World Journal of Hepatology 2024年第5期16卷 791-799页

作者： Thomas daniel-Robin Pradeep Kumar Bernard Benichou jean-Philippe Combal Vivet Therapeutics Paris 75008France Clarivate Noida 201301Uttar PradeshIndia

BACKGROUND Wilson disease(WD)is a progressive,potentially fatal degenerative disease affecting the liver and central nervous *** its low prevalence,collecting data on large cohorts of patients with WD is *** insur-ance claims databases provide powerful tools to collect retrospective data on large numbers of patients with rare *** To describe patients with WD in the United States,their treatment and clinical outcome,using a large insurance claims *** This retrospective,longitudinal study was performed in the Clarivate Real-World Data Product *** patients with≥2 claims associated with an Interna-tional Classification of Diseases 10(ICD-10)diagnostic code for WD(E83.01)between 2016 and 2021 were included and followed until death or study *** were divided into two groups by whether or not they were documented to have received a specific treatment for *** manifestations,hospital-isations,liver transplantation and death were *** Overall,5376 patients with an ICD-10 diagnostic code for WD were *** mean age at inclusion was 41.2 years and 52.0%were men.A specific WD treatment was documented for 885 patients(15.1%),although the number of patients taking zinc salts may be underestimated due to over the counter *** inclusion,the mean age of patients with a documented treatment was 36.6±17.8 years vs 42.2±19.6 years in those without a documented *** follow-up,273 patients(5.1%)*** with the American general population,the standardised mortality ratio was *** proportion of patients with a documented WD-specific treatment who died during follow-up was 4.0%and the mean age at death 52.7 *** Patients treated for WD in the United States had an excess early mortality compared with the American *** findings indicate that there is a significant unmet need for effective treatment for WD in the United States.

关键词： Wilson disease Mortality Treatment Copper chelators Zinc acetate United States

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Epidemiological and Computed Tomography Aspects of Facial Trauma at Kira Hospital in Bujumbura

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Open Journal of Radiology 2024年第2期14卷 83-91页

作者： Sébastien Manirakiza jean Marie Banziriyubusa Stany Harakandi Patrice Barasukana Chantal Murekatete daniel Nduwayo jean Bosco Bizimana Léopold Nzisabira Department of Radiology Teaching Hospital of Kamenge Bujumbura Burundi Department of Anesthesia and Resuscitation Teaching Hospital of Kamenge Bujumbura Burundi Department of Internal Medicine (Neurology) Teaching Hospita lof Kamenge Bujumbura Burundi Department of Surgery (Neurosurgery) Teaching Hospital of Kamenge Bujumbura Burundi

Background: Maxillofacial trauma affects young adults more. The injury assessment is difficult to establish in low-income countries because of the imaging means, particularly the scanner, which is poorly available and less financially accessible. The aim of this study is to describe the epidemiological profile and the various tomodensitometric aspects of traumatic lesions of the face in patients received in the Radiology department of Kira Hospital. Patients and methods: This is a descriptive retrospective study involving 104 patients of all ages over a period of 2 years from December 2018 to November 2019 in the medical imaging department of KIRA HOSPITAL. We included in our study any patient having undergone a CT scan of the head and presenting at least one lesion of the facial mass, whether associated with other cranioencephalic lesions. Results: Among the 384 patients received for head trauma, 104 patients (27.1% of cases) presented facial damage. The average age of our patients was 32.02 years with extremes of 8 months and 79 years. In our study, 87 of the patients (83.6%) were male. The road accident was the circumstance in which facial trauma occurred in 79 patients (76% of cases). These injuries were accompanied by at least one bone fracture in 97 patients (93.3%). Patients with fractures of more than 3 facial bones accounted for 40.2% of cases and those with fractures of 2 to 3 bones accounted for 44.6% of cases. The midface was the site of the fracture in 85 patients (87.6% of cases). Orbital wall fractures were noted in 57 patients (58.8% of cases) and the jawbone was the site of a fracture in 50 patients (51.5% of cases). In the vault, the fractures involved the extra-facial frontal bone (36.1% of cases) and temporal bone (18.6% of cases). Cerebral contusion was noted in 41.2% of patients and pneumoencephaly in 15.5% of patients. Extradural hematoma was present in 16 patients and subdural hematoma affected 13 patients. Conclusion: Computed tomography is

关键词： Road Accident CT-Scan Facial Trauma

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Application of near-infrared spectroscopy for fast germplasm analysis and classification in multi-environment using intact-seed peanut(Arachis hypogaea L.)

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Oil Crop Science 2024年第2期9卷 132-141页

作者： Fentanesh Chekole Kassie Gilles Chaix Hermine Bille Ngalle Maguette Seye Coura Fall Hodo-Abalo Tossim Aissatou Sambou Olivier Gibert Fabrice Davrieux Joseph Martin Bell jean-Francois Rami daniel Fonceka Joel Romaric Nguepjop Department of Plant Biology and Physiology Faculty of SciencesUniversity of Yaounde IYaoundeP.O.Box 337Cameroon Department of Plant Science College of AgricultureWolaita Sodo UniversitySodoP.O.Box 138Ethiopia CIRAD UMR AGAP InstitutMontpellierFrance AGAP Institut Univ MontpellierCIRADINRAEInstitut AgroMontpellierFrance ChemHouse Research Group MontpellierFrance Centre D’Etudes Regional Pour L’Amelioration de L’Adaptation a La Secheresse(CERAAS/ISRA) Route de KhomboleThiesBP 3320Senegal Dispositif de Recherche et de Formation en Partenariat Innovation et Amelioration Varietale en Afrique de L’Ouest(IAVAO)CERAASRoute de KhomboleThiesBP 3320Senegal CIRAD UMR QUALISUDSaint PierreFrance

Peanut is a worldwide oilseed crop and the need to assess germplasm in a non-destructive manner is important for seed nutritional *** this study,Near Infrared Spectroscopy(NIRS)was applied to rapidly assess germplasm variability from whole seed of 699 samples,field-collected and assembled in four genetic and environmentbased sets:one set of 300 varieties of a core-collection and three sets of 133 genotypes of an interspecific population,evaluated in three environments in a large spatial scale of two countries,Mbalmayo and Bafia in Cameroon and Nioro in Senegal,under rainfed *** elemental spectra were gathered on six subsets of seeds of each sample,after three rotation scans,with a spectral resolution of 16 cm-1over the spectral range of867 nm to 2530 *** were then processed by principal component analysis(PCA)coupled with Partial least squares-discriminant analysis(PLS-DA).As results,a huge variability was found between varieties and genotypes for all NIR wavelength within and between *** magnitude of genetic variation was particularly observed at 11 relevant wavelengths such as 1723 nm,usually related to oil content and fatty acid *** yielded the most chemical attributes in three significant PCs(i.e.,eigenvalues>10),which together captured 93%of the total variation,revealing genetic and environment structure of varieties and genotypes into four clusters,corresponding to the four samples *** pattern of genetic variability of the interspecific population covers,remarkably half of spectrum of the core-collection,turning out to be the ***,a PLS-DA model was developed and a strong accuracy of 99.6%was achieved for the four sets,aiming to classify each seed sample according to environment *** confusion matrix achieved for the two sets of Bafia and Nioro showed 100%of instances classified correctly with 100%at both sensitivity and specificity,confirming that their seed quality was different f

关键词： Groundnut Oilseed Near infrared spectroscopy Germplasm analysis Environment Nutritional Breeding

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Nature:CD32a蛋白暴露艾滋病病毒"藏身地"

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现代生物医学进展 2017年第15期17卷 I0004-I0004页

作者： Benjamin Descours, Gaël Petitjean, Raoul Raffel Monsef Benkirane José-Luis López-Zaragoza, Timothée Bruel, Christine Lacabaratz, Yves Levy, Olivier Schwartz jean daniel lelievre José-Luis López-Zaragoza, Christine Lacabaratz, Yves Levy jean daniel lelievre José-Luis López-Zaragoza, Christine Lacabaratz, Yves Levy jean daniel lelievre Timothée Bruel Olivier Schwartz Christina Psomas Jacques Reynes Institut de Génétique Humaine Laboratoire de Virologie Moléculaire UMR9002 CNRS Université de Montpellier Montpellier France Vaccine Research Institute Université Paris-Est Faculté de Médecine INSERM U955 and Assistance Publique-Hôpitaux de Paris Groupe Henri-Mondor Albert-Chenevier Service d’Immunologie Clinique Créteil France Inserm U955 Equipe 16 Créteil France AP-HP Hôpital Henri-Mondor Albert-Chenevier Service d’Immunologie Clinique et Maladies Infectieuses 94000 Créteil France Institut Pasteur Virus and Immunity Unit URA CNRS 3015 Paris France Département Maladies Infectieuses et Tropicales Hôpital Universitaire Montpellier France

《自然》杂志近日发表了法国研究人员的一项最新成果：他们通过实验室研究发现，潜藏艾滋病病毒（HIV）的T细胞表面特定表达着一种名叫CD32a的蛋白，这种蛋白在未感染HIV的T细胞中却没有被检测出来。这意味着，CD32a蛋白可以作为一种生... 详细信息

《自然》杂志近日发表了法国研究人员的一项最新成果：他们通过实验室研究发现，潜藏艾滋病病毒（HIV）的T细胞表面特定表达着一种名叫CD32a的蛋白，这种蛋白在未感染HIV的T细胞中却没有被检测出来。这意味着，CD32a蛋白可以作为一种生物靶标，用来开发出针对”隐身”状态HIV的全新药物。

关键词：艾滋病病毒 a蛋白《自然》杂志研究人员细胞表面 HIV 实验室 T细胞

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Bithalamic Ischemic Stroke in Sickle Cell Patient at the Kamenge University Hospital in Burundi: Case Report

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Open Journal of Medical Imaging 2024年第2期14卷 72-78页

作者： Sébastien Manirakiza daniel Nduwayo Patrice Barasukana Chantal Murekatete jean Bosco Bizimana Alice Ndayishimiye Léopold Nzisabira Hélène Bukuru Department of Radiology Kamenge University Hospital Center University of Burundi Bujumbura Burundi Department of Internal Medicine Neurology Kamenge University Hospital Center University of Burundi Bujumbura Burundi Department of Surgery Neurosurgery Kamenge University Hospital Center University of Burundi Bujumbura Burundi Department of Pediatrics Kamenge University Hospital Center University of Burundi Bujumbura Burundi

Aim: To describe the clinical and radiological diagnostic for a sickle cell patient who got a bithalamic vasculature accident. Observation: This was a 4-year-old female child admitted to the pediatric ward for convulsive seizures in a feverish context. The physical examination did not find signs of meningeal irritation. The effective workup including thick gout, lumbar puncture, complete blood count, and C reactive protein was normal. An electrocencephalogram was not performed due to lack of availability of the device. Based on clinical data and the fact that Burundi is an area of high malaria prevalence, antimalarial and anticonvulsant treatment has been started. An alteration of consciousness (the Glasgow score of 7) had motivated a CT scan. Bithalamic hypodensities in favor of a vascular accident have been demonstrated. Etiological investigation revealed homozygous sickle cell disease ss during hemoglobin electrophoresis. The final evolution has been characterized by a return to normal consciousness. Conclusion: The complexity of the clinical picture following occlusion of Percherons artery makes it difficult to diagnose. Sickle cell disease may favour the occurrence of ischaemic lesions. Cerebral imaging enables the diagnosis to be made with certainty and avoids delays in treatment.

关键词： Stroke Bithalamic Infarction Sickel Cell Percheron Artery

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Evaluating and quantifying the effect of various spruce budworm intervention strategies on forest carbon dynamics in Atlantic Canada

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Forest Ecosystems 2022年第4期9卷 547-557页

作者： Zelin Liu Changhui Peng David AMacLean Louis De Grandpre jean-Noel Candau daniel Kneeshaw School of Geographic Sciences Hunan Normal UniversityChangsha410081China Department of Biological Sciences University of Qu�ebec at MontrealMontrealQC H3C 3P8Canada Faculty of Forestry and Environmental Management University of New BrunswickFrederictonNB E3B 5A3Canada Laurentian Forestry Centre Canadian Forest ServiceNatural Resources CanadaQC G1V 4C7Canada Great Lake Forestry Centre Canadian Forest ServiceNatural Resources CanadaON P6A 2E5Canada

Spruce budworm (SBW) outbreaks are one of the most devastating natural disturbances in spruce-balsam fir forests of eastern North America. Both early intervention strategy (EIS) and foliage protection strategy (FP) are being tested to limit forest losses, but the quantitative impact on forest carbon (C) dynamics is still unclear. In this study, we designed 19 separate scenarios of no intervention or varying success of EIS, FP, and their combination on SBW caused defoliation and mortality. We then used the TRIPLEX-Insect model to quantify their effects on forest C dynamics in the forests of the four provinces of Atlantic Canada. A scenario applying FP to 10%of the area with the greatest potential C losses of living biomass, protecting foliage in 10%of the forests is more realistic than higher proportion of FP given the high cost and large areas involved, resulted in reducing average cumulative net ecosystem productivity (NEP) from 2020 to 2039 by 56%–127%compared to a no outbreak *** results showed that FP would have to be applied everywhere to reduce tree mortality and increase NEP more than 8 years of successful EIS applied. However, if EIS can be successfully implemented for 12 years, it will maintain more forest C than FP applied everywhere during a moderate outbreak. We also found that the combination of EIS followed by FP in 10%of the areas disturbed by the SBW could maintain average cumulative NEP at similar levels to no defoliation in every province of Atlantic Canada. Black/red spruce forests younger than 60years old underwent the smallest changes in C dynamics whether using EIS, FP, or both. This highlights the importance of forest species, forest age, and their interactions on the effectiveness of a treatment during SBW outbreak. Overall, 31%–76%of the study area in Atlantic Canada could convert from a C sink to a source by 2039,if no protective measures are used under the worst-case scenarios, thus contributing to future climate warming.

关键词： Annual defoliation Natural disturbance Forest protection Net ecosystem productivity TRIPLEX-Insect Conifers

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Covalent functionalization/polycarboxylation of tungsten disulfide inorganic nanotubes （INTs-WS2）

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Nano Research 2015年第5期8卷 1454-1463页

作者： daniel Raichman David A. Strawser jean-Paul Lellouche Department of Chemistry Nanomaterials Research Center Institute of Nanotechnology & Advanced Materials Bar-Ilan University Ramat-Gan 5290002 Israel

钨二硫化物的无机的 nanotubes (INTs-WS 2 ) 在普通溶剂并且实际上是不可溶解的惰性，在研究和商业应用妨碍他们的实用性。功能的种类的共有原子价附件到 INT-WS 的表面上 2 是一批评首先在认识到潜力走那 INT-WS 为高效的材料和... 详细信息

钨二硫化物的无机的 nanotubes (INTs-WS 2 ) 在普通溶剂并且实际上是不可溶解的惰性，在研究和商业应用妨碍他们的实用性。功能的种类的共有原子价附件到 INT-WS 的表面上 2 是一批评首先在认识到潜力走那 INT-WS 为高效的材料和产品的 2 提议。尽管一些尝试关于准备修改 nanotubes 被报导了，仅仅表面功能的一个有限范围与这些方法是可能的。我们开发了一个万用的方法，基于一修改，高度 electrophilic 酸的 Vilsmeier-Haack 试剂，结合了 covalently 生产， polycarboxylated 功能的 WS 在极的液体是可分散的 2 nanotubes，包括水。表面 polycarboxylated 壳为另外的 derivatization 提供一个工具，启用到恐水病、吸水的材料的 derivatized nanotubes 的匹配的相容性。Nanocomposites 合并 derivatized INT-WS 2 被期望由于提高的界面的相容性显示出改进性质，由通过起始的 polycarboxylation 步可得到的 functionalization 的班的大数字成为可能。

关键词：碳纳米管共价键合二硫化钨 INT 无机表面官能团高性能材料纳米复合材料

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钢管道加固用碳纤维复合材料的力学与耐久性能研究

钢管道加固用碳纤维复合材料的力学与耐久性能研究

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作者： Honyigloh jean-daniel 哈尔滨工业大学

学位级别：硕士

钢管道一般易损坏和退化,如产生外部冲击和腐蚀。在过去十年中,纤维增强复合材料以其轻质高强、优异的耐腐蚀及疲劳性能在管道方面具有较大应用前景尽管复合材料被验证可有效加固修复管道,但其对管道加固后的力学性能影响尚缺少系统的... 详细信息

钢管道一般易损坏和退化,如产生外部冲击和腐蚀。在过去十年中,纤维增强复合材料以其轻质高强、优异的耐腐蚀及疲劳性能在管道方面具有较大应用前景尽管复合材料被验证可有效加固修复管道,但其对管道加固后的力学性能影响尚缺少系统的评价基于此。本研究采用实验室加速老化方法,系统地研究了Tw树脂及碳纤维增强树脂及碳纤维增强树脂(CFRP)板在蒸馏水和海水/盐中的长期性能演化。探讨了介质和温度共同作用下Tw基CFRP板和Tw基CFRP增强钢环的长期性能。采用动态力学分析(DMA)和力学性能测试(拉伸性能、层间剪切性能),研究了CFRP板材老化过程中树脂基体开裂、纤维/树脂界面脱粘的退化机理,取得了以下的研究成果:(1)随浸泡在蒸馏水和海水溶液中,CFRP板材的拉伸性能、层间剪切强度和玻璃化转变温度逐渐下降,这是由于树脂基体的短期塑化,与长期水解共同作用所致。与蒸馏水环境相比,海水环境下CFRP板材的层间剪切强度退化更加显著。(2)在蒸馏水和海水中浸泡后,两个碳纤维布样本均表现出明显的后固化效应,水分子在复合材料中的扩散降低了碳纤维布的拉伸性能和玻璃化转变温度。浸水对CFRP短梁试件的抗剪强度影响较小。Tw基碳纤维布试样的SBS退化是由裂纹扩展效应引起的。浸泡温度的升高导致热循环,这是短梁强度下降的原因之一。(3)从长期浸泡过程可以看出,两种浸泡介质对Tw基CFRP增强钢环的拉伸性能没有显著影响。在室温和80°C条件下,水浸试样的屈服强度均高于海水浸泡试样。(4)与未加固钢环的工作力相比,温湿耦合环境下Tw基CFRP加固钢环的极限破坏荷载较大.Tw基CFRP加固钢环具有显著的提升作用。

关键词： Tw基CFRP 钢管道湿热耐久性玻璃化转变力学性能界面粘结性能

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How to model the effect of mechanical erosion control practices at a catchment scale?

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International Soil and Water Conservation Research 2021年第3期9卷 370-380页

作者： Elizeu Jonas Didoné jean Paolo Gomes Minella daniel Gustavo Allasia Piccilli Federal University of Santa Maria Department of Soil Science1000 Avenue RoraimaCamobiCEP 97105-900Santa MariaRSBrazil Federal University of Santa Maria Department of Environmental and Sanitary Engineering1000 Avenue RoraimaCamobiCEP 97105-900Santa MariaRSBrazil

Conservation agriculture practices are a crucial factor in mitigating and controlling erosion by *** address water erosion estimates,most environmental models use the USLE,RUSLE,and MUSLE *** practices that affect soil erosion by modifying the flow pattern,such as contour farming,strip farming,or terracing,are represented within these models as a support practice(P)***,due to the difficulty in accurately mapping the P-factor,many studies choose to ignore it,using only the default value P-factor 1 which represents the absence of sowing at the level or cultivation in *** study proposes a methodology that evaluates the current P-factor based on the angle between the crop row orientation and the elevation contour *** method was tested in four areas under soybean crop fields in southern Brazil,totaling 25 km^(2).The reason for choosing four areas is to select different characteristics of rural properties and topographic *** ideal values of the P-factor are expected to be between 0.5 and 0.6;however,in our case,a P-factor greater than 0.8 was obtained in 60%of the area,indicating the low occurrence of contour farming reduces erosion *** results show that policymakers could potentially use this methodology(angle between the crop rows and contour lines)to run soil-erosion risk scenarios for a broader application of contour *** allows the P-factor to be quantified via a thematic map instead of assigning uniform P-factor *** a detailed study of the P-factor on the slopes,there is a better understanding of where to target support practices to reduce erosion.

关键词： Conservation practices Modeling P-Factor Planting efficiency

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Slowβrelaxation in La-based metallic glasses based on mechanical spectroscopy measurements

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Journal of Iron and Steel Research(International) 2017年第4期24卷 397-401页

作者： Ji-chao Qiao jean-marc Pelletier Ning Li daniel Crespo Yao Yao School of Mechanics Civil Engineering and ArchitectureNorthwestern Polytechnical UniversityXi'an 710072ShaanxiChina Universite de Lyon MATEISUMR-CNRS5510Bat.B.PascalINSA LyonF-69621 Villeurbanne cedexFrance Department of Materials Science and Engineering State Key Laboratory for Materials Processing and Die&Mould TechnologyHuazhong University of Science and TechnologyWuhan 430074HubeiChina Departament de Fisica EETACUniversitat Politecnica de CatalunyaCastelldfels 08860BarcelonaSpain

Dynamic mechanical relaxations of La-based metallic glasses were investigated by mechanical *** the framework of the mixing enthalpy of constituent atoms,it was found thatβrelaxation was less evident by the addition of Cu to replace Ni in the LaCuNiAl glassy *** introducing Cu into the LaNiAl metallic glass,the mixing enthalpy was less negative,which led to weakerβrelaxation of the metallic ***αrelaxation of the La-based metallic glasses could be described by a Kohlrausch-Williams-Watts（KWW）function with a Kohlrausch exponentβKWW around *** should be noted that physical aging above the glass transition temperature Tginduced a decrease ofβrelaxation intensity in the La-based metallic glass.

关键词： Metallic glass Mechanical relaxation βrelaxation Mixing enthalpy

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