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Targeted therapy for capillary-venous malformations

Signal Transduction and Targeted Therapy 2024年第7期9卷 3024-3039页

作者： Lola Zerbib Sophia Ladraa Antoine Fraissenon Charles Bayard Marina Firpion Quitterie Venot Sanela Protic Clément Hoguin Amandine Thomas Sylvie Fraitag Jean-paul Duong Sophie Kaltenbach Estelle Balducci Coline Lefevre Patrick Villarese Vahid Asnafi Christine Broissand Nicolas Goudin Ivan Nemazanyy Gwennhael Autret Bertrand Tavitian Christophe Legendre Nadia Arzouk Veronique Minard-Colin Caroline Chopinet Michael Dussiot Denise MAdams Tristan Mirault Laurent Guibaud paul Isenring Guillaume Canaud UniversitéParis Cité ParisFrance INSERM U1151 Institut Necker-Enfants MaladesParisFrance Service d’Imagerie Pédiatrique Hôpital Femme-Mère-EnfantHCLBronFrance CREATIS UMR 5220 Villeurbanne 69100France Service de Radiologie Mère-Enfant Hôpital NordSaint EtienneFrance Service d’Anatomie pathologique Hôpital Necker-Enfants MaladesAP-HPParisFrance Laboratoire d’Oncohématologie Hôpital Necker-Enfants MaladesAP-HPParisFrance Pharmacie Hôpital Necker-Enfants MaladesAP-HPParisFrance Necker Bio-Image Analysis INSERM US24/CNRS UMS 3633ParisFrance Platform for Metabolic Analyses Structure Fédérative de Recherche NeckerINSERM US24/CNRS UMS 3633ParisFrance Plateforme Imageries du Vivant Universitéde ParisPARCCINSERMParisFrance Service de Néphrologie Transplantation AdultesHôpital Necker-Enfants MaladesAP-HPParisFrance Service de Transplantation Hôpital La PitiéSalpétrièreAP-HPParisFrance Department of Pediatric and Adolescent Oncology INSERM 1015Gustave RoussyUniversitéParis-SaclayVillejuifFrance Service de Physiologie&Explorations Fonctionnelles Cardiovasculaires CHU de LilleLille 59000France INSERM U1163 Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutic ImplicationsLaboratoire d’Excellence GR-ExParisFrance Division of Oncology Comprehensive Vascular Anomalies ProgramChildren’s Hospital of PhiladelphiaPhiladelphiaPAUSA Service de Médecine Vasculaire hôpital Européen Georges-PompidouParisFrance Nephrology Research Group L’Hôtel-Dieu de Québec Research CenterDepartment of MedicineFaculty of MedicineLaval UniversityQuebecQC G1R2J6Canada Unitéde médecine translationnelle et thérapies ciblées Hôpital Necker-Enfants MaladesAP-HPParisFrance CNRS UMR8253 Institut Necker-Enfants MaladesParisFrance

Sporadic venous malformations are genetic conditions primarily caused by somatic gain-of-function mutation of PIK3CA or TEK,an endothelial transmembrane receptor signaling through *** malformations are associated with pain,bleedings,thrombosis,pulmonary embolism,esthetic deformities and,in severe cases,life-threatening *** authorized medical treatment exists for patients with venous ***,we created a genetic mouse model of PIK3CA-related capillary venous malformations that replicates patient *** showed that these malformations only partially signal through AKT *** compared the efficacy of different drugs,including rapamycin,a mTORC1 inhibitor,miransertib,an AKT inhibitor and alpelisib,a PI3Kαinhibitor at improving the lesions seen in the mouse *** demonstrated the effectiveness of alpelisib in preventing vascular malformations’occurrence,improving the already established ones,and prolonging *** these findings,we were authorized to treat 25 patients with alpelisib,including 7 children displaying PIK3CA(n=16)or TEK(n=9)-related capillary venous malformations resistant to usual therapies including sirolimus,debulking surgical procedures or percutaneous *** assessed the volume of vascular malformations using magnetic resonance imaging(MRI)for each *** demonstrated improvement in all 25 *** malformations previously considered intractable were reduced and clinical symptoms were *** showed a decrease of 33.4%and 27.8%in the median volume of PIK3CA and TEK malformations respectively,over 6 months on *** conclusion,this study supports PI3Kαinhibition as a promising therapeutic strategy in patients with PIK3CA or TEK-related capillary venous malformations.

关键词： PIK3CA malformations venous

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Smoking cessation prior to elective total joint arthroplasty results in sustained abstinence postoperatively

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World Journal of Orthopedics 2024年第7期15卷 627-634页

作者： Billy Insup Kim Jeffrey O'Donnell Colleen M Wixted Thorsten Markus Seyler William A Jiranek Michael paul Bolognesi Sean Patrick Ryan Department of Orthopedic Surgery Hospital for Special SurgeryNew YorkNY 10021United States Department of Orthopedic Surgery New York UniversityNew YorkNY 10003United States Department of Orthopedic Surgery Duke University HospitalDurhamNC 27560United States

BACKGROUND Tobacco use is a well-documented modifiable risk factor for perioperative *** To determine the tobacco abstinence rates of patients who made cessation efforts prior to a total joint arthroplasty(TJA)*** A retrospective evaluation was performed on 88 self-reported tobacco users who underwent TJA between 2014-2022 and had tobacco cessation dates within 3 mo of *** patients were contacted via phone survey to understand their tobacco use pattern,and patient reported outcomes.A total of 37 TJA patients *** Our cohort was on average 61-years-old,60%(n=22)women,with an average body mass index of 30 kg/m^(2).The average follow-up time was 2.9±1.9 years.A total of 73.0%(n=27)of patients endorsed complete abstinence from tobacco use prior to *** cessation methods were used perioperatively including prescription therapy(13.5%),over the counter nicotine replacement(18.9%),cessation programs(5.4%).At final follow up,43.2%(n=16)of prior tobacco smokers reported complete *** who were able to maintain cessation postoperatively had improved Patient-Reported Outcomes Measurement Information System(PROMIS)-10 mental health scores(49 vs 58;P=0.01),and hip dysfunction and osteoarthritis outcome score for joint replacement(***)scores(63 vs 82;P=0.02).No patients in this cohort had a prosthetic joint infection or required revision *** We report a tobacco cessation rate of 43.2%in patients undergoing elective TJA nearly 3 years *** undergoing TJA who were able to remain abstinent had improved PROMIS-10 mental health scores and *** *** perioperative period provides clinicians a unique opportunity to assist active tobacco smokers with cessation efforts and improve postoperative outcomes.

关键词： Smoking cessation Total joint arthroplasty Outcomes Tobacco use Postoperative abstinence

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iDEAL-CIO: Instant Digital Express Advocated “Magic Lamp” for Cloud Intelligence Outlet

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Intelligent Information Management 2024年第1期16卷 35-63页

作者： Sheldon Liang paul Anthony Miller Brendan Shane Rowe Alexis Rainbow Digital Innovation for Music Education Library and STEM Lane College Jackson TN USA

Instant Digital Express iDEAL-CIO The “Magic Lamp” for Cloud Intelligence Outlet, which has been recommended, combines innovations to satisfy modern users’ needs and efficiently sift through the ever-expanding amount of intelligent content stored in the cloud. One such innovation introduces a ground-breaking concept to remove superfluous and outdated sequential search patterns that overwhelm the user and computer in order to better serve the user in an eclectic & elastic and multidimensional approach to finding, grouping, assimilation, organizing, and delivering archival content. The cloud intelligence outlet (CIO) is presented in this article as the perfect magic lamp option for quick digital express advocacy. The grouping, indexing, folding, and targeting (GIFT) method of multidimensional online synthetic/analytical intelligent content (MOSAIC) for adaptive intelligence is the fundamental intelligent aggregation and automated process of the Magic Lamp. Three perspectives above this new ideal framework are available to observe: The Magic Lamp proposes contextual and multiple analytical tracks to improve cloud intelligence services conceptually. Technically speaking, MOSAIC combines domain-specific services for a wide range of international users, and through the usage of Cloud Intelligence Outlet, GIFT operationally activates grouping, indexing, folding, and targeting to promote decent experience and in-depth research on target for users’ wants. Because of this, iDEAL-CIO works in tandem with cloud extraction, digital transformation, and archival loading to provide improved service through the readily accessible cloud intelligence outlet.

关键词： iDEAL-CIO MOSAIC Multidimensional Online Synthetic/Analytical Intelligent Content

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Comparative Study of Exhaust Emissions from Diesel and Syngas Powered 3.5 kW Compression Ignition Engine with and without Load

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Journal of Power and Energy Engineering 2024年第8期12卷 30-46页

作者： Benson Kariuki paul Njogu Joseph Kamau Robert Kinyua Sameer Bachani Institute of Energy and Environmental Technology Jomo Kenyatta University of Agriculture & Technology Nairobi Kenya Department of Mechanical and Automotive Engineering Technical University of Mombasa Mombasa Kenya

Despite diesel engines being highly efficient, with low fuel consumption and reduced carbon dioxide emissions, they emit relatively high levels of particulate matter and oxides of nitrogen (NOx) due to high exhaust gas temperatures. Engine emissions show the quality and completeness of combustion. This paper aims to present the results of a study comparing exhaust emissions from a diesel and syngas powered engine. Syngas was produced from co-firing coal and biomass in a gasifier then cleaned, cooled and applied as an alternative fuel in an engine operated from 0 - 100% load. Exhaust-emissions were monitored at this load conditions. The exhaust-temperature was measured using thermocouples and the emission gases were analyzed using Testo 350. The emissions were lower and decreased as the engine load increased, except for sulphur dioxide and NOx. The study shows that levels of carbon monoxide, were higher in a range of 46.5 - 80.2%, while carbon dioxide was 3.3 - 18% higher compared to those from diesel. Hydrocarbon emissions were 480 and 1250 ppm for diesel and syngas respectively. The study reveals that the engine operates optimally at higher loads since hydrocarbons and oxides of carbon are low due to complete combustion at higher temperatures. Exhaust gas temperature was higher in the syngas fuel and increased as the engine load increased in the range of 455.83 - 480.03˚C which influenced the formation of NOx. NOx from diesel was found to be higher, ranging from 32.5 - 40.5%, compared to those from syngas with an engine load of 75%. The study observed that relative to diesel, the emissions of sulfur dioxide at 50% engine load were lower in a range of 23.7 - 57.1%. Emissions of hydrocarbons depended on the degree of substitution of diesel and engine load. The study therefore shows that, relative to diesel, emissions decreased when syngas was used with upgraded syngas from Prosporis juliflora presenting as the best alternative followed by Hyphanae compressa, and last

关键词： Emissions Engine Load Temperature Neat-Diesel Syngas

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Levels of Polycyclic Aromatic Hydrocarbons (PAHs) in Healthcare Waste Incinerators’ Bottom Ash from Five County Hospitals in Kenya

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Journal of Environmental Protection 2024年第3期15卷 318-337页

作者： Muriithi Jackson Githinji paul Mwangi Njogu Zipporah Nganga Mohamed Karama Institute of Tropical Medicine and Infectious Diseases (ITROMID) Jomo Kenyatta University of Agriculture and Technology Nairobi Kenya Institute of Energy and Environmental Technology Jomo Kenyatta University of Agriculture and Technology Nairobi Kenya Academic Research and Student Affairs South Eastern Kenya University Kitui Kenya Public Health and Epidemiology Umma University Kajiado Kenya

Health-care waste contains potentially harmful microorganisms and compounds which can infect and affect hospital patients, healthcare workers, the general public and environment. Therefore, management of health care waste requires safe handling, treatment and disposal procedures. While incineration reduces the volume and quantity of waste for final disposal, it leads to the production of fly and bottom ashes laden with toxic incomplete combustion products such as Polycyclic Aromatic Hydrocarbons (PAHs), dioxins, furans and heavy metals. This exposes workers who handle and dispose the bottom ashes, hospital patients, the general public and environment. The goal of this study was to determine the total and individual levels of 16 most prevalent and toxic PAHs. Bottom ash samples were collected from incinerators in five county hospitals in Kenya, namely;Moi-Voi, Narok, Kitale, Makindu and Isiolo. Bottom ash samples were collected over a period of six months from the five hospitals. The samples were then sieved, homogenised and stored at 4°C in amber coloured glass containers. The PAHs were extracted using 30 ml of a hexane-acetone solvent (1:1) mixture by ultrasonication at room temperature (23°C) for 45 minutes. The PAHs were then analyzed with a GC-MS spectrophotometer model (Shimadzu GCMS-QP2010 SE) connected to a computer work station was used for the PAHs analysis. The GC-MS was equipped with an SGE BPX5 GC capillary column (30 m × 0.25 mm × 0.25 μm) for the separation of compounds. Helium was used as the carrier gas at a flow rate of 15.5 ml/minute and 14.5 psi. 1 μl of the sample was injected at 280°C, split mode (10:1). The oven programming was set for a total runtime of 40 minutes, which included: 100°C (2-minute hold);10°C /min rise to 200°C;7°C /min rise to 249°C;3°C /min rise to 300°C (2-minute hold). The interface temperature was set at 290°C. Analysis was done in Selected Ion Monitoring (SIM) mode and the peak areas of each of the PAHs were collected from

关键词： PAHs GC-MS Healthcare Wastes Disposal Incinerator Bottom Ash

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Development and Application of Integrated Indicators for Assessing Healthcare Waste Management Systems in Kenyan Hospitals

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Open Journal of Applied Sciences 2024年第4期14卷 1080-1120页

作者： Muriithi Jackson Githinji paul Mwangi Njogu Zipporah Nganga Mohamed Karama Institute of Tropical Medicine and Infectious Diseases (ITROMID) Jomo Kenyatta University of Agriculture and Technology Nairobi Kenya Institute of Energy and Environmental Technology Jomo Kenyatta University of Agriculture and Technology Nairobi Kenya Academic Research and Student Affairs South Eastern Kenya University Kitui Kenya School of Public Health Amref International University Nairobi Kenya

Healthcare waste management (HCWM) is an important aspect of healthcare delivery globally because of its hazardous and infectious components that have potential for adverse health and environmental impacts. The paper introduces a set of indicators for assessing HCWM systems in hospitals. These indicators are: HCWM policies and standard operating procedures, management and oversight, logistics and budget support, training and occupational health and safety, and treatment, disposal and waste treatment equipment housing. By plotting a mark on a continuum which is defined as good and poor on the extremes and is connected with all other marks in a spoke arrangement, it’s possible to describe a baseline for HCWM in any specific hospital. This baseline can be used to improve awareness of the actors and policy-makers, compare the same hospital at a different point in time, to compare observations by different evaluators and to track improvements. Results suggest that in Kenya, the application of such indicators is useful for evaluating which priorities should be addressed to improve outcomes in HCWM systems. Systematic sampling technique was used to identify and collect data by use of observational checklist, interviews, visual verification and review of documents and a HCWM assessment tool. The objective is to suggest an integrated management tool as a method to identify prevailing problems with a HCWM system. The method can be replicated in other contexts worldwide, with a focus on the developing world. The integrated indicators focus on management of HCW and not its potential impact on human health and environment, an area recognized to be critical for future research.

关键词： Developing Countries HCW Management Indicators Management Tools

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滇金丝猴夜宿树的选择及夜宿地的利用方式

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兽类学报 2024年第1期44卷 37-49页

作者：任宝平吴瑞东黎大勇 paul AGarber 李明海南师范大学生命科学学院热带岛屿生态学教育部重点实验室海南省热带动植物生态学重点实验室海口571158 中国国科学院动物研究所动物生态与保护生物学院重点实验室北京100101 云南大学国际河流与生态学研究院昆明650091 西华师范大学西南野生动植物资源保护教育部重点实验室南充637009 Department of Anthropology and Program in Ecology Evolutionand Conservation BiologyUniversity of IllinoisUrbana 61801USA 大理大学国际生物多样性与灵长类保护中心大理671003 中国科学院动物进化与遗传前沿交叉卓越创新中心昆明650223

野生灵长类夜宿地的利用方式可以明确地反映一个物种特有的生境利用方式和生存之道。2003年12月至2004年10月,我们利用可自动脱落GPS无线电项圈对云南省丽江市金丝厂的一个滇金丝猴(Rhinopithecus bieti)群体的活动进行了持续跟踪记录... 详细信息

野生灵长类夜宿地的利用方式可以明确地反映一个物种特有的生境利用方式和生存之道。2003年12月至2004年10月,我们利用可自动脱落GPS无线电项圈对云南省丽江市金丝厂的一个滇金丝猴(Rhinopithecus bieti)群体的活动进行了持续跟踪记录。本研究着重于对所研究猴群夜宿树的选择和夜宿地的利用方式的考查,并结合可能影响夜宿地选择和利用的环境因素,比如天气、季节、日均温度等做了系统分析。研究群计有180余只个体,家域面积约27.8 km^(2)。GPS项圈记录到夜宿树的有272个夜晚,由此我们确认了131个夜宿地。其中70个(54.3%)夜宿地仅利用了一次,剩余的则不同程度地多次利用(2~9次)。在这些重复利用的夜宿地中,持续利用同一夜宿点的情形共发生了19次,其中连续3个夜晚在同一夜宿地过夜的现象出现了3次,剩下的16次是连续利用同一夜宿地2次。这种连续利用同一夜宿地的情况占重复利用同一夜宿的7.0%,发生频率不高,而且几乎都出现在冬季(84.0%)。滇金丝猴对于同一夜宿地的重复造访的时间间隔约50 d。一旦发生连续重复利用的情况,猴群当天的移动距离显著缩短(527 m vs.884 m),降低了群体移动所必需的能量消耗。明显地,滇金丝猴夜宿点的选择受其当天下午和第二天早上觅食点的位置的影响。鉴于较大的群体和明显回避夜宿地重复利用的特性,提示这是猴群对家域内食物分布的行为响应,避免对同一地点的过度利用造成食物的不足。采用大量(131个)而分散的夜宿地利用方式会保证猴群能够获得充足的食物供应。此外,相较于其他树种,滇金丝猴更喜欢在云南铁杉(Tsuga dumosa)树上过夜,而且尽量不以夜宿点作为觅食点,可能与卫生和安全有关。

关键词：过夜树夜宿地移动模式滇金丝猴

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Nonlinear Rydberg exciton-polaritons in Cu2O microcavities

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Light(Science & Applications) 2024年第4期13卷 619-627页

作者： Maxim Makhonin Anthonin Delphan Kok Wee Song paul Walker Tommi Isoniemi Peter Claronino Konstantinos Orfanakis Sai Kiran Rajendran Hamid Ohadi Julian Heckötter Marc Assmann Manfred Bayer Alexander Tartakovskii Maurice Skolnick Oleksandr Kyriienko Dmitry Krizhanovskii Department of Physics and Astronomy University of SheffieldSheffield S37RHUK. Department of Physics and Astronomy University of ExeterStocker RoadExeter EX44PYUK. SUPA School of Physics and AstronomyUniversity of St AndrewsSt Andrews KY169SSUK. Fakultät Physik TU DortmundAugust-Schmidt-Straße 444227 DortmundGermany

Rydberg excitons(analogues of Rydberg atoms in condensed matter systems)are highly excited bound electron-hole states with large Bohr *** interaction between them as well as exciton coupling to light may lead to strong optical nonlinearity,with applications in sensing and quantum information ***,we achieve strong effective photon–photon interactions(Kerr-like optical nonlinearity)via the Rydberg blockade phenomenon and the hybridisation of excitons and photons forming polaritons in a Cu2O-filled *** pulsed resonant excitation polariton resonance frequencies are renormalised due to the reduction of the photon-exciton coupling with increasing exciton *** analysis shows that the Rydberg blockade plays a major role in the experimentally observed scaling of the polariton nonlinearity coefficient as∝n4.4±1.8 for principal quantum numbers up to n=*** high principal quantum numbers studied in a polariton system for the first time are essential for realisation of high Rydberg optical nonlinearities,which paves the way towards quantum optical applications and fundamental studies of strongly correlated photonic(polaritonic)states in a solid state system.

关键词： density Rydberg polar

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Identification of the Diffusion Coefficients of Pollutants ( N O 3 - and Mn2+) through the Walls of Concrete Tank and Vulnerability of the Stored Water Quality

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Engineering（科研） 2024年第9期16卷 246-274页

作者： Narcisse Malanda Bienvenu Ebata Ndion Christian Tathy Thibault Mongo paul Louzolokimbembe Nation School Superior Polytechnic Marien Ngouabi University Brazzaville Republic of Congo Superior Institute Architecture Town Planning Building and Public Works University of Dnis Sassou Nguesso Kintl Republic of Congo

The populations of urban centers in Congo-Brazzaville have decided to develop various methods of water storage (concrete or masonry underground tanks) for domestic use, due to shortages in the distribution of water through the public network. However, questions remain as to the physico-chemical quality of the water stored in these tanks, when these structures are built in wet and relatively polluted areas. This paper presents a model of pollutant diffusion through the cementitious matrix (concrete) of tank walls simulated at a buried reservoir. The results of the experimental and numerical simulations show that certain concrete parameters, such as porosity, permeability and diffusivity, have a significant influence on the transfer of pollutants through the concrete walls, thus altering the physico-chemical quality of the stored water. The numerical models (1D) used to predict pollutant transfer and the quality of the stored water are consistent with those of the optimal control for identifying the diffusion coefficient. Major ion concentrations appear to be correlated with system porosity and diffusion coefficient. Nevertheless, the identification of the diffusion coefficient from the optimal control method, based on an explicit numerical resolution of a finite volume PDE for the approximation of the experiment, is not consistent with that of the optimal control method.

关键词： Buried Tank Water Concrete Modelling Optimal Control Diffusion Coefficient

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Surface Metallization of Glass Fiber(GF)/Polyetheretherketone(PEEK) Composite with Cu Coatings Deposited by Magnetron Sputtering and Electroplating

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Journal of Wuhan University of Technology(Materials Science) 2024年第1期39卷 213-220页

作者：钟利金凡亚朱剑豪 TONG Honghui DAN Min Southwest Institute of Physics Chengdu 610000China City University of Hong Kong Tat Chee AvenueKowloonHong Kong 999077China

Surface metallization of glass fiber(GF)/polyetheretherketone(PEEK)[GF/PEEK] is conducted by coating copper using electroplating and magnetron sputtering and the properties are determined by X-ray diffraction(XRD), scanning electron microscopy(SEM), and electron backscatter diffraction(EBSD).The coating bonding strength is assessed by pull-out tests and scribing in accordance with GB/T *** results show that the Cu coating with a thickness of 30 μm deposited on GF/PEEK by magnetron sputtering has lower roughness, finer grain size, higher crystallinity, as well as better macroscopic compressive stress,bonding strength, and electrical conductivity than the Cu coating deposited by electroplating.

关键词： surface metallization Cu coating magnetron sputtering electroplating

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