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Cholesteryl ester storage disease of clinical and genetic characterisation: A case report and review of literature

world journal of Clinical Cases 2020年第9期8卷 1642-1650页

作者： elias Badal Rashu Anders Ellekær junker Karen Vagner Danielsen Emilie Dahl Ole Hamberg Line Borgwardt Vibeke Brix Christensen Nicolai j wewer Albrechtsen Lise L Gluud Gastrounit Copenhagen University Hospital HvidovreHvidovre 2650Denmark Department of Hepatology RigshospitaletCopenhagen UniversityCopenhagen 2100Denmark Centre of Genomic Medicine RigshospitaletCopenhagen UniversityCopenhagen 2100Denmark Department of Paediatrics and Adolescent Medicine RigshospitaletCopenhagen UniversityCopenhagen 2100Denmark Novo Nordisk Foundation Center for Protein Research Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagen 2200Denmark Department for Clinical Biochemistry RigshospitaletUniversity of CopenhagenCopenhagen 2200Denmark

BACKGROUND Cholesteryl ester storage disease(CESD)is a rare genetic *** symptoms and severity are highly *** is a systemic disease that can lead to the accumulation of fat and inflammation in the liver,as well as gastrointestinal and cardiovascular *** majority of patients require liver transplantation due to decompensated *** replacement therapy has been approved based on a randomized *** study aims to clinically and genetically evaluate two siblings with CESD who underwent liver transplantation,as well as their first-degree family *** SUMMARY The siblings were compound heterozygous for the missense variant in LIPA exon 8,c.894G>A,(***298Gln)and a single base pair deletion,c.482del(***161Ilefs*19).Analyses of single nucleotide polymorphisms showed variants with an increased risk of fatty liver disease and fibrosis for both ***,both patients show signs of recurrence of CESD in the liver after transplantation and additional gastrointestinal and cardiovascular signs of *** family members who were LIPA heterozygous had a lysosomal acid lipase activity below the reference *** of these carriers,a seven-year-old boy,was found to have severe dyslipidemia and was subsequently treated with *** Our study underlines that CESD is a multi-organ disease,the progression of which may occur post-liver *** findings underline the need for monitoring of complications and assessment of possible further treatment.

关键词： Lysosomal acid lipase deficiency Lysosomal storage disease Non-alcoholicfatty liver disease Liver transplantation Sebelipase alfa Case report

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Endoscopically managed sunflower bezoar

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Laparoscopic, Endoscopic and Robotic Surgery 2020年第2期3卷 48-51页

作者： Samih Hwalla elias Saikaly Melissa Kyriakos Saad Ihab El Hajj Saint George Hospital University Medical Center BeirutLebanon

Rectal bezoar is *** is important to admit the patient with huge colorectal phytobezoars to hospital for monitoring,especially if patient needs several endoscopic trials for *** presented a case of 14-year-old male patient with phytobezoars of sunflower seed extending from the recto sigmoid junction till the *** patient was successfully managed with endoscopic evacuation.

关键词： Bezoar Endoscopic disimpaction Colorectal surgery

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The Big Data Challenge:How to Develop a winning Strategy

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机械设计与制造工程 2012年第7X期41卷 53-55页

作者： Howard elias EMC Information Infrastructure and Cloud Services

Developing a winning Big Data strategy is challenging because it is as much about getting ahead of the trend and acquiring talent as it is about investing in new *** be successful you will need Data Scientists on your team,professionals who are adept with the analytical and visualization tools required to process and recognize patterns in data and who are equally comfortable with business concepts and ***'s Howard elias recommends three steps to help ensure your organization has the people it needs.

关键词： The Big Data Challenge

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Antioxidant and anti-diabetic activities in commercial and homemade pomegranate molasses in Lebanon

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Food Bioscience 2022年第2期46卷 338-345页

作者： Mira Bou Dargham jocelyne Matar Boumosleh Antoine Farhat Salwa Abdelkhalek elias Bou-Maroun Leina El Hosry Department of Nursing and Health Sciences Notre Dame University-LouaizeZouk MosbehLebanon Department of Rehabilitation and Health Services University of North TexasDentonTexasUSA Department of Health and Medical Sciences Khawarizmi International College Univ.Bourgogne Franche-Comt´e AgroSup DijonUMR A 02.102Proc´ed´es Alimentaires et MicrobiologiquesF-21000DijonFrance

Pomegranate fruit and its derived products are rich sources of bioactive *** have many biological *** molasses(PM)is a thick,traditional Middle Eastern syrup used in many recipes for Lebanese and international *** is a highly nutritious product which makes it of great *** purpose of this study was to assess the phytochemical composition,antioxidant and anti-diabetic properties of homemade and commercial PM consumed in Lebanon using samples collected from households in rural areas(n=4)and the market(n=28).The objectives of this study were to determine their total phenolic content using the Follin-Ciocalteu method,their total flavonoid content using aluminum chloride method,their antioxidant activity using DPPH radical scavenging as well as ferrous ion chelating assays and their anti-diabetic activity usingα-amylase andα-glucosidase inhibitory activities with acarbose,a known anti-diabetic drug,used as the standard *** PM samples exhibited a higher antioxidant activity than commercial samples,with the most active homemade PM sample having IC_(50)values of 0.09 mg/mL(DPPH radical scavenging assay)and 46.78%ferrous ion chelating assay).Homemade PM samples also showed higher anti-diabetic activity than commercial samples,with the most active homemade PM sample having an IC_(50)values of 0.63 mg/mL(α-amylase inhibitory activity)and of 0.41 mg/mL(α-glucosidase inhibitory activity)and being almost as active as acarbose with both enzymes(1.5 times).Significant inverse strong/moderate correlations were observed between total phenolic content/total flavonoid content and the IC_(50)value of DPPH radical scavenging assay,indicating positive associations between total phenolic and flavonoid contents and antioxidant *** addition,significant negative moderate correlations were observed between total phenolic/flavonoid contents andα-amylase inhibitory activity,indicating positive associations between total phenoli

关键词： Pomegranate molasses Antioxidant activity Ferrous ion chelating assay Anti-diabetic activity α-amylase inhibitory assay α-glucosidase inhibitory assay

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Blending with transition metals improves bioresorbable zinc as better medical implants

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Bioactive Materials 2023年第2期20卷 243-258页

作者： Yingchao Su jiayin Fu juncen Zhou elias Georgas Shaokang Du Yi-Xian Qin Yadong wang Yufeng Zheng Donghui Zhu Department of Biomedical Engineering Stony Brook UniversityStony BrookNYUSA Nancy E.and Peter C.Meinig School of Biomedical Engineering Cornell UniversityIthacaNYUSA Department of Cardiology Sir Run Run Shaw HospitalZhejiang UniversityHangzhouZhejiangChina Academy for Advanced Interdisciplinary Studies Peking UniversityBeijingChina School of Materials Science and Engineering Peking UniversityBeijingChina

Zinc(Zn)is a new class of bioresorbable metal that has potential for cardiovascular stent material,orthopedic implants,wound closure devices,***,pure Zn is not ideal for these applications due to its low mechanical strength and localized degradation *** is the most common/effective way to overcome this ***,the choice of alloying element is crucial to ensure the resulting alloy possesses sufficient mechanical strength,suitable degradation rate,and acceptable ***,we proposed to blend selective transition metals(i.e.,vanadium-V,chromium-Cr,and zirconium-Zr)to improve Zn’s *** selected transition metals have similar properties to Zn and thus are beneficial for the metallurgy process and mechanical ***,the biosafety of these elements is of less concern as they all have been used as regulatory approved medical implants or a component of an implant such as Ti6Al4V,CoCr,or Zr-based dental *** study showed the first evidence that blending with transition metals V,Cr,or Zr can improve Zn’s properties as bioresorbable medical *** addition,three in vivo implantation models were explored in rats:subcutaneous,aorta,and femoral implantations,to target the potential clinical applications of bioresorbable Zn implants.

关键词： Degradation Cardiovascular Orthopedic Subcutaneous Antibacterial

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Growth performance,welfare traits and meat characteristics of broilers fed diets partly replaced with whole Tenebrio molitor larvae

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Animal Nutrition 2023年第2期13卷 90-100页

作者： Stylianos Vasilopoulos Ilias Giannenas Soumela Savvidou Eleftherios Bonos Christos I.Rumbos elias Papadopoulos Paschalis Fortomaris Christos G.Athanassiou Laboratory of Nutrition Faculty of Veterinary MedicineAristotle University of Thessaloniki54124 ThessalonikiGreece Institute of Animal Science Hellenic Agricultural Organisation-DEMETERParalimni Giannitsa58100Greece Laboratory of Animal Science Nutrition and BiotechnologyDepartment of AgricultureSchool of AgricultureUniversity of IoanninaKostakioi Artas47100ArtaGreece Laboratory of Entomology and Agricultural Zoology Department of AgricultureCrop Production and Rural EnvironmentUniversity of ThessalyPhytokou str.38446N.IoniaVolosGreece Laboratory of Parasitology and Parasitic Diseases Faculty of Veterinary MedicineAristotle University of Thessaloniki54124ThessalonikiGreece Laboratory of Animal Husbandry Faculty of Veterinary MedicineAristotle University of Thessaloniki54124ThessalonikiGreece

The role of insects in animal nutrition has been reconsidered during recent years,paving the way for an increasing market for edible *** protein and amino acid balance make them a promising source of protein for replacing high value *** mealworm,Tenebrio molitor L.(TM;Coleoptera:Tenebrionidae)larvae,have shown positive effects on broiler performance in several research studies and have a strong potential as a sustainable alternative protein source for monogastric *** study aimed to assess the effect of replacing various ratios of basal diets with *** larvae on broiler performance as well as on several meat and welfare *** the study,120 one-day-old male chicks(Ross 308)were randomly allocated in 3 treatments and 4 replications(10 birds per pen).Birds of the control group(basal diet)were fed with typical commercial maize and soymeal-based rations in mash *** other 2 groups were treated with the same diet,after replacing 5%and 10%with dried TM larvae,*** d 35(end of trial),meat samples were collected and *** weight,feed intake,body weight gain and feed conversion ratio during the periods of 1 to 10 d(starter period),11 to 24 d(grower period),25 to 35 d(finisher period)and 1 to 35 d(total period)were ***-dermatitis,diarrhoea,feather score and litter conditions were also assessed during the *** results indicated that TM larvae inclusion in the broilers'diet positively affected body weight gain values,as well as the carcass yield,the meat composition and various welfare ***,the dietary treatments with TM larvae favourably affected meat composition and colour parameters,whereas there were also some positive effects on lipid and protein *** fatty acids were decreased by the dietary supplementation whereas the polyunsaturated fatty acids to SFA ratio *** general,the study showed that whole TM larvae addition can provide a promising alternat

关键词： Tenebrio molitor larvae Broiler Performance welfare Meat trait

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中国药品定价和补偿政策及其效果

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中国卫生政策研究 2017年第2期10卷 5-15页

作者： jia Hu elias Mossialos 英国伦敦政治经济学院

背景:近年来,中国药物政策存在着药品费用快速增长,以及质量、定价和提供能力等方面的问题,其解决的关键在于理清其中的定价和补偿问题。方法:本文较为系统收集了药物领域的学术文献和灰色文献,以及中国政府的官方统计数据,并结合中国... 详细信息

背景:近年来,中国药物政策存在着药品费用快速增长,以及质量、定价和提供能力等方面的问题,其解决的关键在于理清其中的定价和补偿问题。方法:本文较为系统收集了药物领域的学术文献和灰色文献,以及中国政府的官方统计数据,并结合中国国务院发展研究中心举办的研讨会信息,概述了中国的药品定价和补偿情况。结果:通过一个融合了供应方、代理方和需求方多方政策的框架,分析了定价与补偿政策。在目前药品政策的相互影响下,出现了药品价格高、质量低、不合理开药等问题。分立的监管环境也不利于药品政策的改革。结论:提高药品质量标准、市场集中度、政府补贴,以质量为导向的招标、扩大零差率政策的实施范围、建立补偿与合理开药的联动机制以及促进卫生技术评估与比较效能研究来改善定价和补偿政策。另外,整合广泛的监管分立体制、改善透明度和打击腐败等问题将有助于确保制定出具有一致性的、循证的政策。

关键词：药品政策定价补偿中国

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特应性皮炎患者皮肤的水通透屏障功能改变及其临床意义

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临床皮肤科杂志 2007年第10期36卷 671-673页

作者：蔄茂强 Peter M elias 美国加利福尼亚大学旧金山分校医学院加利福尼亚旧金山ca94121

皮肤的生理指标包括皮肤的水通透屏障功能、角质层的水分含量以及皮肤表面的pH值等,这些生理指标反映了机体和皮肤局部的生理状态。除角质层的水分含量降低以及皮肤表面的pH值升高外,特应性皮炎(AD)患者皮肤屏障功能明显降低。这一变化... 详细信息

皮肤的生理指标包括皮肤的水通透屏障功能、角质层的水分含量以及皮肤表面的pH值等,这些生理指标反映了机体和皮肤局部的生理状态。除角质层的水分含量降低以及皮肤表面的pH值升高外,特应性皮炎(AD)患者皮肤屏障功能明显降低。这一变化是AD表皮病变所致,其可影响表皮和真皮的功能。皮肤生理的改变程度与病情有关,通过改善皮肤屏障功能可以缓解AD病情。因此,正确地了解AD患者皮肤屏障功能的改变对于病情的判断和疾病的防治有一定的临床指导意义。

关键词：皮炎特应性屏障功能皮肤含水量角质层 pH值

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Is iron overload in alcohol-related cirrhosis mediated by hepcidin?

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world journal of Gastroenterology 2009年第46期15卷 5864-5866页

作者： Tariq Iqbal Azzam Diab Douglas G ward Matthew.j Brookes Chris Tselepis jim Murray Elwyn elias Department of Gastroenterology University Hospital BirminghamVincent Drive Depart ment of Cancer Studies University of BirminghamVincent Drive Department of Hematology University Hospital BirminghamVincent Drive

In this case report we describe the relationship between ferritin levels and hepcidin in a patient with alcohol-related spur cell anemia who underwent liver *** demonstrate a reciprocal relation-ship between serum or urinary hepcidin and serum ferritin,which indicates that inadequate hepcidin production by the diseased liver is associated with elevated serum *** ferritin level falls with increasing hepcidin production after *** inflammatory indices(IL6)nor erythropoietin appear to be related to hepcidin expression in this *** suggest that inappropriately low hepcidin production by the cirrhotic liver may contribute substantially to elevated tissue iron stores in cirrhosis and speculate that hepcidin replacement in these patients may be of therapeutic benefi t in the future.

关键词： Alcohol Iron Anaemia Hepcidin Cirrhosis

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Preparedness,knowledge,and perception of nursing students about climate change and its impact on human health in India

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Regional Sustainability 2024年第1期5卷 107-118页

作者： Ramya Kundayi RAVI Priya BABY Nidhin elias jisa George THOMAS Kathyayani Bidadi VEERABHADRAIAH Bharat PAREEK College of Nursing Medical and Health Sciences UniversityRas Al Khaimah11172United Arab Emirates College of Nursing National Institute of Mental Health and Neuro sciencesBangalore560011India College of Nursing Amrita Institute of Medical SciencesKochi682041India College of Nursing All India Institute of Medical SciencesBhopal462020India Saraswati Professional and Higher Education College of Nursing Mohali140413India

Higher education plays an important role in all aspects of *** climate change and sustainability into the curriculum is important to prepare future health-care professionals for climate change and sustainability actions to build resilient health *** this study,we evaluated the preparedness,knowledge,and perception regarding climate change and its impact on human health among Indian nursing students.A quantitative descriptive survey was conducted among 644 undergraduate nursing students studying in three institutes in the southern,northern,and western parts of *** used a structured online questionnaire with established validity and reliability to collect *** data were analyzed using Statistical Package for the Social Sciences version 26 *** results showed that more than half of the students reported experiencing excessive increase(362;56.2%)and decrease(374;58.1%)in temperature in recent *** more than three quarters of the students(494;76.7%)accepted the general truth that climate change is mainly caused by human activities,only less than half of the students(309;48.0%)perceived the impact of climate change to be *** quarters of the students(483;75.0%)had a high level of knowledge regarding climate *** students who had a high level of knowledge about climate change exerted a high perceived impact of climate change(χ^(2)=75.47;Pwere highly confident about engaging in climate change-related conversation with *** of the students who participated in the study had basic knowledge about climate change and its impact on human ***,there is an observed gap between knowledge and their preparedness and confidence to engage in climate change ***,we propose to strengthen the nursing curriculum by integra

关键词： Climate change Curriculum Nursing students Human health India

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