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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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Re-evaluation of recent research on metabolic utilization of energy in poultry: Recommendations for a net energy system for broilers

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Animal Nutrition 2024年第1期16卷 62-72页

作者： jean Noblet Aye-Cho Tay-Zar Shu-Biao Wu Pairat Srichana Pierre Cozannet Pierre-Andre Geraert Mingan Choct INRAE Charoen Pokphand Foods Public Company Limited (CPF) School of Environmental and Rural Science University of New England Adisseo France S.A.S.

Different energy systems have been proposed for energy evaluation of feeds for domestic animals. The oldest and most commonly used systems take into account the fecal energy loss to obtain digestible energy(DE), and fecal, urinary and fermentation gases energy losses to calculate metabolizable energy(ME). In the case of ruminants and pigs, the net energy(NE) system, which takes into account the heat increment associated with the metabolic utilization of ME, has progressively replaced the DE and ME systems over the last 50 years. For poultry, apparent ME(AME) is used exclusively and NE is not yet used widely. The present paper considers some important methodological points for measuring NE in poultry feeds and summarizes the available knowledge on NE systems for poultry. NE prediction equations based on a common analysis of three recent studies representing a total of 50 complete and balanced diets fed to broilers are proposed; these equations including the AME content and easily available chemical indicators have been validated on another set of 30 diets. The equations are applicable to both ingredients and complete diets. They rely primarily on an accurate and reliable AME value which then represents the first limiting predictor of NE value. Our analysis indicates that NE would be a better predictor of broiler performance than AME and that the hierarchy between feeds is dependent on the energy system with a higher energy value for fat and a lower energy value for protein in an NE system. Practical considerations for implementing such an NE system from the commonly used AME or AMEn(AME adjusted for zero nitrogen balance) systems are presented. In conclusion, there is sufficient information to allow the implementation of the NE concept in order to improve the accuracy of feed formulation in poultry.

关键词： Poultry Feed evaluation Net energy Energy system

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From Control Theory to Gravitational Waves

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Advances in Pure Mathematics 2024年第2期14卷 49-100页

作者： jean-Francois Pommaret CERMICS Ecole des Ponts Paris Tech Paris France

When D:ξ→η is a linear ordinary differential (OD) or partial differential (PD) operator, a “direct problem” is to find the generating compatibility conditions (CC) in the form of an operator D1:η→ξ such that Dξ = η implies D1η = 0. When D is involutive, the procedure provides successive first-order involutive operators D1,...,Dn when the ground manifold has dimension n. Conversely, when D1 is given, a much more difficult “inverse problem” is to look for an operator D:ξ→η having the generating CC D1η = 0. If this is possible, that is when the differential module defined by D1 is “torsion-free”, that is when there does not exist any observable quantity which is a sum of derivatives of η that could be a solution of an autonomous OD or PD equation for itself, one shall say that the operator D1 is parametrized by D. The parametrization is said to be “minimum” if the differential module defined by D does not contain a free differential submodule. The systematic use of the adjoint of a differential operator provides a constructive test with five steps using double differential duality. We prove and illustrate through many explicit examples the fact that a control system is controllable if and only if it can be parametrized. Accordingly, the controllability of any OD or PD control system is a “built in” property not depending on the choice of the input and output variables among the system variables. In the OD case and when D1 is formally surjective, controllability just amounts to the formal injectivity of ad(D1), even in the variable coefficients case, a result still not acknowledged by the control community. Among other applications, the parametrization of the Cauchy stress operator in arbitrary dimension n has attracted many famous scientists (G. B. Airy in 1863 for n = 2, J. C. Maxwell in 1870, E. beltrami in 1892 for n = 3, and A. Einstein in 1915 for n = 4).

关键词： Differential Operator Differential Sequence Killing Operator Riemann Operator Bianchi Operator Cauchy Operator Control Theory Controllability Elasticity General Relativity

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Severe asthma exacerbation:Changes in patient characteristics,management,and outcomes from 1997 to 2016 in 40 ICUs in the greater Paris area

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Journal of Intensive Medicine 2024年第2期4卷 209-215页

作者： Romy Younan jean Loup Augy Bertrand Hermann Bertrand Guidet Philippe Aegerter Emmanuel Guerot Ana Novara Caroline Hauw-Berlemont Amer Hamdan Clotilde Bailleul Francesca Santi jean-Luc Diehl Nicolas Peron Nadia Aissaoui Intensive Care Unit AP-HPGeorges Pompidou European HospitalUniversitéde ParisParisFrance Intensive Care Unit AP-HPSaint Antoine HospitalUniversités de SorbonneUniversitéPierre et Marie CurieParisFrance INSERM U1136 ParisFrance Versailles Saint-Quentin-en-Yvelines University INSERM U1018Groupe Interrégional de Recherche Clinique et d'InnovationÎle-de-FranceFrance Respiratory Medicine Department AP-HPGeorges Pompidou European HospitalUniversitéde ParisParisFrance Innovative Therapies in Hemostasis INSERM UMR-S1140Universitéde ParisParisFrance Intensive Care Unit and Biosurgical Research Lab(Carpentier Foundation) AP-HPGeorges Pompidou European HospitalParisFrance Paris-Cardiovascular-Research-Center INSERM U970ParisFrance

Background Despite advances in asthma treatments,severe asthma exacerbation(SAE)remains a life-threatening condition in adults,and there is a lack of data derived from adult patients admitted to intensive care units(ICUs)for *** current study investigated changes in adult patient characteristics,management,and outcomes of SAE over a 20-year period in 40 ICUs in the greater Paris *** In this retrospective observational study,admissions to 40 ICUs in the greater Paris area for SAE from January 1,1997,to December 31,2016 were *** primary outcome was the proportion of ICU admissions for SAE during 5-year *** outcomes were ICU and hospital mortality,and the use of mechanical ventilation and *** analysis was performed to assess factors associated with ICU *** A total of 7049 admissions for SAE were *** each 5-year period,the proportion decreased over time,with SAE accounting for 2.84%of total ICU admissions(n=2841)between 1997 and 2001,1.76%(n=1717)between 2002 and 2006,1.05%(n=965)between 2007 and 2011,and 1.05%(n=1526)between 2012 and *** median age was 46 years(interquartile range[IQR]:32–59 years),55.41%were female,the median Simplified Acute Physiology Score II was 20(IQR:13–28),and 19.76%had mechanical *** use of mechanical ventilation remained infrequent throughout the 20-year period,whereas the use of catecholamine *** and hospital mortality rates *** associated with ICU mortality were renal replacement therapy,catecholamine,cardiac arrest,pneumothorax,acute respiratory distress syndrome,sepsis,and invasive mechanical ventilation(IMV).Non-survivors were older,had more severe symptoms,and were more likely to have received *** ICU admission for SAE remains uncommon,and the proportion of cases decreased over *** a slight increase in symptom severity during a 20-year period,ICU and hospital mortality *** requirin

关键词： Epidemiology Intensive care unit Mechanical ventilation Severe asthma exacerbation

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Pd_(20)Pt_(20)Cu_(20)Ni_(20)P_(20)高熵金属玻璃的热稳定性和热塑成形性

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稀有金属材料与工程 2024年第1期53卷 1-7页

作者： Cardinal Sandrine Pelletier jean-Marc Kato Hidemi 里昂大学里昂国立应用科学学院法国里昂F-69621 东北大学金属材料研究所日本仙台980-8577

采用差示扫描量热法、X射线衍射和热力学分析研究了Pd_(20)Pt_(20)Cu_(20)Ni_(20)P_(20)高熵金属玻璃(HEMG)的热稳定性和热力学性能。结果表明,与其他经典贵金属基金属玻璃相比,Pd_(20)Pt_(20)Cu_(20)Ni_(20)P_(20)HEMG具有相当的性能... 详细信息

采用差示扫描量热法、X射线衍射和热力学分析研究了Pd_(20)Pt_(20)Cu_(20)Ni_(20)P_(20)高熵金属玻璃(HEMG)的热稳定性和热力学性能。结果表明,与其他经典贵金属基金属玻璃相比,Pd_(20)Pt_(20)Cu_(20)Ni_(20)P_(20)HEMG具有相当的性能和鲜明的特点。

关键词：高熵合金金属玻璃热塑性成形结晶

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Matrix Assisted Laser Desorption Ionization Time of Flight(MALDI-TOF)-Mass Spectrometry and^(13)C-NMR-Identified New Compounds in Paraberlinia bifoliolata(Ekop-beli)Bark Tannins

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Journal of Renewable Materials 2024年第3期12卷 553-568页

作者： Liliane Nga Benoit Ndiwe Achille Bernard Biwolé Antonio Pizzi jean Jalin Eyinga Biwole Joseph Zobo Mfomo Laboratory of Forest Resources and Wood Valorization Training Unit in Engineering SciencesPost Graduate School of Fundamental and Applied SciencesUniversity of DoualaP.O.Box 1872DoualaCameroun Laboratory of Mechanics Training Unit in Engineering SciencesPost Graduate School of Fundamental and Applied SciencesUniversity of DoualaP.O.Box 2701DoualaCameroun Laboratory of Studies and Research on Wood Material(LERMAB) University of LorraineNancyFrance

Extracts of plant origin,particularly tannins,are attracting growing interest for the sustainable development of materials in the industrial *** discovery of new tannins is therefore *** aim of this work was to contribute to the understanding of the properties of Paraberlinia bifoliolata tannin by Matrix Assisted Laser Desorption Ionization Time of Flight Mass Spectroscopy MALDI-TOF/MS and Carbon 13 Nuclear Magnetic Resonance(13C NMR).The chemical composition of tannin extracted from Paraberlinia bifoliolata bark was determined,as was the mechanical strength of the resin hardened with Acacia nilotica *** by successive water extraction was 35%.MALDI-TOF/MS analysis revealed the presence of three new compounds in this tannin,previously unknown in this family of *** are 3-hydroxyproline acid,N-methyl-4-hydroxypipecolic acid and N-methyl-5-dihydroxypipecolic *** identification of the above molecules means that this tannin can be used for industrial applications,as a resin in the manufacture of particleboard and in the formulation of green corrosion *** information is reinforced by 13C NMR spectrometry,which indicates the presence of several polyflavonoid units,confirming the condensed nature of the *** analysis of the resin formed by the purified tannin of Paraberlinia bifoliolata to which a vegetal biohardener has been added provided a Modulus of Elasticity(MOE)value of 4840 MPa at 150℃,confirming its possible use as a binder resin in the manufacture of wood panels as well as for the formulation of a corrosion inhibitor.

关键词： Tannin Paraberlinia bifoliolata Central African wood species MALDI-TOF/MS 13C NMR bark extracts thermomechanical behaviour wood-binder

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A Global Group of Friends

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Beijing Review 2024年第29期67卷 26-27页

作者： jean Paul Vargas the Sustainable Development Innovation Laboratory Central American Institute of Public Administration the Center for Historical Research of Central America University of Costa Rica

China's Global Development Initiative(GDI)is emerging as a transformative force in the international *** President Xi Jinping first publicized the conceplal the United Nations General Assembly in September *** its announcemen,the GDT's main components and objectives,aimed at fostering cunnectily and coperation between coninents,hawe atractod workdwide atentin.

关键词： Global foster objectives

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Evaluation of Colorectal Cancer Management in Eastern DR Congo. About 55 Cases Were Collected from January 2002 to December 2016 Panzi General Referral Hospital

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Journal of Cancer Therapy 2024年第4期15卷 179-189页

作者： Bafunyembaka Muka Alain Kitumaini Munyahali John Ciruza Balegana jean-Claude Lobe Lottin michel Luhiriri Ndanda Levi Kavira Isse Somo Prisca Amisi Mangaza Emilie Alumeti Munyali Desire Ahuka Ona Longombe Albert Department of Surgery H.G.R Panzi/Universit&#233 Evang&#233liqueen Afrique Bukavu DR Congo

A retrospective study was conducted at the HGR de Panzi in eastern DR Congo to determine the profile of colorectal cancer (CRC) management based on 55 cases out of 129 digestive cancers diagnosed in the digestive surgery, endoscopy, and pathology department of the HGR de Panzi from January 2002 to December 2016. The prevalence of CRC was 42.6%. The mean age of patients was 50.8 years, with a range of 20 to 81 years. The male to female ratio was 2.2. In 69.1% of cases, the patients had a low socioeconomic status, and 52.7% had a history of hemorrhoids. Rectorrhagia revealed CCR in 49.1% of cases. The mode of discovery was predominantly emergency in 69.1% of cases, and 58.20% of CRCs were diagnosed by an initial endoscopy of the lower colon. Adenocarcinoma was found in 94.5% of cases, and 45% of cases required abdominoperineal amputation. Various postoperative complications were experienced by 54.5% of patients, and 25.5% of patients died. To decrease the occurrence of these types of cancers, it is necessary to focus on early detection in the population and enhance access to primary healthcare for everyone.

关键词： Assessment Management Colorectal Cancer

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New Insights in the Biodegradability and the Ecotoxicological Effects of Solar Products Containing Mineral and Chemical UV-Filters on Marine Zoo- and Phytoplanktons: An in silico and in vitro Study

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Journal of Cosmetics, Dermatological Sciences and Applications 2024年第1期14卷 98-111页

作者： jean-Claude Hubaud Karim Mekideche jean-Eric Branka Luc Lefeuvre HELIOSCIENCE Cité de la cosmétique 2 rue Odette Jasse Marseille France EPHYSCIENCE 498ter route de Saint Joseph de Porterie Nantes France Laboratoires Dermatologiques d’Uriage Neuilly-sur-Seine France

Background: Cosmetic formulations, and particularly solar products which contain mineral and chemical UV-filters, are often suspected of causing harmful effects on marine fauna and flora. After the publication of our work in 2019 concerning the ecotoxicological effects of such formulations on corals (Seriatopora hystrix), we here provide some new information about the biodegradability and the ecotoxicological effects of these products on marine zoo- and phytoplankton. Therefore, we choose to realize in silico and in vitro studies of the biodegradability of several solar products but also to evaluate the ecotoxicological effects of these products on one phytoplankton, i.e. Phaeodactylum tricornutum, and one zooplankton, i.e. Acartia tonsa, of a great importance for sea species survival (notably as sources of food). Materials and methods: Two different approaches were used to study the biodegradability of the tested products: One in silico method and an in vitro one. 2 solar products were involved in the in silico study which consisted in the determination of the degradation factor (DF) of each ingredient of the tested formulas in order to finally obtain their estimated biodegradability percentage. Already available data concerning each ingredient coupled to a computer model developed with one of our partners were used to achieve this study. The in vitro study involved 8 formulas containing UV-filters and was led by following the OECD 301 F guidelines. Ecotoxicological studies of 7 of the formulas containing UV-filters were for their part realized by following the ISO 10253 guidelines for the experiments led with Phaeodactylum tricornutum, and the ISO 14669 guidelines for the experiments led with Acartia tonsa. In these studies, the effect of each tested product on crustaceans’ mortality and algal growth inhibition was assessed. Results: The in silico study predicted that formulas containing chemical UV-filters display a high biodegradability (superior to the threshol

关键词： UV-Filters Chemical UV-Filter Biodegradability Ecotoxicology in silico in vitro Zooplankton Phytoplankton Phaeodactylum tricornutum Acartia tonsa

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Joint Management of Shared Resources, Issues and Conflicts of the Nso and Oku Tribes in the Ngongbaa Montane Forest Area, NWR of Cameroon

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Natural Resources 2024年第6期15卷 149-172页

作者： jean-Louis Tatah Elvis Kah Department of Geography Faculty of Arts Letters and Social Sciences University of Yaounde I Yaounde Cameroon School of Geology and Mining Engineering University of Ngaoundere Ngaoundere Cameroon

Wherever people live together in or near homelands that harbour joint natural resources such as forest, water, minerals, oil etc., they must collaborate, co-exist and share these resources irrespective of their genetic, social, cultural, and political peculiarities. This is not always the case when self-interest, ethnic divisions, elitist politics, economic interest and power tussle set in. The Nso and Oku people who reside in the Ngongbaa Forest area constitute an example of joint management of resources where at one period, their relationship over the management of the Oku Mountain Forest was cordial and at another, the relationship became conflictive. This study was designed to study the influence of the KIFP forest management policies on the Nso indigenous forest Management system in Ngongbaa Forest, and its implications. The data collected for the study was secured through the administration of a questionnaire to residents of 10 villages adjacent to the Ngongbaa Forest, and forest users who carried out livelihood activities there between 1963 and 2015. These forest user groups include beekeepers, rat trappers, grazers, mushroom gatherers, ground honey/tree honey harvesters, carvers, etc. The investigation also extended to the traditional leaders of Nso and Oku who are in charge of the land tenure systems. The findings show that the Oku Mountain Forest is jointly owned by the Nso and Oku people. The Nso own the eastern half of the forest known as Ngongbaa Forest while the Oku own the western half, known as the Kilum Forest. The cordial relationship that existed between these people ensured the conservation of the forest. But the conflicts that later set in, especially due to the creation of the Kilum Mountain Forest Project in 1987 later changed to the Kilum Ijim Forest Project (KIFP) in 1992 sidelined the local communities which was contrary to the dispositions of the new Cameroon forest law enacted in 1994. Thus, the indigenous conservation practices became ig

关键词： Conflict Joint Management Shared Resources Ngongbaa Kilum Conservation

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