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Sturge–Weber syndrome:an update for the pediatrician

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World Journal of Pediatrics 2024年第5期20卷 435-443页

作者： Emilie Dingenen damien Segers Hannelore De Maeseneer Dirk Van Gysel Ghent University Faculty of Medicine and Health Sciences GhentBelgium Department of Pediatrics O.L.Vrouw Hospital AalstMoorselbaan 1649300AalstBelgium Interdisciplinary Unit of Pediatric Dermatology Vrije Universiteit Brussel(VUB)Universitair Ziekenhuis Brussel(UZ Brussel)BrusselsBelgium

Sturge–Weber syndrome(SWS)is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary *** usually presents as a facial port-wine birthmark,with a varying presence of leptomeningeal capillary malformations and ocular vascular *** latter may lead to significant neurological and ocular morbidity such as epilepsy and *** is most often caused by a somatic mutation involving the G protein subunit alpha Q or G protein subunit alpha 11 gene causing various alterations in downstream signaling *** specifically conducted a comprehensive review focusing on the current knowledge of clinical practices,the latest pathophysiological insights,and the potential novel therapeutic avenues they *** sources A narrative,non-systematic review of the literature was conducted,combining expert opinion with a balanced review of the available literature.A search of PubMed,Google Scholar and Embase was conducted,using keywords“Sturge–Weber Syndrome”OR“SWS”,“Capillary malformations”,“G protein subunit alpha 11”OR“G protein subunit alpha Q”.Results One of the hallmark features of SWS is the presence of a port-wine birthmark at birth,and forehead involvement is most indicative for *** most common ocular manifestations of SWS are glaucoma and choroidal *** presents in either in infancy(0–3 years of age)or later in *** complications are common in SWS,occurring in about 70%–80%of patients,with seizures being the most common *** significantly impacts the quality of life for patients and their families,and requires a multidisciplinary approach for diagnosis and ***,no disease-modifying therapies exist,and treatment is mostly focused on symptoms or complications as they *** SWS remains a complex and heterogeneous *** research is needed to optimize diagnostic and therapeutic strategies,and to transl

关键词： Capillary malformations G-protein Gq-G11 Port-wine birthmark Neurocutaneous disorder Sturge-Weber syndrome

Virtual brain twins: from basic neuroscience to clinical use

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National Science Review 2024年第5期11卷 72-86页

作者： Huifang E.Wang Paul Triebkorn Martin Breyton Borana Dollomaja Jean-Didier Lemarechal Spase Petkoski Pierpaolo Sorrentino damien Depannemaecker Meysam Hashemi Viktor K.Jirsa Aix Marseille Université Institut National de la Santéet de la Recherche Médicale Institut de Neurosciences des Systèmes(INS) UMR1106 Service de Pharmacologie Clinique et Pharmacosurveillance AP–HM

Virtual brain twins are personalized, generative and adaptive brain models based on data from an individual's brain for scientific and clinical use. After a description of the key elements of virtual brain twins,we present the standard model for personalized whole-brain network models. The personalization is accomplished using a subject's brain imaging data by three means:(1) assemble cortical and subcortical areas in the subject-specific brain space;(2) directly map connectivity into the brain models, which can be generalized to other parameters; and(3) estimate relevant parameters through model inversion, typically using probabilistic machine learning. We present the use of personalized whole-brain network models in healthy ageing and five clinical diseases: epilepsy, Alzheimer's disease, multiple sclerosis, Parkinson's disease and psychiatric disorders. Specifically, we introduce spatial masks for relevant parameters and demonstrate their use based on the physiological and pathophysiological hypotheses. Finally, we pinpoint the key challenges and future directions.

关键词： virtual brain twin personalized modeling inference neuroscience brain disorder

Primordial black hole mass functions as a probe of cosmic origin

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Science China(Physics,Mechanics & Astronomy) 2024年第5期67卷 164-171页

作者： Yi-Fu Cai Chengfeng Tang Geyu Mo Sheng-Feng Yan Chao Chen Xiao-Han Ma Bo Wang Wentao Luo damien A.Easson Antonino Marcianò Deep Space Exploration Laboratory/School of Physical Sciences University of Science and Technology of ChinaHefei 230026China CAS Key Laboratory for Researches in Galaxies and Cosmology/Department of Astronomy School of Astronomy and Space ScienceUniversity of Science and Technology of ChinaHefei 230026China Istituto Nazionale di Fisica Nucleare(INFN) Sezione di MilanoMilano 20146Italy DiSAT Universitàdegli Studi dell'InsubriaComo 22100Italy Jockey Club Institute for Advanced Study The Hong Kong University of Science and TechnologyHong KongChina Department of Physics Arizona State UniversityTempe 85287-1504USA Department of Physics&Center for Field Theory and Particle Physics Fudan UniversityShanghai 200433China Laboratori Nazionali di Frascati INFN Via Enrico Fermi 541 Frascati(Roma)00044Italy INFN sezione Roma Tor Vergata Rome I-00133Italy School of Physics University of Electronic Science and Technology of ChinaChengdu11731China

We discuss a novel window to probe the origin of our universe via the mass functions of primordial black holes(PBHs).The mass functions of PBHs are simply estimated using the conventional Press-Schechter formalism for two paradigms of cosmic origin,including inflationaryΛCDM and bounce *** standard inflationaryΛCDM model cannot generate an appreciable number of massive PBHs;however,non-trivial inflation models with blue-tilted power spectra at small scales and matter bounce cosmology provide formation mechanisms for heavy PBHs,which in turn,may seed the observed supermassive black holes(SMBHs).By fitting the SMBH mass functions at high redshift(z~6)derived from Sloan Digital Sky Survey(SDSS)and Canada-France High-z Quasar Survey(CFHQS)quasars,for two paradigms of cosmic origin,we derive constraints on the PBH density fraction fPBHat z~6 and the characteristic mass M_(★),with the prior assumption that all SMBHs stem from *** demonstrate that this newly proposed procedure,relying on astronomical measurements that utilize deep-field surveys of SMBHs at high redshift,can be used to constrain models of cosmic ***,although not the main focus of this paper,we evolve the mass function from z~6 to z~0 through an assumption of 3×10^(8)-year Eddington’s accretion,and give a rough estimation of fPBHat z~0.

关键词： origin cosmic massive

Antimicrobial stewardship and targeted therapies in the changing landscape of maternal sepsis

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Journal of Intensive Medicine 2024年第1期4卷 46-61页

作者： Nishel M Shah Esmita Charani damien Ming Fook-Choe Cheah Mark R Johnson Department of Metabolism Digestion and ReproductionImperial College LondonChelsea and Westminster HospitalLondonUK Health Protection Research Unit in Healthcare Associated Infection and Antimicrobial Resistance Imperial College LondonLondonUK Division of Infectious Diseases and HIV Medicine Department of MedicineGroote Schuur HospitalUniversity of Cape TownCape TownSouth Africa Department of Infectious Diseases Imperial College LondonChelsea and Westminster HospitalLondonUK Department of Paediatrics Faculty of MedicineUniversiti Kebangsaan Malaysia Medical CentreKuala LumpurMalaysia

Pregnant and postnatal women are a high-risk population particularly prone to rapid progression to sepsis with significant morbidity and mortality ***,severe maternal infections can have a serious detrimental impact on neonates with almost 1 million neonatal deaths annually attributed to maternal infection or *** this review we discuss the susceptibility of pregnant women and their specific physiological and immunological adaptations that contribute to their vulnerability to sepsis,the implications for the neonate,as well as the issues with antimicrobial stewardship and the challenges this poses when attempting to reach a balance between clinical care and urgent ***,we review advancements in the development of pregnancy-specific diagnostic and therapeutic approaches and how these can be used to optimize the care of pregnant women and neonates.

关键词： Sepsis Pregnancy Immunology Cardiovascular Personalised medicine Antibiotic stewardship

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全球蜂群数量和蜂蜜产量呈上升趋势

中国蜂业 2024年第4期75卷 52-53,57页

作者：罗照明(编译) 孟丽峰(编译) Phiri Bernard J Fèvre damien Hidano Arata 中国蜂产品协会北京100050 中国农业科学院蜜蜂研究所北京100093 不详

蜜蜂在社会经济上具有重要意义,因为它们在农作物授粉中发挥着关键作用,并生产对社会和许多行业至关重要的各种蜂产品,它们对生态和社会的可持续发展具有重要贡献。具有社会经济价值的蜂产品包括蜂蜜、蜂蜡、蜂胶、花粉、蜂王浆等,被广... 详细信息

蜜蜂在社会经济上具有重要意义,因为它们在农作物授粉中发挥着关键作用,并生产对社会和许多行业至关重要的各种蜂产品,它们对生态和社会的可持续发展具有重要贡献。具有社会经济价值的蜂产品包括蜂蜜、蜂蜡、蜂胶、花粉、蜂王浆等,被广泛应用于食品、化妆品、药品,甚至用于工业中。因此,全球蜂群数量即将或持续下降可能会产生广泛的社会经济影响。Bernard ***等基于联合国粮农组织(FAO)60年来收集的数据,包括蜂群数量、蜂蜜产量、蜂蜡产量,分析并评估了全球60年蜂业变化。

关键词：蜂蜜产量社会经济价值蜂产品蜂群数量蜂业社会经济影响蜂王浆蜂蜡

Performance Evaluation of Novel Eco-Materials Composed of Millet Husks, Rice Husks, and Polystyrene

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Open Journal of Composite Materials 2024年第1期14卷 33-43页

作者： Paul damien Amadji Edem Chabi Valéry Kouandété Doko Céphas Houndedji Emmanuel Olodo Laboratory of Applied Energy and Mechanics (LEMA) Polytechnic School of Abomey-Calavi University of Abomey-Calavi Abomey-Calavi Benin Laboratory of Rural Engineering School of Rural Engineering National University of Agriculture Kétou Benin

Managing agricultural waste and expanded polystyrene (EPS) poses significant environmental and economic challenges. This study aims to create composites from millet husks, rice husks, and recycled EPS, using a manufacturing method that involves dissolving the polystyrene in a solvent followed by cold pressing. Various particle sizes and two binder dosages were investigated to assess their influence on the physico-mechanical properties of the composites. The mechanical properties obtained range from 2.54 to 4.47 MPa for the Modulus of Rupture (MOR) and from 686 to 1400 MPa for the Modulus of Elasticity in Bending (MOE). The results indicate that these composites have potential for applications in the construction sector, particularly for wood structures and interior decoration. Moreover, surface treatments could enhance their durability and mechanical properties. This research contributes to the valorization of agricultural and plastic waste as eco-friendly and economical construction materials.

关键词： Bio-Based Composites Millet Husk Rice Husk Expanded Polystyrene Waste Valorization Sustainable Construction

Study of Rice Husks and Expanded Polystyrene Composites for Construction Applications

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Open Journal of Civil Engineering 2024年第3期14卷 456-468页

作者： Edem Chabi Valéry Kouandété Doko Sena Peace Hounkpè Paul damien Amadji Emmanuel Olodo Laboratory of Rural Engineering School of Rural Engineering National University of Agriculture Ketou Benin Laboratory of Applied Energy and Mechanics (LEMA) Polytechnic School of Abomey-Calavi University of Abomey-Calavi Abomey-Calavi Benin Department of Civil Engineering Higher National School of Public Works (ENSTP) National University of Sciences Technologies Engineering and Mathematics Abomey Benin

In the current context of environmental challenges, this study focuses on developing innovative and eco-friendly composites using rice husk and recycled expanded polystyrene. This dual-responsibility approach valorizes a by-product like rice husk, often considered waste, and reuses polystyrene, a plastic waste, thereby contributing to CO2 emission reduction and effective waste management. The manufacturing process involves dissolving recycled polystyrene into a solvent to create a binder, which is then mixed with rice husk and cold-compacted into composite materials. The study examines the impact of two particle sizes (fine and coarse) and different proportions of recycled polystyrene binder. The results show significant variations in the mechanical characteristics of the composites, with Modulus of Rupture (MOR) values varying from 2.41 to 3.47 MPa, Modulus of Elasticity (MOE) ranging from 223.41 to 1497.2 MPa, and Stiffness Coefficient (K) from 5.04 to 33.96 N/mm. These characteristics demonstrate that these composites are appropriate for various construction applications, including interior decoration, panel claddings, and potentially for furniture and door manufacturing when combined with appropriate coatings. This study not only highlights the recycling of agricultural and plastic waste but also provides a localized approach to addressing global climate change challenges through the adoption of sustainable building materials.

关键词： Rice Husk Recycled Expanded Polystyrene Eco-Friendly Composites Waste Valorization Sustainable Construction

Rainfall Dynamics and Landslides in Urban Areas of Gabon: The Case of Libreville-Est (Gabon)

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Journal of Geoscience and Environment Protection 2024年第8期12卷 239-264页

作者： Jospin Lewis Backita Moussounda André Géraud Le Brun Bivegue-Bivegue Fern Lièle Mboumbou Makanga Jean damien Maloba Makanga Department of Environmental and Marine Geographic Sciences Faculty of Arts and Humanities Omar Bongo University Laboratory of Spatial Analysis and Tropical Environments Libreville Gabon

Due to its abundant rainfall, the city of Libreville, which concentrates more than half of Gabon’s population, is frequently confronted with the impacts of natural disasters such as floods and landslides. This study attempts to identify the complex relationships between the dynamics of land use and the role of rainfall in the occurrence of landslides. On the one hand, it uses statistics on landslides compiled from information taken from general news bulletins and, on the other, daily rainfall data obtained from the National Meteorological Department. The study revealed that the Libreville East sector, dominated by Mount Nkol Ogoum, one of Libreville’s most prominent landforms, is affected by a land-use dynamic in which human settlement has been progressing for some thirty years, to the detriment of the original vegetation which, among other things, helped to stabilise the soil on the hillsides and the marshy areas at the foot of the slopes. The result is not only an uncontrolled occupation of the land, but also a major landslide every two years in this part of the city, causing significant loss of life and property. However, an analysis of the time series shows little rainfall variability, marked in particular by a predominance of negative anomalies, and the occurrence of a few exceptional daily rainfall peaks. Similarly, the period from 20 October to 20 November, which receives the most rainfall, also appears to be the most conducive to landslides.

关键词： Libreville Land Use Rainfall Natural Hazards Landslide

BIERMER’s Disease: About 4 Cases Diagnosed at Regional University Hospital of Ouahigouya (Chur/Ohg) and Literature Review

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Open Journal of Gastroenterology 2024年第9期14卷 322-329页

作者： Zanga damien Ouattara Stève Léonce Zoungrana Saïdou Santi Emeline Agathe Carama Fabien Dembele Bafima Désiré Diarra Alain Bougouma Department of Gastroenteroly Ouahigouya Regional University Hospital Ouahigouya Burkina Faso Department of Neurology Bogodogo University Hospital Ouagadougou Burkina Faso Department of Gastroenterology Yalgado Oudraogo University Hospital Center Ouagadougou Burkina Faso

Megaloblastic pernicious anemia is an autoimmune disorder, considered rare in African context. The objective of this study was to report four clinical cases collected at the CHUR/OHG, and to review the literature. The study population consisted of two men and two women. The clinical manifestations were mainly neurological and hematological. The neurological signs were mainly paresthesia. One patient presented memory problems. On the biological level, macrocytic anemia and vitamin B12 deficiency were reported in two cases prior to treatment while the other two without serological assay of vitamin B12 were put on trial treatment. Anti-intrinsic factor antibodies were positive in three patients. The Schilling test was not used. Upper gastrointestinal endoscopy revealed atrophic fundic gastritis in all four patients who received treatment through intramuscular injection of hydroxocobalamin (vitamin B12). The evolution was favorable after one month of treatment in all cases. The literature review is dominated by clinical case reports, the largest cohorts of which are from the Maghreb.

关键词： Anemia Pernicious Megaloblastic Vitamin B12