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Targeted therapy for capillary-venous malformations

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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

Interactions between Cryptosula and Watersipora (Bryozoa： Cheilostomata) on a ship＇s hull in Qingdao Harbour (South Yellow Sea) after five and a half years of immersion

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Chinese Journal of Oceanology and Limnology 2017年第5期35卷 1179-1188页

作者：刘会莲 Kamil Zágor?EK 王世伟马士德 paul D.TAYLOR Institute of Oceanology Chinese Academy of Sciences Department of Geography Technical University of Liberec Department of Earth Sciences Natural History MuseumCromwell Road London

The distributions of two ubiquitous fouling cheilostome bryozoans,Cryptosula pallasiana(Moll,1803)and W atersipora sp.,on a ship moored for almost six years in Qingdao Bay show diff erences with respect to illumination,C ryptosula being dominant on the side of the ship which was exposed to the sun and Watersipora dominating on the other side which was in shadow for most of the *** interactions for substrate space were nearly always won by Watersipora,which succeeded in overgrowing the edges of C ryptosula colonies regardless of the side of the *** for the superiority of W atersipora in spatial competition with Cryptosula could include faster growth rate and the stronger feeding currents created by the larger lophophores of Watersipora.

关键词：苔藓动物门唇口目相互作用船舶南黄海青岛港浸泡竞争优势

Electric-field driven stability control of skyrmions in an ultrathin transition-metal film

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npj Computational Materials 2022年第1期8卷 992-1000页

作者： Souvik paul Stefan Heinze Peter grünberg Institut and Institute for Advanced Simulation Forschungszentrum Jülich and JARA52425JülichGermany Institute of Theoretical Physics and Astrophysics Christian-Albrechts-Universität zu KielLeibnizstrasse 1524098KielGermany

To realize future spintronic applications with magnetic skyrmions—topologically nontrivial swirling spin structures—it is essential to achieve efficient writing and deleting capabilities of these ***-field assisted nucleation and annihilation is a promising route,however,the understanding of the underlying microscopic mechanisms is still ***,we show how the stability of individual magnetic skyrmions in an ultrathin transition-metal film can be controlled via external electric *** demonstrate based on density functional theory that it is important to consider the changes of all interactions with electric field,i.e.,the pair-wise exchange,the Dzyaloshinskii–Moriya interaction,the magnetocrystalline anisotropy energy,and the higher-order exchange *** energy barriers for electric-field assisted skyrmion writing and deleting obtained via atomistic spin simulations vary by up to a factor of three more than the variations of the interactions calculated from *** surprising effect originates from the electric-field dependent size of metastable skyrmions at a fixed magnetic *** large changes in lifetimes allow the possibility of electric-field assisted thermally activated writing and deleting of skyrmions.

关键词： field stability film

Geodynamic evolution of the Tethyan lithosphere as recorded in the Spontang Ophiolite,South Ladakh ophiolites(NW Himalaya,India)

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Geoscience Frontiers 2022年第1期13卷 326-349页

作者： Mallika K.Jonnalagadda Mathieu Benoit Shivani Harshe Romain Tilhac Raymond A.Duraiswami Michel grégoire Nitin R.Karmalkar Interdisciplinary School of Science Savitribai Phule Pune UniversityPune 411007India Department of Geology Savitribai Phule Pune UniversityPune 411007India Géosciences Environnement Toulouse CNRS-CNES-IRD-UniversitéPaul SabatierObservatoire Midi Pyrénées31400 ToulouseFrance Instituto Andaluz de Ciencias de la Tierra(IACT) CSIC–Universidad de Granada18100 ArmillaGranadaSpain Vice-Chancellor’s Office Savitribai Phule Pune UniversityPune 411007India

The Spontang Ophiolite complex represents the most complete ophiolite sequence amongst the South Ladakh ophiolites and comprises mantle rocks(depleted harzburgites,dunites and minor lherzolites)as well as crustal rocks(basalt,isotropic gabbros,layered gabbros etc.).In the present study,detailed geochemistry(whole rock as well as mineral chemistry)and Sr-Nd isotopic analyses of thirty-six ultramaficmafic samples have been attempted to constraint the evolution and petrogenetic history of the Tethyan oceanic ***,trace-element and REE patterns of the peridotites and their minerals indicate that the lherzolites experienced lower degrees of partial melting resembling abyssal peridotites(at higher temperatures,TREE=$1216℃)than the harzburgites(6%–8%versus 15%–17%).Elevated eNd(t)and variable^(87) Sr/^(86) Sr(t)ratios along with REE patterns suggest that the Spontang mafic rocks display N-MORB affinity with negligible participation of oceanic sediments in their genesis are originated from a depleted upper mantle with little contribution from subduction-related ***-type Neotethyan oceanic crust is associated with the earliest phase of subduction(of older Jurassic age)through which a younger intra-oceanic island arc(Spong arc)subsequently *** REE display typical U-shaped patterns,suggesting that these rocks have been metasomatized by LREE-enriched *** the other side,mafic rocks are characterized by heterogeneous(Nb/La)PMand(Hf/Sm)PMand relatively homogeneous eNd(t),indicating interaction of subduction-related melts with the upper mantle during the initiation of subduction,in Early Cretaceous times.

关键词： Peridotites Mafic rocks Partial melting Metasomatism Ladakh

Unleashing the Power of Moiré Materials in Neuromorphic Computing

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Chinese Physics Letters 2023年第12期40卷 131-132页

作者： John paul Strachan Peter grünberg Institute(PGI-14) Neuromorphic Compute NodesForschungszentrum JülichJülichGermany and RWTH Aachen UniversityAachenGermany

Reservoir computing has been an intriguing paradigm in the field of artificial intelligence and machine learning that draws inspiration from the complex dynamics of recurrent neural networks found in biological systems. Unlike traditional neural networks, reservoir computing separates the training of a fixed, randomly connected ‘reservoir’layer from a simpler ‘readout’ layer. This distinctive architecture allows the reservoir to process information in a highly dynamic and nonlinear manner.

关键词： networks neural computing

Recent advances in metasurface design and quantum optics applications with machine learning,physics-informed neural networks,and topology optimization methods

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光:科学与应用(英文版) 2023年第8期12卷 1494-1509页

作者： Wenye Ji Jin Chang He-Xiu Xu Jian Rong Gao Simon groblacher H.paul Urbach Aurele J.L.Adam Department of Imaging Physics Delft University of TechnologyLorentzweg 12628 CJ DelftThe Netherlands Department of Quantum Nanoscience Delft University of TechnologyLorentzweg 12628 CJ DelftThe Netherlands Shaanxi Key Laboratory of Flexible Electronics(KLoFE) Northwestern Polytechnical University(NPU)127 West Youyi RoadXi'an 710072China SRON Netherlands Institute for Space Research Niels Bohrweg 42333 CA LeidenThe Netherlands

As a two-dimensional planar material with low depth profile,a metasurface can generate non-classical phase distributions for the transmitted and reflected electromagnetic waves at its ***,it offers more flexibility to control the wave front.A traditional metasurface design process mainly adopts the forward prediction algorithm,such as Finite Difference Time Domain,combined with manual parameter ***,such methods are time-consuming,and it is difficult to keep the practical meta-atom spectrum being consistent with the ideal *** addition,since the periodic boundary condition is used in the meta-atom design process,while the aperiodic condition is used in the array simulation,the coupling between neighboring meta-atoms leads to inevitable *** this review,representative intelligent methods for metasurface design are introduced and discussed,including machine learning,physics-information neural network,and topology optimization *** elaborate on the principle of each approach,analyze their advantages and limitations,and discuss their potential *** also summarize recent advances in enabled metasurfaces for quantum optics *** short,this paper highlights a promising direction for intelligent metasurface designs and applications for future quantum optics research and serves as an up-to-date reference for researchers in the metasurface and metamaterial fields.

关键词： optics quantum topology

Management of syndesmotic injuries:What is the evidence?

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World Journal of Orthopedics 2016年第11期7卷 718-725页

作者： Marc Schnetzke Sven Y Vetter Nils Beisemann Benedict Swartman paul A grützner Jochen Franke Department for Trauma and Orthopaedic Surgery MINTOS-Medical Imaging and Navigation in Trauma and Orthopaedic SurgeryBG Trauma Center Ludwigshafen at Heidelberg University Hospital

Ankle fractures are accompanied by a syndesmotic injury in about 10% of operatively treated ankle fractures. Usually, the total rupture of the syndesmotic ligaments with an external rotation force is associated with a Weber type B or C fracture or a Maisonneuve fracture. The clinical assessment should consist of a comprehensive history including mechanism of injury followed by a specific physical examination. Radiographs, and if in doubt magnetic resonance imaging, are needed to ascertain the syndesmotic injury. In the case of operative treatment the method of fixation, the height and number of screws and the need for hardware removal are still under discussion. Furthermore, intraoperative assessment of the accuracy of reduction of the fibula in the incisura using fluoroscopy is difficult. A possible solution might be the assessment with intraoperative three-dimensional imaging. The aim of this article is to provide a current concepts review of the clinical presentation, diagnosis and treatment of syndesmotic injuries.

关键词： Ankle sprain Syndesmotic injury Syndesmotic screw Ankle TightRope Three-dimensional

Epileptic Seizures in Neonates Treated with Hypothermia for Hypoxo-Ischemic Encephalopathy in Brazzaville, Congo: Types and Evolution

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Neuroscience & Medicine 2023年第4期14卷 63-75页

作者： Josué Euberma Diatewa Dinah Happhia Boubayi Motoula-Latou Benoîte gracia Diatewa Gabrielle Ontsira grâce Kadidja Cléona Nkounkou-Milandou Eliot Prince Galiéni Sounga-Banzouzi Ghislain Armel Mpandzou Jean Robert Mabiala-Babela paul Macaire Ossou-Nguiet Neurology Department University Hospital of Brazzaville Brazzaville Republic of Congo Faculty of Health Sciences Marien Ngouabi University Brazzaville Republic of Congo Neonatology Department Blanche Gomez Mother and Child Hospital Brazzaville Republic of Congo Neonatology Department University Hospital of Brazzaville Brazzaville Republic of Congo Neurology Department Loandjili General Hospital Pointe Noire Republic of Congo

Background: Moderate to severe hypoxic-ischemic encephalopathy (HIE) in neonates is often treated with hypothermia. However, some neonates may experience epileptic seizures during therapeutic hypothermia (TH). Data on the electrophysiologic and evolutionary aspects of these seizures are scarce in African countries. Objectives: To determine the types of epileptic seizures caused by HIE in neonates in Brazzaville;to describe the evolution of background EEG activities during TH and rewarming;to report the evolution of epileptic seizures. Methods: This was a cross-sectional, descriptive study conducted from January 2020 to July 2022. It took place in Brazzaville in the Neonatology Department of the Blanche Gomez Mother and Child Hospital. It focused on term neonates suffering from moderate or severe HIE. They were treated with hypothermia combined with phenobarbital for 72 hours. Results: Among 36 neonates meeting inclusion criteria, there were 18 boys and 18 girls. Thirty-one (86.1%) neonates had grade 2 and 5 (13.9%) grade 3 HIE. In our neonates, HIE had induced isolated electrographic seizures (n = 11;30.6%), electroclinical seizures (n = 25;69.4%), and 6 types of background EEG activity. During TH and rewarming, there were 52.8% of patients with improved background EEG activity, 41.7% of patients with unchanged background EEG activity, and 5.5% of patients with worsened background EEG activity. At the end of rewarming, only 9 (25%) patients still had seizures. Conclusion: Isolated electrographic and electroclinical seizures are the only pathological entities found in our studied population. In neonates with moderate HIE, the applied therapeutic strategy positively influences the evolution of both seizures and background EEG activity. On the other hand, in neonates with severe HIE, the same therapeutic strategy is ineffective. .

关键词： Epileptic Seizures Neonate Hypoxo-Ischemic Encephalopathy Therapeutic Hypothermia Antiepileptic Drugs Brazzaville

全球星(GLOBALSTAR)的姿态和轨道控制

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控制工程（北京） 1999年第3期 20-26页

作者： Alexander R Brüderle E gr.gor.E Schrempp W. Widmann H 潘科炎

本文简介由48颗低轨道卫星组成的全球星通信卫星的姿态和轨道控制。姿态控制功能使卫星天线指向地球,同时使卫星按预定的方式绕偏航轴转动。这就是说,尽管卫星有52°轨道倾角,仍要通过绕太阳帆板轴的另一种旋转机动(即偏航机动)来控制帆... 详细信息

本文简介由48颗低轨道卫星组成的全球星通信卫星的姿态和轨道控制。姿态控制功能使卫星天线指向地球,同时使卫星按预定的方式绕偏航轴转动。这就是说,尽管卫星有52°轨道倾角,仍要通过绕太阳帆板轴的另一种旋转机动(即偏航机动)来控制帆板,使之始终垂直于太阳方向定向。文中还介绍了其他工作模式:如从900km轨道自主升轨到1400km的自动变轨模式,还有故障检测和恢复算法等。Globalstar控制系统的基本单元包括:采用磁强计以及太阳和地球敏感器的姿态控制单元;采用四个反作用轮的偏航机动控制单元,用两个磁力矩器为飞轮卸载。DSS公司(多尼尔卫星系统公司)在其全球星姿态和轨道控制系统设计中采用了与其科学卫星ROSAT(伦琴卫星)极相似的设计方案,ROSAT卫星已在轨运行了7年多时间。全球星的软件和分系统试验以其成熟的科学和通信卫星的方案为基础,但试验的自动化程度更高,以满足飞行部件大批量生产的要求。

关键词：全球星通信卫星姿态控制轨道控制