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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

Late Quaternary clay minerals off Middle Vietnam in the western South China Sea: Implications for source analysis and East Asian monsoon evolution

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Science China Earth Sciences 2007年第11期50卷 1674-1684页

作者： christophe COLIN Laboratoire IDES UMR 8148 CNRS Université de Paris XI Orsay 91405 France

High-resolution clay mineral records combined with oxygen isotopic stratigraphy over the past 450 ka during late Quaternary from Core MD05-2901 off Middle Vietnam in the western South China Sea are re-ported to reconstruct a history of East Asian monsoon evolution. Variations in Illite, chlorite, and kaolinite contents indicate a strong glacial-interglacial cyclicity, while changes in smectite content present a higher frequency cyclicity. The provenance analysis indicates a mixture of individual clay minerals from various sources surrounding the South China Sea. Smectite derived mainly from the Sunda shelf and its major source area of the Indonesian islands. Illite and chlorite originated mainly from the Mekong and Red rivers. Kaolinite was provided mainly by the Pearl River. Spectral analysis of the kaolin-ite/(illite+chlorite) ratio displays a strong eccentricity period of 100 ka, implying the ice sheet-forced win-ter monsoon evolution; whereas higher frequency changes in the smectite content show an ice sheet-forced obliquity period of 41 ka, and precession periods of 23 and 19 ka and a semi-precession period of 13 ka as well, implying the tropical-forced summer monsoon evolution. The winter monsoon evolution is generally in coherence with the glacial-interglacial cyclicity, with intensified winter monsoon winds during glacials and weakened winter monsoon winds during interglacials; whereas the summer monsoon evolution provides an almost linear response to the summer insolation of low latitude in the Northern Hemisphere, with strengthened summer monsoon during higher insolation and weakened summer monsoon during lower insolation. The result suggests that the high-latitude ice sheet and low-latitude tropical factor could drive the late Quaternary evolution of East Asian winter and summer monsoons, respectively, implying their diplex and self-contained forcing mechanism.

关键词： clay minerals,provenance,East Asian monsoon,late Quaternary,Middle Vietnam,South China Sea

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连接中欧,塑造未来

今日中国 2024年第10期73卷 22-23页

作者：邓敬熙瑞士德科集团

中欧双方强强联手,将培养面向未来、推动经济增长的人才队伍。备受期待的2024中欧人才论坛重点关注人才发展的未来,为进一步增进中欧关系、加强中欧交流提供了一个宝贵平台。长期以来,中欧交往一直注重相互尊重,并在贸易、科技、教育和... 详细信息

中欧双方强强联手,将培养面向未来、推动经济增长的人才队伍。备受期待的2024中欧人才论坛重点关注人才发展的未来,为进一步增进中欧关系、加强中欧交流提供了一个宝贵平台。长期以来,中欧交往一直注重相互尊重,并在贸易、科技、教育和文化等各领域开展深度合作。在中欧培养适应性强、创新水平高、有韧性的人才,对于推动中欧关系持续蓬勃发展至关重要。我们今天面临的全球性挑战—从科技的快速进步到环境问题的不断出现—都在告诉我们,人类需要一支技术娴熟、能够灵活适应环境变化的人才队伍。

关键词：人才队伍中欧关系深度合作全球性挑战相互尊重强强联手灵活适应面向未来

Generalized LMMSE Filtering with Out-of-Sequence Observations in Arbitrary Constant Delay

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Chinese Journal of Electronics 2019年第6期28卷 1217-1226页

作者： LEI Ming BAEHR christophe JING Zhongliang School of Aeronautics and Astronautics Shanghai Jiao Tong University Météo-France National Meteorological Research Centre/CNRS

Focusing on the problem of state estimation in the presence of sensor faults and Out-of-sequence measurement(OOSM) observations synchronously, we derive a formulation of Linear minimum mean squared error(LMMSE) filter with the arbitrary time delay of OOSM, the generalization of the present work lies in simultaneous treatment of correlated faults together with OOSMs that arrive at an arbitrary delay in a linear-optimal manner. The approach is demonstrated in a numerical comparison.

关键词： Out-of-sequence measurement(OOSM) Linear minimum mean squared error(LMMSE) Arbitrary time delay Sensor faults Fault-tolerant estimation Multisensor fusion

A note on the regularity of the holes for permeability property through a perforated domain for the 2D Euler equations:Dedicated to Professor Jean-Yves Chemin on the Occasion of His 60th Birthday

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Science China Mathematics 2019年第6期62卷 1121-1142页

作者： christophe Lacave Chao Wang Institut Fourier Université Grenoble AlpesCNRS School of Mathematical Sciences Peking University

For equations of order two with the Dirichlet boundary condition, as the Laplace problem, the Stokes and the Navier-Stokes systems, perforated domains were only studied when the distance between the holes d_ε is equal to or much larger than the size of the holes ε. Such a diluted porous medium is interesting because it contains some cases where we have a non-negligible effect on the solution when(ε, d_ε) →(0, 0).Smaller distances were avoided for mathematical reasons and for these large distances, the geometry of the holes does not affect or rarely affect the asymptotic result. Very recently, it was shown for the 2D-Euler equations that a porous medium is non-negligible only for inter-holes distances much smaller than the sizes of the *** this result, the boundary regularity of holes plays a crucial role, and the permeability criterion depends on the geometry of the lateral boundary. In this paper, we relax slightly the regularity condition, allowing a corner, and we note that a line of irregular obstacles cannot slow down a perfect fluid in any regime such thatε ln d_ε→ 0.

关键词： ideal fluids homogenization in perforated domains shrinking obstacles and the porous medium domains with corners

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Paleoclimate and Paleoenvironment of Gonghe Basin, Qinghai-Tibet Plateau, During the Lastdeglacial: Weathering, Erosion and Vegetation Cover Affect Clay Mineral Formation

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Acta Geologica Sinica(English Edition) 2014年第2期88卷 647-660页

作者： SUN Qingfeng christophe COLIN Faculty of Geography Northwest Normal University Laboratoire des Interactions et Dynamique des Environnements de Surface (IDES) UMR 8148CNRS Université de Paris-Sud

With the analysis of the sources and formation mechanism of the clay minerals in the sediment core from the Dalianhai lake in the Gonghe Basin,northeastern Tibet-Qinghai Plateau,clay mineral composition proxies,grain-size and carbonate contents have been employed for high-resolution study in order to reconstruct East Asian Summer Monsoon （EASM） over the northeastern Tibet-Qinghai Plateau during the *** study also extended to establish a relationship between vegetation cover changes and erosion during the last 14.5 ka with pollen record and clay mineral ***/kaolinite and smectite/（illite＋chlorite） ratios allow us to assess hydrolysis conditions in lowlands and/or physical erosion process in highlands of the Gonghe *** 12.9 Cal ka BP,both mineralogical ratios show low values indicative of strong physical erosion in the basin with a dominant cold and dry *** 12.9 Cal ka BP,an increase in both mineralogical ratios indicates enhanced chemical weathering in the basin associated with a warm and humid *** beginning of Holocene is characterized by high smectite/（illite＋chlorite） and smectite/kaolinite ratios that is synchronous as with deposition of many peat laminae,implying the best warm and humid conditions specifically between 8.0 to 5.5 Cal ka *** time interval after 5.0 Cai ka BP is characterized by a return to high physical erosion and low chemical weathering with dry climate conditions in the *** variations of clay mineral assemblages with previous pollen results,we observe a rapid response in terms of chemical weathering and physical erosion intensity to a modification of the vegetation cover in the basin.

关键词： clay minerals vegetation Gonghe Basin Tibet-Qinghai Plateau Asian monsoon Holocene

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Predictive-reactive Strategy for Flowshop Rescheduling Problem:Minimizing the Total Weighted Waiting Times and Instability

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Journal of Systems Science and Systems Engineering 2021年第3期30卷 253-275页

作者： Ayoub Tighazoui christophe Sauvey Nathalie Sauer Universitéde Lorraine LGIPMF-57000MetzFrance

Due to the fourth revolution experiencing,referred to as Industry 4.0,many production firms are devoted to integrating new technological tools to their manufacturing *** of them,is rescheduling the tasks on the machines responding to ***,for static scheduling,the efficiency criteria measure the performance of scheduling systems,in dynamic environments,the stability criteria are also used to assess the impact of jobs *** this paper,a new performance measure is investigated for a flowshop rescheduling *** one considers simultaneously the total weighted waiting time as the efficiency criterion,and the total weighted completion time deviation as the stability *** fusion could be a very helpful and significant measure for real life industrial *** disruption types are considered:jobs arrival and jobs ***,a Mixed Integer Linear Programming(MILP)model is developed,as well as an iterative predictive-reactive strategy for dealing with the online *** last,two heuristic methods are proposed and discussed,in terms of solution quality and computing time.

关键词： Rescheduling flowshop predictive-reactive strategy weighted waiting time stability weighted completion time deviation

Climate-influenced vector-borne diseases in Africa: a call to empower the next generation of African researchers for sustainable solutions

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贫困所致传染病(英文) 2024年第2期13卷 83-92页

作者： Obame-Nkoghe Judica?l Agossou Adjoavi Esse Mboowa Gerald Kamgang Basile Caminade Cyril Duke Dawn C. Githeko Andrew Karanja Ogega Obed M. Elloué Nestor Engone Sarr Fatou Bintou Nkoghe Dieudonné Kengne Pierre Ndam Nicaise T. Paupy christophe Bockarie Moses Otomo Patricks Voua Molecular and Cellular Biology Laboratory (LabMC) Biology Department University of Science and Technology of Masuku BP Franceville Gabon Ecology and Health Research Unit Interdisciplinary Center for Medical Research of Franceville BP Franceville Gabon Department of Zoology and Entomology Faculty of Natural and Agricultural Sciences University of the Free State Private Bag x13 Phuthaditjhaba Republic of South Africa Laboratory of Pharmacology and Improved Traditional Medicines Department of Animal Physiology Faculty of Science and Technology University of Abomey-Calavi BP Cotonou Benin The African Center of Excellence in Bioinformatics and Data-Intensive Sciences Infectious Diseases Institute College of Health Sciences Makerere University P. O Box Kampala Uganda Africa Centers for Disease Control and Prevention African Union Commission Roosevelt Street P.O. Box WKAddis Ababa Ethiopia Centre for Research in Infectious Diseases P.O. Box Yaoundé Cameroon Earth System Physics Department The Abdus Salam International Centre for Theoretical Physics Trieste Italy Africa Research Excellence Fund Charterhouse Street London EC1MHR UK Community Health Support Program P.O. Box Kisumu Kenya The African Academy of Sciences Nairobi Kenya Center for Phylosophical Studies and Research (CERP) Omar Bongo University (UOB) BP Libreville Gabon UMRED Health Training and Research Unit University of Iba Der Thiam of Thiès BP Thiès Senegal National Parasitic Diseases Control Program Ministry of Health Libreville Gabon MIVEGEC Univ. Montpellier CNRS IRD Montpellier France MERIT IRD Paris Cité University Paris France Department of Parasitology Noguchi Memorial Institute for Medical Research University of Ghana LG Accra Ghana School of Community Health Sciences Njala University Bo Sierra Leone

We look at the link between climate change and vector-borne diseases in low- and middle-income countries in Africa. The large endemicity and escalating threat of diseases such as malaria and arboviral diseases, intensified by climate change, disproportionately affects vulnerable communities globally. We highlight the urgency of prioritizing research and development, advocating for robust scientific inquiry to promote adaptation strategies, and the vital role that the next generation of African research leaders will play in addressing these challenges. Despite significant challenges such as funding shortages within countries, various pan-African-oriented funding bodies such as the African Academy of Sciences, the Africa Research Excellence Fund, the Wellcome Trust, the U.S. National Institutes of Health, and the Bill and Melinda Gates Foundation as well as initiatives such as the African Research Initiative for Scientific Excellence and the Pan-African Mosquito Control Association, have empowered (or are empowering) these researchers by supporting capacity building activities, including continental and global networking, skill development, mentoring, and African-led research. This article underscores the urgency of increased national investment in research, proposing the establishment of research government agencies to drive evidence-based interventions. Collaboration between governments and scientific communities, sustained by pan-African funding bodies, is crucial. Through these efforts, African nations are likely to enhance the resilience and adaptive capacity of their systems and communities by navigating these challenges effectively, fostering scientific excellence and implementing transformative solutions against climate-sensitive vector-borne diseases.

关键词： Climate change Vector-borne diseases Africa Environmental justice Research and development Pan-African funding initiatives

Co-Morbidities Associated with Prematurity in Two Referral Hospitals in Cameroon

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Open Journal of Pediatrics 2024年第4期14卷 738-753页

作者： Diomede Noukeu Njinkui Dominique Enyama Yolande Djike Fokam Cyrelle O. Mefotse Saha Beatrice Moudze Kaptue Charlotte Eposse Koube Annick A. Tchouamo Sime christophe Akazong Adjahoung Marthe E. Barla Edgar Mandeng Ma Linwa Seraphin Nguefack Faculty of Medicine and Pharmaceutical Sciences of the University of Dschang Dschang Cameroon Faculty of Health Sciences University of Buea Buea Cameroon Faculty of Health Sciences University of Mountains Bangangte Cameroon Douala Laquintinie Hospital Douala Cameroon Faculty of Medicine and Pharmaceutical Sciences University of Douala Douala Cameroon Douala General Hospital Douala Cameroon Faculty of Medicine and Biomedical Sciences University of Yaound I Yaound Cameroon

Introduction: Prematurity is the leading cause of neonatal death in Africa. More than a million children die each year due to co-morbidities related to prematurity. In addition to being one of the causes of neonatal deaths, the health problems associated with prematurity can also lead to severe lifelong impairment in those who survive. Objectives: This paper aims to determine the epidemiology and identify co-morbidities of prematurity in the neonatology units of the Douala General Hospital (DGH) and the Laquintinie Hospital of Douala (LHD). Patients and Methodology: We conducted an analytical retrospective cohort study from January 2015 to January 2018 in the neonatology department of the GDH and the LHD, which are considered reference hospitals for the management of preterm babies in Cameroon. We included all newborns aged less than 37 weeks admitted to the neonatology units of the GDH and the LHD. The descriptive component was based on the analysis of quantitative variables using measures of central tendency. The analytical component was evaluated using Spearman correlations and the Chi-square and Fisher tests. Simple and multiple logistic regressions measured factors predictive of mortality. The Kaplan Meier survival curve used the Log Rank test and significance at p ≤ 0.05. Results: We recorded 908 preterm newborns in neonatal service and 1,124 preterm deliveries in maternity, representing an incidence of 32.5% in neonatal unit and 10.6% in maternity. 51% of whom were girls, given a sex ratio M/F of 0.9. Hypertension was the main prenatal pathology (9.1%), while premature rupture of membranes: PROM (35.5%) and eclampsia/pre-eclampsia (18.6%) were the most common obstetrical pathologies. 75.9% of deliveries were vaginal with 65.2% being performed in our referral hospitals. Gestational age ranged from 22 to 36 weeks, with a mean of 32.4 weeks. Late preterm birth rate was 53.7%, and birth weight varied between 590 and 3200 g with an average of 1747 ± 479 g. The per

关键词： Epidemiology Co-Morbidities Prematurity Douala