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Targeted therapy for capillary-venous malformations

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

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SOME RECURRENCE FORMULAS FOR BOX SPLINES AND CONE SPLINES

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Analysis in Theory and Applications 2004年第4期20卷 297-306页

作者： patrick j. Van Fleet Department of Mathematics University of St. Thomas St. Paul MN 55105 USA

A degree elevation formula for multivariate simplex splines was given by Micchelli and extended to hold for multivariate Dirichlet splines in [8]. We report similar formulae for multivariate cone splines and box splines. To this end, we utilize a relation due to Dahmen and Micchelli that connects box splines and cone splines and a degree reduction formula given by Cohen, Lyche, and Riesenfeld in [2].

关键词： box spline cone spline degree elevation

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Determination of fractional energy loss of waves in nearshore waters using an improved high-order Boussinesq-type model

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Chinese journal of Oceanology and Limnology 2009年第3期27卷 621-629页

作者：何海伦宋金宝 patrick j. Lynett 李爽 Institute of Oceanology Chinese Academy of Sciences Key Laboratory of Ocean Circulation and Waves(KLOCAW) Chinese Academy of Sciences Graduate School Chinese Academy of Sciences Department of Civil Engineering Texas A&M University

Fractional energy losses of waves due to wave breaking when passing over a submerged bar are studied systematically using a modified numerical code that is based on the high-order Boussinesq-type *** model is first tested by the additional experimental data,and the model's capability of simulating the wave transformation over both gentle slope and steep slope is ***,the model's breaking index is replaced and *** new breaking index,which is optimized from the several breaking indices,is not sensitive to the spatial grid length and includes the bottom *** tests show that the modified model with the new breaking index is more stable and efficient for the shallow-water wave ***,the modified model is used to study the fractional energy losses for the regular waves propagating and breaking over a submerged *** results have revealed that how the nonlinearity and the dispersion of the incident waves as well as the dimensionless bar height(normalized by water depth) dominate the fractional energy *** is also found that the bar slope(limited to gentle slopes that less than 1:10) and the dimensionless bar length(normalized by incident wave length) have negligible effects on the fractional energy losses.

关键词： breaking waves Boussinesq-type equations nearshore wave modeling fractional energy loss

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Stability Characterization, Kinetics and Mechanism of Degradation of Dantrolene in Aqueous Solution: Effect of pH and Temperature

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Pharmacology & Pharmacy 2012年第3期3卷 281-290页

作者： Saeed R. Khan Mobin Tawakkul Vilayat A. Sayeed patrick faustino Mansoor A. Khan Division of Product Quality Research Office of Pharmaceutical Science Food and Drug Administration Life Science Building 64 10903 New Hampshire Avenue Silver Spring MD 20993 USA Office of Generic Drugs Food and Drug Administration 7500 Standish Place Rockville MD 20855 USA.

The mechanism of degradation of dantrolene in aqueous buffer solutions was studied at various pH values in the range of pH 1.2-9.5 and at temperatures ranging from 25℃ to 75℃ to determine the optimum pH and temperature requirements for its stability and eventual product performance over the human gastrointestinal pH range. Dantrolene was analyzed by reversed phase ultra-performance liquid chromatographic (UPLC). Chromatographic separation was achieved on a Waters Acquity UPLC system using a Waters BEH C18 analytical column and Waters BEH C18 guard column. The compounds were eluted with a linear acetonitrile gradient (25%-75%) over three minutes with a buffer composition of 2.0 mM of sodium acetate at pH 4.5 for degradation studies. The flow rate was maintained at 0.5 mL/min. Column temperature was maintained at 35℃. Injection volume was 4 μL and the degradation products were detected by a photodiode array (PDA) detector at 375 nm. Degradation products, including compound B and C were analyzed by mass spectroscopy (MS) and nuclear magnetic resonance spectroscopy (NMR) and the degradation pathways were proposed. Degradation of dantrolene followed pseudo first–order kinetics and a V-shaped pH-rate profile over the pH range 1.2-9.5. The maximum stability was observed at pH 7.4 and 37℃. Although the focus of this paper was on the mechanism of hydrolysis of dantrolene, the poor aqueous solubility of dantrolene, the developed understanding can be utilized to improve the quality of the formulation and the risk associated with the extravasation of dantrolene sodium solution in its current form.

关键词： Dantrolene UPLC pH Impurity Degradation Compound B C.

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Chronic peripheral inflammation: a possible contributor to neurodegenerative diseases

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Neural Regeneration Research 2021年第9期16卷 1711-1714页

作者： patrick Süβ Addison j.Lana johannes C.M.Schlachetzki Department of Molecular Neurology Friedrich-Alexander-University Erlangen-NürnbergUniversity Hospital ErlangenErlangenGermany Department of Cellular and Molecular Medicine University of California San DiegoLa JollaCAUSA

The contribution of chronic peripheral inflammation to the pathogenesis of neurodegenerative diseases is an outstanding question. Sustained activation of the peripheral innate and adaptive immune systems occurs in the context of a broad array of disorders ranging from chronic infectious diseases to autoimmune and metabolic diseases. In addition, progressive systemic inflammation is increasingly recognized during aging. Peripheral immune cells could potentially modulate the cellular brain environment via the secretion of soluble molecules. There is an ongoing debate whether peripheral immune cells have the potential to migrate into the brain under certain permissive circumstances. In this perspective, we discuss the possible contribution of chronic peripheral inflammation to the pathogenesis of age-related neurodegenerative diseases with a focus on microglia, the resident immune cells of the brain parenchyma.

关键词： aging Alzheimer's disease inflammation innate immune system microglia neurodegeneration Parkinson's disease

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A fine-scale assessment of the ecosystem service-disservice dichotomy in the context of urban ecosystems affected by alien plant invasions

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Forest Ecosystems 2019年第4期6卷 323-338页

作者： Luke j.Potgieter Mirijam Gaertner patrick j.O’Farrell David M.Richardson Centre for Invasion Biology Department of Botany and ZoologyStellenbosch University

Background: Natural resources within and around urban landscapes are under increasing pressure from ongoing urbanisation, and management efforts aimed at ensuring the sustainable provision of ecosystem services(ES) are an important response. Given the limited resources available for assessing urban ES in many cities, practical approaches for integrating ES in decision-making process are ***: We apply remote sensing techniques(integrating LiDAR data with high-resolution multispectral imagery)and combined these with supplementary spatial data to develop a replicable approach for assessing the role of urban vegetation(including invasive alien plants) in providing ES and ecosystem disservices(EDS). We identify areas denoting potential management trade-offs based on the spatial distribution of ES and EDS using a local-scale case study in the city of Cape Town, South Africa. Situated within a global biodiversity hotspot, Cape Town must contend with widespread invasions of alien plants(especially trees and shrubs) along with complex socio-political challenges. This represents a useful system to examine the challenges in managing ES and EDS in the context of urban plant ***: Areas of high ES provision(for example carbon sequestration, shade and visual amenity) are characterized by the presence of large trees. However, many of these areas also result in numerous EDS due to invasions of alien trees and shrubs – particularly along rivers, in wetlands and along the urban edge where tall alien trees have established and spread into the natural vegetation(for example increased water consumption, increased fire risk and reduced soil quality). This suggests significant trade-offs regarding the management of species and the ES and EDS they ***: The approach applied here can be used to provide recommendations and to guide city planners and managers to fine-tune management interventions at local scales to maximise the provision of ES.

关键词： Biodiversity Biological invasions Ecosystem disservices Ecosystem services Remote sensing Trade-offs Tree invasions Urban plant invasions

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Cathodic activated stainless steel mesh as a highly active electrocatalyst for the oxygen evolution reaction with self-healing possibility

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journal of Energy Chemistry 2020年第10期29卷 153-160页

作者： Gui-Rong Zhang Liu-Liu Shen patrick Schmatz Konrad Krois Bastian j.M.Etzold Ernst-Berl-Institut für Technische und Makromolekulare Chemie Technische Universit鋞Darmstadt64287 DarmstadtGermany

The oxygen evolution reaction(OER)represents one of the major bottlenecks for broad-based applications of many clean energy storage/conversion *** key to solving this problem lies in developing high-performing,cost effective and stable catalysts for the ***,we demonstrate that ubiquitous stainless steel mesh(SSM)materials activated by a facile cathodization treatment can be employed as a high performing OER catalyst,as showcased by the impressively low overpotentials of 275 and 319 mV to reach the benchmark current densities of 10 and 100 mA cm^−2(1.0 M KOH),*** SSM also exhibits excellent performance in a two-electrode water electrolyzer,which requires a low cell voltage of 1.58 at 10 mA cm^−2 and outperforms many of water electrolyzers using earth-abundant OER ***,cathodized SSM with minor performance degradation after the stability test can also be readily healed by subjecting it to an additional cathodization *** is disclosed that the superior performance of cathodized SSMs stems from the surface enrichment of OER active Ni(oxy)hydroxide,facile gas-bubble removal and transportation over the unique mesh-structured surfaces,while the abundant reservoir of nickel in the bulk allows healing of the catalyst by a facile cathodization.

关键词： Oxygen evolution reaction Water splitting Stainless steel mesh Cathodization Mass transfer limitation Self-healing

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A Thickness Gauge for the Lithosphere Based on Ce/Yb and Sm/Yb of Mantle–Derived Magmatic Rocks

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Acta Geologica Sinica(English Edition) 2018年第6期92卷 2120-2135页

作者： WU Zhenhan patrick j. BAROSH ZHANG Qichao WU jiwen YANG Yan Chinese Academy of Geological Sciences Beijing 100037China P.j.Barosh and Associates 103 Aaron Ave.BristolRI02809USA Institute of Karst Geology Chinese Academy of Geological SciencesGuilin 541004China

A new method for determining the partial melting depth of mantle-derived magma and lithospheric thickness in continental regions is derived from REE geochemistry. This effective technique uses variations in the Ce/Yb and Sm/Yb ratios found in mainly volcanic rocks in continental China. The ratios change with the depth of origin consistent with the correlation between lithospheric thickness and the Ce/Yb and Sm/Yb ratios found in oceanic basalt. These ratios increase exponentially with the depth of origin, the lithospheric thickness, of a wide variety of Cenozoic volcanic basalt and Paleozoic kimberlite in the North China Craton, northeastern China continent and vicinity. This functional relationship with depth is shown in a plot of the ratios that forms a concordia curve, which is closely expressed by formulas using 8–degree polynomials. These provide a more accurate gage in measuring the lithospheric thickness than the traditional geophysical methods. When applied to volcanic rock of different ages it also reveals how the thickness has changed over time and thus, greatly aids the understanding of the tectonic history. Relations between the COcontent, mineral reactions and pressure in the upper asthenosphere beneath the base of the lithosphere appears to affect the proportions of REE in partial melts and brings about a close correlation between lithospheric thickness and the Ce/Yb and Sm/Yb ratios in mantle–derived magmatic rock. This thickness gauge, for both continental and oceanic lithosphere, provides a new approach in analyzing the lithospheric thickness in different tectonic settings and geologic times.

关键词： Ce/Yb and Sm/Yb ratios mantle–derived magmatic rock lithospheric thickness correlation analysis North China Craton

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Low Gate Voltage Operated Multi-emitter-dot H^+ Ion-Sensitive Gated Lateral Bipolar junction Transistor

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Chinese Physics Letters 2015年第2期32卷 27-30页

作者：袁珩张冀星张晨张宁徐丽霞丁铭 patrick j.C Science and Technology on Inertial Laboratory Beihang University School of Instrumentation Science and Opto-electronics Engineering Beihang University

A low gate voltage operated multi-emitter-dot gated lateral bipolar junction transistor （BjT） ion sensor is proposed. The proposed device is composed of an arrayed gated lateral BjT, which is driven in the metal-oxidesemiconductor field-effect transistor （MOSFET）-BjT hybrid operation mode. Further, it has multiple emitter dots linked to each other in parallel to improve ionic sensitivity. Using hydrogen ionic solutions as reference solutions, we conduct experiments in which we compare the sensitivity and threshold voltage of the multi-emitter-dot gated lateral BjT with that of the single-emitter-dot gated lateral BjT. The multi-emitter-dot gated lateral BjT not only shows increased sensitivity but, more importantly, the proposed device can be operated under very low gate voltage, whereas the conventional ion-sensitive field-effect transistors cannot. This special characteristic is significant for low power devices and for function devices in which the provision of a gate voltage is difficult.

关键词： BjT MOSFET Ion-Sensitive Gated Lateral Bipolar junction Transistor Low Gate Voltage Operated Multi-emitter-dot H

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Tectonics and Topography of the Tibetan Plateau in Early Miocene

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Acta Geologica Sinica(English Edition) 2014年第2期88卷 410-424页

作者： WU Zhenhan YANG Yan patrick j. BAROSH WU Zhonghai ZHANG Yaoling Chinese Academy of Geological Sciences P.j. Barosh and Associates Institute of Geomechanics Chinese Academy of Geological Sciences

Early Miocene stratigraphy,major structural systems,magmatic emplacement,volcanic eruption,vegetation change and paleo-elevation were analyzed for the Tibetan Plateau after regional geological mapping at a scale of 1：250,000 and related researches,revealing much more information for tectonic evolution and topographic change of the high plateau caused by Indian-Asian continental *** deposits of dolostone,dolomite limestone,limestone,marl,sandstone and conglomerate of weak deformation formed extensively in the central Tibetan Plateau,indicating that vast lake complexes as large as 100,000-120,000 km2 existed in the central plateau during Early *** assemblages contained in the lacustrine strata indicate the disappearance of most tropical-subtropical broad-leaved trees since Early Miocene and the flourishing of dark needle-leaved trees during Early *** vegetation changes adjusted for latitude and global climate variations demonstrate that the central Tibetan Plateau rose to ca.4,000-4,500 m and the northeastern plateau uplifted to ca.3,500-4,000 m before the Early *** thrust and crustal thickening occurred in the areas surrounding central Tibetan Plateau in Early Miocene,formed Gangdise Thrust System （GTS） in the southern Lhasa block,Zedong-Renbu Thrust （ZRT） in the northern Himalaya block,Main Central Thrust （MCT） and Main Boundary Thrust （MBT） in the southern Himalaya block,and regional thrust systems in the Qaidam,Qilian,West Kunlun and Songpan-Ganzi *** basins formed in Early Miocene along major thrust systems,*** Siwalik basin along MCT,Yalung-Zangbu Basin along GTS and ZRT,southwestern Tarim depression along West Kunlun Thrust,and large foreland basins along major thrust systems in the northeastern margin of the *** volcanic eruptions formed in the Qiangtang,Hoh-Xil and Kunlun blocks,porphyry granites and volcanic eruptions formed in the Nainqentanglha and Gangdise Mts.,and leuco

关键词： Lacustrine deposits vast lakes elevation change regional thrust system Early Miocene Tibetan Plateau

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