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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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Phenylpropanoid Biosynthesis

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Molecular Plant 2010年第1期3卷 2-20页

作者： thomas Vogt Leibniz-lnstitute of Plant Biochemistry Department of Secondary Metabolism Weinberg 3 D-06120 Halle (Saale) Germany

The general phenylpropanoid metabolism generates an enormous array of secondary metabolites based on the few intermediates of the shikimate pathway as the core unit. The resulting hydroxycinnamic acids and esters are am- plified in several cascades by a combination of reductases, oxygenases, and transferases to result in an organ and devel- opmentally specific pattern of metabolites, characteristic for each plant species. during the last decade, methodology driven targeted and non-targeted approaches in several plant species have enabled the identification of the participating enzymes of this complex biosynthetic machinery, and revealed numerous genes, enzymes, and metabolites essential for regulation and compartmentation. Considerable success in structural and computational biology, combined with the an- alytical sensitivity to detect even trace compounds and smallest changes in the metabolite, transcript, or enzyme pattern, has facilitated progress towards a comprehensive view of the plant response to its biotic and abiotic environment. Trans- genic approaches have been used to reveal insights into an apparently redundant gene and enzyme pattern required for functional integrity and plasticity of the various phenylpropanoid biosynthetic pathways. Nevertheless, the function and impact of all members of a gene family remain to be completely established. This review aims to give an update on the various facets of the general phenylpropanoid pathway, which is not only restricted to common lignin or flavonoid biosynthesis, but feeds into a variety of other aromatic metabolites like coumarins, phenolic volatiles, or hydrolyzable tannins.

关键词： Phenylpropanoid biosynthetic pathway flavonoid anthocyanin tannin coumarin volatiles lignin.

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What would a US green hydrogen energy economy look like?

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Clean Energy 2023年第5期7卷 1148-1172页

作者： thomas Tonon Independent researcher Ph.D.Aerospace and Mechanical SciencesPrinceton University1971.11 Bolfmar AvenuePrinceton JunctionNJ 08550USA

detailed description is given for a hypothetical US hydrogen economy with solar and wind energy supplying virtually all current energy needs and with electrolytic hydrogen the energy carrier and storage *** fuels provide nonfuel products(plastics,chemicals,cement and asphalt).Only current technologies are considered and hydrogen storage accommodates generation intermit-tency and variability,using pit storage of high-pressure vessels in open air,yielding daily storage round-trip energy installation costs of 722 and 538$/kWh for electric and thermal,respectively;and for power,2351 and 2240$/kW for electric and thermal,*** long-duration storage,the costs are 94.1 and 23.8$/kWh and 937 and 845$/kW,*** energy generation 20%over baseline accommodates low-season generation,obviates much required storage and ensures that reserves are topped off;96%of US 2022 total energy consumption is provided *** the default scenario(demand energy portions:half photovoltaic,quarter on-shore wind and quarter offshore wind),the surface area for the farms(including offshore surface)requires~4.6%of the US 48-state land *** 350 pit storage sites provide both daily and long-duration storage,with the latter accounting for complete loss of generation for 4 days over a quarter of the *** pipelines and a renewed electric grid transmit and distribute *** installation cost of the public infrastructure is~$27.8 trillion for the default *** scenarios show significant in-frastructure and cost savings when batteries are used for transportation and/or utility storage,provided current insufficiencies can be ***,cost levels in money,surface and infrastructure are within existing levels already achieved in historical events and modern living.

关键词： hydrogen economy renewable energy hydrogen storage hydrogen pipelines sustainable net zero

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A new institution devoted to insect science： The Florida Museum of Natural History, McGuire Center for Lepidoptera and Biodiversity

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Insect Science 2012年第3期19卷 426-428页

作者： Akito Y. Kawahara thomas C. Emmel Jacqueline Miller Andrew d. Warren McGuire Center for Lepidoptera and Biodiversity Florida Museum of Natural History University of Florida Gainesville Florida USA

The Florida Museum of Natural History＇s McGuire Center for Lepidoptera and Biodiversity, on the University of Florida campus in Gainesville, Florida, has become one of the world＇s largest institutions for research on butterflies and moths, and an important research facility for insect science. The facility was constructed by combining the staff and merging the Lepidoptera holdings from the Allyn Museum of Entomology, the Florida State Collection of Arthropods and other University of Florida collections, and now includes over ten million specimens from all over the world, rivaling some of the largest Lepidoptera research collections globally. The facility includes a team of domestic and international researchers studying many areas of lepidopterology, including behavior, biodiversity, biogeography, ecology, genomics, physiology, systematics and taxonomy. In this paper, we introduce the McGuire Center, its staff, and the many research activities for researchers across entomological disciplines.

关键词： Florida Museum of Natural History Lepidoptera lepidopterology McGuireCenter moth new research facility

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Recent result from the A2 real photon facility at MAMI

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Chinese Physics C 2009年第12期33卷 1120-1126页

作者： Andreas thomas Institut fr Kernphysik University Mainz J.-J. Becherweg 45 D-55099 Mainz Germany

The upgraded MAMI C accelerator is delivering electrons with an energy of 1558 MeV routinely. The A2 collaboration is doing experiments with energy marked polarised real photons produced via ＂Bremsstrahlung＂. Recent results from the GdH-experiment using a longitudinal polarised frozen spin target in combination with the dAPHNE detector and from the Crystal Ball experiment at MAMI are presented.

关键词： photonuclear reactions polarization phenomena in reactions polarized and other targets leptoninduced reactions

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World Journal of Gastroenterology 2020年第22期26卷 2931-2947页

作者： Tea L Laursen thomas d Sandahl Konstantin Kazankov Jacob George Henning Gronbæk department of Hepatology and Gastroenterology Aarhus University HospitalAarhus N DK-8200Denmark Storr Liver Centre Westmead Institute for Medical ResearchWestmead HospitalSydney NSW 2145Australia University of Sydney Sydney NSW 2145Australia

More than five years ago,the treatment of hepatitis C virus infection was revolutionized with the introduction of all-oral direct-acting antiviral(dAA)*** proved highly efficient in curing patients with chronic hepatitis C(CHC),including patients with *** new dAA treatments were alleged to induce significant improvements in clinical outcome and prognosis,but the exact cause of the expected benefit was ***,little was known about how the underlying liver disease would be affected during and after viral *** this review,we describe and discuss the liver-related effects of the new treatments in regards to both pathophysiological aspects,such as macrophage activation,and the time-dependent effects of therapy,with specific emphasis on inflammation,structural liver changes,and liver function,as these factors are all related to morbidity and mortality in CHC *** seems clear that antiviral therapy,especially the achievement of a sustained virologic response has several beneficial effects on liver-related parameters in CHC patients with advanced liver fibrosis or *** seems to be a timedependent effect of dAA therapy with viral clearance and the resolution of liver inflammation followed by more discrete changes in structural liver *** improvements lead to favorable effects on liver function,followed by an improvement in cognitive dysfunction and portal ***,the data provide knowledge on the several beneficial effects of dAA therapy on liverrelated parameters in CHC patients suggesting short-and long-term improvements in the underlying disease with the promise of an improved longterm prognosis.

关键词： Chronic hepatitis C Antiviral treatment Inflammation Liver fibrosis Liver cirrhosis Metabolic liver function Galactose elimination capacity Urea synthesis capacity Portal hypertension Hepatic encephalopathy

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Is thirty-seven years sufficient for full return of the ant biota following restoration?

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Ecological Processes 2013年第1期2卷 183-194页

作者： Jonathan d Majer Brian Heterick thomas Gohr Elliot Hughes Lewis Mounsher Andrew Grigg Curtin Institute for Biodiversity and Climate Curtin UniversityPO Box U1987PerthWA 6845Australia Alcoa of Australia Ltd PO Box 172PinjarraWA 6208Australia

Introduction:An assessment of whether rehabilitated mine sites have resulted in natural or novel ecosystems requires monitoring over considerable periods of time or the use of space-for-time substitution(chronosequence)***:To provide an assessment of ecosystem recovery in areas mined for bauxite in 1975,the ant fauna of one area planted with Eucalyptus resinifera,one seeded with mixed native species,one topsoiled but unrestored,and a forest reference was subjected to a‘long-term’study by sampling monthly and latterly annually between 1976 and 1989 using pitfall *** plots were resampled in 2012.A companion‘short-term’chronosequence study was performed in 1979 in 28 bauxite mines of various ages and restored by a range of different methods,plus three forest *** order to examine the assertion that the observed differences between restored areas and forest references will lessen with time,sampling using comparable methods was repeated in 2012 in seven of the original plots,representing progressive advances in rehabilitation technology:planted pines;planted eastern states eucalypts;planted native eucalypts;planted eucalypts over seeded understorey;and planted eucalypts on fresh,double-stripped topsoil,plus two forest reference ***:Ant and other invertebrate richness in the long-term study was initially superior in the seeded plot,with little difference between the planted and unplanted *** was concluded that although composition of the ant fauna had converged on that of the forest over the 14-year period,differences still *** 2012 resampling revealed that ant species richness and composition had deteriorated in the seeded plot,while values in the unplanted plot,which now supported naturally colonised trees and an understorey,had *** between all rehabilitated plots and forest still *** with the long-term study,the rate of fauna return and the type of ants present in the short-term study p

关键词： Bauxite mining Ant bioindicator Long-term study Chronosequence Seeding Planting Succession

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Locoregional therapies and their effects on the tumoral microenvironment of pancreatic ductal adenocarcinoma

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World Journal of Gastroenterology 2022年第13期28卷 1288-1303页

作者： thomas Lambin Cyril Lafon Robert Andrew drainville Mathieu Pioche Frédéric Prat LabTAU INSERMCentre Léon BérardUniversitéLyon 1Univ LyonLyon 69003France department of Gastroenterology Hospices Civils de LyonEdouard Herriot HospitalLyon 69008France Service d’Endoscopie digestive Hôpital BeaujonAssistance Publique-Hôpitaux de ParisClichy 92110France INSERM U1016 Institut CochinUniversitéde ParisParis 75014France

Pancreatic ductal adenocarcinoma(PdAC) is expected to become the second leading cause of death from cancer by 2030. despite intensive research in the field of therapeutics, the 5-year overall survival is approximately 8%, with only 20% of patients eligible for surgery at the time of diagnosis. The tumoral microenvironment(TME) of the PdAC is one of the main causes for resistance to antitumoral treatments due to the presence of tumor vasculature, stroma, and a modified immune response. The TME of PdAC is characterized by high stiffness due to fibrosis, with hypo microvascular perfusion, along with an immunosuppressive environment that constitutes a barrier to effective antitumoral treatment. While systemic therapies often produce severe side effects that can alter patients’ quality of life, locoregional therapies have gained attention since their action is localized to the pancreas and can thus alleviate some of the barriers to effective antitumoral treatment due to their physical effects. Local hyperthermia using radiofrequency ablation and radiation therapy-most commonly using a local high single dose-are the two main modalities holding promise for clinical efficacy. Recently, irreversible electroporation and focused ultrasound-derived cavitation have gained increasing attention. To date, most of the data are limited to preclinical studies, but ongoing clinical trials may help better define the role of these locoregional therapies in the management of PdAC patients.

关键词： Pancreatic ductal adenocarcinoma Tumoral microenvironment Stroma Hyperthermia Radiation therapy High-intensity focused ultrasound

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Personalized targeted therapy for esophageal squamous cell carcinoma

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World Journal of Gastroenterology 2015年第25期21卷 7648-7658页

作者： Xiaozheng Kang Keneng Chen Yicheng Li Jianying Li thomas A d'Amico Xiaoxin Chen Key Laboratory of Carci-nogenesis and Translational Research (Ministry of Education) The First Department of Thoracic Surgery Peking University Cancer Hospital and Institute Cancer Research Program Julius L. Chambers Biomedical Biotechnology Research Institute North Carolina Central University Euclados Bioinformatics Solutions division of Thoracic Surgery Duke University Medical Center Center for Esophageal disease and Swallowing Division of Gastroenterology and Hepatology Department of Medicine University of North Carolina at Chapel Hill

Esophageal squamous cell carcinoma continues to heavily burden clinicians worldwide. Researchers have discovered the genomic landscape of esophageal squamous cell carcinoma, which holds promise for an era of personalized oncology care. One of the most pressing problems facing this issue is to improve the understanding of the newly available genomic data, and identify the driver-gene mutations, pathways, and networks. The emergence of a legion of novel targeted agents has generated much hope and hype regarding more potent treatment regimens, but the accuracy of drug selection is still arguable. Other problems, such as cancer heterogeneity, drug resistance, exceptional responders, and side effects, have to be surmounted. Evolving topics in personalized oncology, such as interpretation of genomics data, issues in targeted therapy, research approaches for targeted therapy, and future perspectives, will be discussed in this editorial.

关键词： Cancer heterogeneity Cultured tumorcells driver mutation drug side effects Esophagealsquamous cell carcinoma Exceptional responder Highthroughputnucleotide sequencing Neoplasm drugresistance Personalized medicine Xenograft model

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Associations between Bodyweight and Clinical Outcome in Patients Post-Fontan Procedure: A Systematic Review

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Congenital Heart disease 2022年第6期17卷 617-639页

作者： Emma Payne thomas Wilson Marjan Haghighi Zoe McCallum Yves d’Udekem Julian Ayer The University of Sydney SydneyAustralia The University of Melbourne MelbourneAustralia The Royal Children’s Hospital MelbourneAustralia The Royal Prince Alfred Hospital SydneyAustralia The Children’s National Hospital WashingtonUSA

Background: Patients born with a single ventricle circulation commonly experience growth failure in early life,which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectoryand clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, asystematic review was performed in MEdLINE, EMBASE, the Cochrane Library, and Scopus of studies of patientswith clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluatedby Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional ***: Of 527 studies that underwent title and abstract screening, 15 were selected for final review. An increasedrisk of adverse post-Fontan outcomes was found for low weight patients, consistent with findings in *** there is some evidence to suggest increased mortality in overweight adult patients, studies are conflictingas to whether overweight status is associated with increased heart failure. Increased BMI is associated with diminishedexercise capacity and deceased physiological functioning. Negative weight trajectory is associated withadverse outcomes in the peri-Fontan period, whereas a positive weight trajectory is associated with increased Fontanfailure in adulthood. Abnormal BMI (high or low) is associated with increased heart failure and poorer performancein quality-of-life scores. Conclusions: Bodyweight is a modifiable risk factor for poor clinical outcomein patients with a single ventricle circulation. Recognizing associations between bodyweight and Fontan pathophysiologymay help to define patient-centered exercise and diet interventions that minimize patient morbidityand mortality.

关键词： Fontan bodyweight congenital heart disease single ventricle

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