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PLCG2 Mutation in a Patient Presenting with Type 2 Autoimmune Hepatitis

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Journal of Clinical and Translational Hepatology 2024年第7期12卷 685-687页

作者： Antoine Gardin Léa Gaigne Aude Magérus Frédéric Rieux-Laucat Emmanuel Jacquemin Pediatric Hepatology and Liver Transplantation Unit National Reference Centre for Inflammatory Diseases of the Bile Ducts and Autoimmune HepatitisFILFOIEERN RARE LIVERBicêtre HospitalAssistance Publique–Hôpitaux de ParisUniversity Paris-SaclayLe Kremlin-BicêtreFrance inserm UMR 1193 HepatinovParis-Saclay UniversityOrsayFrance Laboratory of Immunogenetics of Pediatric Autoimmune Diseases INSERM UMR 1163Imagine InstituteParis CitéUniversityParisFrance

The combination of defective immune tolerance and external triggers is thought to underlie the pathophysiology of autoimmune hepatitis.1,2 Some polymorphisms associated with susceptibility to autoimmune hepatitis have been reported,and some cases have been described in patients with monogenic primary immune deficiencies or systemic diseases.

关键词： autoimmune Autoimmune immune

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

Stem cell transplantation in cerebrovascular accidents:A global bibliometric analysis(2000-2023)

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World Journal of Stem Cells 2024年第9期16卷 832-841页

作者： Jad El Masri Ahmad Afyouni Maya Ghazi Karim Hamideh Israe Moubayed Abdo Jurjus Hanine Haidar Ruzanna Petrosyan Pascale Salameh Hassan Hosseini École Doctorale Sciences de la Vie et de la Santé UniversitéParis-Est CréteilCréteil 94010France inserm U955-E01 Institut Mondor de Recherche BiomédicaleUniversitéParis-Est CréteilCréteil 94000France Faculty of Medical Sciences Lebanese UniversityBeirut 1533Lebanon Department of Anatomy Cell Biology and Physiological SciencesFaculty of MedicineAmerican University of BeirutBeirut 1107Lebanon Department of Neurology Faculty of MedicineLebanese American UniversityBeirut 1102Lebanon Department of Pathology Faculty of MedicineLebanese American UniversityBeirut 1102Lebanon Faculty of Pharmacy Lebanese UniversityBeirut 1102Lebanon Faculty of Medicine Lebanese American UniversityBeirut 1102Lebanon Department of Primary Care and Population Health University of Nicosia Medical SchoolNicosia 2408Cyprus Institut National de SantéPublique d'Épidémiologie Clinique et de Toxicologie-Liban Beirut 1103Lebanon Department of Neurology Henri Mondor HospitalAP-HPCréteil 94000France

BACKGROUND Cerebrovascular accident(CVA)is a major global contributor to death and *** part of its medical management,researchers have recognized the importance of promising neuroprotective strategies,where stem cell transplantation(SCT)is thought to confer advantages via trophic and neuroprotective *** To evaluate the current state of research on SCT in patients with CVA,assess key trends and highlight literature *** PubMed was screened for SCT in CVA-related articles in October 2023,for each country during the period between 2000 and *** the World Bank data,total population and gross domestic product were collected for ***_1.6.19 was used to create the VOS figure using the results of the same *** and tables were obtained using Microsoft Office *** A total of 6923 studies were identified on SCT in CVA,making 0.03%of all published studies ***,68%were conducted in high-income countries,with a significant focus on mesenchymal stem *** journal“Stroke”featured the largest share of these articles,with mesenchymal SCT having the highest rate of inclusion,followed by hematopoietic *** time,there has been a noticeable shift from in vitro studies,which assess stem cell proliferation and neurogenesis,to in vivo studies aimed at evaluating efficacy and ***,the number of reviews increased along this *** This bibliometric analysis provides a comprehensive guide for physicians and researchers in the field through an objective overview of research activity,and highlights both current trends and *** a potential therapeutic role in CVA,more research is needed in the future to focus on different aspects of SCT,aiming to reach a better treatment strategy and improve life quality in patients.

关键词： Bibliometric analysis PubMed Stem cell transplantation Cerebrovascular accidents Stroke

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法国人群中结肠直肠癌根治术后的监测模式

世界核心医学期刊文摘（胃肠病学分册） 2006年第3期2卷 26-27页

作者： Boulin M. Lejeune C. Le Teuff G. C. BonithonKopp 郑世成 inserm emi 01-06 Facultéde Médecine 7 Boulevard Jeanne d'Arc 21079 Dijon Cedex France.Dr

PURPOSE: Despite controversies, postoperative surveillance of colorectal cancer is generally recommended. This registry-based study was designed to assess the adherence of surveillance practices to French guidelines and identify three-year surveillance patterns and their demographic and clinical determinants. METHODS: All patients (N = 409) diagnosed with first colorectal cancer in 1998 and alive without recurrence at least six months after curative surgery were identified from a population-based registry. Medical charts from multiple sources were reviewed to collect exhaustive information on follow-up procedures used during a three-year period. Multiple correspondence and cluster analyses were used to identify surveillance patterns. RESULTS: The proportion of patients with a lower surveillance than that recommended was 35 percent for clinical examination, 65 percent for abdominal ultrasound, 52 percent for chest x-ray, and 20 percent for colonoscopy. Cluster analysis identified three patterns called minimal, moderate, and intensive surveillance patterns, which included 47, 24, and 29 percent of the patients respectively. The main independent predictors of both moderate and intensive surveillance patterns vs. minimal pattern were advanced tumor stage, chemotherapy, and radiation therapy. Younger age also was strongly associated with the intensive surveillance pattern, and the presence of symptoms with the moderate surveillance pattern. CONCLUSIONS: Adherence of surveillance practices to French guidelines seems relatively poor. Surveillance patterns are mainly explained by patient age, tumor stage, and treatment modalities.

关键词：结肠直肠癌监测模式根治术肿瘤晚期结肠镜检查根治性手术聚类分析对应分析肿瘤分期术后监

Lipid-derived radical-trapping antioxidants suppress ferroptosis

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Life Metabolism 2024年第2期3卷 6-8页

作者： Ruoxi Zhang Guido Kroemer Daolin Tang Department of Surgery UT Southwestern Medical CenterDallasTX 75390United States Centre de Recherche des Cordeliers INSERM U1138Equipe labellisée-Ligue contre le cancerUniversitéParis CitéSorbonne UniversitéInstitut Universitaire de FranceParis 75006France Metabolomics and Cell Biology Platforms Gustave Roussy Cancer CampusVillejuif 94800France Institut du Cancer Paris CARPEM Department of BiologyHôpital Européen Georges PompidouAssistance Publique-Hôpitaux de ParisParis 75015France

Ferroptosis,characterized by lipid peroxidation-mediated cell demise,is governed by a nuanced interplay of lipid species influencing its *** recent publications in Nature discovered 7-dehydrocholesterol,a cholesterol precursor,as a radical-trapping antioxidant that can suppress ferroptosis,thereby presenting a novel metabolic target to improve ferroptosis-related cancer therapy.

关键词： Lipid peroxidation cholesterol

How does chronic dry eye shape peripheral and central nociceptive systems?

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Neural Regeneration Research 2021年第2期16卷 306-307页

作者： Adrian Guerrero-Moreno Darine Fakih Stéphane Melik Parsadaniantz Annabelle Réaux-Le Goazigo Sorbonne Université INSERMCNRSInstitut de la VisionParisFrance

Dry eye disease(DED)is a multifactorial disease characterized by a loss of homeostasis of the tear film and accompanied by ocular symptoms,in which tear film instability and hyperosmolarity,ocular surface inflammation... 详细信息

关键词： homeostasis al. ocular

Mediator contributes to IgH locus VDJ rearrangements by promoting usage of most distal V segments

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Cellular & Molecular Immunology 2020年第4期17卷 407-409页

作者： Iman Dalloul Zeinab Dalloul Sandrine Le Noir François Boyer Bernardo Reina-San-Martin Jeanne Cook-Moreau Michel Cogné UMR CNRS 7276 INSERM U1262Universite de LimogesLimoges 87025France inserm U1258 UMR CNRS 7104Institut de Genetique et de Biologie Moleculaire et CellulaireUniversitéde StrasbourgIllkirch 67400France

INTRODUCTION The immunoglobulin heavy(IgH)chain locus includes a large cluster of V,D and J segments spanning thousands of kilobases in mammals and its expression implies long-range 3D-regulation of various gene modifications.1 V(D)J recombination occurs during early B-cell ontogeny.2 It is regulated by multiple transcription factors and cis-regulatory elements,and most notably by the intronic 5′Eμelement located in the JH to Cμintron.3 The intergenic control region 1(IGCR1)located between the VH and D gene clusters inhibits rearrangement of the most DH-proximal VH gene segments,while promoting usage of distal VH gene segments.4 Other downstream elements of the locus,flanking its 3′end might have more subtle roles:while the large 3′regulatory region(3′RR)shows strong internal synergies2 and behaves as a superenhancer in mature B *** deletion showed no influence on the VDJ repertoire and rather increased transcription of unrearranged V segments.3–5 Insulators binding CTCF farther downstream of the 3′RR were shown,however,to promote usage of distal VH segments.6 Altogether,there are strong indications that elements involved in long-distance regulation of transcription might also influence VDJ ***-frame splicing of VDJ exons is also needed for full Ig expression.7 Given the functional importance of gene architecture and long-range interactions for the IgH locus physiology,we explored the dependence of the VDJ repertoire on Med1,a known actor of long-range enhancer interactions.

关键词： B cells mediator VDJ repertoire

脯氨酰内肽酶对乳糜泻的麦胶蛋白肽解毒作用有限

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世界核心医学期刊文摘（胃肠病学分册） 2006年第1期2卷 25-26页

作者： Matysiak-Budnik T. Candalh C. Cellier C. 孟欣颖 inserm emi-0212 Faculté Necker-Enfants Malades 156 rue de Vaugirard 75730 Paris France Dr.

Background& Aims: The resistance of prolamines to digestive enzymes is thought to be a key contributor to the pathogenesis of celiac disease by promoting the intestinal entrance of peptides able to trigger inflammation in at- risk individuals. Oral administration of a bacterial prolyl- endopeptidase (PEP) therefore was proposed as a treat- ment for celiac disease. To delineate the feasibility of this treatment, the effect of PEP on gliadin peptides was assessed in vitro, and ex vivo during their transport across intestinal biopsy specimens of active celiac disease patients. Methods: In vitro degradation by PEP of 3H- labeled gliadin peptides 56- 88 (33- mer) and 31- 49, was analyzed by radio- reverse- phase high- performance liquid chromatography and mass spectrometry. For ex vivo studies, PEP and 3H- peptides were added together onto the mucosal side of duodenal biopsy specimens mounted in Ussing chambers, and peptide transport and digestion were assessed by radio- reverse- phase high- performance liquid chromatography. Results: Gliadin peptides were degraded partly by 20 mU/mL PEP both in vitro and ex vivo. This concentration of PEP decreased the amount of intact peptides 31- 49 and 56- 88 crossing the intestinal biopsy specimens of celiac disease patients, but could not prevent the intestinal passage of toxic or immunostimulatory metabolites. PEP concentrations of at least 500 mU/mL for 3 hours were required to achieve full detoxification of peptides and to prevent intestinal transport of active fragments. Conclusions: After prolonged exposure to high concentrations of PEP, the amount of immunostimulatory gliadin peptides reaching the local immune system in celiac patients is decreased. These results provide a basis to establish whether such conditions are achievable in vivo.

关键词：乳糜泻麦胶蛋白解毒作用内肽酶氨酰肠黏膜体外试验黏膜面高效液相色谱法醇溶蛋白

New insights into the cell-and tissue-specificity of glucocorticoid actions

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Cellular & Molecular Immunology 2021年第2期18卷 269-278页

作者： Linda Quatrini Sophie Ugolini Department of Immunology IRCCS Bambino GesùChildren’s HospitalRomeItaly Aix Marseille Univ CNRSINSERMCentre d’Immunologie de Marseille-LuminyMarseilleFrance

Glucocorticoids(GCs)are endogenous hormones that are crucial for the homeostasis of the organism and adaptation to the external *** of their anti-inflammatory effects,synthetic GCs are also extensively used in clinical ***,almost all cells in the body are sensitive to GC *** a result,these mediators have pleiotropic effects,which may be undesirable or detrimental to human ***,we summarize the recent findings that contribute to deciphering the molecular mechanisms downstream of glucocorticoid receptor *** also discuss the complex role of GCs in infectious diseases such as sepsis and COVID-19,in which the balance between pathogen elimination and protection against excessive inflammation and immunopathology needs to be tightly *** understanding of the cell type-and context-specific actions of GCs from the molecular to the organismal level would help to optimize their therapeutic use.

关键词： Glucocorticoids inflammation immune response