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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

Clinical and Bacteriological Profile of Infections in Sickle Cell Children in Two Referral hospitals in Niamey, Niger

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Open Journal of Pediatrics 2024年第1期14卷 36-42页

作者： Kamaye Moumouni Samaila Aboubacar Garba Moumouni Georges Thomas Ibrahim Mamoudou Abdou djafar Mamane halima hamadou Ibrahim hamani Issaka djibrilla Almoustapha Amadou Yacouba Abdourahamane Marou Soumana Boubacar Moussa Saley Sahada Bade Malam Abdou Soumana Alido service de Pédiatrie A H&#244pital National de Niamey Niamey Niger Faculté des Sciences de la Santé Université Abdou Moumouni de Niamey Niamey Niger service de Pédiatrie H&#244pital national Amirou Boubacar Diallo Niamey Niger service d’Onco-hématologie H&#244pital national de Niamey Niamey Niger service de Biologie Médicale H&#244pital national Amirou Boubacar Diallo Niamey Niger

Introduction: Infections are significant causes of mortality in sickle cell children in resource-limited countries. This study aimed to determine the clinical profile and bacterial ecology of infections in children with sickle-cell disease in two referral hospitals in Niamey. Patients and methods: A retrospective descriptive study was conducted from January 2018 to July 2020 in two referral hospitals in Niamey. All children aged one (1) to 15 years with sickle cell disease admitted for suspected infection, including at least one bacterial culture, were studied. Bacteriological analysis was performed using the appropriate culture media, using BactAlert (Reference 4700003 BTA3d60 BioMérieux). Results: Over 36-months, 350 children with a mean age of 10.9 months were admitted. The sex ratio was 1.2. The SS electrophoretic profile was the most common (93.4%). Immunization status was up to date in 66% of patients. Fever was the common reason for consultation (55.1%). Infection was confirmed in 62 patients (17.7%). The primary diagnoses were bacterial gastroenteritis (24.2%) and urinary tract infection (19.4%). Blood cultures were isolated from Salmonella typhi (13.0%) and Escherichia coli (8.7%). Klebsiella spp (7.1%) and Escherichia coli (5.0%) were detected in cytobacteriological examination of urine. Salmonella typhi (23.5%) and Escherichia coli (5.9%) were isolated on coproculture. Conclusion: Bacterial ecology appears not different from that usually observed in sickle-cell children. Salmonella and Escherichia coli were predominant.

关键词： Sickle Cell disease Child Infection Niger

Effectiveness and safety of therapeutic plasma exchange to modify the functionality of heparin-induced thrombocytopenia antibodies and correct profound thrombocytopenia:A case report and literature review

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Journal of Intensive Medicine 2023年第4期3卷 373-375页

作者： Audrey Graser Anne Bauters Jean-Luc Auffray Caroline Vayne François Provot Merce Jourdain Laurent Robriquet department of Intensive Care Medicine CHU LilleLille F-59000France service d'hémostase-Transfusion Institut d'HématologieCentre de Biologie Pathologie GénétiqueBoulevard du Professeur Jules LeclercqCentre Hospitalier Universitaire de LilleLille 59037France service de Cardiologie Institut Coeur PoumonBoulevard du Professeur Jules LeclercqCentre Hospitalier Universitaire de LilleLille 59037France service d'hématologie-hémostase Centre Hospitalier Universitaire de ToursUniversitéde ToursTours EA4245 T2IFrance Pôle de Néphrologie Hôpital Albert CalmetteBoulevard du Professeur Jules LeclercqCentre Hospitalier Universitaire de LilleLille 59037France department of Intensive Care Medicine Univ LilleInsermCHU LilleLille F-59000France

Introduction heparin-induced thrombocytopenia(hIT)is a rare but life-threatening complication of heparin *** IgG antibodies recognize heparin-modified platelet factor 4(PF4/h IgG)and activate the platelets,increasing ... 详细信息

关键词： heparin antibodies thrombocytopenia

Commentary Potential Enhancement by 3-deazauridine of the Antiviral Activity of Molnupiravir in Patients with COVId-19

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Advances in Infectious diseases 2023年第2期13卷 210-215页

作者： Richard L. Momparler département de Pharmacologie-Physiologie Université de Montréal and Service de Hématologie/Oncologie Centre de Recherche CHU Sainte-Justine Montréal Québec Canada

Background: Molnupiravir, N4-hydroxycytidine-5’-isopropyl ester, is an oral prodrug of N4-deoxycytidine (NhC), a nucleoside analog, which has in vitro activity against SARS-CoV-2. NhC is phosphorylated in cells to NhC triphosphate (NhC-TP), which is incorporated into viral RNA, leading to epigenetic catastrophe of the viral genome and inhibition of viral replication. The antiviral activity against SARS-CoV-2 is dependent on the number of molecules of NhC-TP incorporated into viral RNA. Clinical studies in patients with COVId-19 showed that treatment with molnupiravir for 5 days decreases the risk of hospitalization and death as compared with placebo. Objective: It should be possible to enhance the antiviral activity of NhC-TP against SARS-CoV-2 by the use of the biochemical modulator, 3-deazauridine (3dU). 3dU is an inhibitor of CTP synthetase. Inhibition of this enzyme results in a reduction in the intracellular pool size of CTP. Since NhC-TP competes with CTP for incorporation into viral RNA in the reaction catalyzed by the SARS-CoV-2 viral RNA-dependent RNA polymerase, the reduction in the level of CTP should result in a significant enhancement of the incorporation of NhC-TP into viral RNA and an enhancement of its antiviral activity. Methods: Analysis of the publications of 3dU and cytosine nucleoside analogues support the hypothesis that 3dU enhances the pharmacological action of the analogues. Results: 3-dU increased the incorporation of 5-azacytidine into RNA and 5-aza-deoxycytidine into dNA of leukemic cells with an enhancement of their antineoplastic action. 3-dU potentiated the antiviral activity against hIV-1 activity by the cytosine nucleoside analogues: 2’-deoxy-3’-thiacytidine (3TC;lamivudine) and 2’,3’-dideoxycytidine (ddC). This anti-hIV-1 activity of 3dU was associated with a reduction in the intracellular pool size of dCTP and increased incorporation of triphosphates of 3TC and ddC into dNA by the hIV-1 reverse transcriptase. The reduction of CTP l

关键词： Molnupiravir N4-hydroxycytidine-5’-Triphosphate 3-deazauridine COVId-16 Antiviral Therapy

The antiangiogenic phloroglucinol hyperforin inhibits the secretion of proMMP-2, proMMP-9 and VEGF-A during apoptosis of primary acute myeloid leukemia cells

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Journal of Cancer Metastasis and Treatment 2021年第1期7卷 588-603页

作者： Faten Merhi Ruoping Tang Ollivier Legrand Florence Nguyen-Khac Santos A.Susin Brigitte Bauvois Centre de Recherche des Cordeliers INSERMSorbonne UniversitéUSPCUniversitéParis DescartesUniversitéParis DiderotParis F-75006France department of Life&Earth Sciences Faculty of Sciences(I)Lebanese UniversityBeirutLebanon Assistance Publique-hôpitaux de Paris Groupe Hospitalier Saint-AntoineService d’Hématologie CliniqueParis F-75012France service Génétique des Tumeurs Département de Biologie et de Pathologie MédicaleGustave Roussy Cancer CamusVillejuif F-94805France Assistance Publique-hôpitaux de Paris Groupe Hospitalier Pitié-SalpêtrièreService d’Hématologie BiologiqueParis F-75013France.

Aim:Angiogenesis is observed in acute myeloid leukemia(AML).AML cells abnormally proliferate and are resistant to *** regulators of angiogenesis,VEGF-A and matrix metalloproteinases(MMPs)2 and 9 are markers of disease status in *** natural phloroglucinol hyperforin(hF)displays antitumoral properties of potential pharmacological ***,we investigated the effects of hF on MMP-2/9 and VEGF-A expression and survival of primary AML ***:Blood and bone marrow samples were collected in 45 patients with distinct subtypes defined by French American British classification,i.e.,M0,M1,M2,M3,M4,and *** of MMPs and VEGF-A in leukemic blood cells and culture supernatants were determined by RT-PCR,ELISA,and gelatin zymography(MMPs).The balance between cell death and survival was assessed by flow cytometry with analysis of phosphatidylserine externalization and caspase-3 ***:The administration of hF promoted a caspase-associated apoptosis in primary AML blasts(from blood and bone marrow),but not normal blood cells and *** addition,hF inhibited the levels of secreted proMMP-2,proMMP-9,and VEGF-A without altering *** induction of apoptosis by hF significantly paralleled the inhibition of MMP-2/9 and VEGF-A release by *** differences were seen in response to the deleterious effects of hF between AML cells of distinct ***:Our results suggest that hF,through its proapoptotic and potential antiangiogenic properties(by inhibiting MMP-2/9 and VEGF-A)on primary AML cells,might be a useful experimental agent,in combination with existing drugs,for new therapeutic approaches in the treatment of this incurable disease.

关键词： Acute myeloid leukemia apoptosis hyperforin matrix metalloproteinase VEGF secretion

Arsenic trioxide induces regulatory functions of plasmacytoid dendritic cells through interferon-α inhibition

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Acta Pharmaceutica Sinica B 2020年第6期10卷 1061-1072页

作者： Yishan Ye Laure Ricard Lama Siblany Nicolas Stocker Frédéric de Vassoigne Eolia Brissot Baptiste Lamarthée Arsène Mekinian Mohamad Mohty Béatrice Gaugler Florent Malard Sorbonne Université INSERMCentre de Recherche Saint-Antoine(CRSA)Paris F-75012France Bone Marrow Transplantation Center the First Affiliated HospitalSchool of MedicineZhejiang UniversityHangzhou 310003China AP-hP Hôpital Saint-AntoineService d’Hématologie Clinique et Thérapie CellulaireParis F-75012France AP-hP Hôpital Saint-AntoineService de Médecine Interne et de l'Inflammation-(DHU i2B)Paris F-75012France

Arsenic trioxide(As2O3)is recently found to have therapeutic potential in systemic sclerosis(SSc),a life-threatening multi-system fibrosing autoimmune disease with type I interferon(IFN-I)*** activated plasmacytoid dendritic cells(pdCs)are responsible for IFN-I secretion and are closely related with fibrosis establishment in *** this study,we showed that high concentrations of As2O3 induced apoptosis of pdCs via mitochondrial pathway with increased BAX/BCL-2 ratio,while independent of reactive oxygen species ***,at clinical relevant concentrations,As2O3 preferentially inhibited IFN-αsecretion as compared to other cytokines such as TNF-α,probably due to potent down-regulation of the total protein and mRNA expression,as well as phosphorylation of the interferon regulatory factor7(IRF7).In addition,As2O3 induced a suppressive phenotype,and in combination with cytokine inhibition,it down-regulated pdCs’capacity to induce Cd4+T cell proliferation,Thl/Th22 polarization,and B cell differentiation towards ***,chronically activated pdCs from SSc patients were not resistant to the selective IFN-αinhibition,and regulatory phenotype induced by ***,our data suggest that As2O3 could target pdCs and exert its treatment efficacy in SSc,and more autoimmune disorders with IFN-I signature.

关键词： Arsenic trioxide Plasmacytoid dendritic cell Immunotherapy Systemic sclerosis IFN-I

Single-cell profiling reveals the trajectories of natural killer cell differentiation in bone marrow and a stress signature induced by acute myeloid leukemia

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Cellular & Molecular Immunology 2021年第11期18卷 1290-1304页

作者： Adeline Crinier Pierre-Yves dumas Bertrand Escalière Christelle Piperoglou Laurine Gil Arnaud Villacreces Frédéric Vély Zoran Ivanovic Pierre Milpied Émilie Narni-Mancinelli Éric Vivier Aix Marseille University CNRSINSERMCentre d’Immunologie de Marseille-LuminyMarseilleFrance ChU Bordeaux Service d’Hématologie Clinique et de Thérapie CellulaireBordeauxFrance Bordeaux University BordeauxFrance Institut National de la Santéet de la Recherche Médicale U1035BordeauxFrance APhM Hôpital de la TimoneMarseille-ImmunopôleMarseilleFrance Établissement Français du Sang Nouvelle Aquitaine BordeauxFrance Innate Pharma Research Laboratories Innate PharmaMarseilleFrance

Natural killer(NK)cells are innate cytotoxic lymphoid cells(ILCs)involved in the killing of infected and tumor *** human and mouse NK cells from the spleen and blood,we previously identified by single-cell RNA sequencing(scRNAseq)two similar major subsets,NK1 and *** the same technology,we report here the identification,by single-cell RNA sequencing(scRNAseq),of three NK cell subpopulations in human bone *** analysis identified a subset of resident Cd56^(bright) NK cells,NK0 cells,as the precursor of both circulating Cd56dim NK1-like NK cells and Cd56^(bright) NK2-like NK cells in human bone marrow and spleen under physiological *** profiles of bone marrow NK cells from patients with acute myeloid leukemia(AML)exhibited stress-induced repression of NK cell effector functions,highlighting the profound impact of this disease on NK cell *** marrow NK cells from AML patients exhibited reduced levels of Cd160,but the Cd160high group had a significantly higher survival rate.

关键词： NK cells scRNASeq AML

Wrist Septic Arthritis due to <i>Streptococcus pneumoniae</i>Revealing a Selective Immunoglobulin G4 Subclass deficiciency: A Case Report

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Open Journal of Orthopedics 2015年第11期5卷 350-354页

作者： Atchi Walla Anani Abalo Malewé Kolou service de Chirurgie Orthopédique et Traumatologique CHU Sylvanus Olympio Lomé Togo service d’Immuno-hématologie CHU Campus Lomé Togo

The pneumococcal septic arthritis is uncommon in healthy adults. In most cases, it occurs in patients with immune deficiency. Its exceptional location at the wrist can maintain doubt with rheumatic causes which are more frequent. IgG4 deficiency found in this patient was the only cause that could be impugned. We report the case of a young woman of 46 years without any evident cause of immunodeficiency and any particular risk factor, managed for septic arthritis of the wrist due to Streptococcus pneumoniae. This case without any apparent risk factors demonstrates that pneumococcal infection in a more distant articulation of the ENT is still possible. Scan has been a very significant contribution in confirming the positive diagnosis that leads to surgical drainage. The approach of the systematic search for the gateway must be done. In the study of the patient’s immune status, the realization of the protein profile could be interesting and then guide the practitioner in the decision to vaccinate the patient against Streptococcus pneumoniae if he was not. We recommend looking always for a cause of immunodeficiency including primary Ig defiencies even among adult.

关键词： Streptococcus pneumoniae Septic Arthritis Wrist IgG4 Subclass deficiency

Colorectal cancer and immunity:what we know and perspectives

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World Journal of Gastroenterology 2014年第14期20卷 3738-3750页

作者： Simon Pernot Magali Terme Thibault Voron Orianne Colussi Elie Marcheteau Eric Tartour Julien Taieb service d’hépatogastro-entérologie et d’oncologie digestive H?pital Européen Georges-PompidouUniversité Paris DescartesSorbonne Paris Cité INSERM U970 Paris Cardiovascular Research CenterUniversité Paris-Descartes service d’Immunologie biologique H?pital Européen Georges-PompidouUniversité Paris Descartes

Strong evidence supports the concept of immunosurveillance and immunoediting in colorectal *** particular,the density of T Cd8+and Cd45+lymphocyte infiltration was recently shown to have a better prognostic value than the classic tumor node metastasis classification *** immune subsets,as macrophages,natural killer cells or unconventionnal lymphocytes,seem to play an important *** of regulatory T cells(Tregs)or immunosuppressive molecules such as Pd-1 or CTLA-4 and downregulation of antigen-presenting molecules are major escape mechanisms to antitumor immune *** development of these mechanisms is a major obstacle to the establishment of an effective immune response,but also to the use of *** im-munotherapy is not yet routinely used in colorectal cancer,we now know that most treatments used(chemotherapy and biotherapy)have immunomodulatory effects,such as induction of immunogenic cell death by chemotherapy,inhibition of immunosuppression by antiangiogenic agents,and antibody-dependent cytotoxicity induced by ***,many immunotherapy strategies are being developed and tested in phaseⅠtoⅢclinical *** most promising strategies are boosting the immune system with cytokines,inhibition of immunoregulatory checkpoints,vaccination with vectorized antigens,and adoptive cell *** of antitumor immune response and combination of the different approaches of immunotherapy may allow the use of effective immunotherapy for treatment of colorectal cancer in the near future.

关键词： Colorectal cancer Immunotherapy Immunity Immunoreg