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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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Evaluation of Doppler-ultrasonography in the diagnosis of transjugular intrahepatic portosystemic shunt dysfunction: A prospective study

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World Journal of Hepatology 2017年第27期9卷 1125-1132页

作者： Charlotte Nicolas Amelie Le Gouge Louis d’Alteroche Jean Ayoub monica Georgescu Vincent Vidal Denis Castaing Jean-Pierre Cercueil Patrick Chevallier Jerome Roumy Herve Trillaud Louis Boyer Vincent Le Pennec Christophe Perret Bruno Giraudeau Jean-marc Perarnau STIC-TIPS group Service d’Hepato-Gastroenterologie Hopital TrousseauCHRU Tours CIC CHRU de Tours INSERm United’echographie-doppler Hopital TrousseauCHRU Tours Service de radiologie Hopital de la Timone Centre Hepato-Biliaire Hopital Paul Brousse Service de radiologie CHRU Dijon Service d’imagerie medicale Diagnostique et interventionnelle Hopital de l’ArchetⅡNice Service de radiologie et Echographie CHRU Poitiers Service d’imagerie medicale Hopital Saint Andre CHRU Bordeaux Service d’imagerie viscerale et vasculaire CHRU Clermont Ferrand Service de radiologie CHRU Caen Service de radiologie CHRU Nantes 不详

AIm To prospectively evaluate the performance of Dopplerultrasonography(US) for the detection of transjugular intrahepatic portosystemic shunt(TIPS) dysfunction within a multicenter cohort of cirrhotic *** This study was conducted in 10 french teaching hospitals. After TIPS insertion, angiography and liver Doppler-US were carried out every six months to detect dysfunction(defined by a portosystemic gradient ≥ 12 mm Hg and/or a stent stenosis ≥ 50%). The association between ultrasonographic signs and dysfunction was studied by logistic random-effects models, and the diagnostic performance of each Doppler criterion was estimated by the bootstrap method. This study was approved by the ethics committee of *** Two hundred and eighteen pairs of examinations performed on 87 cirrhotic patients were analyzed. Variables significantly associated with dysfunction were: The speed of flow in the portal vein(P = 0.008), the reversal of flow in the right(P = 0.038) and left(P = 0.049) portal branch, the loss of modulation of portal flow by the right atrium(P = 0.0005), ascites(P = 0.001) and the overall impression of the operator(P = 0.0001). The diagnostic performances of these variables were low; sensitivity was m Doppler-US. CONCLUSION The performance of Doppler-US for the detection of TIPS dysfunction is poor compared to angiography. New tools are needed to improve diagnosis of TIPS dysfunction.

关键词： Transjugular intrahepatic portosystemic shunt Dysfunction Doppler-ultrasonography

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meso-Rex bypass for the management of extrahepatic portal vein obstruction in adults(with video)

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Hepatobiliary & Pancreatic Diseases International 2022年第1期21卷 25-32页

作者： martin Brichard Samuele Iesari Jan Lerut Raymond Reding Pierre Goffette Laurent Coubeau Service de Chirurgie et Transplantation Abdominale Cliniques Universitaires Saint-LucUniversitéCatholique de Louvain10 Avenue HippocrateBrussels 1200Belgium Pôle de Chirurgie Expérimentale et Transplantation Institut de Recherche Expérimentale et CliniqueUniversitéCatholique de LouvainBrusselsBelgium Department of Clinical Sciences and Applied Biotechnology University of L’AquilaL’AquilaItaly Department of General Surgery and Kidney Transplantation Fondazione IRCCS Ca’Granda Ospedale Maggiore PoliclinicoMilanItaly Service de radiologie et imagerie médicale Cliniques Universitaires Saint-LucUniversitéCatholique de LouvainBrusselsBelgium

Background:Extrahepatic portal vein obstruction(EHPVO)results in severe portal hypertension(PHT)leading to severely compromised quality of ***,pharmacological and endoscopic management is unable to solve this *** hepatic portal flow using meso-Rex bypass(mRB)may solve *** procedure,uncommon in adult patients,is considered the treatment of choice for EHPVO in ***:From 1997 to 2018,8 male and 6 female adults,with a median age of 51 years(range 22-66)underwent mRB procedure for EHPVO at the University Hospitals Saint-Luc in Brussels,***-toms of PHT were life altering in all but one patient and consisted of repetitive gastro-intestinal bleedings,sepsis due to portal biliopathy,and/or severe abdominal *** surgical technique consisted in interposition of a free venous graft or of a prosthetic graft between the superior mesenteric vein and the Rex recess of the left portal ***:median operative time was 500 min(range 300-730).median follow-up duration was 22 months(range 2-169).One patient died due to hemorrhagic shock following percutaneous transluminal interven-tion for early graft *** morbidity,defined as Clavien-Dindo score≥III,was 35.7%(5/14).Shunt patency at last follow-up was 64.3%(9/14):85.7%(6/7)of pure venous grafts and only 42.9%(3/7)of prosthetic *** relief was achieved in 85.7%(12/14)who became asymptomatic after ***:Adult EHPVO represents a difficult clinical condition that leads to severely compromised quality of life and possible life-threatening *** such patients,mRB represents the only and last resort to restore physiological portal vein *** successful in a majority of patients,this procedure is associated with major morbidity and mortality and should be done in tertiary centers expe-rienced with vascular liver surgery to get the best results.

关键词： meso-Rex bypass Extrahepatic portal vein obstruction Extrahepatic portal hypertension Portal vein thrombosis Surgical procedure

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Endovascular Stents in Arterial Injury after Radiotherapy

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Journal of Huazhong University of Science and Technology(medical Sciences) 1998年第4期18卷 253-256,261页

作者：刘鹏程 PerreaultPierre OtalPhilippe ColombierDanial BerregiJean-Paul BastideCyri1 CercueilJean-Pierre KrauseDenis RousseauHelve JoffreFrancis Department of Radiology Tongji Hospital Tongji Medical University Wuhan Service de radiologie Hopital de Rangueil CHRU de Toulouse France Service de radiologie Hbpital Cardiologure CHRU Lille France Service de radiologie du Bocage CHRU de Dijon France Service et imagerie medical Hbpital Gabriel Montpied CHRU de Clermont Fettand France

We evaluated preliminary success and patency of stenting for the treatment of radiation - induced arterial diseases. Thirteen stents were placed in 8 patients to treat occlusion (n = 3), aneurysm (n = 1), residual stenosis (n = 2), multiple stenoses (n = l), and delayed restenosis after previous balloon angioplas-ty (n=l). Interventional procedure was successfully performed in 8 patients for their arterial lesions after radiotherapy. Six patients underwent interventional procedure once or twice. Two patients underwent PTA 4 times. Five of these patients demonstrated primary patency with relief of clinical symptoms with a mean follow-up of 2 years (range: 8 - 60 months). Clinical improvement was noted in the other patients. Our results suggest that stent placement by single or multiple techniques may have immediate effect on arterial lesions caused by radiation and can be considered as a therapeutic option of choice in these cases.

关键词： arteries transluminal angioplasty radiation injurious effect stents and prostheses

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Duplication of the Inferior Vena Cava, Azygos Continuation and Retro Aortic Renal Vein: Fortuitous Discovery

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Forensic medicine and Anatomy Research 2021年第3期9卷 31-40页

作者： Géraud Léra Akpo Ndeye Bigué mar Soukeyna Abouabdillah Nfaly Badji Hamidou Deme Youssoupha Kasse Ibrahima Niang mamadou Lamine Gueye marcel mbarga Ibrahima Faye Abdoulaye Dione Diop Assane Ndiaye Sokhna Ba Diop El Hadji Niang Service de radiologie Imagerie Médicale de l’h&#244pital Ariste Le Dantec Dakar Sénégal Laboratoire d’Anatomie UFR des Sciences de la Santé Université Iba Der Thiam de Thiès Thiès Sénégal Service de radiologie Imagerie Médicale de l’h&#244pital Fann Dakar Sénégal Service de radiologie Imagerie Médicale de l’h&#244pital Dalal Jam Dakar Sénégal Laboratoire d’Anatomie UFR des Sciences de la Santé Université Assane SECK de Ziguinchor Ziguinchor Sénégal

We present a rare case associating inferior vena cava’s (IVC) complex variations. Anatomical, embryological and clinical aspects are discussed. Duplication’s discovery was fortuitous during an abdominal ultrasound and was confirmed by the CT scan. The latter also highlighted azygos continuation, retro aortic renal vein and an iliac venous anomaly. These variants are important to know in order to be able to differentiate them from lymphadenopathy, para-vertebral masses, and aortic pathologies. It is necessary to report them before any retroperitoneal surgery or interventional vascular procedure.

关键词： Duplication IVC Continuation Azygos Tomodensitometry

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Idarubicin vs doxorubicin in transarterial chemoembolization of intermediate stage hepatocellular carcinoma

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World Journal of Gastroenterology 2020年第3期26卷 324-334页

作者： Gael Stephane Roth Yann Teyssier melodie Abousalihac Arnaud Seigneurin Julien Ghelfi Christian Sengel Thomas Decaens Clinique Universitaire d’Hepato-Gastroenterologie et Oncologie Digestive CHU Grenoble-AlpesGrenoble 38043France Facultede medicine Universite Grenoble-AlpesDomaine de la MerciLa Tronche 38700France Institute for Advanced Biosciences-INSERm U1209/CNRS UmR 5309/Universite Grenoble-Alpes Site Santé-Allée des AlpesLa Tronche 38700France Clinique Universitaire de radiologie et imagerie médicale CHU Grenoble-AlpesGrenoble 38043France Departement de Sante Publique-CHU Grenoble-Alpes Grenoble 38043France Department of Hepatology and Gastroenterology Grenoble-Alpes University HospitalGrenoble 38043France

BACKGROUND Liver cancer is the fifth most common cancer and the second cause of cancerrelated deaths *** chemoembolization(TACE)is the best treatment of intermediate hepatocellular carcinoma(HCC).Doxorubicin is the most commonly used drug despite a low level of *** To compare the objective response rate of idarubicin-based TACE(Ida-TACE)against doxorubicin-based TACE(Dox-TACE)in intermediate stage *** Between January 2012 and December 2014,all patients treated with TACE at our academic hospital were *** criteria were patients with Child-Pugh score A or B,a performance status below or equal to 1,and no prior *** lipiodol TACE or drug-eluting beads TACE could be performed with 10 mg of idarubicin or 50 mg of *** patient treated with idarubicin was matched with two doxorubicin-treated *** TACE response was assessed by independent radiologists according to the mRECIST *** Sixty patients were treated with doxorubicin and thirty with *** were 93%and 87%of cirrhotic patients and 87%and 70%of Child-Pugh A in the doxorubicin and idarubicin groups,*** median number of HCC per patient was two in both groups with 31%and 26%of single nodules in doxorubicin and idarubicin groups,*** response rate after first TACE was 76.7%and 73.3%(P=0.797)with 41.7%and 40.0%complete response in doxorubicin and idarubicin groups,***-free survival was 7.7 mo in both groups,and liver transplant-free survival was 24.9 mo and 21.9 mo in doxorubicin and idarubicin groups,*** profiles were similar in both groups,with grade 3-4 adverse events in 35%of Dox-TACE and 43%of *** Ida-TACE and Dox-TACE showed comparable results in terms of efficacy and ***-TACE may represent an interesting alternative to Dox-TACE in the management of patients with intermediate stage HCC.

关键词： Hepatocellular carcinoma Transarterial chemoembolization Idarubicin Doxorubicin Intermediate stage

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First Evaluation of Zygomaticus major muscle Elastic Properties Using a US Elastography Technique

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Journal of Biomedical Science and Engineering 2019年第11期12卷 459-468页

作者： Redouane Ternifi Philippe Pouletaut Stéphanie Dakpé Sylvie Testelin Bernard Devauchelle Fabrice Charleux Jean-marc Constans Sabine F. Bensamoun Centre de Recherches de Royallieu UMR CNRS 7338 Biomechanics and Bioengineering Université de Technologie de Compiègne Sorbonne University Compiègne France maxillofacial Surgery Department CHU Amiens France EA 7516 Chimère Université Picardie Jules Verne Amiens France Fédération Hospitalo-Universitaire SURFACE Amiens France Institut Faire Faces Amiens France ACRIm-Polyclinique Saint Côme Radiologie Médicale Compiègne France imagerie et radiologie médicale Amiens France

Facial expressions are linked to movements of muscles, which can be altered by pathological diseases. Assessment of facial muscle deficits is subjective (palpation) and operator-dependent, and these deficits are currently estimated with clinical scales. Thus, the quantification of facial muscle elastic properties is a key for the clinical adaption and evaluation of treatments for facial paralysis. We herein present a novel application of shear wave elastography (SWE) based on an ultrasound protocol to assess the morphological (thickness and texture) and elastic (Young’s modulus) properties of the zygomaticus major (Zm) muscle. Fifteen healthy volunteers underwent SWE tests, and the ultrasound acquisitions were obtained using a new linear transducer (SLH20-6, spatial resolution: 38 μm) and compared to those obtained using an SL10-2 probe (spatial resolution: 50 μm). The probe position was placed along the muscle fiber orientation. A semi-automatic method was developed to quantify the Zm muscle elasticity, and the repeatability was analyzed at one-week intervals. The mean elasticity for the two probes was about 15 kPa. The SLH20-6 probe yielded a higher mean elasticity (approximately 6 kPa) and less homogeneous echogenicity than the SL10-2 probe. Two distinct groups of texture profiles as a function of the transducer were obtained. This study will provide some guidance for clinical practices and will allow the construction of a reference database that could be used to evaluate treatments and develop numerical models of facial expression.

关键词： Facial muscle Shear Wave Elastography Zygomaticus major Texture Analysis Ultrasound

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Dose Optimization in Computer Tomography Pediatric Cranial Scans

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Open Journal of Radiology 2019年第4期9卷 181-193页

作者： Clémence Alla Takam Odette Samba Aurelle Tchagna Kouanou Daniel Tchiotsop Eddy Fotso Kamdem Emilienne Guegang Emmanuel Fongang Unité de Recherche de matière Condensée d’Electronique et de Traitement du Signal (URmACetS) Faculty of Science University of Dschang Dschang Cameroon Service de Radiothérapie Hôpital Général de Yaoundé Yaoundé Cameroo Department of Research Development Innovation and Training InchTech’s Yaoundé Cameroon Unité de Recherche d’Automatique et d’Informatique Appliquée (URAIA) IUT-FV de Bandjoun Université de Dschang Dschang Cameroon Service de radiologie et d’imagerie médicale Hôpital Général de Yaoundé Yaoundé Cameroon

Background and Objective: Nowadays, Computer Tomography is one of the best radiological imaging technics which can give right diagnostic information, among the detection of multiphasic adenomas, the detection of cardiac, cerebral and vascular abnormalities. Although these good qualities, this technic is too radiant for the patient. In this paper, we based on the irradiation doses delivered from the current protocols to find a practical method of their optimization during the pediatric cranial scan. materials and methods: This work relies on a collection of data from patients in the hospitals, so that analyze them, give the conclusions and, propose an optimal practical method to decrease the irradiation doses. To collect data, we performed a prospective study of seventeen months (from December 2017 to may 2019) carried out simultaneously in three hospitals of the city: The Centre medical la Cathédrale (H1), the Yaoundé Central Hospital (H2) and the Yaoundé Gyneaco-Obstetric and pediatric hospital (H3). This study included a total of 192 cases of cerebral trauma, of which 11 cases excluded for incomplete information. The dosimetry quality control (CTDIvol) using the PmmA phantoms of 16 cm and 32 cm fulfilled. The scanographic parameters of the patient acquisition protocol were recorded and analyzed. Some of those parameters were modified and entered the CT with the help of a biomedical engineer to reduce the delivered dose. The relationship between CTDIvol and kV is statistically significant (p Results: Among patients, 172 are boys, and the remaining 9 are girls all were in the 0 to 15 age group. CTDIvol values varied from 34.2 mGy to 107.8 mGy and PDLs from 107.8 *** to 2214.5 *** in H1. In H2, CTDIvol varied from 5.8 mGy to 44 mGy and PDLs from 91.4 *** to 665.5 ***. CTDIvol varied between 9.34 mGy to 92.81 mGy and PDLs from 162.38 *** to 2713.67 *** in H3. All values are taken at 75th percentile, with or without contrast injection. Conclusion: The

关键词： Computed Tomography Dose Optimization Pediatric Cranial Scans Protocol

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半月板后角撕裂的薄层mRI诊断—拉链征的初步研究

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国际医学放射学杂志 2011年第2期 193-193页

作者： P.Y.Savoye J.N.Ravey C.Dubois L.P.Barbier A.Courvoisier D.Saragaglia 白荣杰 Clinique universitaire de radiologie et d'imagerie médicale

目的 "拉链征"是最近提出的名词,用来描述半月板外侧壁至板股韧带(mFL)附着点之间的半月板撕裂,撕裂发生于前交叉韧带断裂时。本研究的目的是对前交叉韧带损

关键词： mRI 半月板撕裂板股韧带外侧半月板后角后交叉韧带断裂

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超声检查了解马里共和国molodo地区儿童血吸虫病发病情况

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世界核心医学期刊文摘（胃肠病学分册） 2005年第11期1卷 30-30页

作者： Ké ita A.D. Sangho H. Sacko m. 张诗峰 Service de radiologie et d'imagerie mé dicale CHU Point G BP 333 Bamako Mali

Aim - To study schistomasiasis infection in school children in molodo, an irrigated rice growing region of mali, by determining the prevalence of schistomasiasis and lesions identified by ultrasonography among children living in this region. methods - This cross sectional study included 346 children aged 7 to 14 years selected at random from five schools in molodo. We tested for hematuria using urine dipsticks and searched for Schistosoma haematobium eggs in urine and S. mansoni eggs in stools. Ultrasonography of the liver, spleen and urinary tract was performed. Results - The prevalences of Schistosoma haematobium and S. mansoni infection were 72% (range: 66.9- 76.6% ) and 68.2% (range: 60.9- 71.2% ) respectively; 55.1% of the children had co-infection. Ultrasonography of the urinary tract revealed an irregular bladder wall as the most frequent abnormality (3.4% of children). Abdominal ultrasonography demonstrated type B hepatic fibrosis in four children (1.1% ), type C in one (0.3% ) and type D in one (0.3% ). Conclusion - Few schistosomiasis lesions were detected by ultrasonography compared with the prevalence of S. haematobium and S. mansoni infections. This observation is probably related to mass treatment programs conducted during a national anti-schistosomiasis program.

关键词： molodo 埃及血吸虫卵超声检查曼氏血吸虫膀胱壁双重感染灌溉稻横断面研究肝纤维化治疗程序

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