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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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maxillo-mandibular rehabilitations with very early osteointegrated dental implants for severe hypodontia and anodontia related to ectodermal dysplasia

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Open Journal of Stomatology 2012年第4期2卷 260-268页

作者： Philippe martin Christian Paulus hopital femme mère enfant Service de Chirurgie Maxillo-Faciale et Stomatologie PédiatriquesLyonFrance

The ectodermal dysplasias are rare diseases with hypodontia, hypotrichosis and hypohidrosis. The subject's life is considerably constrained and this from an early age, with major difficulties for the integration and acceptance of conventional prosthetic occlusal rehabilitation. The use of implants is an integral part of early treatment, in the regions of stable growth, that is to say symphysis. In two childs of 5 and 6 years we have made implant-borne prosthetic rehabilitation in the maxilla and the mandible. Aesthetic and social evaluation were positive. We have restored the normal oro-facial functions for the correct development of skeletal bases. They acted as an external fixator intraoral, stimulating the growth by the function. Our question was: can we leave a child throughout his childhood and adolescence with a not suitable removable prosthesis, under the pretext of growth unfinished?

关键词： Ectodermal Dysplasia Anodontia Hypodontia Osteointegrated Dental Implants Facial Growth

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Single-Centre Descriptive Epidemiological Study of Emergency Haemodialysis Patients in the Haemodialysis Unit of the CHU Point G (mali)

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Open Journal of Nephrology 2023年第3期13卷 246-255页

作者： Hamadoun Yattara Nouhoum Coulibaly Abdoul Karim Traoré Seydou Sy Atabieme Kodio Karamoko Djiguiba mahamadoun Kougoulba moctar Coulibaly Sah Dit Baba Coulibaly modi Sidibé Abdou messoum Dolo Yohana Koné moustapha Tangara Alkaya Touré Aboubacar Sididki Fofana magara Samaké Sahare Fongoro Point G University Hospital Bamako Mali regional Hospital of Segou Segou Mali hopital mère-enfant le Luxembourg Bamako Mali mali Gavardo Hospital Bamako Mali Infirmary Garnison de Kati Kati Mali Sikasso regional Hospital Sikasso Mali Kayes regional Hospital Sikasso Mali Faculty of medicine and Ondotostomatology of Bamako Bamako Mali

Introduction: Haemodialysis is an exchange of solutes and water between the patient’s blood and a dialysis solution with a composition close to that of normal extracellular fluid, through a semi-permeable membrane. It is used in the treatment of acute or chronic renal failure. The aim was to study the clinical and paraclinical aspects and the outcome of patients starting dialysis treatment for renal failure in an emergency setting. method: This was a prospective study from 1 January to 31 July 2020, with a sample of 62 patients receiving haemodialysis in an emergency setting in the nephrology department of the Point G University Hospital. results: The study showed that the sex ratio was 1.38 in favour of men and that the mean age of patients was 36.82 years. The group of patients with a low socio-economic standard of living dominated, accounting for 71% of cases. Hypertension and anaemia were the predominant risk factors in 77.4% and 87.3% respectively. Hyperphosphaemia, hypocalcaemia and secondary hyperparathyroidism accounted for 94.7%, 98.2% and 97.7% respectively. Left ventricular hypertrophy was 29.27% on electrocardiogram and 22% on cardiac ultrasound. Isolated cardiomegaly was found in 30.76% of patients. The main indications for dialysis were uraemic syndrome (69.2%), pericardial friction (27.4), anuria for 48 hours (20.1%), hyperkalaemia (17.7%) and acute lung oedema (16.1%). The mean duration of haemodialysis was less than one month in one out of two cases. There was no significant association between age and time on dialysis (p = 0.178). The death rate was 20.97%. Case fatality was higher in patients whose duration of dialysis was less than one month than in the others (p = 0.0006). Conclusion: CKD is a public health problem in mali. It affects young people, especially males. Low economic income is an obstacle to the management of this disease.

关键词： Haemodialysis Emergency CHU Point G

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Inflammation is king in liver resection for hepatocellular carcinoma

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Hepatobiliary Surgery and Nutrition 2021年第6期10卷 839-841页

作者： Xavier muller Kayvan mohkam Jean-Yves mabrut Department of General Surgery and Liver Transplantation Croix-Rousse University HospitalHospices Civils de LyonUniversity of Lyon ILyonFrance Cancer research Center of Lyon INSERM U1052LyonFrance Department of Pediatric Surgery and Liver Transplantation Hôpital Femme Mère EnfantUniversitéClaude Bernard Lyon 1Hospices Civils de LyonBronFrance

We read with great interest the paper by miyata et ***:The Impact of Histologic Liver Inflammation on Oncology and the Prognosis of Patients Undergoing Hepatectomy for Hepatocellular Carcinoma(1).In this single center retrospective study,the authors included 500 patients undergoing curative surgical liver resection for primary hepatocellular carcinoma(HCC)without any neoadjuvant *** liver specimen underwent postoperative pathological analysis to identify fibrosis but also lymphocyte infiltration and hepatocyte necrosis as surrogate marker for hepatic *** hepatic inflammation was graded from A0:no necro-inflammatory reaction,to A3:severe necro-inflammatory *** follow-up was 47.3 ***,19.4%of the patients had severe liver inflammation A3(n=97)and presented with higher postoperative morbidity and reduced overall and recurrence-free survival compared to patients with none to moderate inflammation A0-A2(n=403).Furthermore,inflammation grade A3 was an independent predictor of HCC recurrence independent of underlying liver disease and fibrosis grade.

关键词： inflammation hepatocellular Carcinoma

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malignant Transformation of Cerebellar Pleomorphic Xanthoastrocytoma: A Case report

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World Journal of Neuroscience 2017年第4期7卷 376-382页

作者： Oumar Coulibaly mahamadou Dama Oumar Diallo Quenum Kouassi Jean marie maurin Kisito Li Gu Boubacar Sogoba moussa Denou Sieleche Auxence Christ Youssouf Sogoba mamadou Salia Diarra Alpha Singuépiré Drissa Kanikomo hopital du mali Bamako Mali Unité de Neurochirurgie Centre Hospitalier Départemental et Universitaire de Parakou Parakou Benin The First Affiliated Hospital of medical School of Zhejiang University Zhejiang China hopital Gabriel Touré Bamako Mali hopital mère-enfant “Le Luxembourg” Bamako Mali

Pleomorphic xanthoastrocytoma (PXA) generally occurs in children and young adults and is classified as a low-grade astrocytic tumor with a potential favorable prognosis. But these data must be interpreted with much caution, because of some rapid progression or malignant transformation during the postoperative follow-up. We report herein a rare case of a cerebellar PXA manifested as a benign lesion at first time in a 39 year-old woman, but with malignant transformation two years later. In this paper, we discuss the clinical signs, radiological findings and the therapeutic data on the subject according to literature review.

关键词： Pleomorphic Xanthoastrocytoma Cerebellar malignant

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Surgical management of 4 Cases of Craniofacial Fibrous Dysplasia

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World Journal of Neuroscience 2021年第4期11卷 279-286页

作者： Youssouf Sogoba moussa Diallo Boubacar Sogoba Djènè Kourouma Izoudine Blaise Koumaré mamadou Diallo Oumar Coulibaly mahamadou Dama mamadou Salia Diarra Seybou Hassane Diallo Salimata Diallo Oumar Diallo Youssoufa maiga Drissa Kanikomo Depatment of Neurosurgery H&#244pital Gabriel Touré Bamako Mali Department of Neurosurgery Hopital du Mali Bamako Mali Department of Neurosurgery Hopital Mère-enfant Bamako Mali Depatment of Neurology H&#244pital Gabriel Touré Bamako Mali

Background: Fibrous dysplasia is an uncommon skeletal disorder in which normal bone and marrow are replaced with fibro-osseous tissue. The disease comprises 2.5% of all bone tumors and 7.5% of all benign bone neoplasm. It is the progressive, slowly developing disease and the optimum treatment remains unclear in many cases. Aim: In this study, the authors report their experience in the surgical treatment of four cases of craniofacial fibrous dysplasia. Cases presentation: The study involved 4 patients with craniofacial fibrous dysplasia. There were 3 men and a woman. The patients were 10, 17, 20 and 8 years old. No patient had a focal neurological deficit. The CT scan appearance was compatible with Fibrous dysplasia in all patients. The site of disease was frontal in one case and parietal in the other 3 cases. Cosmetic surgical treatment was performed in all patients. Cranioplasty was performed in one patient and planned for the other three. Conclusion: Fibrous Dysplasia is a benign slow growing disease that may cause as well as clinical symptom and aesthetical discomfort. Radical resection, if possible, is the only technique to obtain resolution of the disease.

关键词： Fibrous Dysplasia Surgical Treatment Bone Tumor

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Thymic langerhans cell histiocytosis in children:A case report

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Open Journal of Pediatrics 2012年第4期2卷 303-305页

作者： marie Poncelet Camille Chabert Jean Pierre Pracros Perrine marec Berard Pediatric Institute of Hematology and Oncology Institut d’Hématologie et d’Oncologie Pédiatrique(IHOP)LyonFrance Pediatric Unit of Radiology Hopital Femme Mere Enfant(HFME)LyonFrance

Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of the disease involving the bone, the skin or the pituitary gland have been well described whereas others, such as thymic histiocytosis, are still poorly understood. Here, we report a case of isolated Langerhans cell histiocytosis of the thymus in an infant with non-specific symptoms. We provide a description of the disease and discuss patient management.

关键词： Langerhans Cell Histiocytosis Children Thymus

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Burden of celiac disease in the mediterranean area

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World Journal of Gastroenterology 2011年第45期17卷 4971-4978页

作者： Luigi Greco Laura Timpone Abdelhak Abkari mona Abu-Zekry Thomas Attard Faouzi Bouguerrà Paskal Cullufi Aydan Kansu Dusanka micetic-Turk Zrinjka miak Eleftheria Roma Raanan Shamir Selma Terzic European Laboratory for Food Induced Diseases Federico Ⅱ University of Naples hopital des enfant Ibn Rochd de Casablanca Children's Hospital Gastrointestinal Unit Cairo University University of malta Medical School hopital des enfants de Tunis Department of Gastroenterology and Hepatology University Hospital Center "Mother Teresa" Department of Pediatric Gastroenterology Ankara University Dikimevi/Mamak University medical Centre Paediatric Department Children's Hospital Zagreb Athens University Aghia Sophia Children's Hospital Schneider Children's medical Center of Israel Tel-Aviv University University Clinical Center Tuzla Children's Hospital

AIm: To estimate the burden of undiagnosed celiac disease (CD) in the mediterranean area in terms of morbidity, mortality and health cost. mETHODS: For statistics regarding the population of each country in the mediterranean area, we accessed authoritative international sources (World Bank, World Health Organization and United Nations). The prevalence of CD was obtained for most countries from published reports. An overall prevalence rate of 1% cases/total population was finally estimated to represent the frequency of the disease in the area, since none of the available conf idence intervals of the reported rates significantly excluded this rate. The distribution of symptoms and complications was obtained from reliable reports in the same cohort. A standardized mortality rate of 1.8 was obtained from recent reports. Crude health cost was estimated for the years between symptoms and diagnosis for adults and children, and was standardized for purchasing power parity to account for the different economic prof iles amongst mediterranean countries. reSULTS: In the next 10 years, the mediterranean area will have about half a billion inhabitants, of which 120 million will be children. The projected number of CD diagnoses in 2020 is 5 million cases (1 million celiac children), with a relative increase of 11% compared to 2010. Based on the 2010 rate, there will be about 550 000 symptomatic adults and about 240 000 sick children: 85% of the symptomatic patients will suffer from gastrointestinal complaints, 40% are likely to have anemia, 30% will likely have osteopenia, 20% of children will have short stature, and 10% will have abnormal liver enzymes. The estimated standardized medical costs for symptomatic celiac patients during the delay between symptom onset and diagnosis (mean 6 years for adults, 2 years for children) will be about €4 billion (€387 million for children) over the next 10 years. A delay in diagnosis is expected to increase mortality: about 600 000 celiac pat

关键词：地中海地区负担腹腔疾病医疗费用世界卫生组织死亡率总人口

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Sacrospinous Fixation in the Gynecology Department of Hôpital du mali

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Open Journal of Obstetrics and Gynecology 2021年第1期11卷 20-26页

作者： Traoré Alassane Sissoko Abdoulaye Coulibaly mamadou Bakary Traoré Soumana Oumar Sima mahamadou Bocoum Amadou Traoré Soumaila Touré moustapha Tegueté Ibrahima Traoré Youssouf Obstetrics and Gynecology Department at hopital du mali Bamako Mali Obstetrics and Gynecology Department at the Teaching Hospital mère-enfant Bamako Luxembourg Obstetrics and Gynecology Department at the referral Health Center Commune V Bamako Mali Obstetrics and Gynecology Department at the Teaching Hospital Point G Bamako Mali Obstetrics and Gynecology Department at the Teaching Hospital Gabriel Touré Bamako Mali Obstetrics and Gynecology Department at the regional Hospital of Sikasso Sikasso Mali

Sacrospinous fixation (SSF) or Richter’s intervention (RI) aims to treat genital prolapse by securing the posterior vaginal wall to the small sacrospinous ligament. It is performed by low approach and includes a dissection of the pararectal space, visual exposure of the sacrospinous ligament and a needle with strait needle holder with nonabsorbable threads. It is often associated with a more complex corrective procedure, including cystocele cure, vaginal hysterectomy and posterior myorrhaphy. The objective of this study is to report the results of SSF in the gynecology department of hopital du mali. A descriptive study was conducted from September 2014 to September 2015 concerning 37 patients operated on for uterine prolapse (UP). All patients with grade III UP were included in our study in whom a unilateral hysterectomy (UH) and sacrospinous fixation (SSF) were performed. All the patients were scheduled. Preoperatively they had benefited from an assessment and a pre-anesthetic consultation. Hospitalization of at least 24 hours prior to the operation was required. Postoperative follow-up was two years with a physical examination at 3 months, 9 months and 15 months, and phone calls between physical consultations. During the study period, we performed 37 RIs. The mean age of the patients was 48 years with extremes of 41 to 73 years. The large multiparity was found out in 35 cases (94.59%), the pauciparous were two with 3 deliveries for each. Long labor of more than 18 hours was found out in 9 patients (24.32%) and home delivery in 13 cases (35.13%). The duration of the occurrence of prolapse was at least two years and 35 patients were going through menopause. The type of anesthesia used for the surgery was spinal anesthesia for all patients. The average duration of the operation was 90 minutes. Complications occurred in three patients or 8.10% of cases, two cases of acute urine retention and one case of hematoma of the para-rectal space. The medium time of hospital st

关键词： Sacrospinous Fixation Genital Prolapse Hôpital du mali

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Impaired Executive Function in Everyday Life: A Predictor of OCD relapse?

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Journal of Behavioral and Brain Science 2019年第3期9卷 90-107页

作者： Nathalie Fournet Ouafae Achachi Arnaud Roy Jérémy Besnard Céline Lancelot Didier Le Gall martine Bouvard Université Grenoble Alpes Université Savoie Mont Blanc CNRS LPNC Grenoble France Laboratoryof Psychology des Pays de la Loire EA4638 Université Bretagne Loire—Université d’Angers Angers France reference Center of Learning Disorders—Centre Référent des Troubles d’Apprentissage Centre de Compétence Nantais de Neurofibromatose H&#244 pital femme-enfant-Adolescent CHU de Nantes Nantes France

Objectives: In the present study, we set out to establish whether executive function in everyday life is impaired in obsessive-compulsive disorder patients, and if it is more impaired (or not) in patients who relapsed after cognitive behavioural therapy than in treatment-na?ve obsessive-compulsive disorder patients. method: The Behavior Rating Inventory of Executive Function— Adult Version (BRIEF-A) was used to measure executive function in everyday life in three groups of 19 participants: treatment-na?ve obsessive-compulsive disorderpatients, obsessive-compulsive disorder patients having relapsed after CBT, and healthy controls. results: The BRIEF-A results revealed an impairment in executive function in the treatment-na?ve and relapsed obsessive-compulsive disorder groups, relative to the healthy control group. There was no significant difference in executive function between the two groups of patients. Conclusions: These results show that impaired executive function is not associated with relapse in patients with obsessive-compulsive disorder having undergone cognitive behavioural therapy.

关键词： Obsessive Compulsive Disorder relapse Executive Function Cognitive Behavioural Therapy

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