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Fate of Right Coronary Artery Occlusion after a Surgically Repaired Aorto- Ventricular Tunnel in a Neonate

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Congenital heart Disease 2024年第3期19卷 267-273页

作者： Sherif Negm Loic Mace Fedoua El Louali Beatrice Desnous Philipe Aldebert Virginie Fouilloux Marien Lenoir Department of Pediatric Cardiac Surgery Hôpital Timone enfantAssistance Public-Hopitaux MarseilleAix-Marseille UniversitéMarseilleFrance Department of Cardiothoracic Surgery Tanta UniversityTantaEgypt Department of Pediatric Cardiology Hôpital Timone enfantAssistance Public-Hopitaux MarseilleAix-Marseille UniversitéMarseilleFrance Department of Neurology Hôpital Timone enfantAssistance Public-Hopitaux MarseilleAix-Marseille UniversitéMarseilleFrance

The aorto-ventricular tunnel is a rare congenital cardiac *** present a case of aorto-ventricular tunnel diagnosed via fetal *** surgery was performed on the 2nd day of life to close the tun-nel,located just in front of the right coronary ostium,due to the patient’s unstable health *** post-operative period revealed complete occlusion of the right coronary *** to the patient’s stability,we opted not to reintervene on the right coronary *** patient fully recovered without the need for further coronary *** cases of patients with an aorto-ventricular tunnel(AVT)and associated coronary lesions,it is crucial to exercise caution when intervening in the coronary arteries.

关键词： Aorto-ventricular tunnel aorta right coronary artery

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

Soluble p75 neurotrophic receptor as a reliable biomarker in neurodegenerative diseases: what is the evidence?

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Neural Regeneration Research 2024年第3期19卷 536-541页

作者： Georges Jourdi Samuel Fleury Imane Boukhatem Marie Lordkipanidzé Research Center Montreal Heart InstituteMontrealQCCanada Faculty of Pharmacy Universitéde MontréalMontrealQCCanada UniversitéParis Cité INSERMInnovative Therapies in HaemostasisParisFrance Service d’hématologie Biologique AP-HPHôpital LariboisièreParisFrance

Neurodegenerative diseases are often misdiagnosed,especially when the diagnosis is based solely on clinical *** p75 neurotrophic receptor(p75^(NTR))has been studied as an index of sensory and motor nerve development and *** cleavable extracellular domain(ECD)is readily detectable in various biological fluids including plasma,serum and *** is evidence for increased p75NTR ECD levels in neurodegenerative diseases such as Alzheimer’s disease,amyotrophic lateral sclerosis,age-related dementia,schizophrenia,and diabetic *** p75^(NTR) ECD could be used as a biomarker for diagnosis and/or prognosis in these disorders,and whether it could potentially lead to the development of targeted therapies,remains an open *** this review,we present and discuss published studies that have evaluated the relevance of this emerging biomarker in the context of various neurodegenerative *** also highlight areas that require further investigation to better understand the role of p75^(NTR) ECD in the clinical diagnosis and management of neurodegenerative disorders.

关键词： Alzheimer’s disease amyotrophic lateral sclerosis biomarker dementia diabetic neuropathy nerve growth factor receptor(NGFR) neurodegeneration p75^(NTR) schizophrenia

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

Diabetic Macroangioapathy at the Internal Medicine Department of the Abass Ndao hospital Center (About 359 Cases)

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Journal of Diabetes Mellitus 2024年第1期14卷 1-12页

作者： Sow Djiby Djiba Boundia Ndour Michel Assane Diedhiou Demba Dieng Mouhamed Diallo Ibrahima Mané Fall Ndiouga Gueye Khoudia Gadji Fatou Kiné Sarr Anna Diop Said Nourou Ndour Mbaye Maimouna Department of Internal Medicine and Medical Specialties H&#244pital Abass Ndao Dakar Senegal

Introduction: Macroangiopathy plays an important role, with a high prevalence of morbidity and mortality in diabetic patients. The aim was to study the epidemiological, clinical, paraclinical, therapeutic and evolutionary profile of macroangiopathy in diabetic patients in the internal medicine department of the Abass Ndao hospital. Patients and methods: This was a descriptive and analytical cross-sectional study. Our investigations were recruited over a 7-year period (January 1, 2016 to December 31, 2022). Results: Three hundred and fifty-nine (359) patients (10.22%) were enrolled. The mean age was 62.83 years, with extremes ranging from 17 to 98 years. The [60 - 69] age group was more representative (37.32%). Women accounted for 180 cases (50.1%), with a sex ratio (m/f) of 0.99. The average duration of diabetes was 11.86 years. Average consultation time was 38.07 days, with extremes ranging from 1 to 368 days. Average hospital stay was 7.65 days. Inaugural diabetes was noted in 12 cases (3.34%). Type 2 diabetes accounted for 95.82% (n = 344) of patients. hypertension was present in 150 patients (41.8%). Patients with 2 risk factors accounted for 173 cases (48.18%). Nineteen patients had already had a stroke (5.29% of cases). Fourteen (14) patients (4.2%) were amputees. Obliterative arteriopathy of the lower limbs (AOMI) was noted in 193 patients (54%). Stroke was noted in 101 patients (28%). Ischemic heart disease (IhD) was noted in 38 patients (11%). AOMI was more common in males (110 patients, 57%) than in females (43%). Seventy-three (73) patients (20.3%) died. Predictors of death were age over 60 and the existence of more than two cardiovascular risk factors. Conclusion: Diabetic macroangiopathy is a major cause of morbidity and mortality. The development and implementation of a prevention and management program is essential.

关键词： Macroangiopathy Diabetes Risk Factors Senegal

含二硫化硒洗发水对中-重度头皮脂溢性皮炎外用糖皮质激素/水杨酸治疗后的疗效维持作用

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中华皮肤科杂志 2024年第5期57卷 483-484页

作者：陈旭魏宇昊牛悦青 L'Oréal Research and Innovation Saint-Ouen France. Centre Sabouraud Hôpital Saint-Louis 1 avenue Claude-Vellefaux 75010 Paris France. CPCAD Hôpital l'Archet Nice France. L'Oréal Research and Innovation Aulnay-sous-Bois France. Cosmetic Active International Levallois-Perret France.

脂溢性皮炎是一种慢性炎症性皮肤病,好发于头皮、面部和躯干等皮脂腺分布丰富的部位。头皮脂溢性皮炎主要表现为红斑和片状鳞屑,程度从轻度鳞屑到弥漫性大片黏着性鳞屑不等,伴有明显的瘙痒,并容易反复发作。目前认为脂溢性皮炎的发生与... 详细信息

脂溢性皮炎是一种慢性炎症性皮肤病,好发于头皮、面部和躯干等皮脂腺分布丰富的部位。头皮脂溢性皮炎主要表现为红斑和片状鳞屑,程度从轻度鳞屑到弥漫性大片黏着性鳞屑不等,伴有明显的瘙痒,并容易反复发作。目前认为脂溢性皮炎的发生与亲脂性马拉色菌定植增加以及遗传、环境和全身健康状况等因素相关,上述因素共同导致皮脂腺代谢活动异常、皮脂成分改变、表皮屏障功能障碍,最终加重炎症反应。

关键词：头皮脂溢性皮炎二硫化硒洗发水亲脂性炎症反应马拉色菌皮脂腺黏着性

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Theoretical and experimental investigation of the resonance responses and chaotic dynamics of a bistable laminated composite shell in the dynamic snap-through mode

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Applied Mathematics and Mechanics(English Edition) 2024年第4期45卷 581-602页

作者： Meiqi WU Pengyu LV hongyuan LI Jiale YAN huiling DUAN Wei ZhANG State Key Laboratory for Turbulence and Complex Systems Department of Mechanics and Engineering ScienceBIC-ESATCollege of EngineeringPeking UniversityBeijing 100871China Nanchang Innovation Institute of Peking University Nanchang 330096China Department of Mechanics Guangxi UniversityNanning 530004China

The dynamic model of a bistable laminated composite shell simply supported by four corners is further developed to investigate the resonance responses and chaotic *** existence of the 1:1 resonance relationship between two order vibration modes of the system is *** resonance response of this class of bistable structures in the dynamic snap-through mode is investigated,and the four-dimensional(4D)nonlinear modulation equations are derived based on the 1:1 internal resonance relationship by means of the multiple scales *** hopf bifurcation and instability interval of the amplitude frequency and force amplitude curves are *** discussion focuses on investigating the effects of key parameters,e.g.,excitation amplitude,damping coefficient,and detuning parameters,on the resonance *** numerical simulations show that the foundation excitation and the degree of coupling between the vibration modes exert a substantial effect on the chaotic dynamics of the ***,the significant motions under particular excitation conditions are visualized by bifurcation diagrams,time histories,phase portraits,three-dimensional(3D)phase portraits,and Poincare ***,the vibration experiment is carried out to study the amplitude frequency responses and bifurcation characteristics for the bistable laminated composite shell,yielding results that are qualitatively consistent with the theoretical results.

关键词： bistable laminated composite shell dynamic snap-through mode hopf bifurcation chaotic dynamics vibration experiment

Phase field modeling of ferroelastic variant switching in yttria-stabilized t'zirconia with strain gradient elasticity and interface tension

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Science China(Technological Sciences) 2024年第5期67卷 1443-1457页

作者： ZhOU QianQian WEI YueGuang ZhOU YiChun YANG Li College of Engineering BIC-ESATPeking UniversityBeijing100871China School of Advanced Materials and Nanotechnology Xidian UniversityXi’an710126China Frontier Research Center of Thin Films and Coatings for Device Applications Academy of Advanced Interdisciplinary ResearchXidian UniversityXi’an710126China

The 6–8 wt%yttria-stabilized zirconia with a tetragonal structure(t’-YSZ)is extensively employed in thermal barrier *** exceptional fracture toughness of t’-YSZ can be attributed to its distinctive ferroelastic toughening *** and interface tension play a critical role in ferroelastic variant switching at the micro-and *** paper presents an original thermodynamically consistent phase field(PF)theory for analyzing ferroelastic variant switching at the micro-and nano-scale of t’-*** theory incorporates strain gradient elasticity using higher-order elastic energy and interface tension tensor via geometric nonlinearity to represent biaxial tension resulting from interface ***,a mixed-type formulation is employed to implement the higher-order theory through the finite element *** an interface in equilibrium,the effects of strain gradient elasticity result in a more uniform distribution of stresses,whereas the presence of interface tension tensor significantly amplifies the stress magnitude at the *** introduction of an interface tension tensor increases the maximum value of stress at the interface by a factor of 4 to *** nucleation and evolution of variants at a pre-existing crack tip in a mono-phase t’-YSZ have also been *** strain gradient elasticity is capable of capturing the size effect of ferroelastic variant switching associated with microstructures in ***,when the grain size approaches that of the specimen,the critical load required for variant switching at the crack tip increases,resulting in greater dissipation of elastic energy during ferroelastic variant ***,the interface tension accelerates the evolution of *** presented framework exhibits significant potential in modeling ferroelastic variant switching at the micro-and nano-scale.

关键词： thermodynamics strain gradient elasticity interface tension phase field method ferroelastic variant switching

Risk Factors for Neonatal Mortality at the Institute of Nutrition and Child health of the Donka/Guinea-Conakry National hospital

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Open Journal of Pediatrics 2024年第2期14卷 327-337页

作者： Bangoura Mmah Aminata Kolié Ouo Ouo Camara Salématou hassimiou Baldé Mariama Bangoura Kaba Diop Mamadou Moustapha Camara Emmanuel Diallo Fatoumata Binta Doukouré Mamadou Aliou Mamadou Mouctar Sow Bémy Pé Néabey Institut de Nutrition et de Santé de l’Enfant Conakry Guinea Service de Pédiatrie hô pital National Donka Conakry Guinea Service de Pédiatrie hô pital National d’Ignace Deen Conakry Guinea

Introduction: The birth of a baby is a moment of joy and celebration. however, the neonatal period is a very delicate phase of life. Neonatal mortality rates remain high in low-income countries. In Guinea, after 20 years, this rate has fallen from 34.2% in 1998 to 32% in 2018. Objective: To identify the main risk factors for neonatal mortality. Methods: This was an observational, analytical case-control study, lasting 6 months from January 1 to June 30, 2019, conducted at the Institut de Nutrition et de la Santé de l’Enfant (INSE) at Donka National hospital. Results: We collected 242 cases and 242 controls, i.e. a total of 484 records. 748 patients were registered, with 32.35% deaths. 82.86% of deaths occurred in the early neonatal period. Statistical analysis revealed the main risk factors: prematurity (RQ 7.39 95% CI 3.27 - 16.61 p = 0.0000003), hypothermia (RQ 2.29 95% CI 1.51 - 3.46 p = 0.0001), acute fetal distress (RQ 2.13 95% CI 1.33 - 3.43 p = 0.0016), low birth weight (QR 1.91 95% CI 1.12 - 3.24 p = 0.016), home birth (QR 3.26 95% CI 1.25 - 8.46 p = 0.015). Conclusion: Neonatal mortality is a health problem in the INSE neonatology department. To reduce the mortality rate in this referral facility, it is essential to equip it and provide ongoing training for staff.

关键词： Neonatal Mortality INSE Guinea-Conakry