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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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Surgical Management of Perforated Colon Tumours in Yaoundé: A Multicentre Retrospective Review of 30-Day Postoperative Outcome

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Surgical Science 2024年第5期15卷 330-341页

作者： Eric patrick Savom Richard II Mbele Colman Tamboh Tankou Mahamat Yannick Ekani Boukar Cédric Paterson Atangana Fred Dikongue Dikongue Daniel Biwole Biwole Guy Aristide Bang Arthur Essomba Yaoundé General Hospital Yaound&#233 Cameroon Faculty of Medicine and Biomedical Sciences University of Yaound&#233 I Yaound&#233 Cameroon Faculty of Health Sciences University of Buea Buea Cameroon Faculty of Medicine and Biomedical Sciences University of Dschang Dschang Cameroon Yaoundé Central Hospital Yaound&#233 Cameroon Yaoundé University Teaching Hospital Yaound&#233 Cameroon

Introduction: Colon cancer is often diagnosed late in our context and there is a high prevalence of complicated forms, this including perforation. The optimal surgical treatment remains controversial. The aim of this study was to improve the short-term postoperative outcomes of patients operated upon for perforated colon cancer, by identifying factors associated with complications occurring within 30 days after surgery. Patients and methods: We carried out an analytical observational study, with a retrospective collection of data from the files of patients operated upon for an in situ tumoral colon perforation. This was done in four referral hospitals in the city of Yaoundé. The period was from the 1st of January, 2012 to the 31st of December, 2021. Cox regression identified the factors associated with postoperative morbidity and mortality. Results: We collected files of 46 patients. The mean age was 54.7 ± 19.9 years with a sex ratio of 1.4. The clinical presentation on admission was dominated by the presence of signs of peritoneal irritation (91.3%) and bowel obstruction (28.3%). The diagnosis was made preoperatively in 16 cases (34.8%). The surgical procedures consisted of colectomy with anastomosis in 28 cases (60.9%) and without anastomosis in 14 cases (30.4%). Postoperative morbidity was 60.9% dominated by surgical site infection (37%). Postoperative mortality was 39.1%. The existence of generalized peritonitis was associated with the occurrence of postoperative complications (p = 0.019). The main factors associated with postoperative mortality were resection with primary anastomosis (p = 0.027), enterocutaneous fistula (p = 0.005). Conclusion: Colic resection with primary anastomosis or ideal colectomy is associated with an increased risk of mortality and should therefore not be performed in this setting.

关键词： Perforated Colon Cancer Postoperative Morbidity and Mortality Colectomy Acute Generalized Peritonitis

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Climatic and non-climatic factors driving the livelihood vulnerability of smallholder farmers in Ahafo Ano North District,Ghana

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Regional Sustainability 2024年第3期5卷 24-39页

作者： Frank BAFFOUR-ATA Louisa BOAKYE Moses Tilatob GADO Ellen BOAKYE-YIADOM Sylvia Cecilia MENSAH Senyo Michael KWAKU KUMFO Kofi Prempeh OSEI OWUSU Emmanuel CARR Emmanuel DZIKUNU patrick DAVIES Department of Environmental Science Kwame Nkrumah University of Science and TechnologyKumasiAK-039-5028Ghana Department of Meteorology and Climate Science Kwame Nkrumah University of Science and TechnologyKumasiAK-039-5028Ghana

Smallholder farmers in Ahafo Ano North District,Ghana,face multiple climatic and non-climatic *** study assessed the factors contributing to the livelihood vulnerability of smallholder farmers in this district by household surveys with 200 respondents and focus group discussions(FGDs)with 10 *** Mann–Kendall trend test was used to assess mean annual rainfall and temperature trends from 2002 to *** relative importance index(RII)value was used to rank the climatic and non-climatic factors perceived by *** socioeconomic characteristics affecting smallholder farmers’perceptions of climatic and non-climatic factors were evaluated by the binary logistic regression *** showed that mean annual rainfall decreased(P>0.05)but mean annual temperature significantly increased(P<0.05)from 2002 to 2022 in the *** key climatic factors perceived by smallholder farmers were extreme heat or increasing temperature(RII=0.498),erratic rainfall(RII=0.485),and increased windstorms(RII=0.475).The critical non-climatic factors were high cost of farm inputs(RII=0.485),high cost of healthcare(RII=0.435),and poor condition of roads to farms(RII=0.415).Smallholder farmers’perceptions of climatic and non-climatic factors were significantly affected by their socioeconomic characteristics(P<0.05).This study concluded that these factors negatively impact the livelihoods and well-being of smallholder farmers and socioeconomic characteristics influence their perceptions of these ***,to enhance the resilience of smallholder farmers to climate change,it is necessary to adopt a comprehensive and context-specific approach that accounts for climatic and non-climatic factors.

关键词： Smallholder farmers Livelihood vulnerability Climate change Socioeconomic characteristics Food security Ghana

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Cardiac Surgery at Yaoundé General Hospital by a Local Team: Activity Report from September 2022 to January 2024

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World Journal of Cardiovascular Surgery 2024年第6期14卷 87-94页

作者： Laurence Carole Ngo Yon Charles Mve Mvondo Hermann Nestor Tsague Kengni Zephanie Kobe Fokalbo Sépolin Lowe Mireille Dakleu Alain patrick Menanga William Ngatchou Djomo Vincent De Paul Djientcheu Department of Cardiothoracic and Vascular Surgery Yaound General Hospital Yaound Cameroon Department of Surgery and Specialities Faculty of Medicine and Pharmaceutical Sciences University of Douala Douala Cameroon Department of Clinical Sciences Faculty of Medicine and Pharmaceutical Sciences University of Douala Douala Cameroon Department of Clinical Sciences Faculty of Medicine and Biomedical Sciences University of Yaound Yaound Cameroon Department of Surgery and Specialities Faculty of Medicine and Biomedical Sciences University of Yaound Yaound Cameroon

Introduction: The practice of cardiac surgery (CS) in sub-Saharan Africa faces a number of challenges. We report on the surgical activities of the first 17 months of the program launched at the Yaoundé General Hospital (YGH), describing the types of interventions and the early results. Patients and Methods: This was a descriptive cross-sectional study including patients who underwent CS from September 2022 to January 2024 at the YGH. Clinical, operative and postoperative data were collected and analysed retrospectively by R software. Results: A total of 37 patients underwent CS during the study period, divided into 23 (62.2%) open-heart and 14 (37.8%) closed-heart cases. Males predominated (sex ratio 1.17). The median age and interquartile range (IQR) were 36 years (9 - 51). Isolated valve surgery, in 15 (40.5%) cases, was the most common procedure in open-heart surgery cases. Closed-heart surgery involved closure of the patent ductus arteriosus in 9 (24.3%) cases, coronary artery bypass grafting in 3 (8.1%) cases, and pericardectomy in 2 (5.4%) cases. The median duration of extracorporeal circulation and aortic cross-clamping was 101 min (IQR 84 - 143) and 74 min (IQR 54 - 112) respectively. The median duration of mechanical ventilation was 2 hours (IQR 2 - 3). The median intensive care unit stay and median hospital stay were 2 days (IQR 2 - 3) and 7 days (IQR 5 - 8) respectively. Operative mortality was 2 (5.4%). Conclusion: Cardiac surgery remains a major challenge for African populations. The early results achieved by the local team are satisfactory. An increase in surgical activity should be strongly encouraged to facilitate local training and ensure the sustainability of the activity.

关键词： Cardiac Surgery Sub-Saharan Africa Cameroon

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Laparoscopic Surgery of Incisional Hernia: Technique and Short-Term Results in Three Surgical Units in Cameroon

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Surgical Science 2024年第4期15卷 232-243页

作者： Guy Aristide Bang Eric patrick Savom Achille Aimé Bekolo Otiti Fred Dikongue Dikongue Mahamat Yannick Ekani Boukar Richard II Mbele Cédric Paterson Atangana Daniel Biwole Biwole Arthur Essomba Yaoundé Teaching Hospital Yaound&#233 Cameroon Faculty of Medicine and Biomedical Sciences of the University of Yaoundé I Yaound&#233 Cameroon Yaoundé General Hospital Yaound&#233 Cameroon Faculty of Medicine and Biomedical Sciences of the University of Dschang Dschang Cameroon Faculty of Health Sciences University of Buea Buea Cameroon Yaoundé Central Hospital Yaound&#233 Cameroon

Introduction: Incisional hernia is a common medical situation and its treatment has always been a challenge for general surgeons. If laparoscopic repair has become a gold standard elsewhere, it is increasingly done in our milieu, but no data has been found. The aim of this study was to review this surgery done in our country so far, to describe the operative technique and to evaluate the postoperative outcomes. Patients and Methods: We conducted an observational descriptive study with retrospective collection of data from the 1st of July 2018 to 30th of June 2022, in three hospitals in Cameroon. Data on socio-demographic and clinical characteristics, surgical technique and postoperative outcomes of patients who had a laparoscopic repair of their incisional hernia were collected. Results: We reviewed 20 files. There were 14 women with an average age of 54.6 years. The average BMI was 32.8 kg/m2. Thirteen patients had a moderate size hernia and the hernia was localized at the midline in 18 cases. Only composite biface meshes were used with dimensions chosen to obtain at least 5 cm overlap. Spiral tacks were the only fixation means used. We registered 3 complications. One case of generalized peritonitis secondary to missed digestive perforation, one case of seroma and one case of postoperative ileus. We registered no death. Conclusion: Laparoscopic repair of incisional hernias is feasible in a resource-limited setting like ours. Compliance with the operative technique and recommendations is important to obtain short-term results closed to that put forward by literature.

关键词： Incisional Hernia Laparoscopy Repair Composite Biface Mesh Postoperative Outcomes

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Effect of Biofertilizers on the Agromorphological Parameters of Three Okra Cultivars in Southern Côte d’Ivoire

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Agricultural Sciences 2024年第4期15卷 408-422页

作者： Guinagui N’doua Bertrand Nomel Meless Patrice Sanogo Souleymane Kouassi N’dri Pacôme Kouadio Edouard Yves Gilchrist Dao Jonas patrick Koné Dapah Sara Fatim Koné Daouda Fatogoma Sorho Plant Physiology and Pathology Teaching and Research Unit UFR Biosciences F&#233lix Houphou&#235t-Boigny University Abidjan C&#244te d&#8217Ivoire WASCAL Center/African Center of Excellence on Climate Change Biodiversity and Sustainable Agriculture (WASCAL/CEA-CCBAD) Abidjan C&#244te d&#8217Ivoire

Okra (Abelmoschus esculentus L.) is an herbaceous plant of the Malvaceae family. In Côte d’Ivoire, okra production is estimated to be over 193,000 tons. This low production is largely due to poor soils and hardly covers the needs of the population. To remedy this, growers systematically use mineral fertilizers. However, these fertilizers pollute the environment. To find an alternative to chemical fertilization and increase production, the effect of biofertilizers (Spaawet, Retone, Super Gro) compared with NPK mineral fertilizer was evaluated on Divo, Teriman, and Djonan F1 cultivars. The trial was set up in a factorial block design with three replications. Plant height, number of functional leaves, and crown diameter were assessed at 60 days after sowing (DAS). The time to 50% flowering, production time, and fruit yield were calculated. The results showed that the biofertilizer Retone induced the highest heights and number of functional leaves, with averages of 61.89 cm and 29.88 leaves, respectively. The diameter at the crown (17.77 mm) was highest with the NPK mineral fertilizer, and the shortest 50% flowering time, with an average of 47.61 days, was also obtained with the biofertilizer Retone. The NPK mineral fertilizer produced the longest production time, with an average of 35.25 days. The highest yields were obtained using Retone (11.07 t/ha) and NPK (9.52 t/ha) fertilizers. The “Divo*Retone” interaction produced the highest yield with an average of 12.19 t/ha. The biofertilizer Retone could therefore be used as an alternative fertilizer to chemical fertilization in okra crops, given its effect on the parameters assessed.

关键词： Biofertilizer Retone Mineral Fertilizer Okra Yield Côte d’Ivoire

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The Influence of the Exchange-Correlation Functional on the Non-Interacting Kinetic Energy and Its Implications for Orbital-Free Density Functional Approximations

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物理化学学报 2018年第6期34卷 650-655页

作者： FINZEL Kati BULTINCK patrick Department of Inorganic and Physical Chemistry Ghent University 281 Krijgslaan 9000 Ghent Belgium.

In this work it is shown that the kinetic energy and the exchange-correlation energy are mutual dependent on each *** aspect is first derived in an orbital-free *** is shown that the total Fermi potential depends on the density only,the individual parts,the Pauli kinetic energy and the exchange-correlation energy,however,are orbital dependent and as such mutually influence each *** numerical investigation is performed for the orbital-based non-interacting Kohn-Sham system in order to avoid additional effects due to further approximations of the kinetic *** numerical influence of the exchange-correlation functional on the non-interacting kinetic energy is shown to be of the orderof a few *** chemical purposes,however,the energetic performance as a function of the nuclear coordinates is much more important than total ***,the effect on the bond dissociation curve was studied exemplarily for the carbon *** data reveals that,the mutual influence between the exchange-correlation functional and the kinetic energy has a significant influence on bond dissociation energies and bond ***,the effect of the exchange-correlation treatment must be considered in the design of orbital-free density functional approximations for the kinetic energy.

关键词： The Influence of the Exchange-Correlation Functional the Non-Interacting Kinetic Energy Density Functional Approximations

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The dawn and rise of antimony use in the southern Caucasus

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Science China Earth Sciences 2022年第11期65卷 2037-2056页

作者： Sarah DILLIS patrick DEGRYSE Department of Earth and Environmental Sciences Katholieke Universiteit LeuvenCelestjnenlaan 200 EHeverlee 3001Belgium Faculty of Archaeology Leiden UniversityEinsteinweg 2Leiden 2333 CCThe Netherlands

Antimony(Sb)was utilised over several millennia as the prime material to opacify or decolour glass and glazes,as well as an accompanying element in copper(Cu)*** antimony objects are rare,and mostly confined to Chalcolithic Central Italy and to the first millennia BCE in the southern *** innovation of antimony use in metallurgy seems to be confined to the southern Caucasus,and the invention of it might be even more specifically situated in the Great *** knowledge of mining set the pathway for the initial stage of antimonial copper alloys in the first half of the third millennium BCE and for metallic antimony ornaments in the second half of the third millennium ***,the first major expansion of antimony in the metallurgy of the Racha-Lechkumi district in the southern Caucasus(present-day Georgia)started around 1700 BCE,while its spreading in glassmaking occurred in the Late Bronze Age(LBA).Explanations that place antimony adoption within its broader social context are favoured over those that consider material or geological properties in isolation.A recurring theme is the importance of comparative analysis,both geographically and between the different pyrotechnologies,including the precious metals and glass industries,to explore how social,political,climatic and economic conditions affected adoption and innovation *** these factors are considered to explain why the extraction of antimony blossomed in the Late Bronze Age in the southern Caucasus and to reconstruct a framework of exploitation,distribution/trade and use of antimony in the Caucasus and its neighbouring regions in the south and east.

关键词： Antimony Caucasus Metallurgy Glass Gold

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Optimal p-Ary Codes from One-Weight and Two-Weight Codes over IF_p+ vIF_p

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Journal of Systems Science & Complexity 2015年第3期28卷 679-690页

作者： SHI Minjia SOL patrick School of Mathematical Sciences Anhui University Telecom Paris Tech

This paper is devoted to determining the structures and properties of one-Lee weight codes and two-Lee weight projective codes Ck1,k2,k3 over p IF+ v IFp with type p2k1pk2pk3. The authors introduce a distance-preserving Gray map from( IFp + v IFp)nto2np. By the Gray map, the authors construct a family of optimal one-Hamming weight p-ary linear codes from one-Lee weight codes over IFp+ v IFp, which attain the Plotkin bound and the Griesmer bound. The authors also obtain a class of optimal p-ary linear codes from two-Lee weight projective codes over IFp + vIFp, which meet the Griesmer bound.

关键词： Generator matrix Gray map linear code one-Lee weight code two-Lee weight code.

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Revisiting the dissolution of biogenic Si in marine sediments： a key term in the ocean Si budget

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Acta Geochimica 2017年第3期36卷 429-432页

作者： patrick Frings Department of Geoscience Swedish Museum of Natural History 10405 Stockholm Sweden Earth Surface Geochemistry GFZ German Research Centre for Geosciences 14473 Telegrafenberg Potsdam Germany

Of the　~240*10^(12)mol year^(-1)of biogenic silica(bSi)produced by diatoms and other silicifying organisms,only roughly 3%–4%escapes dissolution to be permanently *** the global scale,how,where and why b Si is preserved in sediment is not well *** help address this,I compile 6245 porewater dissolved Si concentrations from 453 sediment cores,to derive the concentration gradient at the sediment–water interface and thus diffusive fluxes out of the *** range from\0.002 to 3.4 mol m^(-2)year^(-1),and are independent of temperature,depth and *** classified by sediment lithology,predominantly siliceous sediments unsurprisingly have higher mean diffusive fluxes than predominantly calcareous or clay-rich *** with the areal extent of these lithologies,the‘best-guess’global sedimentary b Si recycling flux is69 9 10^(12)mol year^(-1).

关键词： Biogenic silica Diatoms Ocean Si cycle Dissolution

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