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Symptomatic Val122del mutated hereditary transthyretin amyloidosis: Need for early diagnosis and prioritization for heart and liver transplantation

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Hepatobiliary & Pancreatic Diseases International 2021年第4期20卷 323-329页

作者： Adriano-Valerio Schettini Laura Llado JulieK Heimbach JoseGonzalez Costello Marie Tranäng Olivier Van Caenegem Richard C Daly Peter Van den Bergh Carlos Casasnovas Joan Fabregat John J Poterucha maxime Foguenne Bo Göran Ericzon Jan Lerut Institute for Experimental and Clinical Research(IREC) Catholic University of Louvain(UCL)Avenue Hippocrate 55Brussels 1200Belgium Liver Transplant Unit Department of SurgeryHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Transplantation Surgery William J.von Liebig Transplant CenterMayo Clinic2001st St SWRochesterMN 55905USA Advanced Heart Failure and Transplant Unit Cardiology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Familial Amyloidotic Polyneuropathy World Transplant Registry(FAPWTR) Division of Transplantation SurgeryKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden Cardiothoracic Intensive Care Unit Department of Cardiovascular DiseasesUniversity Hospitals Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Cardiovascular Surgery Department Mayo Clinic2001st St SWRochesterMN 55905USA Neuromuscular Reference Centre University Hospital Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Neuromuscular Unit Neurology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Gastroenterology and Hepatology Mayo Clinic2001st St SWRochester MN 08907USA Division of Transplantation Surgery CLINTECKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden

Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils(i.e. amyloid). Apart from the common Val30 Met mutation there is a very heterogeneous group of non-Val30 Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. Methods: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122 del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. Results: Three patients needed a staged(1 patient) or simultaneous(2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac(allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. Conclusions: This is the first report in(liver) transplant literature about the rare Val122 del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.

关键词： Hereditary transthyretin amyloidosis Heart transplantation Liver transplantation Non-Val30Met mutation Val122del mutation Domino liver transplantation

Impact of sarcopenia on tumor response and survival outcomes in patients with hepatocellular carcinoma treated by trans-arterial (chemo)-embolization

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World Journal of Gastroenterology 2022年第36期28卷 5324-5337页

作者： Gael Roth Yann Teyssier maxime Benhamou Mélodie Abousalihac Stefano Caruso Christian Sengel Olivier Seror Julien Ghelfi Arnaud Seigneurin Nathalie Ganne-Carrie Elia Gigante Lorraine Blaise Olivier Sutter Thomas Decaens Jean-Charles Nault Univ.Grenoble-Alpes Grenoble 38058France Department of Hepatology Gastroenterology and Digestive OncologyCHU Grenoble AlpesGrenoble 38043France Institute for Advanced Biosciences INSERM U1209/CNRS UMR 5309Grenoble 38043France Department of Radiology CHU Grenoble AlpesGrenoble 38043France Department of Radiology CHU Avicenne-APHPBobigny 93000France Functional Genomics of Solid Tumors Laboratory Centre de Recherche des Cordeliers-INSERM UMR 1138InsermUniversitéParisParis 75006France Unitéde Formation et de Recherche SantéMédecine et Biologie Humaine UniversitéParis NordParis 93430France Service d'Epidémiologie et Evaluation Médicale CHU Grenoble AlpesGrenoble 38043France Department of Hepatology CHU Avicenne-APHPBobigny 93000France

BACKGROUND At the diagnosis of hepatocellular carcinoma(HCC),more than 90%of HCC patients present cirrhosis,a clinical condition often associated to *** is an indirect marker of malnutrition assessable on computed tomography(CT).AIM To evaluate the prognostic value of sarcopenia in patients with HCC treated by trans-arterial(chemo)-*** Patients with HCC treated by a first session of trans-arterial(chemo)embolization and an available CT scan before treatment were *** was assessed using skeletal muscle index at baseline and at the first radiological *** response was recorded after the first session of treatment using *** Of 225 patients treated by trans-arterial bland embolization(n=71)or trans-arterial chemoembolization(n=154)for HCC between 2007 and 2013,Barcelona Clinic of Liver Cancer stage was A,B,and C in 27.5%,55%,and 16.8%of cases,*** was present in 57.7%of the *** with sarcopenia presented a higher rate of progressive disease(19%vs 8%,P=0.0236),a shorter progression-free survival(8.3 vs 13.2 mo,P=0.0035),and a shorter median overall survival(19.4 mo vs 35.5 mo,P=0.0149)compared with non-sarcopenic ***,patients whose sarcopenia appeared after first transarterial treatment had the worst prognosis(P=0.0004).CONCLUSION Sarcopenia is associated with tumor progression and poor survival outcomes after trans-arterial(chemo)-embolization for HCC.

关键词： Hepatocellular carcinoma Transarterial chemoembolization Bland embolization Sarcopenia Skeletal muscle index

Evaluating Fecal Sieving Tool as an Indicator of Feed Valorization and the Impact of Feeding Strategy on Dairy Cow Performance under Farm Conditions

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Agricultural Sciences 2023年第10期14卷 1420-1435页

作者： Nizar Salah Héloïse Legendre Laurine Faivre maxime Briche Raphael Gourdon Valentin Nenov Phileo by Lesaffre Marcq-en-Baroeul France Huvepharma Flemish Region Antwerp Belgium

The objective of this study was to evaluate the use of a faeces sieving tool “Digescan” as an indicator of feed valorization, and to test the influence of feeding strategy on fecal particle size (PS) distribution and weight, milk performance and the possible association between them. Data from 95 trials were used. During each trial, two periods were identified: before and during live yeast probiotic (LYP) supplementation. The LYP used at 5 g/d/cow was Saccharomyces cerevisiae (CNCM I-4407, 1010 CFU/g, Actisaf® Sc47;Phileo by Lesaffre, France). Milk yield and composition were recorded. Fecal samples were taken at the end of each period and sieved with a set of two wire-mesh screens with pore sizes of 5 mm and 2 mm under running tap water. Yeast probiotic supplementation significantly reduced (P 5 mm was accompanied by 1.2 and 0.65 kg/d decrease in MY, 2.5 and 2 kg/d decrease in ECM and 3.2 and 2.6 kg/d in FCM before and during LYP supplementation, respectively. Fecal particle distribution appears to be a practicable tool to predict influences of feeding systems on feed valorization and performance in dairy cows. Live yeast increases dairy performance and decreases the quantity of fecal particles remaining in the two sieves after rinsing.

关键词： Feces Sieving Yeast Probiotic Dairy Cow Milk Particle Size

Ocular Surgical Pathologies in Children Aged 0 to 15 Years in the Bartimée Ophthalmological Clinic in Conakry, Guinea

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Open Journal of Pathology 2022年第4期12卷 120-129页

作者： maxime Dantouma Sovogui Christophe Zoumanigui M’mah Aminata Bangoura Mössö Sevogui Kokou Vonor Faculty of Health Sciences and Techniques University of Conakry Conakry Guinea Bartimée Ophthalmological Clinic Conakry Guinea Pediatrics Department of the Donka National Hospital Conakry Guinea KARA Regional Hospital Center in Togo Kara Togo

Context: Childhood eye health is a real public health problem in our context, the consequences of which go beyond vision, affecting education, social participation and future economic productivity. The aim was to study the frequency of ocular surgical pathologies in children aged 0 to 15 years. Patients and Methodius: This study covered a 4 years period from January 1, 2016 to December 31, 2020. It was a retrospective study of a descriptive and analytical nature. It concerned all children aged 0 to 15 who had undergone surgery for ocular pathology during the study period and who were included in the operating room register. Results: Out of 4974 patients of all ages operated for ocular surgical pathology, 124 were children aged 0 to 15 years or 2.49%. The 9 - 12 age group was the most represented with 29.03%;the mean age was 7.3 years ± 4.6 with extremes of 3 months and 15 years. A male predominance was observed at 62.90%. Pathologies of the lens, cataract and eyelids, traumatic edema represented the most frequent eye conditions in our patients with respectively 51 cases or 41.13% in the right eye (OD), 54 cases or 53.55% in the left eye (OG) and 14 cases or 11.29% in OD and OG followed by endemic limboconjunctivitis of the tropics (LCET), 10 cases or 3.23%, in OD and hemorrhage under conjunctival 13 cases or 10.48% in OG. Cataract surgery with the Technique Manuel Small Incision Cataract Surgery + Implantation in the posterior chamber (MSICS + ICP) was the most used in 74% of cases. General anesthesia was the most widely used anesthetic method in 100% of cases. The clinical course was favorable at 45.97%. Conclusion: We note a relatively low hospital frequency of ocular surgical pathologies in children, with a male predominance. The early consultation period and the improvement of the technical platform could improve their management.

关键词： Surgeries Conakry Children Guinea Eyepieces

Giant Omphalocele Ruptured: An Operated Case and Review of the Literature

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Open Journal of Pediatrics 2020年第3期10卷 447-451页

作者： Koffi maxime Koffi Akaffou Evelyne Dieth Atafy Gaudens Yapo Brouh da Silva-Anoma Sylvia Pediatric Surgery Mother and Children Hospital of Bingerville Bingerville Cote d’Ivoire General Pediatric Mother and Children Hospital of Bingerville Bingerville Cote d’Ivoire Anesthesiology Mother and Children Hospital of Bingerville Bingerville Cote d’Ivoire

Background: The omphalocele is a congenital defect of closure of the abdominal wall at the level of the umbilical ring with evisceration of the intra-abdominal organs in the form of a bag covered with a membrane. This membrane can rupture and become an extreme emergency that can put the infant’s life at stake. Aim: The purpose of this case report is to present a case of ruptured omphalocele operated successfully and describe the factors of good prognosis. Case Presentation: A female new-born with a giant omphalocele. This omphalocele ruptured during conservative treatment. An emergency surgery was organised and the defect was closed by using a prosthesis. The postoperative course was simple. During the 12 months follow-up, we have seen no complication. Conclusion: The management of ruptured omphaloceles is a surgical and anesthetic challenge. The mortality of this condition can be improved in our context by setting up well-equipped neonatal resuscitation units.

关键词： Ruptured Omphalocele Surgery Tanning

Epidemiological, Clinical and Therapeutic Aspects of Childhood Eye Diseases at the Prefectural Hospital of Macenta in Guinea

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Open Journal of Ophthalmology 2023年第4期13卷 352-359页

作者： maxime Dantouma Sovogui Christophe Zoumanigui M’mah Aminata Bangoura Balla Sovogui Mössö Sevogui Kokou Vonor Faculty of Health Sciences and Techniques University of Conakry Conakry Republic of Guinea Bartimee Ophthalmological Clinic Conakry Republic of Guinea Pediatrics Department of the Donka National Hospital Conakry Republic of Guinea Prefectural Hospital of Macenta Macenta Republic of Guinea KARA Regional Hospital Center Kara Togo

Introduction: Eye health in children is a real public health problem in developing countries. The aim of this work was to determine the frequency of ocular pathologies in children aged 0 to 16 at the prefectural hospital of Macenta in Guinea. Patients and Method: This was a descriptive prospective study. It covered a period of 6 months from October 1 to March 31, 2022 at the prefectural hospital of Macenta in Guinea. Children aged 0 to 16 received during the study period were included. Were excluded, those with an incomplete file and whose parents did not consent. Results: Out of 1816 consultations, 224 children aged 0 to 16 were collected 7.95%;mean age 7.35 years ± 4.6;sex ratio 1.38. The age group of 6 to 11 years was more represented. More than half of the patients were educated 50.4% and came from rural areas 52.7%. Pathologies of the orbit were dominated by orbital cellulitis 13% and those of the annexes by conjunctivitis 40.6%. Keratitis and cataracts were pathologies dominating the cornea and the lens, respectively 5.4% and 8.5%. Atrophy of the eyeball was the most frequent among those of the eyeball, at 3.6%. The most performed surgical procedure was phacophagy in 6.7%. The most common reason for consultation was eye pain, at 33.5%. Both eyes were affected at the same time in the majority 49.5%, and the most represented antecedent was malaria 22.3% of cases. Visual acuity without correction ≥ 3/10 was the most represented 37.5% in the right eye and 34.8% in the left eye. The therapeutic classes used were dominated by antibiotic-corticoid combinations 88.8%. Conclusion: Ocular pathologies in children from 0 to 16 years old constitute a real public health problem. They are dominated by pathologies of the conjunctiva and the lens. Popularization for early management of ophthalmological pathologies would be beneficial. .

关键词： Ocular Pathologies Children Macenta Guinea

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园境呈现:景观视角的建筑绘画入门课程重塑

世界建筑导报 2016年第6期31卷 36-39页

作者： maxime Decaudin(文/图) 法国现代中国研究中心

引言为什么要绘画？对于景观建筑师而言,绘画属于创造景观而不是描绘景观,这主要基于两方面的原因。第一,从艺术历史角度看,景观是人们对于环境感知的教育性反映,这源于长期受到的各种景观再现熏陶。

关键词：景观建筑师建筑绘画基础课程一年级建筑学入门环境感知教育性

Toehold switch based biosensors for sensing the highly trafficked rosewood Dalbergia maritima

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Synthetic and Systems Biotechnology 2022年第2期7卷 791-801页

作者： Paul Soudier Daniel Rodriguez Pinzon Tristan Reif-Trauttmansdorff Hassan Hijazi Maeva Cherriere Catia Goncalves Pereira Doriane Blaise maxime Pispisa Angelyne Saint-Julien William Hamlet Melissa Nguevo Eva Gomes Sophia Belkhelfa Anna Niarakis Manish Kushwaha Ioana Grigoras Evry Paris-Saclay 2020 iGEM team France Universite Paris-Saclay INRAEAgroParisTechMicalis Institute78352Jouy-en-JosasFrance Universite Paris-Saclay Univ EvryLaboratoire Europeen de Recherche pour la Polyarthrite Rhumatoïde-GenhotelEvryFrance Universite Paris-Saclay Univ EvryCNRSCEAGenomique MetaboliqueEvryFrance DNA Script Le Kremlin-BicetreFrance SYNMIKRO Center of Synthetic Microbiology MarburgGermany Universite Grenoble Alpes CEAInsermEDyP-BGEGrenobleFrance National Institute of Industrial Property(INPI) CourbevoieFrance

Nucleic acid sensing is a 3 decades old but still challenging area of application for different biological sub-domains,from pathogen detection to single cell transcriptomics *** many applications of nucleic acid detection and identification are mostly carried out by PCR techniques,sequencing,and their derivatives used at large ***,these methods’limitations on speed,cost,complexity and specificity have motivated the development of innovative detection methods among which nucleic acid biosensing technologies seem *** switches are a particular class of RNA sensing devices relying on a conformational switch of secondary structure induced by the pairing of the detected trigger RNA with a de novo designed synthetic sensing mRNA *** we describe a streamlined methodology enabling the development of such a sensor for the RNA-mediated detection of an endangered plant species in a cell-free reaction *** applied this methodology to help identify the rosewood Dalbergia maritima,a highly trafficked wood,whose protection is limited by the capacity of the authorities to distinguish protected logs from other unprotected but related *** streamlined pipeline presented in this work is a versatile framework enabling cheap and rapid development of new sensors for custom RNA detection.

关键词： Toehold switch Riboregulator Biosensor Rosewood

Cirrhosis: Therapeutic Aspects and Outcome for Hospitalized Patients in Burkina Faso

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Open Journal of Epidemiology 2021年第2期11卷 152-162页

作者： Eric Nagaonlé Somé Félicité W. Nana Isabelle Télarpoa Lompo Drabo maxime Roger Sombié Institut de Recherche en Sciences de la Santé (IRSS) Biomedical and Public Health Department Ouagadougou Burkina Faso Université Joseph Ki-Zerbo UFR des Sciences de la Santé Gastro-Enterology Department Ouagadougou Bukina Faso

Introduction: Cirrhosis represents 27.63% of the hepatobiliary diseases in Burkina Faso. We aimed at studying the therapeutic and evolutionary features of the cirrhosis. Patients and methods: We implemented a cross-sectional and descriptive study, collecting retrospectively the data from 1st January 2012 to 31st March 2014. The diagnosis of cirrhosis was based on 1) clinical criteria (an edema-ascitic syndrome with a heterogeneous hepatomegaly with a sharp inferior border or atrophic liver and a portal hypertension);2) biological criteria (a hepatocellular insufficiency syndrome);and 3) ultrasound imaging suggesting cirrhosis. Qualitative variables were presented as frequencies and percentages while quantitative ones were presented as means. Results: The data of 273 patients representing 33.9% of all hospitalizations were analyzed. The hepatobiliary diseases represented 74.7% of all diagnosis. The participants’ mean age was 46.9 years and the sex ratio, 2.7. The HBs antigen and anti-HCV antibodies were positive in 76.5% and 14.6% of the cases, respectively. Ascites was treated with puncture in 40.2% of patients, a salt-free diet (38.8%) and diuretics (54.2%). Hepatic encephalopathy was treated with lactulose in 73.6% of patients and two patients (1.3%) underwent esophageal varices ligation to treat their gastrointestinal bleeding. Lamivudine, tenofovir, and lamivudine-tenofovir combination were administered to 57.4%, 32.8%, and 10% of HBs Antigen positive patients, respectively. Conclusion: Cirrhosis still mostly affects the young and active male population at the gastro-enterology department of the university teaching hospital Yalgado Ouédraogo. Patients show up at a very advanced stage of the disease in poorly prepared health centres. In this context, prevention by the anti-hepatitis B immunization and early systematic screening

关键词： Cirrhosis Hepatitis B Virus Treatment Prognosis Sub-Saharan Africa