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Importance of clinical suspicion and multidisciplinary management for early diagnosis of a cardiac laminopathy patient:A case report

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World Journal of Clinical Cases 2021年第25期9卷 7472-7477页

作者： Vincenzo Ezio Santobuono Andrea Igoren Guaricci Eugenio Carulli nicola Bozza Martino Pepe Alfredo Ranauro Carlotta Ranieri Maria Cristina Carella Francesco Loizzi nicoletta Resta Stefano Favale Cinzia Forleo Department of Emergency and Organ Transplantation Cardiology Unit of Policlinic University of BariBari 70124BAItaly Division of Medical Genetics Department of Biomedical Sciences and Human OncologyPoliclinic University of BariBari 70124BAItaly

BACKGROUnD Laminopathies are rare diseases,whose cardiac manifestations are heterogeneous and,especially in their initial stage,similar to those of more common conditions,such as ischemic heart *** diagnosis is essential,as these conditions can first manifest themselves with sudden cardiac *** complications usually appear before structural complications;therefore,it is important to take into consideration these rare genetic disorders for the differential diagnosis of brady and tachyarrhythmias,even when left ventricle systolic function is still *** SUMMARY A 60-year-old man,without history of previous disorders,presented in September 2019 to the emergency department because of the onset of syncope associated with *** patient was diagnosed with a high-grade atrioventricular block.A dual chamber pacemaker was implanted,but after the onset of a sustained ventricular tachycardia during physical exertion,a drug eluting stent was implanted on an intermediate stenosis on the left anterior descending artery,which had previously been considered non-haemodynamically *** the follow-up,the treating cardiologist,suspicious of the overall clinical picture,recommended a genetic test for the diagnosis of cardiomyopathies,which tested positive for a pathogenetic mutation of the lamin A/C *** awaiting the result of the genetic test and,later,the pacemaker to be upgraded to a biventricular defibrillator,a remote monitoring device was given to the patient in 2019-related *** case aims to raise awareness of the cardiological manifestations of laminopathies,which can be dangerously misdiagnosed as other, more common conditions.

关键词： Lamin A/C-mutation Cardiolaminopathy Ventricular tachyarrhythmias Remote-monitoring COVID-19 Case report

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Endoscopic sphincterotomy in patients with stenosis of ampulla of Vater:Three-year follow-up of exocrine pancreatic function and clinical symptoms

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World Journal of Gastroenterology 2007年第6期13卷 901-905页

作者： nils Ewald Axel Michael Marzeion Reinhard Georg Bretzel Hans Ulrich Kloer Philip Daniel Hardt Third Medical Depart-ment and policlinic University Hospital Giessen and Marburg Giessen Site D-35392 Giessen Germany

AIM: To investigate retrospectively the long-term effect of endoscopic sphincterotomy (ES) including exocrine pancreatic function in patients with stenosis of ampulla of Vater. METHODS: After diagnostic endoscopic retrograde cholangiopancreatography (ERCP) and ES because of stenosis of the ampulla of Vater (SOD Type Ⅰ), follow-up examinations were performed in 60 patients (mean follow-up time 37.7 mo). Patients were asked about clini-cal signs and symptoms at present and before interven-tion using a standard questionnaire. Before and after ES exocrine pancreatic function was assessed by determina-tion of immunoreactive fecal elastase 1. Serum enzymes indicating cholestasis as well as serum lipase and amy-lase were measured. RESULTS: Eighty percent of patients reported an im-provement in their general condition after ES. The fecal elastase 1 concentrations (FEC) in all patients increased significantly after ES. This effect was even more marked in patients with pathologically low concentrations (nd amylase as well as serum alcaline phosphatase (AP) and gamma-glutamyltranspeptidase (GGT) decreased signifi-cantly after ES. COnCLUSIOn: The results of this study demonstrate that patients with stenosis of the ampulla of Vater can be successfully treated with endoscopic sphincterotomy. The positive effect is not only indicated by sustained improvement of clinical symptoms and cholestasis but also by improvement of exocrine pancreatic function.

关键词： Endoscopic sphincterotomy Fecal elastase Chronic pancreatitis Papillary stenosis Oddi dysfunction

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new insights in the management of the middle hepatic vein dilemma

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Hepatobiliary & Pancreatic Diseases International 2019年第2期18卷 101-102页

作者： Quirino Lai Fabio MelAnDro Gianluca Mennini Massimo Rossi Hepato-Bilio-Pancreatic and Liver Transplant Unit Department of General Surgery and Organ Transplantation Sapienza University of Rome Umberto I Policlinic of Rome

When the first series of adult-to-adult living donor liver transplantation(A2A-LDLT)were performed in Hong Kong[1],it was immediately clear that great technical challenges existed,mainly concerning the middle hepatic ... 详细信息

关键词： new insights middle hepatic vein dilemma

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Shiftwork and Heat Stress in an Intensive Care Unit

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Journal of Huazhong University of Science and Technology(Medical Sciences) 1994年第2期14卷 98-104页

作者：刘理望 Institute of Occupational Medicine Tongji Medical University Wuhan Institute of Occupational Medicine University of Verona Policlinic Hospital Verona Italy

This paper reported the results of investigation on shiftwork and heat stress in an intensive care unit. The aim of this study was to analyse the physiological strain of nurses during the three shifts (Morning, Afternoon,night) in relation to the specific microclimatic conditions and job activities. 8 professional nurses (6 female and 2 male), aged between 21 and 38 years (rnean 29. 8 ±5. 6 years), having from 3 to 18years service, were monitored throughout a complete working cycle of 4 days. They worked on a three shift system at fast rotation. Their working environment was an independent unit for intensive care and expert surgical treatment which was entirely air conditioned. The observation indexes included: twenty-five blood parameters, ten urine parameters, net cardiac cost and relation cardiac cost, etc. The results of the investigation have not envidenced serious alterations of the psycho-physical conditions of the nurses, but do pointed out some problems pertaining both to the environmental and physiological conditions.

关键词： shiftwork heat stress nurse

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Amylase intrapancreatic infusion delays insulin release during an intravenous glucose tolerance test,proof of acini–islet–acinar interactions

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World Journal of Experimental Medicine 2024年第3期14卷 101-107页

作者： Kateryna Pierzynowska Piotr Wychowański Kamil Zaworski Jarosław Woliński Janine Donaldson Dominika Szkopek Katarzyna Roszkowicz-Ostrowska Agata Kondej Stefan G Pierzynowski Department of Biology Lund UniversityLund 22362Sweden Department of Animal Physiology The Kielanowski Institute of Animal Physiology and NutritionPolish Academy of SciencesJabłonna 05110Poland Anara AB Trelleborg 23132Sweden Department of Head and neck and Sensory Organs Division of Oral Surgery and ImplantologyInstitute of Clinical DentistryGemelli Foundation for the University PoliclinicCatholic University of the“Sacred Heart”Rome 00168Italy Department of Oral Surgery Medical University of GdańskGdańsk 80211Poland Laboratory of Large Animal Models The Kielanowski Institute of Animal Physiology and NutritionPolish Academy of SciencesJabłonna 05110Poland School of Physiology Faculty of Health SciencesUniversity of the Witwatersrand(WITS)Johannesburg 2050South Africa Department of Medical Biology Institute of Rural HealthLublin 20090Poland

BACKGROUnD The possible existence of an acini–islet–acinar(AIA)reflex,involving mutual amylase and insulin interactions,was investigated in the current acute experiment on *** To confirm the existence of an AIA reflex and justify the placement of the exocrine and endocrine pancreatic components within the same *** The study was performed on six pigs under general *** intravenous glucose tolerance test was performed,with a bolus infusion of 50%glucose to the jugular vein,while amylase(5000 U/kg)or vehicle intrapancreatic infusions were administered via the pancreaticoduodenalis cranialis artery during 30 min with a 1 mL/min flow *** The amylase infusion to pancreatic arterial circulation inhibited and delayed the insulin release peak which is usually associated with the highest value of blood glucose and is typically observed at 15 min after glucose infusion,for>1 *** intrapancreatic infusion of the vehicle(saline)did not have any effect on the time frame of insulin *** of 1%bovine serum albumin changed the insulin release curve dramatically and prolonged the high range of insulin secretion,far beyond the glucose *** Intrapancreatic arterial infusion of amylase interrupted the integrated glucose–insulin *** confirms an AIA reflex and justifies placement of the exocrine and endocrine pancreatic components within the same organ.

关键词： Amylase Glucose-insulin-amylase interaction Intravenous glucose tolerance test Acini-islet-acinar axis Insulin

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Role of imaging in the evaluation of renal dysfunction in heart failure patients

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World Journal of nephrology 2017年第3期6卷 123-131页

作者： Dario Grande Paola Terlizzese Massimo Iacoviello School of Cardiology Univer- sity of Bari Cardiology Unit Cardiothoracic DepartmentPoliclinic University Hospital

Heart failure and kidney disease share common pathophysiological pathways which can lead to mutual dysfunction,known as cardiorenal *** heart failure patients,renal impairment is related to hemodynamic and nonhemodynamic *** decreased renal blood flow and renal venous congestion due to heart failure could lead to impaired renal *** disease and worsening renal function are independently associated with poor prognosis in heart failure patients,both in acute and chronic clinical *** aim of this review is to assess the role of renal imaging modalities in the evaluation and management of heart failure *** imaging techniques could complete laboratory data,as estimated glomerular filtration rate,exploring different pathophysiological factors involved in kidney disease and adding valuable information about renal structure and *** particular,Doppler examination of arterial and venous hemodynamics is a feasible and non invasive technique,which has proven to be a reliable method for prognostic stratification in patients with cardiorenal *** renal resistance index,a measure related to renal hemodynamics,can be calculated from the Doppler evaluation of arterial ***,the analysis of Doppler venous flow patterns can integrate information from the arterial study and evaluate renal *** imaging modalities are promising,but still confined to research purposes.

关键词： Heart failure Doppler Renal resistance index Hemodynamics Venous Doppler Prognosis Cardiorenal syndrome Chronic kidney disease

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Metastatic Leiomyosarcoma of the Spine Updates in Management and Surgical Strategy

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Open Journal of Modern neurosurgery 2013年第4期3卷 98-103页

作者： Giuseppe Maimone nicola nicassio Irfan Malik Mauro Cambria Department of neurosurgery King’s College Hospital London UK Department of neurosurgery University Policlinic of Messina Messina Italy

Introduction: Leiomyosarcoma (LMS) is a rare malignant tumor arising from the smooth-muscle cells which rarely metastasizes to the bone and even less is found in the spine as a primary localization. In this article the authors describe a case of a peculiar localization of this neoplasm in the upper thoracic spine which required a tailored surgical treatment. Case Report: A 52-year-old female presented with an 8 weeks' history of gradually worsening myelopathy and sensory level in the upper thoracic spine. Pre-operation MRI scans showed an invasive mass neoplasm at T3 and T4 levels involving the vertebral bodies and pedicles with a significant cord compression. A 360° two-step approach was performed. The first operation consisted in a T1-T4 decompressive laminectomy with a sub-total tumor resection and pedicle fixation extending from C7 to T5. A second surgical step was performed through an anterior approach in order to improve the amount of tumor removal. Instrument fixation assured the stabilization of the spinal segment. Post-operative scans demonstrated an optimal decompression. neurological symptoms gradually improved, and the patient benefitted from a gradual reversion of previous symptomatology and could go back to her previous life. Conclusion: LMS represents a challenging tumor due to an extremely aggressive behavior and a considerably high rate of recurrence which requires a tailored approach in terms of surgical strategy and follow-up. A exhaustive review of literature was performed in the attempt to rationalize a surgical strategy and correct management of this extremely rare neoplastic lesion.

关键词： Metastatic Leiomyosarcoma Spinal Metastasis Upper Thoracic Anterior Approach

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non-alcoholic fatty liver disease:Dietary and nutraceutical approaches

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Liver Research 2023年第3期7卷 216-227页

作者： Ludovica Cogorno Elena Formisano Andrea Vignati Amalia Prigione Antonio Tramacere Consuelo Borgarelli Samir Giuseppe Sukkar Livia Pisciotta Department of Experimental Medicine-Medical Pathophysiology Food Science and Endocrinology SectionSapienza University of RomeRomeItaly Department of Internal Medicine University of GenoaGenoaItaly Dietetics and Clinical nutrition Unit IRCCS Policlinic Hospital San MartinoGenoaItaly

non-alcoholic fatty liver disease(nAFLD),defined as the presence of fat accumulation in imaging or histology in more than 5%of hepatocytes and exclusion of other causes for secondary hepatic fat accumulation,is one of the major causes of chronic liver disease *** syndrome is associated with an increased risk of progression from nAFLD to non-alcoholic steatohepatitis(nASH),fibrosis,and forthcoming liver ***,genetic predisposition contributes to the risk of nAFLD *** review explores the role of diets and nutraceuticals in delaying the development and the evolution of nAFLD to chronic liver *** Mediterranean diet,high-protein diet,lowcarbohydrate/high-fat diet,high-carbohydrate/low-fat diet,and intermittent fasting are the dietary approaches investigated given the presence of relevant literature ***,this review focused on nutraceuticals with proven efficacy in ameliorating nAFLD and grouped them into four different categories:plant-based nutraceuticals(Ascophyllum nodosum and Fucus vesiculosus,Silymarin,Berberine,Curcumin,Resveratrol,nigella sativa,Quercetin),vitamin-like substances(vitamin E,vitamin D,vitamin C,coenzyme Q10,inositol),fatty acids(omega-3),and microbiota-management tools(probiotics).

关键词： non-alcoholic fatty liver disease(nAFLD) Metabolic-associated fatty liver disease(MAFLD) non-alcoholic steatohepatitis(nASH) non-alcoholic fatty liver(nAFL) Diets nutraceuticals

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Limited Knowledge of Safe Driving Practice among Drivers with Diabetes in Armenia: Association with Greater Risk of Motor Vehicle Accidents

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Journal of Diabetes Mellitus 2019年第1期9卷 14-23页

作者： Lilit Petrosyan Zhanna Ghazaryan Greta Muradyan Elena Aghajanova Marek Brabece Denisa Janickova Zdarska Katarina Halciakova Jan Polak Brian MFrier Jan Broz n8 policlinic Yerevan State Medical UniversityYerevanArmenia “SourbAstvatsamayr”Medical Centers YerevanArmenia Armenian-American Wellness Center YerevanArmenia Department of Endocrinology Yerevan State Medical University“Muratsan”University HospitalYerevanArmenia Institute of Computer Science The Czech Academy of SciencesPragueCzech Republic Czech Institute of Informatics Roboticsand CyberneticsCzech Technical University in PraguePragueCzech Republic Department of Internal Medicine Second Medical Faculty of Charles UniversityPragueCzech Republic Department for the Study of Obesity and Diabetes Third Medical Faculty of Charles UniversityPragueCzech Republic British Heart Foundation Centre for Cardiovascular Science The Queen’s Medical Research InstituteUniversity of EdinburghEdinburghUK

Aims: The aims of the study were to assess the degree of knowledge and adherence to the recommended safe practice for driving and the risk of road traffic accidents among people with diabetes mellitus in Armenia. Methods: A total of 628 respondents, including 200 drivers, out of 641 consecutive attendees at six diabetes clinics, participated in the survey. A modified British questionnaire on driving and diabetes was used to obtain the relevant data. The information from all 103 drivers treated with insulin and 73 taking sulfonylureas was reviewed. Results: The study revealed that of 176 insulin and sulfonylurea-treated drivers, 161 (91.5%) had never received any advice about safe driving practices. Among the drivers, 156 (88.6%) never measured their blood glucose before, or during driving. The survey revealed that 86 (51.2%) of 168 patients had a history of at least one motor vehicle accident within the previous 5 years. The average road traffic accident rate per person/year was 0.21. Conclusions: The study revealed a lack of knowledge among drivers with diabetes treated with insulin or sulfonylureas concerning recommended safe practices for driving. This was associated with significant hypoglycemia while driving and an elevated rate of road traffic accidents.

关键词： Driving Hypoglycemia Road Traffic Accident

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Urothelial or oral mucosa cells for tissue-engineered urethroplasty: A critical revision of the clinical outcome

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Asian Journal of Urology 2020年第1期7卷 18-23页

作者： Guido Barbagli Axel Heidenreich Vahudin Zugor Leonidas Karapanos Massimo Lazzeri Centro Chirurgico Toscano ArezzoItaly Research Institute for Uronephrology SechenovFirst Moscow State Medical UniversityMoscowRussia University Clinic and policlinic for Urology CologneGermany Istituto Clinico Humanitas IRCCS Clinical and Research HospitalRozzanoItaly

Objective:To report the clinical outcome of urethral reconstruction by cultured urothelial or oral mucosa cells for tissue-engineered ***:We systematically searched for studies reporting the use of tissue-engineered techniques for hypospadias and urethral stricture repair in humans in PubMed and Embase(OvidSP)through January,1990 to June,*** excluded studies based on titles that clearly were not related to the subject,studies in which tissue-engineered biomaterial were used only in laboratory or experimental animals,and in the absence of autologous cultured epithelial *** were also excluded if they were not published in English,had no disease background and adequate ***,we search all relevant abstract presented at two of the main urological meetings in the last 10 years:European Association of Urology(EAU)and American Urological Association(AUA).Results:A total of six articles,reporting the clinical use of tissue-engineered techniques in humans,were fully reviewed in our *** epithelial cells were harvested from the urethra(10 patients),the bladder(11 patients)and the mouth(104 patients).The tissue-engineered grafts were used in children for primary hypospadias repair in 16 cases,and in adults for posterior and anterior urethral strictures repair in 109 ***-engineered grafts were showed working better in children for primary hypospadias repair than in adults for urethral strictures ***:One hundred and twenty-five patients received tissue-engineered urethroplasty using cultured epithelial cells for primary hypospadias or urethral strictures *** studies demonstrate a high degree of heterogeneity respect to epithelial cells(from urethra,bladder,and mouth),type of scaffold,etiology,site of urethral stricture,number of patients,follow-up and outcomes.

关键词： Urethra Urethroplasty Tissue engineering Urethral mucosa Oral mucosa Surgical technique

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