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Silvia priori

Przemysław B Radwański, Hsiang-Ting Ho, Rengasayee Veeraraghavan, Lucia Brunello, Bin Liu, Andriy E Belevych, Sathya D Unudurthi, Michael A Makara, Silvia G Priori, Pompeo Volpe, Antonis A Armoundas, Wolfgang H Dillmann, Bjorn C Knollmann, Peter J Mohler, Thomas J Hund, Sándor Györke
BACKGROUND: Cardiac arrhythmias are a leading cause of death in the US. Vast majority of these arrhythmias including catecholaminergic polymorphic ventricular tachycardia (CPVT) are associated with increased levels of circulating catecholamines and involve abnormal impulse formation secondary to aberrant Ca(2+) and Na(+) handling. However, the mechanistic link between β-AR stimulation and the subcellular/molecular arrhythmogenic trigger(s) remains elusive. METHODS AND RESULTS: We performed functional and structural studies to assess Ca(2+) and Na(+) signaling in ventricular myocyte as well as surface electrocardiograms in mouse models of cardiac calsequestrin (CASQ2)-associated CPVT...
June 2016: JACC. Basic to Translational Science
Marta Cavo, Marco Fato, Leonardo Peñuela, Francesco Beltrame, Roberto Raiteri, Silvia Scaglione
Three-dimensional (3D) cell cultures represent fundamental tools for the comprehension of cellular phenomena both in normal and in pathological conditions. In particular, mechanical and chemical stimuli play a relevant role on cell fate, cancer onset and malignant evolution. Here, we use mechanically-tuned alginate hydrogels to study the role of substrate elasticity on breast adenocarcinoma cell activity. The hydrogel elastic modulus (E) was measured via atomic force microscopy (AFM) and a remarkable range (150-4000 kPa) was obtained...
October 13, 2016: Scientific Reports
Francesco Lodola, Diego Morone, Marco Denegri, Rossana Bongianino, Hiroko Nakahama, Lucia Rutigliano, Rosanna Gosetti, Giulia Rizzo, Alessandra Vollero, Michelangelo Buonocore, Carlo Napolitano, Gianluigi Condorelli, Silvia G Priori, Elisa Di Pasquale
Catecholaminergic Polymorphic Ventricular Tachycardia type 2 (CPVT2) is a highly lethal recessive arrhythmogenic disease caused by mutations in the calsequestrin-2 (CASQ2) gene. We have previously demonstrated that viral transfer of the wild-type (WT) CASQ2 gene prevents the development of CPVT2 in a genetically induced mouse model of the disease homozygous carrier of the R33Q mutation. In the present study, we investigated the efficacy of the virally mediated gene therapy in cardiomyocytes (CMs) differentiated from induced pluripotent stem cells (iPSCs) obtained from a patient carrying the homozygous CASQ2-G112+5X mutation...
October 6, 2016: Cell Death & Disease
Barbara Mosca, Jan Eckhardt, Leda Bergamelli, Susan Treves, Rossana Bongianino, Marco De Negri, Silvia G Priori, Feliciano Protasi, Francesco Zorzato
No abstract text is available yet for this article.
September 23, 2016: Journal of Biological Chemistry
Michael J Ackerman, Silvia G Priori, Anne Dubin, Peter Kowey, Nicholas J Linker, David Slotwiner, John Triedman, George F Van Hare, Michael R Gold
No abstract text is available yet for this article.
September 19, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
Matteo Gabetta, Alberto Malovini, Mauro Bucalo, Elisa Zini, Valentina Tibollo, Silvia G Priori, Simone Vettoretti, Cristiana Larizza, Riccardo Bellazzi, Nicola Barbarini
The i2b2 software is a widely adopted solution for secondary use of clinical data for clinical research, specifically designed for cohort identification. i2b2 is still lacking functionalities for data analysis. The aim of this work is to empower the i2b2 framework enabling clinical researchers to perform statistical analyses for accelerating the process of hypothesis testing. To this aim we have developed a flexible extension of i2b2 able to exploit different statistical engines. We have implemented some first applications for basic statistics and survival analyses, exploiting this extension and accessible through suitable user interfaces designed with a special consideration for usability...
2016: Studies in Health Technology and Informatics
Andrea Mazzanti, Silvia G Priori
No abstract text is available yet for this article.
August 9, 2016: Journal of the American College of Cardiology
Silvia Franco, Nicolas Hoertel, Kibby McMahon, Shuai Wang, Jorge Mario Rodríguez-Fernández, Hugo Peyre, Frédéric Limosin, Carlos Blanco
OBJECTIVE: The present study sought to quantify the generalizability of pharmacologic and psychotherapy clinical trial results in individuals with a DSM-IV diagnosis of posttraumatic stress disorder (PTSD) to a large representative community sample. METHODS: Data were derived from the 2004-2005 National Epidemiologic Survey on Alcohol and Related Conditions (NESARC), a large nationally representative sample of the adult US population. We applied a standard set of exclusion criteria representative of pharmacologic and psychotherapy clinical trials to all adults with a DSM-IV diagnosis of PTSD in the previous 12 months (n = 1,715) and then to a subsample of participants seeking treatment (n = 366)...
August 2016: Journal of Clinical Psychiatry
Alessandro Faragli, Katherine Underwood, Silvia G Priori, Andrea Mazzanti
The identification of patients at risk for sudden cardiac death (SCD) is fundamental for both acquired cardiovascular diseases (such as coronary artery diseases, CAD) and inherited arrhythmia syndromes (such as the long-QT syndrome, LQTS). Genetics may play a role in both situations, although the potential to exploit this information to reduce the burden of SCD varies among these two groups. Concerning acquired cardiovascular diseases, which affect most of the general population, preliminary data suggest an association between genetics and the risk of dying suddenly...
September 2016: Journal of Cardiovascular Electrophysiology
Janice Y Chyou, Daniel Friedman, Marina Cerrone, William Slater, Yu Guo, Daniel Taupin, Sean O'Rourke, Silvia G Priori, Orrin Devinsky
Sudden unexpected death in epilepsy (SUDEP) is the most common cause of epilepsy-related mortality. We hypothesized that electrocardiography (ECG) features may distinguish SUDEP cases from living subjects with epilepsy. Using a matched case-control design, we compared ECG studies of 12 consecutive cases of SUDEP over 10 years and 22 epilepsy controls matched for age, sex, epilepsy type (focal, generalized, or unknown/mixed type), concomitant antiepileptic, and psychotropic drug classes. Conduction intervals and prevalence of abnormal ventricular conduction diagnosis (QRS ≥110 msec), abnormal ventricular conduction pattern (QRS <110 msec, morphology of incomplete right or left bundle branch block or intraventricular conduction delay), early repolarization, and features of inherited cardiac channelopathies were assessed...
July 2016: Epilepsia
Paolo Sfriso, Roberta Priori, Guido Valesini, Silvia Rossi, Carlo Maurizio Montecucco, Anna D'Ascanio, Linda Carli, Stefano Bombardieri, Gaetana LaSelva, Florenzo Iannone, Giovanni Lapadula, Stefano Alivernini, Gianfranco Ferraccioli, Michele Colaci, Clodoveo Ferri, Daniela Iacono, Gabriele Valentini, Luisa Costa, Raffaele Scarpa, Andrea LoMonaco, Valentina Bagnari, Marcello Govoni, Ilaria Piazza, Silvano Adami, Francesco Ciccia, Giovanni Triolo, Elisa Alessandri, Maurizio Cutolo, Luca Cantarini, Mauro Galeazzi, Piero Ruscitti, Roberto Giacomelli, Francesco Caso, Paola Galozzi, Leonardo Punzi
Adult-onset Still's disease (AOSD) is a systemic inflammatory condition of unknown aetiology characterized by typical episodes of spiking fever, evanescent rash, arthralgia, leukocytosis and hyperferritinemia. Given the lack of data in Italian series, we promote a multicentric data collection to characterize the clinical phenotype of Italian patients with AOSD. Data from 245 subjects diagnosed with AOSD were collected by 15 centres between March and May 2013. The diagnosis was made following Yamaguchi's criteria...
July 2016: Clinical Rheumatology
Barbara Mosca, Jan Eckhardt, Leda Bergamelli, Susan Treves, Rossana Bongianino, Marco De Negri, Silvia G Priori, Feliciano Protasi, Francesco Zorzato
We exploited a variety of mouse models to assess the roles of JP45-CASQ1 (CASQ, calsequestrin) and JP45-CASQ2 on calcium entry in slow twitch muscles. In flexor digitorum brevis (FDB) fibers isolated from JP45-CASQ1-CASQ2 triple KO mice, calcium transients induced by tetanic stimulation rely on calcium entry via La(3+)- and nifedipine-sensitive calcium channels. The comparison of excitation-coupled calcium entry (ECCE) between FDB fibers from WT, JP45KO, CASQ1KO, CASQ2KO, JP45-CASQ1 double KO, JP45-CASQ2 double KO, and JP45-CASQ1-CASQ2 triple KO shows that ECCE enhancement requires ablation of both CASQs and JP45...
July 8, 2016: Journal of Biological Chemistry
Kai Klepiszewski, Silvia Venditti, Christian Koehler
The development of a strategic approach to manage pollution of surface waters with pharmaceutical residues is in centre of interest in Europe. In this context a lack of reliable standard procedures for sampling and subsequent assessment of pharmaceutical mass flows in the water cycle has been identified. Authoritative assessment of relevant substance concentrations and flows is essential for environmental risk assessments and reliable efficiency analysis of measures to reduce or avoid emissions of drugs to water systems...
July 1, 2016: Water Research
Antonio Curcio, Andrea Mazzanti, Raffaella Bloise, Nicola Monteforte, Ciro Indolfi, Silvia G Priori, Carlo Napolitano
INTRODUCTION: The 2013 HRS/EHRA/APHRS consensus statement recommends the use of V1 and V2 leads recorded in the second and third intercostal spaces (High-ICS) for diagnosis of Brugada syndrome (BrS) creating a new category of patients discovered only with modified leads. The clinical presentation and the arrhythmic risk in these patients are ill defined. This study was aimed at assessing the role of High-ICS in the analysis of BrS and the clinical profile of the patients diagnosed only when ECG leads are moved to upper intercostal spaces...
August 2016: Journal of Cardiovascular Electrophysiology
Silvia G Priori, Carina Blomström-Lundqvist, Andrea Mazzanti, Nico Blom, Martin Borggrefe, John Camm, Perry Mark Elliott, Donna Fitzsimons, Robert Hatala, Gerhard Hindricks, Paulus Kirchhof, Keld Kjeldsen, Karl-Heinz Kuck, Antonio Hernandez-Madrid, Nikolaos Nikolaou, Tone M Norekvål, Christian Spaulding, Dirk J Van Veldhuisen
No abstract text is available yet for this article.
February 2016: Giornale Italiano di Cardiologia
Jacopo F Imberti, Katherine Underwood, Andrea Mazzanti, Silvia G Priori
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inheritable cardiac disorder associated with exercise- and stress-induced sudden death in young individuals. Although important steps forward have been made in the comprehension and treatment of this disease, several aspects remain unclear. Firstly, from an epidemiological standpoint the actual prevalence of CPVT is still unknown and possibly underestimated. In addition, the diagnostic process remains very challenging and can be supported by genetic analysis in only about half of the cases...
August 2016: Heart, Lung & Circulation
Andrea Mazzanti, Riccardo Maragna, Alessandro Faragli, Nicola Monteforte, Raffaella Bloise, Mirella Memmi, Valeria Novelli, Paola Baiardi, Vincenzo Bagnardi, Susan P Etheridge, Carlo Napolitano, Silvia G Priori
BACKGROUND: Long QT syndrome type 3 (LQT3) is a lethal disease caused by gain-of-function mutations in the SCN5A gene, coding for the alpha-subunit of the sodium channel NaV1.5. Mexiletine is used to block late sodium current and to shorten QT interval in LQT3 patients. OBJECTIVES: The aim of this study was to determine whether mexiletine prevents arrhythmic events (arrhythmic syncope, aborted cardiac arrest, or sudden cardiac death) in LQT3 patients. METHODS: The endpoint of this retrospective cohort study, which studied consecutive LQT3 patients who were referred to our center and treated with mexiletine, was to evaluate the antiarrhythmic efficacy of mexiletine by comparing the number of arrhythmic events per patient and the annual rate of arrhythmic events during observation periods of equal duration before and after the beginning of therapy with mexiletine...
March 8, 2016: Journal of the American College of Cardiology
Rafael Pérez-López, Francisco Macías, Carlos Ruiz Cánovas, Aguasanta Miguel Sarmiento, Silvia María Pérez-Moreno
Phosphogypsum wastes from phosphate fertilizer industries are stockpiled in stacks with high contamination potential. An assessment of the environmental impact, including the use of geochemical tracers such as rare earth elements (REE) and Cl/Br ratios, was carried out in the phosphogypsum stack located at the Estuary of Huelva (SW Spain). Inside the pile, highly polluted acid pore-waters flows up to the edge of the stack, emerging as small fluvial courses, known as edge outflows, which discharge directly into the estuary...
May 15, 2016: Science of the Total Environment
Daniela Laricchiuta, Luana Saba, Paola De Bartolo, Silvia Caioli, Cristina Zona, Laura Petrosini
Although aversive memory has been mainly addressed by analysing the changes occurring in average populations, the study of neuronal mechanisms of outliers allows understanding the involvement of individual differences in fear conditioning and extinction. We recently developed an innovative experimental model of individual differences in approach and avoidance behaviors, classifying the mice as Approaching, Balancing or Avoiding animals according to their responses to conflicting stimuli. The approach and avoidance behaviors appear to be the primary reactions to rewarding and threatening stimuli and may represent predictors of vulnerability (or resilience) to fear...
2016: Scientific Reports
Silvia G Priori, Carina Blomström-Lundqvist, Andrea Mazzanti, Nico Blom, Martin Borggrefe, John Camm, Perry Mark Elliott, Donna Fitzsimons, Robert Hatala, Gerhard Hindricks, Paulus Kirchhof, Keld Kjeldsen, Karl-Heinz Kuck, Antonio Hernandez-Madrid, Nikolaos Nikolaou, Tone M Norekvål, Christian Spaulding, Dirk J Van Veldhuisen
No abstract text is available yet for this article.
February 2016: Revista Española de Cardiología
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