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| 1 | Electrical storm: A clinical and electrophysiological overview显示文摘Electrical storm(ES) is a clinical condition characterized by three or more ventricular arrhythmia episodes leading to appropriate implantable cardioverterdefibrillator(ICD) therapies in a 24 h period. Mostly, arrhythmias responsible of ES are multiple morphologies of monomorphic ventricular tachycardia(VT), but polymorphic VT and ventricular fibrillation can also result in ES. Clinical presentation is very dramatic in most cases, strictly related to the cardiac disease that may worsen electrical and hemodynamic decompensation. Therefore ES management is challenging in the majority of cases and a high mortality is the rule both in the acute and in the long-term phases. Different underlying cardiomyopathies provide significant clues into the mechanism of ES, which can arise in the setting of structural arrhythmogenic cardiomyopathies or rarely in patients with inherited arrhythmic syndrome, impacting on pharmacological treatment, on ICD programming, and on the opportunity to apply strategies of catheter ablation. This latter has become a pivotal form of treatment due to its high efficacy in modifying the arrhythmogenic substrate and in achieving rhythm stability, aiming at reducing recurrences of ventricular arrhythmia and at improving overall survival. In this review, the most relevant epidemiological and clinical aspects of ES, with regard to the acute and long-term follow-up implications, were evaluated, focusing on these novel therapeutic strategies of treatment. | Sergio Conti Salvatore Pala Viviana Biagioli Giuseppe Del Giorno Martina Zucchetti Eleonora Russo Vittoria Marino Antonio Dello Russo Michela Casella Francesca Pizzamiglio Valentina Catto Claudio Tondo Corrado Carbucicchio | 2015 | World Journal of Cardiology2015，7，9： | 9 |
| 2 | liver and the defects of cholesterol and bile acids biosynthesis: rare disorders many diagnostic pitfalls显示文摘In recent decades, biotechnology produced a growth of knowledge on the causes and mechanisms of metabolic diseases that have formed the basis for their study, diagnosis and treatment. Unfortunately, it is well known that the clinical features of metabolic diseases can manifest themselves with very different characteristics and escape early detection. Also, it is well known that the prognosis of many metabolic diseases is excellent if diagnosed and treated early. In this editorial we briefly summarized two groups of inherited metabolic diseases, the defects of cholesterol biosynthesis and those of bile acids. Both groups show variable clinical manifestations but some clinical signs and symptoms are common in both the defects of cholesterol and bile acids. The differential diagnosis can be made analyzing sterol profiles in blood and/or bile acids in blood and urine by chromatographic techniques(GC-MS and LC-MS/MS). Several defects of both biosynthetic pathways are treatable so early diagnosis is crucial. Unfortunately their diagnosis is made too late, due either to the clinical heterogeneity of the syndromes(severe, mild and very mild) that to the scarcity of scientific dissemination of these rare diseases. Therefore, the delay in diagnosis leads the patient to the medical observation when the disease has produced irreversible damages to the body. Here, we highlighted simple clinical and laboratory descriptions that can potentially make you to suspect a defect in cholesterol biosynthesis and/or bile acids, as well, we suggest appropriate request of the laboratory tests that along with common clinical features can help to diagnose these defects. | Gaetano Corso Antonio Dello Russo Monica Gelzo | 2017 | World Journal of Gastroenterology2017，23，29： | 2 |
| 3 | Influence of dietary fiber addition on sensory and theological properties of yogurt显示文摘 | Dello M Staffolo | | 0，，： | 1 |
| 4 | Gaseous neuromodulator in the control of neuroendocrine stress axis显示文摘 | Navarra P Dello Russo C Mancuso C | 2000 | Ann NY Acad Sci2000，917，： | 1 |
| 5 | Concealed cardiomyopathies in competitive athletes with ventricular arrhythmias and an apparently normal heart:role of cardiac electroanatomical mapping and biopsy显示文摘 | Dello Russo A Pieroni M Santangeli P | | 0，，12： | 1 |
| 6 | Evidence that hydrogen sulfide can modulate hypothalamo-pituitary- adrenal axis function: in vitro and in vivo studies in the rat 显示文摘 | Dello Russo C Tringali G Ragazzoni E | 2000 | J Neruoendoerinol2000，12，3： | 1 |
| 7 | A genetic framework for the control of cell division and differentiation in the root meristem显示文摘 | Dello Ioio R Nakamura K Moubayidin L | 2008 | Science2008，322，5906： | 1 |
| 8 | The heat-shock protein 90 inhibitor 17-allylamino-17-demethoxygeldanamycin suppresses glial inflammatory responses and ameliorates experimental autoimmune encephalomyelitis显示文摘 | Dello Russo C Polak PE Mercado PR | 2006 | J Neurochem2006，99，5： | 1 |
| 9 | Drug-refractoryventricular tachycardias after myocarditis:endocardial andepicardial radiofrequency catheter ablation显示文摘 | Dello Russo A Casella M Pieroni M | 2012 | Circ ArrhythmElectrophysiol2012，5，3： | 1 |
| 10 | Experi-ments on standing bubbles in a vertical pipe显示文摘 | GENNARO DELLO Ioio WOODS Andrew W | 2008 | Journalof Fluid Mechanics2008，616，： | 1 |
| 11 | High prevalence of myocarditis mimicking arrhythmogenic right ventricular cardiomyopathy differential diagnosis by electroanatomic mapping-guided endomyocardial biopsy显示文摘 | Pieroni M Dello Russo A Marzo F | | 0，，8： | 1 |
| 12 | Treatment with amiodarone to prevent artrial fibrillation显示文摘 | Santangeli P Dello Russo A Bellocci F | 2009 | JAMA2009，301，10： | 1 |
| 13 | 'Near-zero' fluoro- scopic exposure in supraventricular arrhythmia ablation using the En- site NavXTM mapping system: personal experience and review of the literature显示文摘 | Casella M Pelargonio G Dello Russo A | 2011 | lnterv Cardi Electrophysiol2011，31，： | 1 |
| 14 | Plasma intestinal fatty acid-binding protein levels correlate with morphologic epithe- lial intestinal damage in a human translational ischemia-reperfu- sion model 显示文摘 | Schellekens DH Grootjans JM Dello SA | 2014 | J Clin Gastroentero12014，48，3： | 1 |
| 15 | Gsα^(R201C)and estrogen reveal different subsets of bone marrow adiponectin expressing osteogenic cells显示文摘The Gsα/cAMP signaling pathway mediates the effect of a variety of hormones and factors that regulate the homeostasis of the post-natal skeleton.Hence,the dysregulated activity of Gsαdue to gain-of-function mutations (R201C/R201H)results in severe architectural and functional derangements of the entire bone/bone marrow organ.While the consequences of gain-of-function mutations of Gsαhave been extensively investigated in osteoblasts and in bone marrow osteoprogenitor cells at various differentiation stages,their effect in adipogenically-committed bone marrow stromal cells has remained unaddressed.We generated a mouse model with expression of Gsα^(R201C) driven by the Adiponectin(Adq)promoter.Adq-Gsα^(R201C) mice developed a complex combination of metaphyseal,diaphyseal and cortical bone changes.In the metaphysis,Gsα^(R201C) caused an early phase of bone resorption followed by bone deposition.Metaphyseal bone formation was sustained by cells that were traced by Adq-Cre and eventually resulted in a high trabecular bone mass phenotype.In the diaphysis,Gsα^(R201C),in combination with estrogen,triggered the osteogenic activity of Adq-Cre-targeted perivascular bone marrow stromal cells leading to intramedullary bone formation.Finally,consistent with the previously unnoticed presence of Adq-Cre-marked pericytes in intraosseous blood vessels,Gsα^(R201C) caused the development of a lytic phenotype that affected both cortical(increased porosity)and trabecular(tunneling resorption)bone.These results provide the first evidence that the Adq-cell network in the skeleton not only regulates bone resorption but also contributes to bone formation,and that the Gsα/cAMP pathway is a major modulator of both functions. | Biagio Palmisano Rossella Labella Samantha Donsante Cristina Remoli Emanuela Spica Ilenia Coletta Giorgia Farinacci Michele Dello Spedale Venti Isabella Saggio Marta Serafini Pamela Gehron Robey Alessandro Corsi Mara Riminucci | 2022 | Bone Research2022，10，4： | 1 |
| 16 | Influence of dietary fiber addition on sensory and rheological properties of yogurt显示文摘 | M Dello Staffolo | 2004 | International Dairy Journal2004，14，： | 1 |
| 17 | Evidence that hydrogen sulphide can modulate hypothalamo-pituitary-adrenal axis function:in vitro and in vivo studies in the rat显示文摘 | Dello Russo C Tringali G Ragazzoni E | 2000 | J Neuroendocrinol2000，12，3： | 1 |
| 18 | Gaseous neuromodulat ors in the control of neuroen docrine stress axis显示文摘 | Navarra P C Dello Russo Mancuso C | 2000 | Ann N Y Acad Sci2000，917，1： | 1 |
| 19 | Influence of enhanced alpha-1-acid glycoprotein concentration on protein binding, pharmacokinetics and antiarrhythmic effect of lidocaine in the dog显示文摘 | De Rick AF Belpaire FM Dello C Bogaert MG | 1987 | J Pharmacol Exp Ther1987，241，： | 1 |
| 20 | More on the effectiveness of public spending on health care and education : a covariance structure model 显示文摘 | Emanuele Baldacci Maria Teresa Guin - sui Luiz Dello | 2003 | Journal of International Development2003，，15： | 1 |
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