نتایج جستجو برای: romano ward syndrome

تعداد نتایج: 645734  

2017
Flavia Mayer Laura Faglioni Nera Agabiti Susanna Fenu Francesco Buccisano Roberto Latagliata Roberto Ricci Maria Antonietta Aloe Spiriti Caterina Tatarelli Massimo Breccia Giuseppe Cimino Luana Fianchi Marianna Criscuolo Svitlana Gumenyuk Stefano Mancini Luca Maurillo Carolina Nobile Pasquale Niscola Anna Lina Piccioni Agostino Tafuri Giulio Trapè Alessandro Andriani Paolo De Fabritiis Maria Teresa Voso Marina Davoli Gina Zini

Data on Myelodysplastic Syndromes (MDS) are difficult to collect by cancer registries because of the lack of reporting and the use of different classifications of the disease. In the Lazio Region, data from patients with a confirmed diagnosis of MDS, treated by a hematology center, have been collected since 2002 by the Gruppo Romano-Laziale Mielodisplasie (GROM-L) registry, the second MDS regis...

Journal: :Journal of the American College of Cardiology 2012
Priya Chockalingam Lia Crotti Giulia Girardengo Jonathan N Johnson Katy M Harris Jeroen F van der Heijden Richard N W Hauer Britt M Beckmann Carla Spazzolini Roberto Rordorf Annika Rydberg Sally-Ann B Clur Markus Fischer Freek van den Heuvel Stefan Kääb Nico A Blom Michael J Ackerman Peter J Schwartz Arthur A M Wilde

OBJECTIVES The purpose of this study was to compare the efficacy of beta-blockers in congenital long QT syndrome (LQTS). BACKGROUND Beta-blockers are the mainstay in managing LQTS. Studies comparing the efficacy of commonly used beta-blockers are lacking, and clinicians generally assume they are equally effective. METHODS Electrocardiographic and clinical parameters of 382 LQT1/LQT2 patient...

Journal: :Circulation 1991
D P Zipes

T he long QT interval syndrome (LQTS) has a low incidence in the general population, but, like the Wolff-Parkinson-White (WPW) syndrome , it has a major impact. While the WPW syndrome is the Rosetta stone of reentry, the LQTS may be the Rosetta Stone for ventricular tachyar-rhythmias dependent on sympathetic stimulation. Present Study In a recent issue of Circulation, Schwartz et all provided f...

Journal: :Circulation 2001
P J Schwartz S G Priori C Spazzolini A J Moss G M Vincent C Napolitano I Denjoy P Guicheney G Breithardt M T Keating J A Towbin A H Beggs P Brink A A Wilde L Toivonen W Zareba J L Robinson K W Timothy V Corfield D Wattanasirichaigoon C Corbett W Haverkamp E Schulze-Bahr M H Lehmann K Schwartz P Coumel R Bloise

BACKGROUND The congenital long-QT syndrome (LQTS) is caused by mutations on several genes, all of which encode cardiac ion channels. The progressive understanding of the electrophysiological consequences of these mutations opens unforeseen possibilities for genotype-phenotype correlation studies. Preliminary observations suggested that the conditions ("triggers") associated with cardiac events ...

2015
Milena Bellin Boris Greber

Recessive mutations in the ion channel-encoding KCNQ1 gene may cause Jervell and Lange-Nielsen syndrome (JLNS), a fatal cardiac disease leading to arrhythmia and sudden cardiac death in young patients. Mutations in KCNQ1 may also cause a milder and dominantly inherited form of the disease, long QT syndrome 1 (LQT1). However, why some mutations cause LQT1 and others cause JLNS can often not be u...

Journal: :Journal of the American College of Cardiology 2014
Jie Wu Nobu Naiki Wei-Guang Ding Seiko Ohno Koichi Kato Wei-Jin Zang Brian P Delisle Hiroshi Matsuura Minoru Horie

OBJECTIVES This study sought to explore molecular mechanisms underlying the adrenergic-induced QT prolongation associated with KCNQ1 mutations. BACKGROUND The most frequent type of congenital long QT syndrome is LQT1, which is caused by mutations in the gene (KCNQ1) that encodes the alpha subunit of the slow component of delayed rectifier K(+) current (IKs) channel. We identified 11 patients ...

2008
Yanfei Ruan Silvia G. Priori

The discovery of genetic defects underlying long-QT syndrome (LQTS) has allowed to identify important genotype-phenotype correlations that are now being used for risk stratification. The next challenge is to exploit the new information on the pathophysiology of the disease derived from molecular genetics to devise more effective therapies. The successful response of LQT1 patients to -blockers, ...

Journal: :Kardiologia polska 2010
Magdalena A Szeliga Paula L Hedley Carin P Green Daniel V Møller Michael Christiansen

INTRODUCTION Long QT-syndrome (LQTS) is a genetic cardiac channelopathy characterised by a prolonged QT interval on a surface electrocardiogram (ECG), syncope, T-wave abnormalities, ventricular tachycardia of the torsades de pointes (TdPVT) type (Fig. 1) and an increased risk of sudden death [1]. LQTS has variable clinical presentation and is genetically characterised by incomplete penetrance, ...

2005

I F we consider that agriculture employed a majority of the population in non-industrial societies and is still man's staple occupation, we must ad-mit that the agriculture of Roman Britain has concerned archaeologists and historians much less than it should, for on its vicissitudes depended to no small degree the well-being and decline of the province. The existence of Roman 'villas' was first...

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