Congenital long QT syndrome in children

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Congenital long QT syndrome

Congenital long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval at basal ECG and by a high risk of life-threatening arrhythmias. Disease prevalence is estimated at close to 1 in 2,500 live births. The two cardinal manifestations of LQTS are syncopal episodes, that may lead to cardiac arrest and sudden cardiac death, and electrocardiographic ...

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Long QT syndrome in children with congenital deafness.

The Jervell and Lange Nielson syndrome(JLN) is an infrequent form of long QT syndrome (LQTS) in which prolonged QT interval and congenital deafness exist together. We attempted to identify patients with LQTS among 127 children (age 1.2 to 10 years) with congenital hearing loss. The corrected QT interval was measured from 12 lead electrocardiogram(ECG) , using Bazettes and Friedricia formulae.Th...

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Long QT Syndrome in Children

he advent of genetic testing and the implantable ardioverter-defibrillator (ICD) have so revolutionized the utlook for patients with long QT that the cardiac commuity needs to revise its thinking about long QT syndrome LQTS). This is the underlying message of a report by theridge et al. (1) in this issue of the Journal. Etheridge et al. (1) studied 128 children with LQTS. bout one-half of their...

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Diagnostic miscues in congenital long-QT syndrome.

BACKGROUND Long-QT syndrome (LQTS) is a potentially lethal cardiac channelopathy that can be mistaken for palpitations, neurocardiogenic syncope, and epilepsy. Because of increased physician and public awareness of warning signs suggestive of LQTS, there is the potential for LQTS to be overdiagnosed. We sought to determine the agreement between the dismissal diagnosis from an LQTS subspecialty ...

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Long QT syndrome in children.

T o those of us who were practicing cardiology in the early 1960s, the reports from Jervell and Lange-Nielsen,1 Romano and colleagues,2 and Ward3 were welcome enlightenment for a few of our patients with alarming episodes of syncope and a family history of sudden death. Although Ward reported that one of his original patients responded to ,8-blockade and that form of therapy remains central to ...

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ژورنال

عنوان ژورنال: Medicinski podmladak

سال: 2016

ISSN: 0369-1527

DOI: 10.5937/medpodm1602056c