Prolonged Corrected QT Interval in Patients with Myotonic Dystrophy Type 1

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Prolonged Corrected QT Interval in Patients with Myotonic Dystrophy Type 1

BACKGROUND AND PURPOSE Sudden cardiac death is one of the leading causes of death in patients with myotonic dystrophy type 1 (DM1). It has been proposed that a prolonged QT interval is associated with sudden cardiac death in several neurological diseases, including multiple system atrophy, idiopathic Parkinson's disease, and diabetic autonomic neuropathy. However, analyses of the corrected QT (...

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Genotype–Phenotype Correlations in Iranian Myotonic Dystrophy Type I Patients

Objectives: Myotonic Dystrophy type I (DM1) is a dominantly inherited disorder with a multisystemic pattern affecting skeletal muscle, heart, eye, endocrine and central nervous system. DM1 is associated with the expansion and instability of CTG repeat in the 3chr('39') untranslated region of the myotonic dystrophy protein kinase (DMPK) gene located on chromosome 19q13.3. The aim of this study w...

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Myotonic dystrophy type 1

Myotonic dystrophy type 1 (DM1) is a distal myopathy and a multisystem disease occurring with an incidence of 1/8000, as a result of a CTG trinucleotide repeat expansion in the serinetreonine-protein kinase (DMPK) coding gene on chromosome 19q13.3. In DM1 patients the length of the CTG expansion ranges from 50 to 4000. Disease severity correlates with repeat length and the phenomenon of genetic...

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Routine echocardiography in patients with myotonic dystrophy type 1.

BACKGROUND Myotonic dystrophy type 1 (DM1) is an autosomal-dominant disease. One third of DM1 patients die suddenly, most of them due to the heart conduction abnormalities and arrhythmias. The aim of this study was to analyze echocardiographic findings in a large cohort of DM1 patients. METHODS This retrospective study comprised 111 patients and 71 healthy controls (HCs) matched for gender an...

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Glycemia and corrected QT interval prolongation in young type 1 diabetic patients: what is the relation?

The relationship between a prolonged QT interval and an increased risk of sudden death has been extensively explored in familial long QT syndrome, sudden infant death, and ischemic heart disease (1–3). Several recent studies also describe the relation between corrected QT interval (QTc) prolongation, diabetes complications, and an increased mortality in adults (4,5). We recently described QTc p...

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ژورنال

عنوان ژورنال: Journal of Clinical Neurology

سال: 2013

ISSN: 1738-6586,2005-5013

DOI: 10.3988/jcn.2013.9.3.186