An Unusually Late Presentation of Recurrent Cardiac Syncope in Kearns Sayre Syndrome
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چکیده
منابع مشابه
An Unusually Late Presentation of Recurrent Cardiac Syncope in Kearns Sayre Syndrome
Background: Kearns Sayre Syndrome (KSS) is a rare mitochondrial disorder with systemic involvement characterised by skeletal, smooth and cardiac muscle dysfunction; pigmentary retinal changes; neurological and endocrine dysfunction. KSS arise from a large-scale deletion of mitochondrial DNA which typically occurs as a sporadic event. Recurrent cardiac syncope is one way of the initial presentat...
متن کامل[Kearns-Sayre syndrome: recurrent syncope and atrial flutter].
Kearns-Sayre syndrome is a rare disease linked to mitochondrial inheritance. The characteristic diagnostic triad consists of progressive external ophthalmoplegia, pigmentary degeneration of the retina, and atrioventricular block.1 It may also be associated with mental retardation, ataxia, deafness, muscle weakness, and endocrine disorders, such as diabetes mellitus or hypothyroidism. We present...
متن کامل[Cardiac involvement in Kearns-Sayre syndrome].
The mitochondrial cytopathies or oxidative-phosphorylation diseases are a diverse group of disorders that result from the structural, biochemical, or genetic derangement of mitochondria. Because mitochondrial dysfunction can affect the most highly energy-dependent organs, cardiac involvement is frequent in these diseases. To identify the clinical features of Kearns-Sayre syndrome, an entity ass...
متن کاملCardiac arrest in kearns-sayre syndrome.
The prognosis of progressive ophthalmoplegia in patients with large-scale mitochondrial DNA deletions is highly variable and almost unpredictable. The risk to develop cardiac involvement and sudden cardiac death is strikingly high, especially in patients with Kearns-Sayre syndrome (KSS). The most typical cardiac complications of the disease are conduction defects, which usually begin with left ...
متن کاملKearns-Sayre syndrome
The Kearns-Sayre syndrome is a mitochondrial myopathy characterised by ptosis, chronic progressive external ophthalmoplegia, abnormal retinal pigmentation, and cardiac conduction defects. A unique case is reported in which there was rapid development ofprogressive congestive cardiac failure that required cardiac transplantation. A review of published reports of mitochondrial myopathy shows that...
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ژورنال
عنوان ژورنال: Journal of Cardiovascular Diseases & Diagnosis
سال: 2017
ISSN: 2329-9517
DOI: 10.4172/2329-9517.1000289