1986
DOI: 10.1007/bf00705340
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Fatal mitochondrial cardiomyopathy in Kearns-Sayre Syndrome

Abstract: The clinical and postmortem findings in a 26 year old man with Kearns-Sayre syndrome are described. In the last years of his life he suffered from cardiac arrhythmias and a congestive cardiomyopathy, dying of cardiac pump failure. The heart was enlarged, especially the left ventricle which was fibrotic and excessively dilated. Histological and fine structural investigation revealed an excessive loss of myofibrils and an increase of enlarged mitochondria with lamellar and atypically tubular cristae in widesprea… Show more

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Cited by 32 publications
(8 citation statements)
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“…Several observations support this hypothesis. First, cardiac arrhythmias have been reported in patients suffering from diseases caused by the massive accumulation of mtDNA deletions, such as Kearns-Sayre syndrome (KSS) or progressive external ophthalmoplegia (PEO) (Bates et al, 2012;Fratter et al, 2010;Hü bner et al, 1986;Suomalainen et al, 1992). Second, a mosaic pattern of respiratory deficiency has been observed in the heart of a 26-year-old KSS patient with dilated cardiomyopathy (Mü ller-Hö cker et al, 1992).…”
Section: (Legend Continued On Next Page)mentioning
confidence: 99%
“…Several observations support this hypothesis. First, cardiac arrhythmias have been reported in patients suffering from diseases caused by the massive accumulation of mtDNA deletions, such as Kearns-Sayre syndrome (KSS) or progressive external ophthalmoplegia (PEO) (Bates et al, 2012;Fratter et al, 2010;Hü bner et al, 1986;Suomalainen et al, 1992). Second, a mosaic pattern of respiratory deficiency has been observed in the heart of a 26-year-old KSS patient with dilated cardiomyopathy (Mü ller-Hö cker et al, 1992).…”
Section: (Legend Continued On Next Page)mentioning
confidence: 99%
“…l^^iS^F ig. 4 of BISL-32, but none of them reacted with the same epitopes as did BISL-32. Antibodies 521F2, 673D2 and 673D5 stained structures, which were identified as B-cells by the use of an antihuman-insulin serum from guinea pig ( fig.…”
Section: Resultsmentioning
confidence: 77%
“…Hubner et al (1986) reviewed nine reports of young patients who had died of mitochondral myopathy. The youngest was 6 months old.…”
Section: Discussionmentioning
confidence: 99%
“…Cardiac involvement occurs in 20% of cases of MM (Channer et al 1988). Both the conduction and contractile elements of heart muscle can be the target of the mitochondral lesion, the aetiology of which is unknown (Hubner et al 1986). In patients with KSS, abnormalities of cardiac conduction usually become manifest as a teenager or in the early twenties.…”
Section: Discussionmentioning
confidence: 99%
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