Cardiac Abnormalities in Diabetic Patients With Mutation in the Mitochondrial tRNA^<Leu(UUR)> Gene
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概要
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An A-to-G transition at position 3243 of the mitochondrial DNA is known to be a pathogenic factor for mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS), diabetes and cardiomyopathy. This mutation causes dysfunction of the central nervous system in MELAS. Because the heart, as well as the brain and nervous system, is highly dependent on the energy produced by mitochondrial oxidation, these tissues are more vulnerable to mitochondrial defects. Cardiac abnormalities were assessed in 10 diabetic patients associated with this mutation using echocardiography and ^<123>I-metaiodobenzylguanidine(MIBG) scintigraphy, and compared with 19 diabetic patients without the mutation. Duration of diabetes, therapy, control of blood glucose and diabetic complications, such as diabetic retinopathy and nephropathy, were not different between the 2 groups. Diabetic patients with the mutation had a signficantly thicker interventricular septum (16.8±3.7 vs 11.0±1.6mm, p<0.001) than those without the mutation. Fractional shortening was lower in diabetic patients with the mutation than those without it (30.7±7.0 vs 42.5±6.6, p<0.001). MIBG uptake on the delayed MIBG image was signfuvantly lowe in diabetic patients with the mutatiron than in those without the mutation (mean value of the heart to mediastinum ratio: 1.6±0.2 vs 2.0±0.4, p<0.05). In conclusion, left ventricular hypertrophy with or without abnormal wall motion and severely redeced MIBG uptake may be characteristic in diabetic patients with a mutation in the mitochondrial tRNA^<Leu(UUR)> gene.
- 社団法人日本循環器学会の論文
- 1999-10-20
著者
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Shiotani Hideyuki
Faculty Of Health Science Kobe University Of Medicine
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Ueno Hiroshi
Department Of Anesthesiology Kyoto Prefectural University Of Medicine
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