Glycogen Storage Disease Associated With Left Ventricular Aneurysm in an Elderly Patient.
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- Toda Genji
- The Third Department of Internal Medicine, Nagasaki University School of Medicine
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- Yoshimuta Tsuyoshi
- The Third Department of Internal Medicine, Nagasaki University School of Medicine
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- Kawano Hiroaki
- The Third Department of Internal Medicine, Nagasaki University School of Medicine
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- Yano Katsusuke
- The Third Department of Internal Medicine, Nagasaki University School of Medicine
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Glycogen storage disease (GSD) types II, III, IV, and V may be associated with cardiomyopathy, but, with the exception of type III GSD, adult cases are extremely rare. A 62-year-old man was found to have GSD and a concomitant left ventricular aneurysm. He had been comparatively well until the age of 62 years, although he had suffered a cerebral infarction at the age of 35 years. The damage caused by glycogen deposition was strictly confined to the myocardium. Left ventriculography revealed a left ventricular aneurysm in the apex. The serial change on electrocardiogram, as well as the findings of the echocardiogram and of cardiac catheterization, resembled those of the dilated phase of hypertrophic cardiomyopathy. However, a left ventricular endomyocardial biopsy specimen revealed central vacuolar degeneration of myocytes with depositions of glycogen. The GSD type remains unknown in the present patient, because the activity of debranching enzyme (type III) measured from the skeletal muscle specimen was normal, whereas that of acid maltase (type II) was slightly low. It is possible that there is a specific malfunction of the acid maltase of the myocardium in the present patient. (Jpn Circ J 2001; 65: 462 - 464)
収録刊行物
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- JAPANESE CIRCULATION JOURNAL
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JAPANESE CIRCULATION JOURNAL 65 (5), 462-464, 2001
社団法人 日本循環器学会
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詳細情報 詳細情報について
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- CRID
- 1390282681410025472
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- NII論文ID
- 130004427864
- 110002567089
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- NII書誌ID
- AA00690731
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- COI
- 1:STN:280:DC%2BD38%2FgvFOgsA%3D%3D
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- ISSN
- 13474839
- 00471828
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- PubMed
- 11348054
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- Crossref
- PubMed
- CiNii Articles
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- 使用不可