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Vol.48 No.3 >
Please use this identifier to cite or link to this item:
http://hdl.handle.net/10564/373
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Title: | グルコースクランプ法でインスリン抵抗性の増大が確認された筋緊張性ジストロフィーの1例 |
Other Titles: | A CASE OF MYOTONIC DYSTROPHY WITH INCREASED INSULIN RESISTANCE CONFIRMED BY THE GLUCOSE CLAMP TECHNIQUE |
Authors: | 辻本, 伸麸 金内, 雅夫 尾崎, 博基 木村, 俊明 藤井, 謙裕 土肥, 和紘 村田, 顕也 高柳, 哲也 |
Keywords: | glucose clamp technique insulin resistance myotonic dystrophy |
Issue Date: | 30-Jun-1997 |
Publisher: | 奈良医学会 |
Citation: | 奈良医学雑誌 Vol.48 No.3 p.174-177 |
Abstract: | We report a case of myotonic dystrophy with diabetes mellitus. A 37-year
-old man was admitted for further examination of liver dysfunction, diabetes mellitus, and
myotonic dystrophy. His father, his mother and his sister had been previously diagnosed as
having myotonic dystrophy. He had been mentally retarded since childhood, and suffered
from muscle weakness and hardness of hearing. He was diagnosed as having bilateral
cataracts at the age of 30, when loss of hair gradually started to be noticed. Physical
examination revealed atrophy of bilateral sternocleidomastoid muscles and muscle weak-
ness. Laboratory investigations showed hyperlipidemia, liver dysfunction, hyperglycemia
and primary hypogonadism. ECG showed lst degree of AV block, EMG myotonic discharge
dominantly in distal side. The microscopic findings of muscle biopsy showed hypertrophy
of muscle fibers, predominantly in type 1 fibers. These findings were compatible with the
diagnosis of myotonic dystrophy. DNA analysis of this patient showed an abnormal band
of 6.2 Kb and 3.6 Kb (control; 3.4Kb). His glucose infusion rate by the glucose clamp
technique was decreased to about 6.0 mg/kg/min (normal range : 8.9±1.1). We discuss
the pathogenesis of insulin resistance in patients with myotonic dystrophy based on relevant
studies published in the literature. |
URI: | http://hdl.handle.net/10564/373 |
ISSN: | 04695550 13450069 |
Appears in Collections: | Vol.48 No.3
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