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Second Department of Internal Medicine, Osaka University Medical School, and The Second Department of Pathology (Dr. Hashimoto), Kinki University Medical School. Osaka, Japan.
In a patient with Kearns-Sayre syndrome, concentration of coenzyme Ql0, a component of the mito-chondrial electron transport system, was decreased in serum and in the mitochondrial fraction of skeletal muscle. Serum concentrations of lactate and pyruvate were abnormally high, especially after exercise or oral glucose loading. Levels of folic acid in plasma and CSF were decreased. ECG showed a first-degree atrioventricular block. After administration of coenzyme Ql0 60 to 120 mg daily for 3 months, serum levels of lactate and pyruvate became normal, with improvement of atrioventricular block and ocular movements.
Address correspondence and reprint requests to Dr. Ogasahara, The Second Department of Internal Medicine. Osaka University Medical School, Fukushima 1-1-50 Fukushima-ku, Osaka 553, Japan.
Supported in part by grant No. 82-04 from NCNMMD of the Ministry of Health and Welfare of Japan, and Basic Research Grant from the Muscular Dystrophy Association of the United States.
Accepted for publication June 25, 1984.
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