Plasma Free Fatty Acid Metabolism in Patient with Progressive Muscular Dystrophy

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The metabolism of plasma free fatty acid (FFA) and ketone bodies was studied in 33 patients with generalized muscular atrophy: 18 with Duchenne type progressive muscular dystrophy (PMD), 3 with limb-girdle type PMD, 8 with congenital PMD and 4 with Kugelberg-Welander's disease. The age of patients were ranged between 8 and 18 years. Plasma FFA was assayed by Novak's colorimetric method and β-hydroxybutyric acid by Williamson's enzymatic method.<BR>High level of plasma FFA was observed after overnight fast in 7 Duchenne type PMD, 5 congenital PMD and 3 Kugelberg-Welander's disease. Three patients with limb-girdle type PMD revealed no abnormality in respect to fasting level of plasma FFA. Seventeen of these patients excreted 10 mg/dl or more of ketone bodies in urine after overnight fast. A high level of β-hydroxybutyric acid in blood was also observed in 4 of the 5 Duchenne type dystrophics examined. The data suggested that these patients were easily liable to metabolic acidosis. Hyperketonemia might be caused partly by the increased FFA level in blood, but it may also be possible that human dystrophic muscle can not utilize ketone bodies as suggested by Gould for the muscle of dystrophic mouse.<BR>No significant correlation was disclosed between the fasting plasma FFA level and the severity of muscle involvement in the patients. After ingestion of 50 grams of glucose, high plasma FFA fell below 0.3 mEq/l as in normal control subjects. In 5 patients with Duchenne type PMD, intravenaus infusion of norepinephrine did not induce any mobilization of lipid. Release of FFA from adipose tissue seemed to be increased in these patients by the mechanism which was not yet fully elucidated.
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Plasma Free Fatty Acid Metabolism in Patient with Progressive Muscular Dystrophy

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