Abstract
The responses of plasma insulin and blood intermediary metabolites to oral glucose (75 g) were determined in 10 subjects with myotonic dystrophy. Results were compared with responses in 10 normal control subjects matched for age, sex, and body mass index. Fasting hyperinsulinemia was observed in the myotonic subjects (7.5 ± 1.6 v 2.4 ± 0.4 mU/L; P < .005) and plasma insulin concentration remained significantly higher following oral glucose (F = 38.09; P < .001). Total cumulative insulin release was markedly higher in the myotonic subjects (4,984.3 v 1,286.6 mU/L; P < .0001). Basal blood glucose concentration was normal (4.8 ± 0.2 v 4.7 ± 0.1 mmol/L), although overall blood glucose was elevated in the myotonic subjects following oral glucose ingestion (F = 5.37; P < .05). Glucose tolerance was normal in all subjects. Fasting blood lactate was higher in the myotonic subjects (1.31 ± 0.13 v 0.94 ± 0.08 mmol/L; P < .05) and remained significantly elevated following the ingestion of glucose (F = 7.22; P < .02). Blood pyruvate response was also higher in the myotonic subjects (F = 5.88; P < .05). Basal blood glycerol was elevated in the myotonic subjects (0.12 ± 0.02 v 0.05 ± 0.01 mmol/L; P < .005) and remained elevated following oral glucose (F = 11.31; P < .005). No significant overall differences were observed in ketone bodies, alanine, or fatty acids between the groups. These results indicate that abnormal lipid metabolism is a feature of myotonic dystrophy in addition to disordered carbohydrate metabolism and insulin resistance. These findings are consistent with the multisystem nature of this disorder.
Published Version
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