Abstract
Extract: Peripheral uptake of triglyceride from plasma was investigated by intravenous fat tolerance tests and by postheparin lipoprotein lipase measurements in children with different types of glycogen storage disease.
The patients with a glucose 6-phosphatase deficiency were characterized by a significantly diminished triglyceride elimination rate (5.79 ± 2.78%/min) and 5-min postheparin lipoprotein lipase activity (40.2 ± 23 μEq fatty acid (FA)/liter/min). The patients with a deficiency of debranching enzyme showed a significantly diminished triglyceride elimination rate (4.84 ± 1.61%/min) whereas the 5-min postheparin lipoprotein lipase activities did not significantly differ from the control values (49.6 ± 27.7 μEq FA/liter/min).
The patients with a deficiency of the phosphorylase system showed neither a significantly diminished triglyceride elimination rate (7.34 ± 2.65%/min) nor a diminished 5-min postheparin lipoprotein lipase activity (61.7 ± 30.1 μEq FA/liter/ min).
Triglyceride elimination rates were correlated positively with plasma lipoprotein lipase activities (r = 0.55, P < 0.05).
Speculation: The dietary treatment of hyperlipidemia in hepatic glycogenosis might be based on the outcome of the intravenous fat tolerance test, a low fat clearance being an indication for a low fat, high carbohydrate diet, a normal fat tolerance for a high fat low carbohydrate diet.
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Forget, P., Fernandes, J. & Begemann, P. Triglyceride Clearing in Glycogen Storage Disease. Pediatr Res 8, 114–119 (1974). https://doi.org/10.1203/00006450-197402000-00008
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DOI: https://doi.org/10.1203/00006450-197402000-00008
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