Triglyceride clearing in glycogen storage disease.

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.