Beneficial effects of fish-oil supplements on lipids, lipoproteins, and lipoprotein lipase in patients with glycogen storage disease type l

Abstract

Glycogen storage disease type I (GSD-I) is frequently complicated by severe hyperlipoproteinemia and the increased potential risk of premature atherosclerosis. The effects of fish-oil supplementation [MaxEPA, 10 g.(1.73 m2)-1 for 3 mo] were investigated prospectively in seven hyperlipoproteinemic patients with GSD-I. Hypertriglyceridemia and hypercholesterolemia improved after 3 mo of fish-oil treatment, decreasing 49% (P < 0.005) and 23%, respectively. This was accompanied by a reduction in both low-density-lipoprotein (LDL) cholesterol (25%, P < 0.03) and apolipoprotein B (40%) and by increased high-density-lipoprotein increased (HDL) cholesterol (30%, P < 0.002) and apolipoprotein A-I (31%, P < 0.05). Low pretreatment ratios of HDL to total cholesterol and HDL to LDL, indicators of elevated atherosclerosis risk, increased significantly (P < 0.05). Plasma lipoprotein profile as well as lipoprotein composition [triglyceride (TG) enrichment and cholesteryl depletion] improved. Reduced TG concentrations were due to enhanced fat catabolism, as evidenced by the significantly increased hepatic and extrahepatic lipoprotein lipase activity (P < 0.05). Withdrawal of fish oil for 3 mo was associated with a return to pretreatment abnormalities in plasma lipids and lipoproteins. Fish-oil supplementation thus improves the hyperlipoproteinemia in GSD-I and may significantly reduce the risk of premature atherosclerotic cardiovascular disease.

Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics

“…Both dietary and pharmacological treatments have been studied, including fibrates, statins, niacin, and fish oil. 109,110 The effect of medium-chain triglycerides on lowering cholesterol and triglycerides is currently being studied. 111,112 The use of vitamin E and its effectiveness in reducing the frequency of infection and improving neutropenia has been reported.…”

Disturbed lipid metabolism in glycogen storage disease type 1

“…The introduction of nocturnal gastric drip feeding and resistant cornstarch for maintenance of normoglycaemia at night time was found to lower plasma cholesterol and triglyceride levels [4,13,24,32] but generally not to normal values [4,7,24,32,38]. Treatment with fibrates [13,14] and/or fish oil [14,27] has also been shown to improve hyperlipidaemia, although the effects of these therapies were found to diminish again over time in a number of patients [14,27].…”

Acute Inhibition of Glucose-6-Phosphate Translocator Activity Leads to Increased De Novo Lipogenesis and Development of Hepatic Steatosis Without Affecting VLDL Production in Rats

“…Diabetes is generally associated with hyperlipidemia, which has been found to be mainly due to overproduction of VLDLtriglycerides in type 2 diabetes (9 -11). Deficiency of G6Pase activity, the metabolic basis of glycogen storage disease type I (GSD-1), also leads to abnormalities in lipid metabolism, characterized by severe hypertriglyceridemia and hypercholesterolemia (12)(13)(14)(15). Glycogen storage disease (GSD) is caused by mutations in the genes encoding either the putative translocator (type non-1a) (4,5) or the catalytic subunit (type 1a) (6,16,17) of the G6Pase system.…”