Beneficial effects of fish-oil supplements on lipids, lipoproteins, and lipoprotein lipase in patients with glycogen storage disease type I.
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.
Levy
E
E
Hôpital Sainte-Justine, Department of Nutrition, Université de Montréal, Quebec, Quebec, Canada.
Thibault
L
L
Turgeon
J
J
Roy
C C
CC
Gurbindo
C
C
Lepage
G
G
Godard
M
M
Rivard
G E
GE
Seidman
E
E
D000293:Adolescent; D000328:Adult; D002784:Cholesterol / Q000097:blood / Q000145:classification; D005395:Fish Oils / Q000009:adverse effects / Q000494:pharmacology*; D005953:Glycogen Storage Disease Type I / Q000097:blood* / Q000150:complications; D006801:Humans; D006949:Hyperlipidemias / Q000097:blood / Q000150:complications; D008055:Lipids / Q000097:blood*; D008071:Lipoprotein Lipase / Q000097:blood*; D008074:Lipoproteins / Q000097:blood*; D008297:Male; D011446:Prospective Studies
D005395:Fish Oils; D008055:Lipids; D008074:Lipoproteins; D002784:Cholesterol; D008071:Lipoprotein Lipase