Plasma and lipoprotein fatty acid composition in glycogen storage disease type I.

摘要: Nocturnal intragastric feeding has been shown to be an effective means improve clinical and biochemical features in glycogen storage disease type I (GSD-I). In this study, we investigated the fatty acid patterns a whole plasma circulating lipoproteins patients on therapy. The results demonstrated massive concentration of total acids coupled with higher levels triglycerides, free cholesterol, cholesterol ester phospholipids. This hyperlipidemia involved all without distinction carbon or bond numbers. However, increase was more pronounced for saturated than polyunsaturated acids, as by ratios both oleic linoleic (1.91±0.40 vs 0.80±0.09 controls) ω3+ω6 ω9 families (0.92±0.11 1.66±0.08 controls). very low (VLDL), (LDL) high (HDL) density lipoprotein showed substantial differences composition, reflecting association between abnormal pattern essential deficiency. Furthermore, GSD-I exhibited significant VLDL (17±2 47±7 mg/dl) LDL (124±7 206±24 mg/dl), decrease HDL (49±4 28±3 mg/dl). These data documenting associated increased proportion suggest that nocturnal are at risk atherosclerosis its complications.