title: Plasma antioxidants in pediatric patients with glycogen storage disease, diabetes mellitus, and hypercholesterolemia.
abstract: Oxidative modification of lipoproteins in vessel walls plays a key role in atherogenesis. Patients with glycogen storage disease type Ia (GSD Ia) do not develop premature atherosclerosis despite severe hyperlipidemia. We analyzed antioxidative defense and oxidative stress in plasma and serum of patients with GSD Ia (n = 17) compared to patients with type I diabetes mellitus (DMI, n = 17), familial hypercholesterolemia (FH, n = 18), and healthy controls (n = 20). We measured the total radical-trapping antioxidant parameter (TRAP), single antioxidants (sulfhydryl groups, uric acid, vitamin C, alpha-tocopherol, coenzyme Q10), malondialdehyde, oxidized low density lipoprotein (LDL) antibodies, lipid profile [cholesterol, triglyceride, lipoprotein (a)], homocysteine, and hemoglobin (Hb)A(1C). TRAP levels were elevated in the GSD Ia group (p <.01) and correlated with elevated uric acid levels (r = 0.72, p =.001). None of the other plasma antioxidants correlated with TRAP levels. DMI patients showed decreased sulfhydryl groups (p <.01) and a reduced ubiquinol-10 fraction (p <.01). Malondialdehyde (p <.001) and oxidized LDL autoantibodies (p <.05) were increased in the diabetic group. In FH patients, parameters of oxidative stress and TRAP did not differ from controls. We conclude that in GSD Ia an increased antioxidative defense in plasma may protect against lipid peroxidation and thus against premature atherosclerosis. Furthermore, we demonstrated that in DMI increased oxidative mechanisms are already present in childhood.
authors.affiliation: Department of Pediatrics, University of Hamburg, Hamburg, Germany.
authors.forename: Birgit
authors.initials: B
authors.lastname: Wittenstein
authors.forename: Marcus
authors.initials: M
authors.lastname: Klein
authors.forename: Barbara
authors.initials: B
authors.lastname: Finckh
authors.forename: Kurt
authors.initials: K
authors.lastname: Ullrich
authors.forename: Alfried
authors.initials: A
authors.lastname: Kohlschütter
mesh_terms: D000293:Adolescent; D000328:Adult; D000975:Antioxidants / Q000378:metabolism*; D001205:Ascorbic Acid / Q000097:blood; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D002851:Chromatography, High Pressure Liquid; D003067:Coenzymes; D003922:Diabetes Mellitus, Type 1 / Q000097:blood*; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood*; D006454:Hemoglobins / Q000378:metabolism; D006710:Homocysteine / Q000097:blood; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood*; D007223:Infant; D008077:Lipoproteins, LDL / Q000097:blood; D008297:Male; D008315:Malondialdehyde / Q000097:blood; D013438:Sulfhydryl Compounds / Q000097:blood; D014280:Triglycerides / Q000097:blood; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood; D014527:Uric Acid / Q000097:blood; D014810:Vitamin E / Q000097:blood
chemical_list: D000975:Antioxidants; D003067:Coenzymes; D006454:Hemoglobins; D008077:Lipoproteins, LDL; D013438:Sulfhydryl Compounds; D014280:Triglycerides; C099252:oxidized low density lipoprotein; D006710:Homocysteine; D014451:Ubiquinone; D014810:Vitamin E; D014527:Uric Acid; D008315:Malondialdehyde; D002784:Cholesterol; C024989:coenzyme Q10; D001205:Ascorbic Acid