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Radical trapping in glycogen storage disease 1a

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Abstract

Oxidative mechanisms involving lipid peroxidation in the subendothelium of the arterial vessel wall play a key role in atherogenesis. Despite severe hyperlipidaemia, patients with glycogen storage disease type la (GSD1a) do not develop premature atherosclerosis. Therefore, we analysed parameters of antioxidative defence and oxidative stress in plasma and serum of patients with GSD1a (n=17) and compared them with those of patients with type 1 diabetes mellitus (n=17), familial hypercholesterolaemia (n=18) and healthy controls (n=20). We measured the total radical trapping ability parameter (TRAP), single plasma antioxidants (sulfhydryl-groups, uric acid, vitamin C, alpha-tocopherol, coenzyme-Q10), markers of lipid peroxidation, lipoprotein (a) and homocysteine. Patients with GSD1a showed an elevated TRAP (P<0.01) compared to the three other groups. This can mainly be attributed to elevated uric acid levels (P<0.05 versus control). Lipoprotein (a) was significantly lower in the GSD1a group compared to the three other groups (P<0.05).Conclusion: Patients with glycogen storage disease type 1a show an increased antioxidative defence in plasma which may protect them against lipid peroxidation and thus against premature atherosclerosis. Our finding of low lipoprotein (a) levels in this small group of patients warrants further investigation in a greater number of patients before assessing its role in atherogenesis in glycogen storage disease type 1a.

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Abbreviations

DM1 :

diabetes mellitus type 1

FH :

familial hypercholesterolaemia

GSD1a :

glycogen storage disease type 1a

LDL :

low density lipprotein

Lp(a) :

lipoprotein (a)

MDA :

malondiadehyde

TRAP :

total radical trapping ability parameter

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Authors and Affiliations

  1. Department of Paediatrics, University Hospital Hamburg, Martinistrasse 52, 20246, Hambury, Germany

    Birgit Wittenstein, Marcus Klein, Barbara Finckh, Kurt Ullrich & Alfried Kohlschütter

Authors
  1. Birgit Wittenstein
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  2. Marcus Klein
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  3. Barbara Finckh
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  4. Kurt Ullrich
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  5. Alfried Kohlschütter
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Correspondence to Birgit Wittenstein.

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Published online: 19 September 2002

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Wittenstein, B., Klein, M., Finckh, B. et al. Radical trapping in glycogen storage disease 1a. Eur J Pediatr 161, S70–S74 (2002). https://doi.org/10.1007/BF02679999

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  • DOI: https://doi.org/10.1007/BF02679999

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