Abstract
We describe 2 patients with glycogen storage disease type 1a and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the ~40-fold increase in palmitate synthesis altering lipoprotein saturated fatty acid contents. These findings are potentially relevant for antihyperlipidemic treatment in patients with glycogen storage disease type 1a.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Cholesterol, LDL / metabolism*
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Glycogen Storage Disease Type I / blood
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Glycogen Storage Disease Type I / complications
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Glycogen Storage Disease Type I / genetics
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Glycogen Storage Disease Type I / physiopathology*
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Humans
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Hyperlipidemias / complications*
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Lipoproteins, VLDL / metabolism
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Male
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Oxidation-Reduction
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Triglycerides / blood
Substances
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Cholesterol, LDL
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Lipoproteins, VLDL
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Triglycerides