Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a

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...

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Bibliographic Details
Published in:The Journal of pediatrics 2002-02, Vol.140 (2), p.256-260
Main Authors: Bandsma, Robert H.J., Rake, Jan-Peter, Visser, Gepke, Neese, Richard A., Hellerstein, Marc K., van Duyvenvoorde, Wim, Princen, Hans M.G., Stellaard, Frans, Smit, G.Peter A., Kuipers, Folkert
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Cholesterol, LDL - metabolism
Disorders of blood lipids. Hyperlipoproteinemia
Glycogen Storage Disease Type I - blood
Glycogen Storage Disease Type I - complications
Glycogen Storage Disease Type I - genetics
Glycogen Storage Disease Type I - physiopathology
Humans
Hyperlipidemias - complications
Lipoproteins, VLDL - metabolism
Male
Medical sciences
Metabolic diseases
Oxidation-Reduction
Triglycerides - blood
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Summary: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. (J Pediatr 2002;140:256-60)
ISSN:0022-3476
1097-6833
DOI:10.1067/mpd.2002.121382