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

Bandsma, Robert H J; Rake, Jan-Peter; Visser, Gepke; Neese, Richard A; Hellerstein, Marc K; van Duyvenvoorde, Wim; Princen, Hans M G; Stellaard, Frans et al. · J Pediatr · 2002

case_report · Level V

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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.

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