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. 2009 Apr;154(4):588-91.
doi: 10.1016/j.jpeds.2008.10.048. Epub 2008 Dec 21.

Vascular dysfunction in glycogen storage disease type I

Angelina V Bernier  1 , Catherine E Correia, Michael J Haller, Douglas W Theriaque, Jonathan J Shuster, David A Weinstein
Affiliations

Vascular dysfunction in glycogen storage disease type I

Angelina V Bernier et al. J Pediatr. 2009 Apr.

Abstract

Objective: To determine cardiovascular disease risk in a larger cohort of patients with glycogen storage disease (GSD) I through the use of noninvasive measures of arterial function and anatomy.

Study design: Carotid intima media thickness (IMT), radial artery tonometry, and brachial artery reactivity were performed in 28 patients with GSD I (13F/15M, mean age 23 years) and 23 control subjects (19F/4M, mean age 23 years).

Results: The primary outcome measure, mean left distal IMT was greater in the GSD cohort (0.500+/-0.055 mm) than in the control group (0.457+/-0.039 mm) (P= .002, adjusted for age, sex, and body mass index). Mean augmentation index measured by radial artery tonometry was higher in the GSD cohort (16.4%+/-14.0%) than in the control group (2.4%+/-8.7%) (P< .001). No significant difference was observed between mean brachial artery reactivity in the GSD cohort (6.3%+/-4.9% change) versus control subjects (6.6%+/-5.1% change) (P= .46).

Conclusions: GSD I is associated with arterial dysfunction evident by increased IMT and augmentation index. Patients with GSD I may be at increased risk for cardiovascular disease.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure
Figure
Box-and-whisker plot for IMT, RAT, and BAR. Boxes represent the 25th and 75th percentiles, and the median is represented in each box. The range of observations is depicted by the whiskers.
See this image and copyright information in PMC

References

    1. Wolfsdorf JI, Weinstein DA. Glycogen storage diseases. Rev Endocr Metab Disord. 2003;4:95–102. - PubMed
    1. Levy E, Letarte J, Lepage G, Thibault L, Roy CC. Plasma and lipoprotein fatty acid composition in glycogen storage disease type I. Lipids. 1987;22:381–5. - PubMed
    1. Ubels FL, Rake JP, Slaets JP, Smit GP, Smit AJ. Is glycogen storage disease 1a associated with atherosclerosis? Eur J Pediatr. 2002;161:S62–4. - PubMed
    1. Lee PJ, Celermajer DS, Robinson J, McCarthy SN, Betteridge DJ, Leonard JV. Hyperlipidaemia does not impair vascular endothelial function in glycogen storage disease type 1a. Atherosclerosis. 1994;110:95–100. - PubMed
    1. Nguyen AD, Pan CJ, Weinstein DA, Chou JY. Increased scavenger receptor class B type 1-mediated cellular cholesterol efflux and antioxidant capacity in the sera of glycogen storage disease type Ia patients. Mol Genet Metab. 2006;89:233–8. - PubMed

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