The Turkish Journal of Pediatrics 2007 , Vol 49 , Num 2
Prediction of premature atherosclerosis by endothelial dysfunction and increased Iintima-media thickness in glycogen storage disease types Ia and III
1Departments of Radiology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey
2Departments of Pediatrics, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey
Yekeler E, Dursun M, Emeksiz E, Akkoyunlu M, Akyol Y, Demir F, Gökçay G, Demirkol M. Prediction of premature atherosclerosis by endothelial dysfunction and increased intima-media thickness in glycogen storage disease types Ia and III. Turk J Pediatr 2007; 49: 115-119.

The aim of this study was to investigate the endothelial dysfunction (ED) and carotid intima-media thickness (IMT) in patients with glycogen storage disease (GSD) types Ia and III.

In 22 patients with GSD (13, type Ia; 9, type III) and 18 healthy subjects, endothelial functions of the brachial artery and carotid IMT were evaluated by high-resolution ultrasound. Endothelial-dependent dilatation (EDD) was assessed by establishing reactive hyperemia. EDD and carotid IMTs were compared between the three groups.

Mean cholesterol level was slightly higher in GSD type III patients but the difference was not significant. Triglyceride levels and cholesterol to high density lipoprotein (HDL) ratio were significantly higher in GSD type Ia patients. EDD was significantly impaired in GSD type Ia (13%±8%, P=.001) and type III (15%±6%, P=.005) patients when compared with the healthy subjects (22%±4%). The carotid IMT was significantly higher in both GSD type Ia (0.23±0.03 mm, P=.005) and type III (0.26±0.05 mm, P=.001) patients when compared with the healthy subjects (0.20±0.02 mm).

Both GSD type Ia and type III patients show significant ED and increased IMT, which are predictors of atherosclerosis. Keywords : Glycogen storage diseases, endothelial dysfunction, endothelial-dependent dilatation, carotid intima-media thickness, ultrasound

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