Yekeler Ensar, Dursun Memduh, Emeksiz Eda, Akkoyunlu Murat, Akyol Yakup, Demir Fikri, Gökcay Gülden, Demirkol Mübeccel
Department of Radiology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.
Turk J Pediatr. 2007 Apr-Jun;49(2):115-9.
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.
本研究旨在调查Ⅰa型和Ⅲ型糖原贮积病（GSD）患者的内皮功能障碍（ED）和颈动脉内膜中层厚度（IMT）。对22例GSD患者（13例Ⅰa型，9例Ⅲ型）和18名健康受试者，采用高分辨率超声评估肱动脉内皮功能和颈动脉IMT。通过建立反应性充血来评估内皮依赖性舒张（EDD）。比较三组之间的EDD和颈动脉IMT。Ⅲ型GSD患者的平均胆固醇水平略高，但差异不显著。Ⅰa型GSD患者的甘油三酯水平以及胆固醇与高密度脂蛋白（HDL）的比值显著更高。与健康受试者（22%±4%）相比，Ⅰa型（13%±8%，P = 0.001）和Ⅲ型（15%±6%，P = 0.005）GSD患者的EDD显著受损。与健康受试者（0.20±0.02 mm）相比，Ⅰa型（0.23±0.03 mm，P = 0.005）和Ⅲ型（0.26±0.05 mm，P = 0.001）GSD患者的颈动脉IMT均显著更高。Ⅰa型和Ⅲ型GSD患者均表现出显著的ED和IMT增加，这是动脉粥样硬化的预测指标。
