Bernier Angelina V, Correia Catherine E, Haller Michael J, Theriaque Douglas W, Shuster Jonathan J, Weinstein David A
Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics, University of Florida, Gainesville, FL 32610-0296, USA.
J Pediatr. 2009 Apr;154(4):588-91. doi: 10.1016/j.jpeds.2008.10.048. Epub 2008 Dec 21.
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.
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).
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).
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.
通过使用动脉功能和解剖结构的非侵入性测量方法，确定更大队列的糖原贮积病（GSD）I型患者的心血管疾病风险。
对28例GSD I型患者（13名女性/15名男性，平均年龄23岁）和23名对照者（19名女性/4名男性，平均年龄23岁）进行颈动脉内膜中层厚度（IMT）、桡动脉张力测量和肱动脉反应性检测。
主要结局指标，GSD队列的平均左远端IMT（0.500±0.055mm）大于对照组（0.457±0.039mm）（P = .002，校正年龄、性别和体重指数后）。通过桡动脉张力测量的平均增强指数在GSD队列中（16.4%±14.0%）高于对照组（2.4%±8.7%）（P < .001）。GSD队列的平均肱动脉反应性（变化6.3%±4.9%）与对照者（变化6.6%±5.1%）之间未观察到显著差异（P = .46）。
GSD I型与IMT增加和增强指数升高所显示的动脉功能障碍相关。GSD I型患者心血管疾病风险可能增加。
