糖原贮积病中的血脂和血液流变学改变
Blood lipids and rheological modifications in glycogen storage disease.
作者信息
Keddad K, Razavian S M, Baussan C, Chalas J, Abella A, Levenson J, Simon A, Moatti N, Legrand A
机构信息
Laboratoire Central de Biochimie, Hopital de Bicetre, Le Kremlin-Bicetre, France.
出版信息
Clin Biochem. 1996 Feb;29(1):73-8. doi: 10.1016/0009-9120(95)02014-4.
OBJECTIVES
Hyperlipidemia is a feature of liver glycogen storage disease (GSD). Recent studies have suggested that rheological mechanisms such as elevated erythrocyte aggregation may be involved in the pathogenesis of ischemic syndromes associated with hyperlipidemia.
DESIGN AND METHODS
We investigated erythrocyte aggregation, lipids, and circulatory proteins in the blood of 24 patients affected with GSD, aged from 1 to 23 years (mean = 8) and 26 controls aged from 1 to 28 years (mean = 9).
RESULTS
The aggregation results were much higher in patients than controls. The lipid data showed a mixed hyperlipidemia with predominant hypertriglyceridemia, low HDL-C, apoA-I and LpA-I/A-II, and high apoB as compared with controls. However, the LpA-I was not significantly different from controls.
CONCLUSIONS
In conclusion, patients with GSD presented hyperlipidemia and elevated erythrocyte aggregation such that they are at long-term risk of ischemic complications.
目的
高脂血症是肝糖原贮积病（GSD）的一个特征。最近的研究表明，诸如红细胞聚集增加等流变学机制可能参与了与高脂血症相关的缺血综合征的发病过程。
设计与方法
我们调查了24例年龄在1至23岁（平均8岁）的GSD患者以及26例年龄在1至28岁（平均9岁）的对照者血液中的红细胞聚集、血脂及循环蛋白情况。
结果
患者的聚集结果比对照者高得多。脂质数据显示与对照者相比，患者存在混合性高脂血症，以高甘油三酯血症为主，高密度脂蛋白胆固醇（HDL-C）、载脂蛋白A-I（apoA-I）和脂蛋白A-I/A-II（LpA-I/A-II）水平低，载脂蛋白B（apoB）水平高。然而，LpA-I与对照者无显著差异。
结论
总之，GSD患者存在高脂血症且红细胞聚集增加，因此他们长期有发生缺血性并发症的风险。
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