BLOOD-LIPIDS AND RHEOLOGICAL MODIFICATIONS IN GLYCOGEN-STORAGE-DISEASE

Citation
K. Keddad et al., BLOOD-LIPIDS AND RHEOLOGICAL MODIFICATIONS IN GLYCOGEN-STORAGE-DISEASE, Clinical biochemistry, 29(1), 1996, pp. 73-78
Citations number
35
Categorie Soggetti
Biology,"Chemistry Medicinal
Journal title
ISSN journal
00099120
Volume
29
Issue
1
Year of publication
1996
Pages
73 - 78
Database
ISI
SICI code
0009-9120(1996)29:1<73:BARMIG>2.0.ZU;2-Z
Abstract
Objectives: Hyperlipidemia is a feature of liver glycogen storage dise ase (GSD). Recent studies have suggested that rheological mechanisms s uch as elevated erythrocyte aggregation may be involved in the pathoge nesis of ischemic syndromes associated with hyperlipidemia. Design and Methods: We investigated erythrocyte aggregation, lipids, and circula tory 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 (mea n = 9). Results: The aggregation results were much higher in patients than controls. The lipid data showed a mixed hyperlipidemia with predo minent hypertriglyceridemia, low HDL-C, apoA-I and LpA-I/A-II, and hig h apoB as compared with controls. However, the LpA-I was not significa ntly different from controls. Conclusions: In conclusion, patients wit h GSD presented hyperlipidemia and elevated erythrocyte aggregation su ch that they are at long-term risk of ischemic complications.