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

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.