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.