Blood lipids and rheological modifications in glycogen storage disease.
OBJECTIVE
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.


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.
Keddad
K
K
Laboratoire Central de Biochimie, Hopital de Bicetre, Le Kremlin-Bicetre, France.
Razavian
S M
SM
Baussan
C
C
Chalas
J
J
Abella
A
A
Levenson
J
J
Simon
A
A
Moatti
N
N
Legrand
A
A
D000293:Adolescent; D000328:Adult; D001054:Apolipoproteins A / Q000097:blood; D001055:Apolipoproteins B / Q000097:blood; D001798:Blood Proteins / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D004903:Erythrocyte Aggregation; D005260:Female; D006008:Glycogen Storage Disease / Q000097:blood* / Q000150:complications; D006801:Humans; D006949:Hyperlipidemias / Q000150:complications*; D007223:Infant; D008055:Lipids / Q000097:blood*; D008074:Lipoproteins / Q000097:blood; D008297:Male; D012212:Rheology; D014280:Triglycerides / Q000097:blood
D001054:Apolipoproteins A; D001055:Apolipoproteins B; D001798:Blood Proteins; D008055:Lipids; D008074:Lipoproteins; D014280:Triglycerides