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Strokova et al. Vessel Plus 2019;3:16 I http://dx.doi.org/10.20517/2574-1209.2019.08 Page 3 of 7
[16]
The impact of oxidized lipoproteins on atherogenesis is well known . The role of oxidative stress and
total antioxidant protection in patients with GSD I was examined in a number of studies. In one study,
total reactive antioxidant potential was compared between children with GSD, diabetes, and familial
[17]
hypercholesterolemia. This biomarker was much higher among GSD patients .
Some studies demonstrated the development of premature atherosclerosis and an increased risk of CV
complications associated with GSD [18,19] , whereas others did not reveal the association of GSD with
premature atherosclerosis [1,20] . Moreover, it was found that LDL-cholesterol particles are paradoxically more
[21]
resistant to oxidative stress in patients with GSD I compared to the control group .

A series of studies show the connection between high cholesterol levels in children and adolescents in the
[22]
general population and the risk of atherosclerosis and CV complications in adult life . However, despite
the fact of the existence of dyslipidemia in GSD has been known for several decades, the need for medical
treatment of lipid disorders in GSD is still disputable.

Fibrates, statins, niacin, bile acid sequestrants, ezetimibe, lomitapide, phytosterols, fish oil, etc. are currently
used to correct lipid disorders in adults. The issue of prescribing these medicines to children remains
controversial. Apparently, lipid-lowering therapy should be prescribed if there are strict indications, when
diet therapy does not eliminate high levels of blood lipids, threatening the development of acute pancreatitis
[23]
or atherothrombotic complications .

Thus, the aim of our study was to determine lipid profile patterns in children with different types of glycogen
storage disease.

METHODS
The study included 62 children with GSD (43 boys, 19 girls), mean age 8.29 years. All patients underwent
anthropometry including weight, height adjusted as Z-score to the age with the calculation of body mass
index Z-score and percentiles. The assessment of physical development for overweight and obesity was
carried out using the WHO Anthro and AnthroPlus programs. Fasting total cholesterol, HDL cholesterol,
LDL cholesterol and triglycerides plasma levels were analyzed by Konelab Prime 60i auto-analyzer (Thermo
Scientific, Wilmington, DE, USA) with the internal age-adjusted normal range. Dyslipidemia was defined
as any abnormal total cholesterol, HDL cholesterol, LDL cholesterol or/and triglycerides plasma levels.
Statistical analysis was performed using Statistica for Windows 6.0 (StatSoft Inc., USA). The statistical
significance level was taken as sufficient for P < 0.05.

The study was conducted in agreement with the Declaration of Helsinki, GCP. The special approval from the
Independent Local Board of Ethics Committee was received.

RESULTS
The children were divided into three groups depending on the type of GSD. Nineteen children (31%) had
type I GSD (Group 1), 16 (26%) - type III (Group 2) and 27 (43%) - types VI and IX (Group 3). The groups
were comparable in age. In the group of children with type VI and IX GSD, boys prevailed, since the vast
majority of patients had type IX GSD, which is characterized by the X-linked pattern.

Overweight and obesity were found in 10.5% of children in Group 1, 6.3% in Group 2 and 11.1% in Group 3.
Weight deficit was observed in 21%, 12.5%, and 14.8%, respectively.

Lipid profile disorders were detected in 44 (71%) of 62 children. Dyslipidemia of varying severity was more
specific to patients with type I and III GSD. Higher levels of triglycerides were associated with type I GSD,