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Page 4 of 7 Strokova et al. Vessel Plus 2019;3:16 I http://dx.doi.org/10.20517/2574-1209.2019.08

Table 1. Lipid profile of children with GSD (n = 62)
Total Type I Type III Type VI and IX
GSD type
(n = 62) (n = 19) (n = 16) (n = 27)
Cholesterol, mmol/L 4.7 ± 0.2 4.9 ± 0.5 4.9 ± 0.3 4.4 ± 0.2
HDL cholesterol, mmol/L 0.6 ± 0.03 0.8 ± 0.06 0.8 ± 0.07 1.1 ± 0.1
LDL cholesterol, mmol/L 3.1 ± 0.1 2.8 ± 0.2 3.6 ± 0.3 0.9 ± 0.06
Triglycerides, mmol/L 2.2 ± 0.3 3.9 ± 0.8 2.05 ± 0.3 1.1 ± 0.1

while higher levels of LDL cholesterol were common to type III GSD (P < 0.05) [Table 1]. No changes in the
lipid profile were observed in 18 (29%): one with type I, 4 with type III, and 13 with types VI-IX.

Hypertriglyceridemia and a decrease in the level of HDL cholesterol were most frequently detected
[Figures 1A and B]. Hypertriglyceridemia was specific to type I and III GSD. At the same time, low HDL
cholesterol levels were more common in patients with type I GSD than in patients with type III or type
VI-IX GSD.

Hypercholesterolemia was diagnosed in more than one-third of patients with type I and type III GSD, and
more than one-third of patients with type III demonstrated high levels of LDL cholesterol [Figure 1C].

Thus, hypercholesterolemia, hypertriglyceridemia and low HDL cholesterol are common to type I GSD,
while high levels of LDL cholesterol, hypertriglyceridemia and low HDL cholesterol are typical for type
III GSD. For type VI and IX GSD, lipid metabolism disorders were observed less frequently, and the most
common changes were a decrease in HDL cholesterol.

DISCUSSION
Lipid metabolism disorders, including hypercholesterolemia, hypertriglyceridemia, and decreased levels of
HDL cholesterol were detected in 71% of children with GSD. They were mainly observed in patients with
types I and III of the disease, in which hypertriglyceridemia often occurred. More than one-third of patients
with type I and type III GSD were diagnosed with hypertriglyceridemia, and an equal number of patients
with type III GSD had elevated levels of LDL cholesterol. Patients with GSD VI-IX types performed lower
rates of lipid disorders.

The elevated levels of total cholesterol and LDL cholesterol are closely associated with the early progression
of atherosclerosis and an increased cardiovascular risk in the general population. At the same time, there is
still no clear position on hypertriglyceridemia as a risk factor for cardiovascular diseases .
[24]
According to some studies hypertriglyceridemia can be associated with atherogenesis, firstly, as a marker of
some metabolic disorders, secondly, because of the vascular wall macrophages infiltration due to oxidised
triglyceride-rich lipoproteins receptor activation . There is evidence that elevated triglycerides lead to
[11]
hypercoagulation through stimulation of a plasminogen tissue activator inhibitor, an increase in the content
of VII coagulation factor, and the activation of the transition of prothrombin to thrombin . The Hokanson
[24]
and Austin meta-analysis of prospective studies established that triglyceride levels of up to 5 mmol/L (450 mg/dL)
predicted the risk of coronary heart disease (CHD), especially in women. Thus, in the Framingham study,
[25]
the risk of CHD positively correlated with triglycerides levels .
According to the Copenhagen Male Study covering 2,906 men aged 52-74 years without CHD, the first
myocardial infarction occurred in 229 of them in 8 years of follow-up and the risk of developing CHD
[26]
increased as the initial triglyceride levels increased . A meta-analysis of clinical trials with a total of 46,413
men and 10,864 women examined by Hokanson and Austin showed that triglycerides are an independent
[26]
risk factor for CHD even after adjusting for HDL cholesterol values .