Metabolic disturbances occur more often in patients with Down Syndrome (DS, trisomy 21) than in the health population.
Superoxide dismutase (SOD-1), the main enzyme in the antioxidative system, is coded on chromosome 21. Disturbances in the antioxidative system may play a major role in the development of complications of CHD with arterio-venous shunt.
The aim was to evaluate the activity of SOD-1 and concentration of LPO in children with DS and CHD.
Sixty-two children with DS (mean 6.7 years) were divided for 3 groups:without CHD (DS-0, n=18/CG-0, n=13); CHD with insignificant arterio-venous shunt (DS-1, n=26/CG-1, n=26) and CHD with significant arterio-venous shunt ± pulmonary hypertension (HP) (DS-2, n=18 / CG-2, n=14).) As a control group were 53 healthy children (mean 10.4 years) as a control group (CG).
The activity of SOD-1 and concentration of LPO were evaluated.
The activity of SOD-1 was statistically higher in DS than in CG group, which is genetically determined, but only in children without CHD (DS-0 vs CG-0: 0.0441 U/mg protein vs 0.0319 U/mg protein, P< 0.05).
SOD-1 was higher in CG2 -children than in CG0- children, as well DS as CG. (CG-2 vs CG-0: 0.0608 vs 0.0319 and DS-2 vs DS-0: 0.0568 vs 0.0441; P<0.05).
In CG2 children the activity of SOD-1 was higher even in CG than in DS (CG-2 vs DS-2: 0.0608 vs 0.0568).
The level of LPO in all DS groups was lower than in CG groups (DS-0=1.57 nmol/ml vs CG-0=1.83; DS-1=1.46 vs CG-1=1.58; DS-2=1.41 vs CG-2=1.44).
Conclusions: The high activity of SOD-1 in children with CHD with arterio-venous shunt (as well DS as CG) indicates a disturbances in the antioxidative system in this children.
The higher activity of SOD-1 may play an important role in lipid peroxidation process, which express lower LPO concentrations.
03 - 07 May 2008
European Society of Endocrinology