Endocrine Abstracts (2008) 16 P596

The superoxide dismutase and lipid peroxide in children with Down syndrome and congenital heart disease

Elzbieta Wojcik, Ewa Barg, Malgorzata Gromkowska, Ewa Maslowska, Beata Wikiera, Danuta Kalita, Elzbieta Gamian, Barbara Turczyn & Anna Skoczynska

Wroclaw Medical University, Wroclaw, Poland.

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.

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