THE DISTURBANCES IN IRON TRANSPORT AND STORES AND TOTAL FREE RADICAL TRAPPING ABILITY OF BLOOD PLASMA IN BABIES WITH MITOCHONDRIAL ENCEPHALO MYOPATHIES

Babies with mitochondrial encephalomyopathies had higher ferritin levels than controls. Although plasma iron levels were simi lar in both groups, babies with mitochondrial encephalomyopathies had lower transferrin levels. Thiobarbituric acid reactive substances in plasma of babies with mitochondrial encephalomyopathies were higher thun in controls suggesting increased lipid oxidation. We studied the total radical trapping capacity of the antioxidants in plasma (TRAP) and compared the TRAP level in children with mitochondrial encephalomyopathies with that in healthy controls. I n addition, the concentrations of various known antioxidants were measured and the theoretical contribution of these antioxidants to the TRAP calculated. The measured and calculated TRAP were higher in the control group than the group of children with mitochondrial encephalomyopathies. Uric acid, vitamins E and C and sulphides concentrations were lower in children with mitochondrial encephalomyopathies compared with controls. The main conclusion from this work is that forchildren with mitochondrial encephalomyopathies, plasma contents of vitamin E, vitamin C, and cysteine-rich proteins which are all essential nutrients are too low for optimal activity of antioxidant systems.