The selenium status was investigated in 87 patients of the German Collaborative Study of Phenylketonuria (PKU) (mean age 9.7 years). The selenium values and glutathione peroxidase activity in plasma and erythrocytes were negatively correlated to the quality of dietary management (mean plasma phenylalanine value). Despite a low selenium state, the children showed no clinical sign of deficiency and almost all biochemical parameters checked were normal. In the low selenium state thyroxine values are increased and decline during selenium supplementation, whereas tri-iodothyronine and thyroid stimulating hormone levels remain unchanged. The reduction in glutathione peroxidase activity in plasma was more pronounced than in the erythrocytes pointing to a different availability of both enzymes for selenium. In addition we estimated the selenium status in 29 women with PKU during pregnancy. In 32 healthy pregnant women we observed a decrease in plasma selenium values and the glutathione peroxidase activity in the third trimester, whereas the erythrocyte glutathione peroxidase activity remained stable. In contrast to the healthy women we found in the PKU group a steady decrease of all selenium parameters tested during the whole pregnancy. During the long-term low-dose selenium supplementation in PKU children the glutathione peroxidase activity of plasma and erythrocytes increased. They reached a similar plateau after the application of inorganic or organic selenium compounds. In contrast the selenium values of plasma and whole blood showed only a plateau after the application of sodium selenite. The supplementation with low doses of selenium in the form of selenomethionine increased the plasma and whole blood selenium values constantly within the first 9 months. Therefore selenomethionine supplementation cannot be recommended.
Eighty-seven participants of the German Collaboratory Study for Children with Phenylketonuria (PKU) presented low plasma, whole blood and hair selenium (Se) values, reduced urinary selenium excretion, and decreased plasma and erythrocyte glutathione peroxidase activity in comparison with a healthy reference group (all figures p < 0.001). Aspartate amino transferase and thyroxine (T4) concentrations in plasma were inversely correlated with the selenium blood values of the PKU children. Somatic measurements showed a negative standard deviation score of body height in the PKU children compared with reference values. Despite the different Se supply, the infants did not present any specific Se deficiency symptoms.
A simple, reproducible test was used to quantify muscle weakness in mdx mice, an animal model of Duchenne muscular dystrophy. The effect of bedding on wheat kernels and of dietary supplementation of alpha-tocopherol on the progression of muscle weakness was investigated in mdx mice. When measured during the first 200 d of life, mdx mice developed muscle weakness, irrespective of bedding and diet. When kept on wood shavings and fed a conventional rodent diet, mdx mice showed progressive muscle weakness over the consecutive 200 d, and eventually showed a significant weight loss during the next 200-d observation period. Progression of muscle weakness and weight loss were almost completely prevented in mdx mice that were kept on wheat kernel bedding. In contrast, only incomplete maintenance of muscle strength and body weight was observed in mdx mice kept on wood shavings and fed the alpha-tocopherol-supplemented diet. It is concluded from these experiments that a component of wheat kernels other than alpha-tocopherol is essential to prevent the progression of muscle weakness in mdx mice.
In 78 healthy term babies from Düsseldorf and surroundings spontaneous urine samples were collected on day 108 to day 144 of life and analyzed for the content of iodine and creatinine. 26 babies were breast fed. 23 babies received an iodized hypoallergenic formula and 26 babies different commercial formulas only partly iodized. From 4 parameters to estimate iodine supply of the babies (urinary iodine concentration microgram/dl, iodine/creatinine ratio microgram/g; estimated daily iodine excretion microgram/d, estimated daily iodine excretion corrected for body surface area microgram/dl 1.73 m2) estimated daily iodine excretion was the most useful parameter. Breast fed babies showed a significantly lower estimated daily iodine excretion (median 32 microgram/d) than babies fed the hypoallergenic (52 microgram/d) or a commercial formula (54 micrograms/d). In Germany with an insufficient iodine supply of pregnant and lactating mothers breast fed infants run a risk for an insufficient iodine supply. Furthermore, supplementation of infant formulas with iodine started in 1990/1991 proved to be a sufficient measure to correct iodine deficiency.
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