Evidence that L-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patients.

It is well established that the involvement of reactive species in the pathophysiology of several neurological diseases, including phenylketonuria (PKU), a metabolic genetic disorder biochemically characterized by elevated levels of phenylalanine (Phe). In previous studies, we verified that PKU patients (treated with a protein-restricted diet supplemented with a special formula not containing L-carnitine and selenium) presented high lipid and protein oxidative damage as well as a reduction of antioxidants when compared to the healthy individuals. Our goal in the present study was to evaluate the effect of Phe-restricted diet supplemented with L-carnitine and selenium, two well-known antioxidant compounds, on oxidative damage in PKU patients. We investigated various oxidative stress parameters in blood of 18 treated PKU patients before and after 6 months of supplementation with a special formula containing L-carnitine and selenium. It was verified that treatment with L-carnitine and selenium was capable of reverting the lipid peroxidation, measured by thiobarbituric acid-reactive species, and the protein oxidative damage, measured by sulfhydryl oxidation, to the levels of controls. Additionally, the reduced activity of glutathione peroxidase was normalized by the antioxidant supplementation. It was also verified a significant inverse correlation between lipid peroxidation and L-carnitine blood levels as well as a significant positive correlation between glutathione peroxidase activity and blood selenium concentration. In conclusion, our results suggest that supplementation of L-carnitine and selenium is important for PKU patients since it could help to correct the oxidative stress process which possibly contributes, at least in part, to the neurological symptoms found in phenylketonuric patients.

Influence of lactic acid bacteria on survival of Escherichia coli O157:H7 in inoculated Minas cheese during storage at 8.5 degrees C.

Minas cheese is a typical Brazilian fresh cheese, manufactured by addition of rennin and CaCl2 to milk, followed by draining the curd. The intrinsic characteristics of this product make it favorable for growth of pathogens, including Escherichia coli O157:H7. The influence of the addition of a commercial mesophilic type O lactic culture to this product on the growth of this pathogen during storage at 8.5 degrees C was evaluated. Eight different formulations of Minas cheese were manufactured using raw or pasteurized milk and with or without salt and lactic culture. Individual portions of each formulation were transferred to sterile plastic bags and inoculated with E. coli O157:H7 to yield ca. 10(3) or 10(6) CFU/g. After blending by hand massaging the bags, samples were stored at 8.5 degrees C for up to 14 days. E. coli O157:H7 was counted after 1, 2, 7, and 14 days of storage using 3M Petrifilm Test Kit-HEC. Counts in samples without added lactic culture showed a 2-log increase in the first 24 h and remained constant during the following 14 days. Counts in samples with added lactic culture showed a 0.5-log increase in the first 24 h, followed by a decrease. These variations were statistically significant (P < 0.05). No significant variations (P > 0.05) were obtained for cheese samples manufactured with pasteurized or raw milk, with or without salt. Results indicate that the addition of type O lactic culture may be an additional safeguard to well-established good manufacturing practices and hazard analysis and critical control point programs in the control of growth of E. coli O157:H7 in Minas cheese.