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In a study involving 18 treated phenylketonuria (PKU) patients, supplementation with a combination of L-carnitine and selenium for a period of 6 months, in addition to adherence to a Phe-restricted diet, was found to revert lipid peroxidation (measured by thiobarbituric acid-reactive species), and protein oxidative damage (measured by sulfhydryl oxidation), thereby helping "to correct the oxidative stress process which possibly contributes, at least in part, to the neurological symptoms found in phenylketonuric patients." [ABSTRACT FROM AUTHOR]

In a study involving 36 children with phenylketonuria (who generally adhere to a diet low in protein), between the ages of 1 and 11 years, supplementation with encapsulated fish oil (providing 15 mg/kg body weight DHA per day) for a period of 3 months was found to significantly increase visual evoked potential latencies, suggesting more rapid central nervous system information processing, and significantly improve motor function outcomes and coordination - as compared to healthy controls. All of the PKU subjects at baseline had a good supply of ALA (alpha-linolenic acid, an omega-3 precursor), suggesting that having sufficient ALA does not lead to amounts of DHA needed to support optimal function, leading the authors to conclude that, "DHA appears to be a conditional essential substrate for children with PKU." Furthermore, the authors state, "Because early treated PKU children are healthy, with normal fatty acid turnover, these dat a may indicate a need to supply some DHA to children in general. Further studies are ongoing aiming at establishing quantitative DHA requirements in children." [ABSTRACT FROM AUTHOR]

In a study involving patients with phenylketonuria (PKU) - an inborn error of phenylalanine (Phe) metabolism - low serum levels of L-carnitine were found in patients strictly adhering to a diet designed for patients with PKU which involves severe restriction of protein-enriched foods. The study compared levels of L-carnitine in patients who strictly adhered to the protein-restricted diet and patients who had poor adherence to the protein-restricted diet. Patients who strictly adhered to the diet had significantly lower serum levels of L-carnitine, compared to patients with poor compliance with the diet and as compared to healthy controls. In addition, all of the subjects with PKU, regardless of levels of adherence to the diet, were found to have significantly higher levels of TBARS (an indicator of oxidative stress) and significantly lower levels of total antioxidant reactivity (TAR). In patients with PKU, L-carnitine levels were found to b e inversely associated with TBARS and positively associated with TAR. These results suggest that measurement of L-carnitine levels in patients with PKU should become standard practice, and furthermore, supplementation with L-carnitine in patients with PKU who have low levels of L-carnitine may be needed. [ABSTRACT FROM AUTHOR]

In a study involving 36 early and continuously treated patients with phenylketonuria (PKU) and 29 controls, patients with PKU had significantly lower levels of selenium, coenzyme Q10, and catalase, and significantly higher levels of malondialdehyde, as compared with controls. Cognitive function was assessed as well. Low levels of selenium were associated with lower performance on tests of cognitive function (more omission errors, fluctuating attention, inconsistency of response times, and progressively slower reaction time) among PKU patients. The results of this study suggest that, "...it is important not only to control blood Phe levels in PKU but also other nutritional components such as selenium." [ABSTRACT FROM AUTHOR]