Antioxidant and thyroid hormone status in selenium-deficient phenylketonuric and hyperphenylalaninemic patients

Bakel, M. M. E. van; Printzen, Gert; Wermuth, Bendicht; Wiesmann, Ulrich

doi:10.1093/ajcn/72.4.976

Cited by 87 publications

(63 citation statements)

References 21 publications

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“…One of them is glutathione metabolism, which is influenced by the presence of free radicals. Van Bakel et al (2000) showed in PKU and HPA patients that selenium in their plasma correlated well with their erythrocyte glutathione peroxidase and the antioxidant status was low in both groups of patients. Also, the authors reported that their a-tocopherol, albumin and uric acid levels were not different as compared with controls and recommended selenium supplementation in their diet.…”

Section: Discussionmentioning

confidence: 95%

Effect of diet on plasma total antioxidant status in phenylketonuric patients

et al. 2003

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Background: Phenylketonuria (PKU), an inborn error of phenylalanine (Phe) metabolism, is treated with a low Phe lifelong diet, which is a vegetarian and contains many antioxidants. Aim: The aim of this study was to evaluate the effect of diet on plasma total antioxidant status in our PKU patients. Methods, Results: Twenty-two PKU patients on strict diet (group A), 24 PKU patients who did not accurately follow their dietary control (group B) and 40 healthy children (controls) of comparable age took part in this study. Nutrients, as well as blood levels of lipids, vitamin C, b-carotene and a-tocopherol were evaluated in all groups. Vitamin C intake and its blood levels did not differ between the groups. However, the intake of b-carotene, a-tocopherol (2211 AE 116, 14 AE 1.0 mg=24 h) and their blood levels (0.7 AE 0.09, 34 AE 0.9 mmol=l) in group A were statistically significantly higher (P < 0.001) as compared with those of group B (1352 AE 118, 10 AE 1.0 mg=24 h and 0.49 AE 0.08, 22 AE 0.6 mmol=l) and controls (1290 AE 120, 9.0 AE 0.9 mg=24 h and 0.40 AE 0.09, 24 AE 1.6 mmol=l). Lipid intakes and their blood levels were lower in patients on the strict diet. Plasma total antioxidant status was higher in the same group of patients (group A). Additionally, positive correlations were found between the antioxidant vitamin blood levels and the plasma total antioxidant status in the groups, especially in the group A. PKU patients of group A showed significantly higher antioxidant status (1.6 AE 0.2 mmol=l) as compared with those of group B (1.0 AE 0.19 mmol=l; P < 0.001) and controls (1.01 AE 0.2 mmol=l). Conclusions: The high plasma antioxidant status in patients with PKU, especially in those with a good compliance with their diet, is possibly due to the amounts of antioxidants which are present in their special low Phe vegetarian diet.

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Section: Discussionmentioning

confidence: 95%

Effect of diet on plasma total antioxidant status in phenylketonuric patients

et al. 2003

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“…The natural protein restricted diet of PKU patients may result in some essential nutrient deficiencies; ie selenium (Van Bakel et al, 2000) or cobalamin (Hanley et al, 1996). To prevent these deficiencies, supplementation with several of these micronutrients has been added to the special formulas.…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, the normal glutathione values observed in our PKU patients would further support the beneficial effects of low tHcy and cysteine concentrations. However, this hypothesis should be considered with caution because other factors present in PKU patients may increase oxidative stress, such as selenium deficiency in patients with poor dietary adherence (Van Bakel et al, 2000), or the decreased ubiquinone-10 values observed in serum (Artuch et al, 1999) and cells (Colomé et al, 2002).…”

Section: Discussionmentioning

confidence: 99%

“…In PKU, deficiencies in two important antioxidants, selenium (Van Bakel et al, 2000) and coenzyme Q10 (Artuch et al, 1999), have been reported. These deficiencies were mainly related to poor dietary compliance, and might cause an increase in peroxidative damage manifested in high malondialdehyde (MDA, a product of lipid peroxidation) values, low reduced glutathione concentrations and/or low normal tocopherol concentrations.…”

Section: Introductionmentioning

confidence: 99%

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Plasma thiols and their determinants in phenylketonuria

et al. 2003

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Objective: Treatment of phenylketonuria (PKU) patients consists of a phenylalanine-restricted diet supplemented with a tyrosine-, vitamin-and oligoelement-enriched amino-acid mixture. Vitamins and oligoelements may be deficient when compliance with the supplemented special formula is poor. Plasma thiol concentrations (especially homocysteine) depend mainly on B-vitamin intake. Our aim was to evaluate the plasma thiol concentrations (homocysteine, cysteine and glutathione) and their determinants (methionine, cobalamin and folate) in PKU patients under dietary treatment compared with agematched controls. Design and setting: Cross-sectional study performed in a tertiary care Hospital. Subjects: PKU (42) patients under dietary treatment compared with 42 age-matched controls. Interventions: Plasma total homocysteine, cysteine and glutathione were analyzed by HPLC with fluorescence detection. Plasma phenylalanine and methionine were analyzed by ion exchange chromatography. Serum folate and cobalamin were analyzed by radioimmunoassay procedures. Results: Total homocysteine concentrations were significantly lower in the PKU patients compared with the control group (Students t-test; Po0.0001). Serum folate and cobalamin were significantly higher in the PKU group (t-Student; Po0.0001) compared with controls. A significantly negative correlation was observed between total homocysteine and folate (r ¼ À0.378; P ¼ 0.016), and between cobalamin and phenylalanine concentrations (r ¼ À0.367; P ¼ 0.022) in the PKU group. Conclusions: Plasma total homocysteine values are lower in the PKU group than in the controls, probably because of high folate values. High phenylalanine values, an indicator of poor dietary compliance, are negatively associated with cobalamin, which might be deficient in some cases.

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“…However, the Phe-restricted diet is a considerable risk factor for nutritional deficiencies and requires close supervision to prevent these. Patients may be at risk of mineral and trace element deficiencies such as zinc (Zn), selenium (Se) or iron (Fe) [2,3]. Clinical consequences include anaemia and growth retardation [4].…”

Section: Introductionmentioning

confidence: 99%

Evaluation of Plasma Trace Element and Mineral Status in Children and Adolescents with Phenylketonuria Using Data from Inductively-Coupled-Plasma Atomic Emission and Mass Spectrometric Analysis

Knerr

Blessing²,

Seyferth

et al. 2013

Ann Nutr Metab

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Background: Phenylketonuria (PKU) is caused by a severe phenylalanine hydroxylase deficiency; the mainstay of treatment is a low-phenylalanine diet. A diet which is so restrictive is associated with a risk of nutritional deficiencies. We investigated plasma concentrations for 46 elements, including minerals and trace elements. Methods: We enrolled 20 children and adolescents with PKU and 20 matched controls. Multi-elementary quantification was carried out by solution-based inductively coupled plasma atomic emission spectroscopy (ICP-AES) and ICP mass spectrometry (ICP-MS). Results: With the exception of manganese and aluminium, no significant differences were found for element levels between PKU patients and controls. As a trend, manganese levels were lower in PKU patients than in control subjects (p < 0.05) but were within the reference range. There was a positive linear relationship between manganese and tyrosine levels in subjects with PKU (r² = 0.2295, p < 0.05). If detectable, potentially toxic elements were only identified in ultra-trace quantities in plasma samples of either group; aluminium levels were found to be slightly higher in PKU subjects than in controls (p < 0.01). Conclusion: The combination of ICP-AES and ICP-MS data is a useful diagnostic tool for element quantification at a high analytical rate and for monitoring bio-element status, e.g. in patients on a restrictive diet.

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Antioxidant and thyroid hormone status in selenium-deficient phenylketonuric and hyperphenylalaninemic patients

Abstract: Supplementation with selenium seems to be advisable for patients consuming diets low in natural protein.

Cited by 87 publications

References 21 publications

Effect of diet on plasma total antioxidant status in phenylketonuric patients

Effect of diet on plasma total antioxidant status in phenylketonuric patients

Plasma thiols and their determinants in phenylketonuria

Evaluation of Plasma Trace Element and Mineral Status in Children and Adolescents with Phenylketonuria Using Data from Inductively-Coupled-Plasma Atomic Emission and Mass Spectrometric Analysis

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