Abstract
In Western Countries, the major sources of selenium (SE) are protein rich products such as meat, fish, eggs and bread. One could expect that in patients with phenylketonuria on a protein restricted diet, the SE intake will be dramatically reduced. To objectify this, we decided to measure the daily intake of 5 PKU children's, aged 7 to 19 year, during a PKU summer camp. Daily consumption was duplicated, homogenized and frozen. Se was measured with an atomic absorption spectrometry method after hydride generation. Results: Se intake from allowed natural foods and drinks ranged from 1.3 TO 10.7 μg/d. The synthetic diet products (Phenyldon, PKU2,PKU-Drink, low protein biscuits) provided < 0.5μg/d (detection limit). The mean total SE intake was 2.8±2.2 μg/d (mean±SD.N:35). The mean Se plasma level was 38±13.7 ng/ml (age matched control: 77±13ng /ml) and the glutathion peroxidase activity was 0.062±0.014 U/ml (n=0.301±0.019). These 5 patients with PKU, all symptom free, had a SE daily intake far below the recommended allowances ( 40-60μg/day).
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François, B., Van Caillie-Bertrand, M., Calomme, M. et al. 137 SELENIUM INTAKES IN PHENYLCETONURIC (PKU) CHILDREN ON PHENYLANINE RESTRICTED DIET. CORRELATION WITH BLOOD SELENIUM, PHENYLANINE AND CLUTATHION PEROXIDASE ACTIVITY. Pediatr Res 30, 651 (1991). https://doi.org/10.1203/00006450-199112000-00167
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DOI: https://doi.org/10.1203/00006450-199112000-00167
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