137 SELENIUM INTAKES IN PHENYLCETONURIC |[lpar]|PKU|[rpar]| CHILDREN ON PHENYLANINE RESTRICTED DIET|[period]| CORRELATION WITH BLOOD SELENIUM, PHENYLANINE AND CLUTATHION PEROXIDASE ACTIVITY

摘要

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).