Summary
□Patients and Method: 17 patients (8 female, 9 male; age 8.2±3.7 years) with phenylketonuria under phenylalanin restricted diet were investigated prior to and after 3 months of selenium substitution (sodium selenite, 115 µg Se/m2 BSA/d). Different parameters in blood were determined: selenium, glutathione peroxidase (Gpx) activity, thyroid hormones, blood cell count, lymphocytic antigen expression, muscle function and -enzymes, cardiac ultrasound.
□Results: The main significant results of selenium substitution are: increased plasma-selenium, blood cell selenium, plasma-Gpx activity and left ventricular cardiac index as well as decreased plasma thyroxin, free thyroxin, reverse triiodthyronin, total cholesterol, mean erythrocyte and thrombocyte volume and lymphocytic CD2 expression.
□Conclusion: The data indicate metabolic and functional signs of selenium deficiency in patients with phenylketonuria without selenium substitution. We conclude that, despite of lacking clinical symptoms, a selenium supply in phenylketonuria patients under diet is necessary and should be performed with usefull peroral sodium selenite (115 µg Se/m2 BSA/d) initially, followed by a dosage between 30 and 60 µg Se/m2 BSA/d).
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Kauf, E., Seidel, J., Winnefeld, K. et al. Selen bei Phenylketonuriepatienten. Med Klin 92 (Suppl 3), 31–34 (1997). https://doi.org/10.1007/BF03041959
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DOI: https://doi.org/10.1007/BF03041959