[Selenium in phenylketonuria patients. Effects of sodium selenite administration].
METHODS
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 micrograms 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.


CONCLUSIONS
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 micrograms Se/m2 BSA/d) initially, followed by a dosage between 30 and 60 micrograms Se/m2 BSA/d).
Kauf
E
E
Klinik für Kinder-und Jugendmedizin, Friedrich-Schiller-Universität, Jena.
Seidel
J
J
Winnefeld
K
K
Dawczynski
H
H
Häfer
R
R
Stein
F
F
Vogt
L
L
D000975:Antioxidants / Q000008:administration & dosage*; D002648:Child; D003131:Combined Modality Therapy; D018753:Diet, Protein-Restricted; D005260:Female; D005979:Glutathione Peroxidase / Q000097:blood; D006801:Humans; D008297:Male; D010661:Phenylketonurias / Q000188:drug therapy* / Q000201:enzymology; D012643:Selenium / Q000172:deficiency; D018038:Sodium Selenite / Q000008:administration & dosage*; D013963:Thyroid Hormones / Q000097:blood; D014131:Trace Elements / Q000097:blood; D016277:Ventricular Function, Left / Q000187:drug effects
D000975:Antioxidants; D013963:Thyroid Hormones; D014131:Trace Elements; D005979:Glutathione Peroxidase; D012643:Selenium; D018038:Sodium Selenite