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

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  • Additional Information
    • Transliterated Title:
      Selen bei Phenylketonuriepatienten. Effekte einer Natriumselenitsubstitution.
    • Source:
      Publisher: Urban & Vogel Country of Publication: Germany NLM ID: 8303501 Publication Model: Print Cited Medium: Print ISSN: 0723-5003 (Print) Linking ISSN: 07235003 NLM ISO Abbreviation: Med Klin (Munich) Subsets: MEDLINE
    • Publication Information:
      Publication: Heidelberg : Urban & Vogel
      Original Publication: München [Germany] : Urban & Schwarzenberg, [c1983-
    • Subject Terms:
      Antioxidants/*administration & dosage ; Phenylketonurias/*drug therapy ; Sodium Selenite/*administration & dosage; Child ; Combined Modality Therapy ; Diet, Protein-Restricted ; Female ; Glutathione Peroxidase/blood ; Humans ; Male ; Phenylketonurias/enzymology ; Selenium/deficiency ; Thyroid Hormones/blood ; Trace Elements/blood ; Ventricular Function, Left/drug effects
    • Abstract:
      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 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.
      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 micrograms Se/m2 BSA/d) initially, followed by a dosage between 30 and 60 micrograms Se/m2 BSA/d).
    • References:
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      Nutrition. 1991 May-Jun;7(3):215-21. (PMID: 1802210)
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    • Accession Number:
      0 (Antioxidants)
      0 (Thyroid Hormones)
      0 (Trace Elements)
      EC 1.11.1.9 (Glutathione Peroxidase)
      H6241UJ22B (Selenium)
      HIW548RQ3W (Sodium Selenite)
    • Publication Date:
      Date Created: 19980107 Date Completed: 19971224 Latest Revision: 20191111
    • Publication Date:
      20231215
    • Accession Number:
      10.1007/BF03041959
    • Accession Number:
      9417495