Publication details

Controlled diet in phenylketonuria and hyperphenylalaninemia may cause serum selenium deficiency in adult patients: the Czech experience

Authors

PROCHÁZKOVÁ Dagmar JARKOVSKÝ Jiří VINOHRADSKÁ Hana KONEČNÁ Petra MACHAČOVÁ Lucie DOLEŽEL Zdeněk

Year of publication 2013
Type Article in Periodical
Magazine / Source Biological Trace Element Research
MU Faculty or unit

Faculty of Medicine

Citation
Web http://www.springer.com
Doi http://dx.doi.org/10.1007/s12011-013-9724-6
Field Paediatry
Keywords hyperphenylalaninemia; phenylketonuria; pre-albumin; selenium; zinc; iron
Attached files
Description Phenylketonuria is an inherited disorder of metabolism of the amino acid phenylalanine caused by a deficit of the enzyme phenylalanine hydroxylase. It is treated with a low-protein diet containing a low content of phenylalanine to prevent mental affection of the patient. Because of the restricted intake of high-biologic-value protein, patients with phenylketonuria may have lower than normal serum concentrations of pre-albumin, selenium, zinc and iron. The objective of the present study was to assess the compliance of our phenylketonuric (PKU) and hyperphenylalaninemic (HPA) patients; to determine the concentration of serum pre-albumin, selenium, zinc and iron to discover the potential correlation between the amount of proteins in food and their metabolic control. We studied 174 patients of which 113 were children (age 1-18), 60 with PKU and 53 with HPA and 61 were adults (age 18-42), 51 with PKU and 10 with HPA. We did not prove a statistically significant difference in the concentration of serum pre-albumin, zinc and iron among the respective groups. We proved statistically significant difference in serum selenium concentrations of adult PKU and HPA patients (p=0.006; Mann-Whitney U test). These results suggest that controlled low-protein diet in phenylketonuria and hyperphenylalaninemia may cause serum selenium deficiency in adult patients.

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