title: Controlled diet in phenylketonuria and hyperphenylalaninemia may cause serum selenium deficiency in adult patients: the Czech experience.
abstract: 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.
authors.affiliation: Department of Pediatrics, University Hospital Brno, Medical Faculty of Masaryk University Brno, Cernopolni 9, Brno 62500, Czech Republic. prochazkovad@fnbrno.cz
authors.forename: Dagmar
authors.initials: D
authors.lastname: Procházková
authors.forename: Jiří
authors.initials: J
authors.lastname: Jarkovský
authors.forename: Hana
authors.initials: H
authors.lastname: Vinohradská
authors.forename: Petra
authors.initials: P
authors.lastname: Konečná
authors.forename: Lucie
authors.initials: L
authors.lastname: Machačová
authors.forename: Zdeněk
authors.initials: Z
authors.lastname: Doležel
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018153:Czech Republic; D018753:Diet, Protein-Restricted / Q000009:adverse effects*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010661:Phenylketonurias / Q000097:blood / Q000178:diet therapy; D011446:Prospective Studies; D012643:Selenium / Q000097:blood / Q000172:deficiency
chemical_list: D012643:Selenium