Determination of prealbumin and selenium in serum for monitoring the nutrition status of phenylketonuric and hyperphenylalaninemic patients
Authors:
D. Procházková 1; L. Kolbová 1; J. Jarkovský 2; H. Vinohradská 3; P. Konečná 1; Z. Doležel 1
Authors‘ workplace:
Pediatrická klinika, Lékařská fakulta Masarykovy univerzity Brno a Fakultní nemocnice Brno, přednosta prof. MUDr. Z. Doležel, CSc.
1; Institut biostatistiky a analýz, Lékařská a Přírodovědecká fakulta Masarykovy univerzity, Brno, vedoucí doc. RNDr. L. Dušek, Dr.
2; Oddělení klinické biochemie, Fakultní nemocnice Brno, prim. doc. MUDr. M. Dastych, CSc., MBA
3
Published in:
Čes-slov Pediat 2012; 67 (3): 170-177.
Category:
Original Papers
Overview
Objectives:
Phenylketonuria is an inherited disorder of metabolism of the amino acid phenylalanine caused by a deficit of the enzyme phenylalaninhydroxylase. It is treated with a low-protein diet containing a low content of phenylalanine to prevent mental affection of the patient. It is a highly restrictive therapeutic diet using a mixture of amino acids without phenylalanine (AAM) in combination with low-protein products and low amount of natural proteins. 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 and selenium to discover the potential correlation between the amount of proteins in food and their metabolic control.
Material and methods:
The experimental group contained altogether 174 patients treated at the Department of Pediatrics of Medical Faculty of Masaryk University and University Hospital in Brno, 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. All PKU patients given a low-protein diet took AAM. HPA patients given a low-protein diet were divided into two groups; in the first group AAM was prescribed and in the other it was not.
Results:
We did not prove a statistically significant difference in the concentration of serum pre-albumin among the respective groups. We approved statistically significant difference in serum selenium concentrations of adult PKU and HPA patients (p=0.006; Mann-Whitney U test).
Conclusion:
The preparation of therapeutic restrictive diet for PKU and HPA patients is very difficult, and can cause nutritional deficiency. Patients’ adherence to the diet is not always good. To attend to adherence of the therapeutic restrictive diet and search new ways of treatment and monitoring of the nutrition status of phenylketonuric and hyperphenylalaninemic patients at the same time is necessarily.
Key words:
hyperphenylalaninemia, phenylketonuria, pre-albumin, selenium
Sources
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Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2012 Issue 3
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