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@proceedings{1068272,
author = {Procházková, Dagmar and Kolbová, Lucie and Jarkovský, Jiří and Vinohradská, Hana and Konečná, Petra and Doležel, Zdeněk},
booktitle = {Archives diseases in childhood, 97, suppl. 2, s. 1-58, 2012},
keywords = {phenylketonupria hyperphenylalaninaemia pre-albumin trace elements},
language = {eng},
title = {Detemination of prealbumin, selenium, zinc and iron concentrations in serum for monitoring the nutrition status of phenylketonuric and hyperphenylalaninemic patients},
url = {http://adc.bmj.com},
year = {2012}
}
TY - CONF
ID - 1068272
AU - Procházková, Dagmar - Kolbová, Lucie - Jarkovský, Jiří - Vinohradská, Hana - Konečná, Petra - Doležel, Zdeněk
PY - 2012
TI - Detemination of prealbumin, selenium, zinc and iron concentrations in serum for monitoring the nutrition status of phenylketonuric and hyperphenylalaninemic patients
KW - phenylketonupria hyperphenylalaninaemia pre-albumin trace elements
UR - http://adc.bmj.com
N2 - Background and Aims: Phenylketonuria is an inherited disorder of metabolism of the amino acid phenylalanine (Phe) 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. 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. Methods: The prospective study contained altogether 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. All PKU patients given a low-protein diet took phenylalanine-free amino acid mixtures (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. All probands used AAM from company Nutricia a.s., Czech Republic. For children at age from 1 to 8 years we used classical phenylalanine-free amino acid mixtures, with no flavouring PAM-2, Milupa PKU 2 prima and Milupa PKU 2 mix, which were divided into 3-5 daily doses. One daily dose could be a dosed form of a flavoured powdered preparation, i.e. PKU Anamix Pinneaple/Vanilla or PKU Anamix Chocolate. For patients over age of 8 years classical amino acid mixtures with no flavouring – Milupa PKU 2 secunda, Milupa PKU 3 advanta or P-AM 3 were used in 3-5 daily doses. For patients over age of 9 years one daily dose could be replaced by a dosed form of a flavoured powdered preparation, e.g. PKU Lophlex - orange or by ready to drink formulas, e.g. PKU Lophlex LQ – orange. All the above listed food for special medical purposes is enriched with vitamins and trace elements. HPA probands without AAM prescription limited whole eggs and meat in food. Each of probands kept a therapeutically restrictive diet by the individual dietary schedule worked out by a dietician. Individual compliance with the diet was estimated from regular laboratory test of blood Phe levels and from food records. Results: We did not prove a statistically significant difference in the concentration of serum pre-albumin, zinc and iron 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). Conclusions: The therapeutic restrictive diet for PKU and HPA makes the patient liable to the risk of nutritional deficit. The concentration of serum selenium could be used as indirect marker in evaluation of the risk of late complications of dietary therapy.
ER -
PROCHÁZKOVÁ, Dagmar, Lucie KOLBOVÁ, Jiří JARKOVSKÝ, Hana VINOHRADSKÁ, Petra KONEČNÁ and Zdeněk DOLEŽEL. Detemination of prealbumin, selenium, zinc and iron concentrations in serum for monitoring the nutrition status of phenylketonuric and hyperphenylalaninemic patients. In \textit{Archives diseases in childhood, 97, suppl. 2, s. 1-58, 2012}. 2012. ISSN~1468-2044.
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