<abstract xmlns="http://www.w3.org/1999/xhtml"><sec><h3>Background</h3><p>In patients with phenylketonuria, the central nervous system is adversely affected by noncompliance with diet. The levels of phenylalanine and many different amino acids (AAs) in the plasma of patients with phenylketonuria can be measured simultaneously.</p></sec><sec><h3>Objectives</h3><p>To measure the blood plasma levels of neurotransmitter AAs in a cohort of patients in Sanliurfa province, Turkey, with phenylketonuria for use as a support parameter for the follow-up of patients.</p></sec><sec><h3>Methods</h3><p>The phenylketonurics that we followed (n = 100) were divided into 2 groups according to their compliance with their dietary treatment. Plasma AA analysis results of phenylketonurics were compared with those of healthy children in a control group (n = 50).</p></sec><sec><h3>Results</h3><p>In the diet incompliant group (n = 56), the mean levels of γ-aminobutyric acid (GABA; 0.96 ± 1.07 μmol/L) and glycine (305.1 ± 105.19 μmol/L) were significantly higher than those in the diet compliant group (n = 44; GABA <italic>P</italic> = 0.005, glycine <italic>P</italic> &lt; 0.001) and in the control group (GABA and glycine <italic>P</italic> &lt; 0.001), whereas the mean levels of glutamic acid (39.01 ± 22.94 μmol/L) and asparagine (39.3 ± 16.89 μmol/L) were lower (<italic>P</italic> &lt; 0.001) in the diet incompliant group. A positive correlation was observed between the levels of phenylalanine and GABA and glycine. A negative relationship was found between the levels of phenylalanine and glutamic acid and asparagine.</p></sec><sec><h3>Conclusions</h3><p>A relationship exists between the levels of plasma phenylalanine in a cohort of phenylketonurics in Sanliurfa province, Turkey, and the levels of some excitatory and inhibitory AAs. Excitatory and inhibitory AA levels in plasma may be used as support parameters in the follow-up of patients with phenylketonuria.</p></sec></abstract>

BackgroundIn patients with phenylketonuria, the central nervous system is adversely affected by noncompliance with diet. The levels of phenylalanine and many different amino acids (AAs) in the plasma of patients with phenylketonuria can be measured simultaneously.ObjectivesTo measure the blood plasma levels of neurotransmitter AAs in a cohort of patients in Sanliurfa province, Turkey, with phenylketonuria for use as a support parameter for the follow-up of patients.MethodsThe phenylketonurics that we followed (n = 100) were divided into 2 groups according to their compliance with their dietary treatment. Plasma AA analysis results of phenylketonurics were compared with those of healthy children in a control group (n = 50).ResultsIn the diet incompliant group (n = 56), the mean levels of γ-aminobutyric acid (GABA; 0.96 ± 1.07 μmol/L) and glycine (305.1 ± 105.19 μmol/L) were significantly higher than those in the diet compliant group (n = 44; GABA P = 0.005, glycine P < 0.001) and in the control group (GABA and glycine P < 0.001), whereas the mean levels of glutamic acid (39.01 ± 22.94 μmol/L) and asparagine (39.3 ± 16.89 μmol/L) were lower (P < 0.001) in the diet incompliant group. A positive correlation was observed between the levels of phenylalanine and GABA and glycine. A negative relationship was found between the levels of phenylalanine and glutamic acid and asparagine.ConclusionsA relationship exists between the levels of plasma phenylalanine in a cohort of phenylketonurics in Sanliurfa province, Turkey, and the levels of some excitatory and inhibitory AAs. Excitatory and inhibitory AA levels in plasma may be used as support parameters in the follow-up of patients with phenylketonuria.

Plasma amino acid levels in a cohort of patients in Turkey with classical phenylketonuria

Department of Pediatrics, Faculty of Medicine

Department of Pediatric Metabolism Disorders, Faculty of Medicine

Asian Biomedicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

BackgroundIn patients with phenylketonuria, the central nervous system is adversely affected by noncompliance with diet. The levels of phenylalanine and many...

Plasma amino acid levels in a cohort of patients in Turkey with classical phenylketonuria

Article Category: Original article

Published Online: Aug 04, 2020

Page range: 59 - 65

DOI: https://doi.org/10.1515/abm-2020-0009

Keywords
amino acids, chromatography, diet therapy, neurotransmitter agents, phenylketonuria

© 2020 Kazanasmaz Halil et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Plasma amino acid levels in a cohort of patients in Turkey with classical phenylketonuria

Article Category: Original article

Published Online: Aug 04, 2020

Page range: 59 - 65

DOI: https://doi.org/10.1515/abm-2020-0009

Keywordsamino acids, chromatography, diet therapy, neurotransmitter agents, phenylketonuria

© 2020 Kazanasmaz Halil et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Keywords
amino acids, chromatography, diet therapy, neurotransmitter agents, phenylketonuria