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
© 2020 Kazanasmaz Halil et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Background
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.
Objectives
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.
Methods
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).
Results
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
Conclusions
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.