Akademik Veri Yönetim Sistemi
ASIAN BIOMEDICINE, cilt.14, sa.2, ss.59-65, 2020 (SCI-Expanded, Scopus)
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.-aminobutyric acid (GABA; 0.96 +/- 1.07 mu mol/L) and glycine (305.1 +/- 105.19 mu mol/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 mu mol/L) and asparagine (39.3 +/- 16.89 mu mol/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. 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.