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Table 1 Relevant biochemical and MRS investigations at baseline and after four weeks glutamine supplementation

From: Glutamine supplementation in a child with inherited GS deficiency improves the clinical status and partially corrects the peripheral and central amino acid imbalance

Parameter Baseline End of trial Reference range
Blood    
Ammonia (μmol/L) 66 76 12-48
Glutamine (μmol/L) 74 731 457–857
Glutamate (μmol/L) 58 28 17-69
CSF    
Ammonia (μmol/L) 71 75 12-48
Glutamine (μmol/L) 121 201 333–658
Glutamate (μmol/L) <2.5 <2.5 0–5
Homovanillic acid (nmol/L) 171 188 211–871
Creatine (μmol/L) 84 56 17–87
Free GABA (μmol/L) 0.030 0.037 0.032-0.167
Total GABA (μmol/L) 8.3 7.47 3.3-12.2
Basal ganglia MRS    Reference values from age-matched comparison subjects (n = 3)
NAA (IU) 4.2 4.6 8.3-8.8
Creatine + phosphocreatine (IU) 12.0 10.5 8.4-8.6
Choline (IU) 3.6 2.9 1.7-2.0
Myo-inositol (IU) 6.7 6.4 3.0-4.0
Glutamate (IU) 4.6 4.9 7.3-10.3
Glutamine (IU) 0 1.4 3.1-6.3
GABA (IU) 3.0 2.6 2.4-3.7
  1. Pathological values in bold; GABA: γ-aminobutyric acid; NAA: N-acetylaspartate.