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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.