Table 2 Serum concentrations of amino acids, free carnitine, acyl-carnitine obtained from patients and the respective reference ranges, as well as the correlations of their concentrations with blood ammonia concentrations
constituent | Unit | Total (n = 37) | correlation coefficient | ||||
|---|---|---|---|---|---|---|---|
Reference range | Mean ± SD | Patients exhibiting below the lower limits of the reference range (%) | Patients exhibiting values of 1.3-times or greater than the upper limits of the reference range (%) | r value | p value | ||
Free Carnitine | μmol/L | 36-74 | 33 ± 13 | 41 | 0 | 0.02 | 0.91 |
Acyl-Carnitine | μmol/L | 6-23 | 8 ± 4 | 38 | 0 | −0.24 | 0.15 |
1-Methyl-histidine | nmol/mL | <9.1 | 5 ± 5 | 0 | 8 | −0.09 | 0.66 |
3-Methyl-histidine | nmol/ml | TRA-8.2 | 4 ± 2 | 0 | 5 | −0.30 | 0.08 |
Alanine | nmol/mL | 258.8-615.2 | 458 ± 130 | 3 | 8 | 0.24 | 0.15 |
Anserine | nmol/ml | ND | ND | 0 | 0 | - | - |
Arginine | nmol/mL | 31.8-149.5 | 128 ± 32 | 0 | 27 | −0.27 | 0.10 |
Asparagine | nmol/ml | 43.8-90.6 | 56 ± 11 | 19 | 0 | −0.23 | 0.17 |
Aspartic acid | nmol/mL | TRA-7.2 | 5 ± 2 | 0 | 5 | 0.08 | 0.63 |
Carnosine | nmol/mL | ND | ND | 0 | 0 | - | - |
Citrulline | nmol/mL | 17.9-48.0 | 45 ± 12 | 0 | 27 | −0.42 | 0.01* |
Cystathionine | nmol/mL | ND | 2 ± 0 | 0 | 73 | −0.09 | 0.66 |
Cystin | nmol/mL | 4.7-34.8 | 19 ± 11 | 3 | 14 | −0.25 | 0.14 |
Ethanolamine | nmol/mL | TRA-10.5 | 7 ± 2 | 0 | 3 | 0.15 | 0.37 |
Glutamate | nmol/mL | 12.2-82.7 | 122 ± 59 | 0 | 68 | 0.44 | 〈0.01* |
Glutamine | nmol/mL | 418.0-739.8 | 700 ± 128 | 0 | 38 | −0.41 | 0.01 |
Glycine | nmol/mL | 140.4-427.3 | 394 ± 102 | 0 | 38 | −0.54 | 〈0.01* |
Hdroxylysine | nmol/mL | ND | ND | 0 | ND | - | - |
Histidine | nmol/mL | 63.0-105.2 | 94 ± 15 | 0 | 27 | 0.00 | 0.99 |
Homocystine | nmol/mL | ND | ND | 0 | 0 | - | - |
Hydroxyproline | nmol/mL | TRA-18.8 | 17 ± 5 | 0 | 27 | −0.30 | 0.10 |
Isoleucine | nmol/mL | 37.0-100.4 | 78 ± 15 | 0 | 8 | −0.07 | 0.67 |
Leucine | nmol/mL | 74.2-169.1 | 134 ± 25 | 0 | 8 | 0.05 | 0.76 |
Lysine | nmol/mL | 125.7-281.9 | 237 ± 44 | 0 | 22 | −0.30 | 0.08 |
Methionine | nmol/mL | 15.5-38.6 | 30 ± 5 | 0 | 8 | −0.14 | 0.40 |
Ornithine | nmol/mL | 42.6-141.2 | 86 ± 25 | 0 | 3 | −0.05 | 0.78 |
Phenylalanine | nmol/mL | 43.5-79.8 | 59 ± 9 | 0 | 3 | 0.02 | 0.91 |
Phosphoethanol-amine | nmol/mL | TRA | TRA | 0 | 0 | - | - |
Proline | nmol/mL | 71.3-373.0 | 289 ± 241 | 0 | 11 | −0.16 | 0.36 |
Sarcosine | nmol/mL | TRA | 8 ± 3 | 0 | 51 | −0.34 | 0.15 |
Serine | nmol/mL | 91.5-186.4 | 168 ± 34 | 0 | 24 | −0.04 | 0.81 |
Taurine | nmol/mL | 46.4-128.2 | 110 ± 43 | 3 | 35 | −0.15 | 0.37 |
Threonine | nmol/mL | 74.2-216.1 | 144 ± 35 | 0 | 3 | −0.15 | 0.39 |
Tryptophan | nmol/mL | 36.2-79.3 | 37 ± 10 | 59 | 0 | 0.35 | 0.03* |
Tyrosine | nmol/mL | 38.4-89.4 | 72 ± 18 | 0 | 16 | 0.18 | 0.28 |
Valine | nmol/mL | 156.2-360.4 | 248 ± 41 | 0 | 0 | −0.02 | 0.89 |
α-aminoadipic acid | nmol/mL | ND | ND | 0 | 0 | - | - |
α-amino-n-butyric acid | nmol/mL | 8.1-31.0 | 35 ± 8 | 0 | 70 | −0.10 | 0.06 |
β-Alanine | nmol/mL | TRA-11.8 | 2 ± 1 | 0 | 0 | 0.05 | 0.88 |
β-Amino-iso-butyric acid | nmol/mL | <5.9 | 3 ± 2 | 0 | 5 | −0.18 | 0.32 |
γ-Amino-nbutyric acid | nmol/mL | ND | ND | 0 | 0 | - | - |