Table 1: Plasma amino acid levels at the diagnosis moment, before treatment (sample I) with Pyridoxine treatment, six months after the diagnosis; (sample II) and with pyridoxine treatment and low protein diet; one year after the diagnosis (sample III).
Amino acid | Sample I | Sample II | Sample III | Control | Controlb | GAb |
C (μM) | (2-18 years)a | (n = 22) | (n = 4) | |||
Ornithine | 975 | 1078 | 1692 | 10-163 | 71-81 | 979-1023 |
Arginine | 522 | 66 | 143 | 10-140 | 95-107 | 120-136 |
Lysine | 274 | 238 | 174 | 48-284 | 198-216 | 112-130 |
Histidine | 334 | 224 | 152 | 41-125 | 80-88 | 94-104 |
Valine | 598 | 276 | 303 | 74-321 | 228-246 | 94-214 |
Isoleucine | 76 | 93 | 97 | 22-107 | 65-73 | 53-57 |
Leucine | 305 | 239 | 142 | 49-216 | 130-142 | 103-109 |