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).

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	Control^b	GA^b
C (μM)	Sample I	Sample II	Sample III	(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

^aFrom [41]; ^bFrom [42].