Table 1. Plasma amino acid concentrations.

	HT1	Controls	Mean P-value
Phenylalanine	27 ±14	54 ± 16	0.00462
	33 [16–38]	50 [43–61]
Tyrosine	384 ± 53	64 ± 23	2.67·10−7
	382 [337–432]	60 [49–74]
Valine	226 ± 26	188 ± 54	0.199
	230 [208–246]	181 [155–213]
Isoleucine	59 ± 8	55 ± 22	0.494
	59 [52–64]	52 [41–64]
Leucine	115 ± 18	105 ± 39	0.454
	116 [101–129]	99 [81–121]
Methionine	23 ± 6	21 ± 9	0.401
	21 [18–27]	19 [15–25]
Histidine	81 ± 11	83 ± 22	0.572
	79 [73–89]	81 [70–94]
Threonine	129 ± 37	118 ± 62	0.427
	114 [109–160]	106 [84–134]
Glutamine	480 ± 46	517 ± 121	0.382
	488 [446–507]	513 [451–578]

Plasma amino acid concentrations (μmol/l) in HT1 patients treated with NTBC and diet and controls. Data are presented as Mean ± SD and Median with IQR.