Table 3.

Plasma amino acids at baseline in subjects with CF compared to reference ranges (RR).

Amino acid	Reference range (RR: nmol/mL)	n	Plasma concentration (nmol/mL)	Proportion of subjects (%)
				within RR	< RR	> RR
Alanine	88 – 440	110	384 ± 109 a	84 (76%) b	0	26 (24%)
Arginine	15 – 120	110	76 ± 25	107 (97%)	0	3 (3%)
Asparagine	19 – 106	110	55 ± 12	110 (100%)	0	0
Aspartic Acid	0 – 31	110	6.2 ± 2.5	110 (100%)	0	0
Cysteine	2 – 47	110	44 ± 10	71 (65%)	0	39 (36%)
Glutamic Acid	24 – 163	110	54 ± 24	105 (96%)	4 (4%)	1 (1%)
Glutamine	285 – 832	110	551 ± 89	109 (99%)	0	1 (1%)
Glycine	103 – 424	110	321 ± 78	102 (93%)	0	8 (7%)
Histidine	38 – 132	110	101 (60 – 176) c	98 (89%)	0	12 (11%)
Isoleucine	17 – 106	110	63 ± 13	109 (99%)	0	1 (1%)
Leucine	42 – 188	110	118 ± 21	109 (99%)	0	1 (1%)
Lysine	71 – 217	109	177 ± 30	99 (90%)	0	11 (10%)
Methionine	8 – 49	110	26 ± 9	107 (97%)	0	3 (3%)
Phenylalanine	23 – 95	110	56 ± 10	110 (100%)	0	0
Proline	83 – 346	110	166 (82 – 396)	107 (97%)	1 (1%)	2 (2%)
Serine	50 – 215	110	113 ± 19	110 (100%)	0	0
Threonine	86 – 321	110	142 ± 38	108 (98%)	2 (2%)	0
Tyrosine	22 – 102	110	63 (32 – 134)	108 (98%)	0	2 (2%)
Valine	39 – 321	110	221 (152 – 353)	109 (99%)	0	1 (1%)
Ornithine	13 – 151	110	82 ± 23	110 (100%)	0	0
α-aminobutyric	0 – 43	108	21 ± 8	105 (97%)	0	3 (3%)
Taurine	55 – 204	109	78 ± 22	103 (94%)	6 (6%)	0
Homocysteine d
5 – 10.9 y	0.8 – 6.5	68	4.9 ± 1.2	63 (93%)	0	5 (7%)
11–16.9 y	5.7 – 11.7	39	5.5 ± 1.5	16 (41%)	23 (59%)	0
≥17 y	10.5 – 16.5	3	6.0 ± 0.6	0	3 (100%)	0
BCAA	-	110	403 ± 67	-	-	-
AAA	-	110	121 ± 21	-	-	-
BCAA:AAA ratio	-	110	3.41 ± 0.62	-	-	-

BCAA, branch- chained amino acids (Valine, Leucine, Isoleucine). AAA, aromatic amino acids (Tyrosine, Phenylalanine).

^aContinuous normally distributed variables given as means ± SD

^bCategorical variables are given as numbers (percentages)

^cContinuous variables with significant skewness are given as medians (range)

^dThe reference ranges for homocysteine are given by age group.