Table 2.

Percentiles and cut-off selection (μmol/L) and comparison of Region for the Stork Study (R4S) and Centers for Disease Control cutoff values.¹⁴

Analyte	95%	97%	98%	99%	99.5%	99.9%	Cut off	R4S Cut-off	CDC Cut-off
GLY	465	502	532	585	635	733	<733	767	NA
ALA	327	357	381	419	463	536	<536	507	NA
VAL	113	124	133	148	166	208	<208	212	300
LEU/ILEU	152	166	178	199	221	258	<258	235	290
ORN	119	130	139	153	164	177	<177	NA	NA
ASP	83	95	104	119	131	138	<138	NA	NA
MET (high)	31	35	37	43	50	82	<82	44	75
PHE	71	84	89	99	107	143	<107	97	150
CIT	25	28	31	36	40	73	<73	28	55
TYR	140	184	200	227	241	269	<241	207	350
GLU	484	521	525	530	533	536	<536	551	NA
ARG	25	30	33	36	38	42	<42	32	70
C0 (high)	25	32	35	39	41	44	<44	59	NA
C2	28	36	39	42	45	49	<49	52	NA
C3	2.8	3.7	4.0	4.6	5.3	5.9	<5.9	4.74	5.65
C4	0.4	0.6	0.6	0.8	0.9	1.0	<1.0	0.75	1.30
C5	0.16	0.25	0.30	0.37	0.46	0.70	<0.70	0.39	0.7
C6	0.06	0.08	0.09	0.10	0.10	0.11	<0.10	0.18	0.4
C5DC	0.15	0.20	0.22	0.25	0.28	0.38	<0.38	0.17	0.35
C8	0.06	0.08	0.09	0.11	0.14	0.27	<0.27	0.21	0.45
C10	0.09	0.12	0.13	0.15	0.17	0.55	<0.17	0.26	0.45
C12	0.12	0.16	0.18	0.20	0.24	0.33	<0.33	0.41	NA
C14	0.22	0.28	0.30	0.34	0.38	0.56	<0.38	0.50	0.75
C16	3.54	4.34	4.62	5.09	5.62	6.57	<6.57	6.0	7.50
C18	1.03	1.30	1.40	1.58	1.79	2.51	<1.79	1.7	2.3
C5:1	0	0	0.01	0.02	0.03	0.05	<0.25	0.08	0.25
C5OH	0.25	0.32	0.36	0.42	0.50	0.52	<0.50	0.38	0.80
C8:1	0.07	0.11	0.12	0.16	0.21	0.32	<0.21	NA	NA
C10:1	0.05	0.06	0.07	0.08	0.09	0.10	<0.10	0.18	0.30
C14:1	0.13	0.18	0.20	0.25	0.33	0.37	<0.37	0.37	0.60
C14OH	0.01	0.02	0.02	0.02	0.04	0.04	<0.04	NA	NA
C16:1	0.24	0.30	0.33	0.40	0.46	0.51	<0.53	NA	NA
C16:1OH	0.05	0.06	0.06	0.07	0.08	0.12	<0.12	0.13	NA
C16OH	0.02	0.03	0.04	0.06	0.08	0.11	<0.10	0.08	0.13
C18:1	1.75	2.25	2.47	2.90	3.38	3.76	<3.76	2.5	3.5
C18:1OH	0.02	0.03	0.03	0.05	0.08	0.12	<0.12	0.07	NA
C18OH	0.02	0.03	0.03	0.04	0.04	0.09	<0.09	0.06	0.10
PHE/TYR	1.04	1.35	1.46	1.70	1.96	2.98	<1.96	NA	NA
C3/C2	0.15	0.19	0.20	0.23	0.25	0.41	<0.25	NA	NA
C5/C0	0.01	0.02	0.02	0.03	0.03	0.06	<0.03	NA	NA
C8/C10	1.00	1.50	1.50	2.00	2.50	2.5	<2.05	NA	NA
TSH (μU/mL)	9	10	12	15	21	38	<21	NA	13.6
17OHP (nmol/L)	22	28	33	44	57	93	<93	NA	47.6
Analyte	0.1%	0.25%	0.50%	0.75%	1.00%	Cut off		-----	-----
C0 (Low)	0.0	2.4	4.50	5.20	5.6	>4.50	NA	NA	8.0
Meth (Low)	5.71	6.05	6.45	6.78	6.97	>6.05	NA	NA	NA
GALT (U/dL)	3.3	4.6	5.5	6.1	6.5	>3.3	NA	NA	NA
BTD (U/dL)	18.5	28.2	37.0	43.3	49	>50	NA	NA	NA

GLY: glycine, ALA: alanine, VAL: valine, LEU/ILEU: leucine/isoleucine, ORN: ornithine, ASP: aspartic acid, MET: methionine, PHE: phenylalanine, CIT: citrulline, TYR: tyrosine, GLU: glutamic acid, ARG: arginine, C0: free carnitine, C2: acetylcarnitine, C3: propionylcarnitine, C4: butyryl-/isobutyrylcarnitine, C5: isovaleryl-/2-methylbutyrylcarnitine, C6: hexanoylcarnitine, C5DC: glutarylcarnitine, C8: octanoylcarnitine, C10:decanoylcarnitine, C12: dodecanoylcarnitine, C14: tetradecanolycarnitine, C16: pamitoylcarnitine, C18: stearylcarnitine, C5:1: tiglylcarnitine, C5OH: hydroxyl-isovalerylcarnitine, C8:1: otenylcarnitine, C10:1: dodecenylcarnitine, C14:1: tetradecenylcarnitine, C14OH: hydroxyl tetradecanoylcarnitine, C16:1: hexadecenoylcarnitine, C16:1OH: hydroxyl-hexadecenoylcarnitine, C16OH: hydroxypalmitoylcarnitine, C18:1: oleylcarnitine, C18:1OH: hydroxyl-oleylcarnitine, C18OH: hydroxyl-stearylcarnitine: TSH: thyroid stimulating harmone, 17-OHP: 17-hydroxy progesterone, GALT: galactosemia: BTD: biotinidase deficiency