Table 2.

Levels of KS in controls and untreated patients

A. Di-sulfated KS (ng/mL)
Age (years)	Controls	MPS I	MPS II	MPS III	MPS IV	ML
Newborns	27 ± 47	39 ± 20	84	29 ± 7	n/a	n/a
0–5	33 ± 21	55 ± 30	245 ± 474	92 ± 88	107 ± 139	42
5–10	40 ± 20	n/a	84 ± 41	44 ± 23	126 ± 200	25
10–15	41 ± 19	n/a	295 ± 557	106 ± 165	497 ± 869*	46
15–30	21 ± 11	n/a	122	44 ± 20*	38	n/a
>30	19 ± 15	n/a	n/a	56	56 ± 37	n/a

B. Mono-sulfated KS (ng/mL)
Age (years)	Controls	MPS I	MPS II	MPS III	MPS IV	ML
Newborns	128 ± 161	172 ± 82	208	86 ± 27	n/a	n/a
0–5	188 ± 75	216 ± 127	398 ± 415	257 ± 186	332 ± 408	100
5–10	152 ± 63	n/a	313 ± 163	106 ± 49	225 ± 190	115
10–15	140 ± 69	n/a	560 ± 720	315 ± 383	259 ± 177	182
15–30	67 ± 32	n/a	317	121 ± 60*	96	n/a
>30	49 ± 24	n/a	n/a	143 ± 85	123 ± 61*	n/a

C. Ratio of di-sulfated KS in total KS (%)
Age (years)	Controls	MPS I	MPS II	MPS III	MPS IV	ML
Newborns	17 ± 6	26 ± 15***	27 ± 12***	28 ± 11***	n/a	n/a
0–5	15 ± 8	23 ± 5*	26 ± 10*	25 ± 5***	23 ± 4**	30 ± 2
5–10	21 ± 7	n/a	21 ± 1	24 ± 4	29 ± 8*	18
10–15	23 ± 5	n/a	26 ± 8	30 ± 9*	32 ± 21	20
15–30	23 ± 7	n/a	28	26 ± 4	29	n/a
>30	28 ± 7	n/a	n/a	29	24 ± 9	n/a

^*p < 0.05;

^**p < 0.0005;

^***p < 0.0001

Newborn data from Kubaski et al., 2016.