Table 2.
The levels of urinary DS, HS, and KS, and DMB ratio in different types of MPS (n = 79)
| Type | N | Age (years) | DS (μg/ml) | HS (μg/ml) | KS (μg/ml) | DMB ratio (mg/mmol creatinine) |
|---|---|---|---|---|---|---|
| MPS I | 14 | 16 (0.7–34.9) | 194.6 (62.0–414.6) | 6.2 (0.11–26.8) | 1.3 (0–3.0) | 53.1 (13.6–159.5) |
| MPS II (mild) | 14 | 18.1 (1.4–37.8) | 118.0 (2.7–520.3) | 7.3 (0–42.7) | 1.2 (0–5.54) | 51.6 (10.3–101.4) |
| MPS II (severe) | 9 | 9.1 (0.9–21.5) | 253.8 (37.5–1,214.8) | 446.6 (43.4–1654.4) | 1.0 (0–1.62) | 85.6 (24.8–185.4) |
| MPS III | 11 | 7.4 (3.2–17.1) | 0.4 (0–0.9) | 260.7 (48.6–999.6) | 0.8 (0–3.6) | 46.2 (8.6–101.0) |
| MPS IV | 23 | 15.1 (1.4–29.4) | 0.2 (0–1.3) | 0.1 (0–0.35) | 168.0 (10.54–1,377.5) | 16.7 (0–30.4) |
| MPS VI | 8 | 11.9 (4.4–21.2) | 108.9 (17.0–502.3) | 1.2 (0–3.2) | 0.6 (0–1.88) | 37.0 (21.4–64.9) |
Data are mean (range).
DS: dermatan sulfate; HS: heparan sulfate; KS: keratan sulfate; DMB: dimethylmethylene blue; MPS: mucopolysaccharidosis.