Table 2. Patient, age range and fold-ULN for HS, DS, KS, the cumulative levels of HS, DS and KS and DMB per diagnosis.
MPS subtype | Number of patients | Age range (yrs) | Median fold ULN (range) | ||||
---|---|---|---|---|---|---|---|
HS | DS | KS | HS, DS & KS | DMB | |||
MPS I | 18 | 0.8–32.6 | 12.1 (3.4–2.9) | 101.8 (26.3–03.4) | 0.6 (0.2–1.7) | 10.8 (2.6–50.3) | 4.4 (1.1–0.3) |
MPS II | 16 | 0.2–46.3 | 17.2 (10.0–23.6) | 69.2 (35.3–139.4) | 0.5 (0.4–1.6) | 9.4 (5.3–23.6) | 4.5 (1.7–8.5) |
MPS III | 34 | 3.3–66.9 | 28.3 (17.8–66.9) | 1.3 (0.4–5.0) | 0.5 (0.3–1.6) | 12.0 (6.6–27.6) | 3.1 (0.9–7.3) |
MPS IVA | 10 | 1.1–56.6 | 0.9 (0.4–1.4) | 3.0 (1.0–4.6) | 5.2 (2.4–14.6) | 3.3 (1.6–8.2) | 1.2 (0.6–2.4) |
MPS IVB | 3 | 21.5–51.3 | 1.2 (1.1–1.3) | 1.3 (0.7–2.2) | 6.8 (5.7–10.0) | 3.9 (2.9–5.0) | 0.7 (0.4–0.8) |
MPS VI | 17 | 0.1–57.8 | 0.6 (0.3–2.6) | 31.3 (7.3–243.2) | 0.6 (0.2–1.7) | 3.0 (0.6–15.6) | 1.9 (0.6–9.8) |
MPS VII | 2 | 21.0–21.3 | 1.0–5.4 | 1.5–2.6 | 0.1–0.9 | 0.7–3.5 | 0.5–0.7 |
ML II | 2 | 0.2–0.3 | 0.8–2.8 | 1.6–5.3 | 1.0–1.7 | 0.9–2.1 | 0.8–1.4 |
MLIII | 1 | 12.0 | 1.1 | 2.4 | 1.3 | 1.2 | 0.8 |