Table 1.
Patient demographic and baseline characteristics in 26 patients <19 years of age
| MPS type | n | Age (yr) | Age range (yr) | Height z score | Weight z score | BMI z score | BMD z score | HAZ adjusted BMD z score |
|---|---|---|---|---|---|---|---|---|
| MPS I (H/S*) |
1 |
18.5 |
18.5 |
−7.69 |
−2.13 |
0.53 |
−6.51 |
−1.18 |
| MPS II |
10 |
11.1 (5.2) |
5-18.4 |
−3.05 (1.84) |
−0.19 (1.76) |
1.06 (1.37) |
−2.34 (1.45) |
−0.61 (1.32) |
| MPS IIIB |
2 |
7.2 (0) |
7.2 |
−0.56 (0.06) |
0.36 (0) |
1 (0.05) |
−0.93 (0.17) |
−0.78 (0.20) |
| MPS IVA |
9 |
10.1 (3.2) |
5.8-15.3 |
−6.54 (2.21) |
−2.27 (0.81) |
0.36 (1.47) |
−4.08 (0.88) |
−0.89 (1.29) |
| MPS VI |
4 |
9.1 (2.1) |
6.6-11.7 |
−4.90 (1.52) |
−1.50 (0.31) |
0.72 (0.59) |
−2.57 (1.15) |
−0.33 (1.63) |
| Total | 26 | 10.4 (4.2) | 5-18.5 | −4.53 (2.66) | −1.15 (1.55) | 0.74 (1.23) | −3.03 (1.62) | −0.70 (1.24) |
Values are displayed as mean (standard deviation).
MPS, mucopolysaccharidoses; H/S*, Hurler/Scheie; z score, standard deviation score; BMI, body mass index; BMD, bone mineral density; HAZ, height-for-age z score.