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Table 4 Baseline and follow-up data of the various parameters for 8 MPS patients underwent ERT

From: Assessment of bone mineral density by dual energy x-ray absorptiometry in patients with mucopolysaccharidoses

No. MPS type Gender DXA Age (yr) ERT duration (yr) Height z score Weight z score BMI z score BMD (g/cm2) BMD z score HAZ adjusted BMD z score
The initiation of ERT at pre-pubertal age  
1 MPS II (M*) M Baseline 5 2.5 −1.63 −0.69 −0.17 0.53 −0.81 −0.31
Follow-up 7.5 −1.22 −0.77 −0.39 0.561 −1.48 −1.01
2 MPS VI M Baseline 6.6 3.8 −3.69 −1.69 0.17 0.496 −1.65 −0.26
Follow-up 10.4 −5.92 −1.82 0.11 0.51 −2.75 0.09
3 MPS VI M Baseline 8.7 5.1 −6.52 −1.69 1.29 0.442 −2.35 0.49
Follow-up 13.8 −7.83 −2.55 0.16 0.492 −3.20 1.25
4 MPS VI F Baseline 11.7 7.4 −5.88 −1.57 1.18 0.65 −2.05 1.07
Follow-up 19.1 −8.7 −2.23 2.06 0.671 −3.10 2.83
5 MPS II (M*) M Baseline 13.2 4.5 −2.77 −1.12 0.00 0.636 −2.07 −0.50
Follow-up 17.7 −3.2 −0.68 0.51 0.867 −3.99 −1.76
The initiation of ERT at post-pubertal age  
6 MPS II (M*) M Baseline 17.5 1.0 −4.47 −1.84 −0.62 0.774 −5.06 −2.02
Follow-up 18.5 −3.97 −1.62 −0.49 0.796 −4.80 −1.99
7 MPS I (H/S**) M Baseline 18.5 5.3 −7.69 −2.13 0.53 0.648 −6.51 −1.18
Follow-up 23.8 −7.23 −1.77 1 0.769 −5.11 NA
8 MPS VI F Baseline 22.8 4.5 −8.88 −3.25 −0.11 0.712 −2.82 NA
    Follow-up 27.3 −9.77 −3.14 0.81 0.789 −2.3 NA
  1. MPS, mucopolysaccharidoses; M*, mild form; H/S**, Hurler/Scheie; DXA, dual energy x-ray absorptiometry; ERT, enzyme replacement therapy; z score, standard deviation score; BMI, body mass index; BMD, bone mineral density; HAZ, height-for-age z score; NA, not available.