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Table 2 Changes in mobility, pulmonary function, body mass index and bone mineral density after enzyme replacement therapy at the time of follow-up DXA

From: Improvement of bone mineral density after enzyme replacement therapy in Chinese late-onset Pompe disease patients

 

Duration of ERT (months)

6MWT (m)

Walton score

FVC (L) (% predicted)

BMI (kg/m2)

BMD (g/cm2)

∆BMD (%)

Pre-ERT

Post-ERT

Pre-ERT

Post-ERT

Pre-ERT

Post-ERT

Pre-ERT

Post-ERT

 

Pre-ERT

Post-ERT

Patient 1

62

NA

NA

7

7

0.94 (20%)

1.02 (22%)

14.2

17.0

33% radiusa

0.765

0.825

+7.8

Z-score

−1.9

−1.3

Hip total

0.675

0.749

+11

Z-score

−2.5

−1.9

Patient 2

61

473

630

2

2

1.63 (38%)

2.32 (54%)

16.2

19.6

Spine L1–L4

1.209

1.328

+9.8

Z-score

0.9

2.0

Hip total

0.928

0.939

+1.2

Z-score

−0.5

−0.4

Patient 3

61

429

425

2

2

0.74 (28%)

0.70 (27%)

14.3

13.6

Spine L1–L4

0.959

1.027

+7.1

Z-score

−1.3

−0.7

Hip total

0.576

0.642

+11.5

Z-score

−3.1

−2.4

Patient 4

33

364

428

2.5

2

0.97 (36%)

0.99 (38%)

16.2

20.6

Spine L1–L4

0.940

1.341

+42.7

Z-score

−1.5

1.9

Hip total

0.41

0.773

+84.4

Z-score

−4.3

−1.5

  1. Patients 1 and 2 were brothers, patients 3 and 4 were sisters
  2. FVC forced vital capacity, BMI body mass index, NA not applicable
  3. aPatient 1 had spinal surgery for scoliosis, BMD was measured from forearm instead of lumbar vertebra