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Table 2 Changes in respiratory and muscle strength of P1 (juvenile-onset) after enzyme replacement therapy

From: Comprehensive approach to weaning in difficult-to-wean infantile and juvenile-onset glycogen-storage disease type II patients: a case series

Months of ERT (28 days/month)

Proportion of ventilator time

MRC scale

Invasive BiPAP%

Invasive CPAP%

NIV% or time

UL

LL

0.0

100

0

10 min

3

3+

0.5

29

71

0

3

3+

1.0

21

79

30 min

4

3+

1.5

14

86

0

4+

3+

2.0

0

100

0

5-

4-

2.5–3.5

20

80

0

5-

4

4.0–5.0

0

100

0

5

5-

5.5a

79

21

2 h

5

5

6.0

7

93

0

5

5

6.5–7.0

0

100

0

5

5

7.5

14

86

2d

5

5

8.0–8.5

15

85

0

5

5

9.0

64

14

3d

5

5

9.5–10.5

6

94

0

5

5

11.0

57

0

6d

5

5

11.5–13.5

37

63

0

5

5

14.0–14.5a

100

0

0

5

5

15.0b

29

0

0

5

5

  1. ERT enzyme replacement therapy, P patient no, BiPAP Biphasic positive airway pressure, min minutes, hr hours, d days, CPAP Continuous positive airway pressure, NIV noninvasive ventilator, MRC medical research council, UL Upper-limb muscle strength, LL lower-limb muscle strength
  2. aPulmonary infection; bTracheotomy