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Table 4 Changes in respiratory and muscle strength of P5 (infantile-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%

NIV %

UL

LL

0.0

100

0

4-

4-

0.5–4.5

100

0

4-

4-

4.5–5.5

0

100

4

4

>4.5a

0

0

4+

4+

  1. ERT enzyme replacement therapy, P patient no, BIPAP Biphasic positive airway pressure, NIV noninvasive ventilation, MRC medical research council, UL Upper-limb muscle strength, LL lower-limb muscle strength
  2. aat home