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Changes in respiratory and muscle strength of P5 (infantile-onset) after enzyme replacement therapy
| 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+ |
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
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