Table 3. Respiratory status in patients with infantile and late onset Pompe disease.
In this group of patients, 4/12 (33%) needed assistive ventilation including invasive ventilation in the 2 patients with infantile Pompe disease.
| Patient | FVC (L) | Ventilatory support (onset in years) |
|---|---|---|
| 1 | - | - |
| 2 | 0.96 | Tracheostomy/5 |
| 3 | - | Tracheostomy/0.5 |
| 4 | 4.6 | - |
| 5 | - | - |
| 6 | 5.08 | - |
| 7 | 3.8 | - |
| 8 | 4.06 | - |
| 9 | 1.7 | BiPAP/39 |
| 10 | 3.15 | - |
| 11 | 2.00 | BiPAP/48 |
| 12 | 4.14 | - |
Abbreviations: BiPAP (Bi-Level Positive Air Pressure), FVC forced vital capacity, L liter.