Table 2.
Recommended Frequency of Pulmonary Function Tests for Congenital Myopathies
| Test | Frequency |
|---|---|
| Highly recommended | |
| Spirometry sittinga | 6–12 mo |
| Maximal inspiratory pressure, maximal expiratory pressure | 6–12 mo |
| Peak cough flow | 6–12 mo |
| Overnight oximetry with measurement of CO2 (CBG, transcutaneous CO2, end-tidal CO2) | Annually, if polysomnography not available |
| Recommended | |
| Spirometry supine in addition to sitting | 6–12 mo |
| Comprehensive pulmonary function tests including lung volumes | Annually |
| Oximetry spot check and/or venous bicarbonate | 6–12 mo |
| Attended polysomnography | 1–2 y if stable, sooner if change in baseline status |
a
Spirometry is indicated for children older than 5 years.