Table 1.
Summary of Patients’ Clinical Courses
| Patient | Age at Presentation | SFTPC Mutation | Family History | Age at MV Initiation | Maximum Respiratory Support | Age at Tracheostomy (mo) | LOS (mo) | Age at Decannulation (yr) | Respiratory Treatments | Support at Last Contact (Age) |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 7 d | c.545 G>C, .pGly182Arg | Positive | 2 mo | CMV | 3.5 | 19 | 6 | Clearance, steroids, hydroxychloroquine | Room air (10 yr) |
| 2 | 4 h | c.563 T>A, p. Leu188Gln | Negative | 1 d | HFOV | 3 | 10 | 5 | Clearance, steroids, azathioprine | 0.25 L NC (5 yr) |
| 3 | 10 min | c.567 C>G, p.Cys189Trp | Negative | 4 mo | HFOV | 4.5 | 8 | 2 | Steroids, azithromycin, hydroxychloroquine | Room air (3 yr) |
Definition of abbreviations: CMV = conventional mechanical ventilation; HFOV = high-frequency oscillatory ventilation; LOS = hospital length of stay; MV = mechanical ventilation; NC = nasal cannula; SFTPC = surfactant protein C.