Therapeutic approaches and follow-up of children with SFTPC-associated lung disease
| All patients | Non-BRICHOS domain | BRICHOS domain | |
|---|---|---|---|
| Patients, n | 22 | 16 | 6 |
| Steroids | |||
| Oral | 1 (4.5) | 1 (4.5) | 0 (0) |
| Pulse | 2 (9) | 2 (9) | 0 (0) |
| Oral and pulse | 16 (73) | 10 (45) | 6 (27) |
| Hydroxychloroquine | 11 (50) | 7 (32) | 4 (18) |
| Azithromycin | 5 (23) | 4 (18) | 1 (4.5) |
| Enteral nutrition | 16 (73) | 10 (45) | 6 (27) |
| Median follow-up (range) in years | 3.2 (1–18.3) | 3.8 (1–18.3) | 3.0 (1.8–7.3) |
| Patients symptomatic at the end of follow-up* | 18 (82) | 16 (73) | 2 (9) |
| Supplemental oxygen | |||
| Intermittent or no oxygen | 2 (9) | 2 (9) | 0 (0) |
| Long-term oxygen (>3 months) | 19 (86) | 13 (59) | 6 (27) |
| Patients still on oxygen | 4 (18) | 3 (14) | 1 (4.5) |
| Median duration of oxygen use (range) in months | 34.5 (12–47) | 35 (12–47) | 34 |
| Patients weaned of oxygen | 15 (68) | 10 (45) | 5 (23) |
| duration of oxygen use (range) in months | 10 (3–24) | 12 (3–24) | 7 (4–9) |
The percentage of the total is indicated in parentheses.
↵* Patients symptomatic for one of the following items: cough, dyspnoea, exercise intolerance or recurrent respiratory infections.