Table 3.
CFTR Gain-of-Function (GOF) complex allele [G574A;R668C] in all patients.
| Clinical Category | Females (n.) | Median Age (IQR) | Total n. Patients |
|---|---|---|---|
| Cat. 1 | F (1) | 84 y | 1 |
| Cat. 2 | F (1) | 57 y | 2 |
| M (1) | 75 y | ||
| Cat. 3 | 6(F) | 81.5 y (69.3–85.5) | 15 |
| 9 (M) | 71 y (56.3–76.5) | ||
| Cat. 4 | 2 (F) | 77;82 y | 11 |
| 9(M) | 62 y (54–72.5) | ||
| Cat. 5 | 2 (F) | 43;53 y | 4 |
| 2(M) | 57;64 y | ||
| Cat. 6 | 2 (F) | 44;45 y | 8 |
| 6 (M) | 57.5 y (42.5–63.5) |
Clinical category: 1, death; 2, hospitalized, receiving invasive mechanical ventilation; 3, hospitalized, receiving continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) ventilation; 4, hospitalized, receiving low-flow supplemental oxygen; 5, hospitalized, not receiving supplemental oxygen; 6, not hospitalized.