Table 4.
Patient | CFTR Mutations | Peak VO2 (ml/min)a | Peak VO2/kga | Peak VO2 (% pred)a |
---|---|---|---|---|
1/M | F508del/W1282X | 2658/2798 | 41.86/43.05 | 85/90 |
2/F | F508del/G542X | 1379/1652 | 25.16/29.5 | 71/86 |
3/F | F508del/W1282X | 1822/1762 | 31.41/30.91 | 87/92 |
4/M | F508del/F508del | 1066/1574 | 17.77/24.29 | 37/52 |
5/M | F508del/Y1014C | 2350/2226 | 42.96/41.38 | 91/86 |
6/M | R1158X/G85E | 2133/1746 | 36.78/27.28 | 73/58 |
7/F | G85E/G85E | 1090/1272 | 32.06/30.5 | 87/86 |
Abbreviations: CFTR, Cystic fibrosis transmembrane regulator; F, female; kg, kilograms; M, male; min, minute; ml, milliliters; pred, predicted; VO2, oxygen uptake.
Before/after Trikafta.