TABLE 1.
Baseline | Censored # | |
Gender | ||
Male | 9137 (52.5) | |
Female | 8266 (47.5) | |
Mutation category ¶ | ||
Class 1 | 4062 (23.3) | |
Class 2 | 15043 (86.4) | |
Class 3 | 1007 (5.8) | |
Class 4 | 1068 (6.1) | |
Class 5 | 1120 (6.4) | |
Unknown | 668 (3.8) | |
F508del only | 7827 (45.0) | |
Least severe mutation | ||
Class 1/2 | 13614 (78.2) | |
Class 3 | 950 (5.5) | |
Class 4/5 | 2171 (12.5) | |
Unknown | 668 (3.8) | |
Age | ||
12 to <18 years | 6554 (37.7) | 3694 (21.2) |
≥18 years | 10849 (62.3) | 13709 (78.8) |
FEV1pp | ||
Normal | 3923 (22.5) | 4313 (24.8) |
Mild | 4840 (27.8) | 5125 (29.4) |
Moderate | 4959 (28.5) | 5597 (32.2) |
Severe | 1210 (6.9) | 2256 (13.0) |
Unknown | 2471 (14.2) | 112 (0.6) |
BMI | ||
Underweight | 1135 (6.5) | 1282 (7.4) |
Normal | 11517 (66.2) | 10940 (62.9) |
Overweight | 2345 (13.5) | 2852 (16.4) |
Obese | 839 (4.8) | 1045 (6.0) |
Unknown | 1567 (9.0) | 1284 (7.4) |
Chronic macrolides | 7620 (43.8) | 8192 (47.1) |
NTM positive | 4077 (23.4) | |
Culture positive for MAB only | 1203 (6.9) | |
Culture positive for MAC only | 2066 (11.9) | |
Culture positive for other species only | 741 (4.3) | |
Culture positive for multiple species | 67 (0.4) | |
Any modulator therapy | 7384 (42.4) | |
Ever received ivacaftor monotherapy | 1893 (10.9) | |
Ever received combination therapy | 5869 (33.7) | |
Neither | 10019 (57.6) |
Data are presented as n (%). FEV1pp: percentage predicted forced expiratory volume in 1 s; BMI: body mass index; NTM: nontuberculous mycobacteria; MAB: Mycobacterium abscessus; MAC: Mycobacterium avium complex. #: censor: NTM-positive culture or patient's last time point in dataset; ¶: ≥1.