Table 1.
2016 | 2017 | |||||||||
---|---|---|---|---|---|---|---|---|---|---|
No AF diagnosis (n = 2,591) |
One positive AF (n = 439) |
At least two positive AF (n = 571) |
P value no AF vs. one AF one AF vs. ≥ two AF no AF vs. ≥ two AF |
No AF diagnosis (n = 2,999) |
One positive AF (n = 481) |
At least two positive AF (n = 578) |
P value no AF vs. one AF one AF vs. ≥ two AF no AF vs. ≥ two AF |
|||
Median age (years, range) |
16.0 (0.0–78.8) | 22.0 (1.0–73.0) | 25.0 (3.0–74.0) |
< .0001 0.0003 < .0001 |
16.0 (0.0–79.0) | 23.0 (0.0–70.0) | 25.0 (6.0–75.0) |
< .0001 0.0013 < .0001 |
||
Male (%) | 53.4 | 50.8 | 47.3 |
0.3166 0.2684 0.0083 |
52.3 | 54.9 | 50.4 |
0.2826 0.1408 0.4013 |
||
Median BMI percentile (range): children |
32.0 (15.0–56.0) | 24.0 (11.0–46.0) | 27.0 (10.0–44.0) |
0.0017 0.8511 0.0061 |
33.0 (15.0–56.0) | 26.0 (10.0–48.0) | 26.0 (10.0–46.0) |
0.0183 0.7625 0.0109 |
||
Median BMI (range): adults | 21.1 (2.5–46.2) | 21.4 (13.9–34.6) | 20.9 (12.4- 30.8) |
0.9722 0.1298 0.0417 |
21.1 (13.3–46.1) | 20.8 (15.0–37.9) | 21.0 (13.6–32.0) |
0.3455 0.5051 0.0356 |
||
Genotype F508del homozygote (%) F508del heterozygote (%) Other genotype (%) |
41.8 35.5 22.6 |
50.3 33.0 16.6 |
48.9 34.5 16.7 |
0.0014 0.8790 0.0014 |
42.5 35.8 21.7 |
47.3 37.9 14.8 |
47.7 35.4 17.0 |
0.0021 0.5309 0.0165 |
||
Pancreatic insufficiency (%) | 95.9 | 95.4 | 96.0 |
0.6520 0.6805 0.9450 |
95.2 | 96.5 | 96.2 |
0.2088 0.8150 0.2831 |
||
ABPA (%) | 5.8 | 8.0 | 9.8 |
0.0714 0.3127 0.0004 |
6.2 | 12.3 | 9.2 |
0.0714 0.3127 0.0004 |
||
CFRD (%) | 16.4 | 20.3 | 24.9 |
0.0714 0.3127 0.0004 |
17.2 | 20.6 | 27.0 |
0.0714 0.3127 0.0004 |
||
Liver disease (%) | 28.8 | 38.7 | 41.9 |
< .0001 0.3148 < .0001 |
26.4 | 35.1 | 38.2 |
< .0001 0.2977 < .0001 |
||
Arthritis and/or arthropathy (%) | 3.9 | 4.6 | 6.3 |
0.4891 0.2286 0.0091 |
3.3 | 5.0 | 6.8 |
0.0691 0.2286 0.0001 |
||
Therapy (%) | ||||||||||
Continuous antibiotics | 53.3 | 71.5 | 79.0 |
< .0001 0.0061 < .0001 |
54.0 | 72.8 | 81.5 |
< .0001 0.0007 < .0001 |
||
Tobramycin (inhalative) | 18.6 | 26.0 | 29.8 |
0.0003 0.1825 < .0001 |
18.5 | 26.2 | 31.0 |
< .0001 0.0876 < .0001 |
||
Colistin (inhalative) | 23.4 | 33.5 | 40.3 |
< .0001 0.0269 < .0001 |
22.1 | 39.1 | 41.7 |
< .0001 0.3889 < .0001 |
||
Aztreonam (inhalative) | 11.5 | 21.2 | 24.5 |
< .0001 0.2125 < .0001 |
12.4 | 20.2 | 23.9 |
< .0001 0.1481 < .0001 |
||
Bronchodilatators (inhalative) | 82.4 | 88.4 | 93.9 |
0.0020 0.0020 < .0001 |
82.9 | 91.9 | 94.5 |
< .0001 0.0957 < .0001 |
||
Steroids | 46.0 | 57.6 | 64.6 |
< .0001 0.0235 < .0001 |
46.4 | 54.3 | 63.5 |
0.0014 0.0023 < .0001 |
||
Inhalative steroids | 33.0 | 45.8 | 51.5 |
< .0001 0.0723 < .0001 |
33.4 | 43.7 | 52.4 |
< .0001 0.0045 < .0001 |
||
Oral steroids | 8.1 | 11.6 | 13.0 |
0.0140 0.5207 0.0002 |
8.9 | 9.6 | 12.3 |
0.6384 0.1597 0.0110 |
||
Antimycotics | 10.5 | 13.2 | 12.1 |
0.0966 0.5921 0.2811 |
11.1 | 12.9 | 13.7 |
0.2611 0.7106 0.0813 |
||
CFTR modulator ivacaftor | 3.1 | 2.1 | 2.8 |
0.2338 0.4458 0.7189 |
3.3 | 4.0 | 2.9 |
0.4409 0.3670 0.6836 |
||
CFTR modulator lumacaftor | 4.6 | 10.7 | 14.2 |
< .0001 0.0994 < .0001 |
8.5 | 12.5 | 16.8 |
0.0048 0.0495 < .0001 |
||
Bacterial co-colonization with Pseudomonas aeruginosa, Staphylococcus aureus and non-tuberculous mycobacteria (%) | ||||||||||
Pseudomonas aeruginosa (PA)* | 36.7 | 52.2 | 62.2 |
< .0001 0.0014 < .0001 |
36.5 | 54.3 | 63.5 |
< .0001 0.0023 < .0001 |
||
Mucoid PA | 20.5 | 29.2 | 30.7 |
< .0001 0.6083 < .0001 |
19.8 | 31.4 | 35.5 |
< .0001 0.1623 < .0001 |
||
multiresistant PA | 11.7 | 16.2 | 21.2 |
0.0090 0.0439 < .0001 |
12.9 | 16.8 | 20.4 |
0.0188 0.1381 < .0001 |
||
Staphylococcus aureus (SA) | 65.7 | 69.0 | 67.6 |
0.1725 0.6310 0.3826 |
64.8 | 70.5 | 68.9 |
0.0148 0.5683 0.0596 |
||
Non-tuberculous mycobacteria | 1.2 | 3.2 | 8.8 |
0.0010 0.0003 < .0001 |
1.9 | 6.7 | 9.0 |
< .0001 0.1599 < .0001 |