Subjects |
17 (262) |
22 (351) |
43 (674) |
5 (72) |
3 (51) |
8 (125) |
2 (37) |
10-year rate of any death |
4.60 |
5.40 |
13.50 |
20.80 |
37.30 |
45.60 |
51.40 |
Proposed name |
Classic CF/ lowP. aeruginosa |
Nonclassic CF/ late diagnosis |
Classic CF/ moderate |
Classic CF/ pneumothorax/ NTM |
Classic CF/ ABPA |
Classic CF/ respiratory insufficiency |
Classic CF/ B. cepacia |
Respiratory disease |
|
|
|
|
|
|
|
Lung function |
Mild |
Mild |
Moderate/severe |
Severe |
Moderate/severe |
Very severe |
Moderate/severe |
Courses ofi.v.antibiotics per year (median) |
0 |
0 |
2 |
1 |
2 |
4 |
1 |
Respiratory complications |
Very low |
Very low |
Haemoptysis 15% |
Haemoptysis 13.9%; pneumothorax 54.2% |
Haemoptysis 15%; ABPA/steroids |
Haemoptysis 22.4%; pneumothorax 10.4% |
Haemoptysis 15.7% |
Pancreatic insufficiency rates |
Very high |
Low |
Very high |
Very high |
Very high |
Very high |
Very high |
Diabetes rates |
Very low |
Very low |
High |
Low |
High |
High |
High |
Airway infection |
LowP. aeruginosa |
LowP. aeruginosa |
HighP. aeruginosa |
P. aeruginosa; high NTM |
P. aeruginosa; MRSA |
P. aeruginosa; lowB. cepacia |
HighB. cepacia |
Outcome at 5years |
|
|
|
|
|
|
|
Any death |
0.8 (2) |
1.4 (5) |
7.1 (48) |
9.7 (7) |
21.6 (11) |
36.8 (46) |
32.4 (12) |
Death without transplantation |
0.4 (1) |
1.1 (4) |
4.9 (33) |
6.9 (5) |
19.6 (10) |
18.4 (23) |
21.6 (8) |
Lung transplantation |
3.1 (8) |
4.3 (15) |
10.4 (70) |
19.4 (14) |
25.5 (13) |
55.2 (69) |
24.3 (9) |
Death after lung transplantation |
0.4 (1) |
0.3 (1) |
2.2 (15) |
2.8 (2) |
2.0 (1) |
18.4 (23) |
10.8 (4) |
Outcome at 10years |
|
|
|
|
|
|
|
Any death |
4.6 (12) |
5.4 (19) |
13.5 (91) |
20.8 (15) |
37.3 (19) |
45.6 (57) |
51.4 (19) |
Death without transplantation |
3.1 (8) |
3.7 (13) |
7.4 (50) |
9.7 (7) |
27.5 (14) |
23.2 (29) |
35.1 (13) |
Lung transplantation |
14.5 (38) |
11.4 (40) |
27.6 (186) |
36.1 (26) |
31.4 (16) |
67.2 (84) |
32.4 (12) |
Death after lung transplantation |
1.5 (4) |
1.7 (6) |
6.1 (41) |
11.1 (8) |
9.8 (5) |
22.4 (28) |
16.2 (6) |