Patient number | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 |
---|---|---|---|---|---|---|---|---|
Sex | F | M | F | M | F | F | M | F |
Mutation | dF508/ dF508 | dF508/ dF508 | dF508/ Nk | dF508/ R553X | dF508/ S466X | dF508/ Nk | dF508/ Nk | dF508/ R553X |
Age at first detection (y) | 41.1 | 33.0 | 10.0 | 11.0 | 21.7 | 8.0 | 22.3 | 27.6 |
Age at last detection (y) | 43.0 | 36.9 | 17.8 | 11.0 | 28.4 | 8.6 | 29.2 | 28.4 |
Follow up (y) * | 3.1 | 5.9 | 7.8 | 2.4 | 6.7 | 5.3 | 6.9 | 0.8 |
Trichosporon species | Nk | Asahii | Inkin | Nk | Mycotoxinivorans | Nk | Nk | Nk |
Other isolated organisms from sputum (at first detection) | Ps ae (chron), Ps ae muc | E col, Prot mirab | None | Norcard farc, Inqu lin, Burc cep, Asp fum, Penicill sp | Ps ae (chron), Cand alb | Acinet baum, fungi (not spec), Ps ae (chron) | Ps ae (chron), Ps ae muc, Cand alb | Ps ae muc (chron), Cand alb |
Other isolated organisms from sputum (throughout observation period) | Pseudoall boyd (rep), Cand glab (rep), Cand alb (rep), Cand paraps (rep), Enteroc spp. (rep), Asp fum (rep) | Prot mirab (rep), Asp fum (rep) | Asp fum (rep), Sten mal (rep), Cand alb (rep), Sa (rep) | Inqu lin (rep), Burc cep (rep), Penicill sp (rep), Acinet fum (rep), Ps put (once) | E coli (rep), Sten malt (rep), Asp flav (rep) | Acinet baum (rep), Pet sord (rep) | Asp fum (rep), Prot mirab (rep), Sa (rep), Enteroc spp (rep), Cand alb (rep) | Cand paraps (rep) |
Antibiotic regimen prior to first detection (agent; regimen) | ||||||||
Oral | Cip (cont) | Cefur (cont), Cip (2 w on/off) | Cep (cont), Cip (2 w on/off) | Ceph (cont), Cip (2 w on/off) | Ceph (cont) | Ceph (cont), cip (2 w on/off) | Ceph (cont) | Cot (cont) |
Inhaled | Tob/col (2 w on/off) | None | Col (2 w on/off) | Tob/col (2 w on/off) | Gern (2 w on/off) | None | Gern (2 w on/off) | Gern/col (2 w on/off) |
iv (courses/y) | 2 | 2 | 1 | 1 | 2 | 1 | 1 | 2 |
Diabetes mellitus (at first detection) | Yes | Yes | no | No | No | No | No | No |
ABPA (years prior to first detection) | 5y, 2y | 3y | no | 3y, 1y | No | 1 y after first detection | No | At first detection |
Nebulized/inhaled steroids prior to first detection (duration) | Yes (5y, rep) | No | No | Yes (2.5y) | Yes (2y) | Yes (1y) | No | Yes (from first detection on) |
Intermittent systemic steroids prior to first detection | Yes | Yes | No | Yes | Yes | No | No | Yes (from first detection on) |
Lung function FEV 1 (% pred) ** | Decline | Stable | Decline | Increase | Decline | Increase | Decline | Np |
At first detection | 61.2 | 49.3 | 81.2 | 84.3 | 86.3 | 68.2 | 40.1 | 61.3 |
At end of observation | 43.1 | 47.2 | 57.3 | 88.3 | 79.0 | 79.6 | 30.0 | Np |
BMI (kg/m 2 ) *** | Decline | Stable | Increase | Increase | Increase | Increase | Decline | Np |
At first detection | 18.9 | 25.8 | 13.7 | 15.5 | 19.6 | 15.1 | 19.3 | 21.5 |
At end of observation | 18.2 | 25.8 | 17.8 | 16.3 | 19.9 | 18 | 18.5 | Np |
IgG (g/l) **** | Increase+ | Stable | increase | Stable | Stable | Stable | Increase | Np |
At first detection | 18.8 | 13.0 | 8.1 | 8.2 | 10.4 | 9.5 | 13.4 | 9.2 |
At end of observation | 22.4 | 13.7 | 13.1 | 10.2 | 11.7 | 9.6 | 17.1 | Np |
CRP (mg/l)***** | Increase | Na | Increase | Stable | Stable | Stable | Decrease | Np |
At first detection | 6.2 | 11.1 | <1.0 | <1.0 | 3.5 | <1.0 | 19.9 | 17.9 |
At end of observation | 30.7 | Na | 11.7 | <1.0 | 5.4 | 4.1 | 6.7 | Np |