Combined | Horsley [13, 36, 37] | O’Neill [8, 9] | Ratjen# | Robinson [27, 38] | Scrase [39] | Welsh [29] | Yammine [30] | |
Exacerbations n | 176 | 32 | 21 | 31 | 34 | 16 | 21 | 21 |
Age at admission years | 15 (5–56) | 23 (11–44) | 24 (12–56) | 14 (7–18) | 14 (8–17) | 11 (8–17) | 17 (9–20) | 13 (5–19) |
Male | 76 (43.2) | 20 (62.50) | 10 (47.6) | 11 (35.5) | 12 (35.3) | 5 (31.3) | 9 (42.9) | 9 (42.9) |
LCI at admission | 12.5 (6.9–21.7) | 15.0 (10.7–21.7) | 11.9 (7.7–19.8) | 12.4 (9.1–19.0)¶ | 9.9 (6.9–14.8) | 14.0 (10.2–18.8) | 12.8 (8.4–17.9) | 13.7 (8.3–19.4) |
FEV1 at admission % pred | 59.2 (17.9–105.4) | 53.2 (32.1–86.2) | 45.4 (17.9–93.4) | 59.2 (28.4–103.1) | 67.2 (25.1–95.4) | 48.0 (22.6–105.4) | 64.1 (18.6–95.1) | 74.1 (54.2–104.6) |
Treatment duration days | 14 (8–31) | 14 (9–22) | 15 (8–20) | 14 (11–31) | 15 (12–20) | N/A | 13 (8–18) | 14 (10–28) |
Time between tests days | 12 (7–29) | 13 (7–21) | 13 (7–16) | 12 (8–16) | 13 (9–14) | 11.5 (8–18) | 10 (7–14) | 13 (7–29) |
Complications | ||||||||
Pancreatic insufficiency | 151 (94.4) | 29 (90.6) | 21 (100) | 29 (93.6) | 33 (97.1) | N/A | 18 (85.7) | 21 (100) |
CFRD | 25 (18.0) | 11 (34.4) | 4 (19.1) | 2 (6.5) | 5 (14.7) | N/A | N/A | 3 (14.3) |
ABPA | 14 (10.53) | 1 (3.1) | 0 | 1 (3.3) | 6 (17.6) | N/A | N/A | 6 (28.6) |
Microbiology | ||||||||
P. aeruginosa | 89 (64.0) | 21 (65.6) | 13 (81.3) | 18 (58.1) | 33 (97.1) | N/A | 6 (28.6) | 11 (52.4) |
S. aureus | 78 (66.7) | 16 (50.0) | 9 (56.3) | 26 (86.7) | 18 (52.9) | N/A | N/A | 18 (85.7) |
MRSA | 8 (6.8) | 3 (9.4) | 1 (6.3) | 2 (6.5) | 3 (8.8) | N/A | N/A | 0 (0) |
H. influenzae | 32 (27.4) | 5 (15.6) | 1 (6.3) | 15 (50.0) | 3 (8.8) | N/A | N/A | 9 (42.9) |
B. cepacia complex | 10 (8.5) | 7 (21.9) | 2 (12.5) | 0 (0) | 2 (5.9) | N/A | N/A | 1 (4.8) |
S. maltophilia | 30 (25.6) | 7 (21.9) | 2 (12.5) | 11 (36.7) | 2 (5.9) | N/A | N/A | 10 (47.6) |
Data are presented as median (range) or n (%), unless otherwise stated. LCI: lung clearance index; FEV1: forced expiratory volume in 1 s; % pred: % predicted; CFRD: cystic fibrosis-related diabetes; ABPA: allergic bronchopulmonary aspergillosis; P. aeruginosa: Pseudomonas aeruginosa; S. aureus: Staphylococcus aureus; MRSA: methicillin-resistant S. aureus; H. influenzae: Haemophilus influenzae; B. cepacia: Burkholderia cepacia; S. maltophilia: Stenotrophomonas maltophilia; N/A: not available. #: unpublished data. ¶: LCI measured using both sulfur hexafluoride (SF6) and nitrogen (N2) in 16 subjects; LCI is presented from the subjects that completed SF6 measurements; the corresponding median (range) for the N2 measurements was 14.8 (9.2–22.2).