Demographic and clinical characteristics comparing all the children who participated in ACFBAL and those who participated in the follow up CF-FAB study
| Characteristics | ACFBAL (n=168) | CF-FAB (n=99) |
| At ACFBAL enrolment | ||
| Patients assessed n | 168 | 99 |
| Age months | 3.7±1.7 | 3.6±1.6 |
| Males | 88 (52) | 51 (52) |
| Homozygous CFTR Phe508 deletion | 111 (66) | 69 (70) |
| Pancreatic insufficient | 144 (86) | 84 (85) |
| Meconium ileus | 32 (19) | 24 (24) |
| Weight kg | 5.7±1.4 | 5.5±1.5 |
| Weight z-score | −0.77±1.1 | −0.87±1.2 |
| Weight z-score <−2 sd | 23 (14) | 17 (17) |
| Sites | ||
| New Zealand | 26 (15) | 17 (17) |
| Australia | ||
| Queensland (two centres) | 60 (36) | 44 (44) |
| Victoria (two centres) | 40 (24) | 17 (17) |
| New South Wales (two centres) | 37 (22) | 21 (21) |
| South Australia | 5 (3.0) | 0 (0) |
| Highest education level of either parent | ||
| Patients assessed n | 156 | 92 |
| Completed year 10 | 24±15 | 12±13 |
| Completed secondary education | 39±25 | 23±25 |
| Trade | 76±49 | 45±49 |
| Completed tertiary education | 17±11 | 12±13 |
| Exposure to environmental tobacco smoke | ||
| During pregnancy | 35±21 | 19±19 |
| Concurrent smoking in the household | n=167; 53 (32) | n=98; 29 (30) |
| Preterm delivery (<37-weeks) | n=166; 17 (10) | n=98; 12 (12) |
| Presence of respiratory symptoms | ||
| Cough | 73 (43) | 46 (46) |
| Wheeze | n=166; 17 (10) | n=97; 10 (10) |
| At ACFBAL final outcome | ||
| Patients assessed n | 157 | 99 |
| Age years | 5.1±0.19 | 5.1±0.13 |
| Weight z-score | −0.18±0.85 | −0.19±0.75 |
| BMI z-score | 0.02±0.88 | −0.02±0.76 |
| FEV1 z-score | n=127; −0.58±1.3 | n=81; −0.59±1.3 |
| Pathogenic bacterial counts in BAL ≥103 CFU·mL−1 | ||
| Patients assessed n | 151 | 95 |
| None | 49 (32) | 27 (28) |
| Pseudomonas aeruginosa | 16 (11) | 15 (16) |
| Other bacterial pathogens | 86 (57) | 53 (56) |
| During ACFBAL study | ||
| Ever had Pseudomonas aeruginosa eradication | 83 (53) | 56 (57) |
| At follow-up CT scan | (n=99) | |
| Age years | N/A | 13±1.5 |
| Weight z-score | N/A | −0.32±0.71 |
| BMI z-score | N/A | −0.20±0.68 |
| FEV1 z-score | N/A | −1.4±1.4 |
| Pathogenic bacterial counts in sputum ≥103 CFU·mL−1 | N/A | |
| None | 63 (64) | |
| Pseudomonas aeruginosa | 9 (9.1) | |
| Other bacterial pathogens | 27 (27) |
Data are presented as mean±sd or n (%), unless otherwise stated. ACFBAL: Australasian Cystic Fibrosis Bronchoalveolar Lavage; BAL study: bronchoalveolar lavage; BMI: body-mass index; CF-FAB: Cystic Fibrosis-Follow-up of the ACFBAL study; CFTR: cystic fibrosis transmembrane conductance regulator; CFU: colony-forming units; CT: computed tomography; FEV1: forced expiratory volume in 1 s; N/A: not applicable.