Table 1

Subject characteristics (n=62)

Cystic fibrosis (CF) (n=46)Controls (n=16)p Value
Sex, F:M27:1910:60.789
Age, years7.8 (0.2–16.8)6.3 (0.2–16.4)0.334
FEV1, % predicted*53 (29–84)98 (61–131)0.001
FVC, % predicted*74 (32–99)93 (85–137)0.006
CFTR genotype (n, %)F508del/F508del (n=27, 59%); F508del/G542X (n=2, 4%); F508del/1717-1G>A (n=2, 4%); F508del/other (n=6, 13%); other or unknown (n=9, 20%).NDND
Indication for bronchoscopy (n)

  • Microbiological surveillance at the time of chest exacerbation† (n=23)

  • Routine microbiological surveillance at the time of new CF diagnosis; child well (n=8) or during chest exacerbation (n=7)

  • Microbiological surveillance at the time of line or gastrostomy insertion or removal; child well (n=2) or during chest exacerbation (n=6)

  • Investigation of stridor (n=5; laryngo- and/or tracheomalacia found in 4 cases, double aortic arch found in one case)

  • Investigation of recurrent croup (n=4; laryngo- and/or tracheomalacia found in 2 cases, large adenoids and tonsils found in 1 case, no pathology found in one case)

  • Investigation of noisy breathing (n=2; no pathology found in either case, diagnosis of vocal cord dysfunction made in one case)

  • Investigation of haemoptysis (n=2; no pathology found in either case)

  • Investigation of recurrent cough (n=1; tracheomalacia found)

  • Confirm diagnosis of vascular ring (n=1)

  • Research bronchoscopy at the time of cardiac catheterisation (n=1)

ND
Pathogens in BAL fluid

  • Viruses:

  • Parainfluenza virus 3 (n=2; 4%)

  • Bacteria:

  • Pseudomonas aeruginosa (n=11; 24%)

  • Other (n=6; 13%)‡

  • Fungi:

  • Aspergillus (n=13; 28%)§

  • Candida albicans (n=4; 9%)

No virus detection, no bacterial growthND

  • Values expressed as median (range) unless otherwise indicated.

  • ↵* Data available for 29 children with CF and six control children.

  • ↵† Chest exacerbation based on the decision to hospitalise and treat with intravenous antibiotics.26

  • ↵‡ Staphylococcus aureus (n=2), Haemophilus influenzae (n=1), Mycobacterium chelonae (n=2), Stenotrophomonas maltophilia (n=1).

  • ↵§ Aspergillus fumigatus (n=12), Aspergillus niger (n=1).

  • BAL, bronchoalveolar lavage; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; ND, not determined.