Patient characteristics (N = 17) | Number (percentage) or median value [min-max] |
---|---|
Male gender | 13 (76.5%) |
Age (years) | 19 [6–34] |
CFTR genotype: F508del/F508del | 3 (17.6%) |
F508del/other or unknown | 12 (70.6%) |
Other or unknown | 2 (11.8%) |
BMI (Z-score) | −0.76 [−2.81–0.39] |
Insulin-dependent diabetes | 3 (17.6%) |
Exocrine pancreatic insufficiency | 16 (94.1%) |
Nutritional support (gastrostomy/enteral tube) | 1 (5.9%) |
Liver cirrhosis | 1 (5.9%) |
ABPA | 3 (17.6%) |
Respiratory function tests | |
Median FEV1 (% predicted) | 83.0 [26–113] |
Median FVC (% predicted) | 92.0 [36–109] |
Burkholderiaspecies | |
BCC | 14 (82.4%) |
B. multivorans | 6 (35.3%) |
B. cenocepaciaa | 4 (23.5%) |
B. contaminans | 1 (5.9%) |
B. vietnamiensis | 1 (5.9%) |
B. ambifaria | 1 (5.9%) |
Unidentified BCCb | 1 (5.9%) |
B. gladioli | 3 (17.6%) |
Co-pathogens | |
Methi-S Staphylococcus aureus | 7 (41.2%) |
Methi-R Staphylococcus aureus | 5 (29.4%) |
Pseudomonas aeruginosa | 6 (35.3%) |
Fungi | 4 (23.5%) |
Stenotrophomonas maltophilia | 3 (17.6%) |
Non-tuberculous Mycobacteria | 2 (11.8%) |
Achromobacter spp. | 0 (0%) |