Table 1.
Characteristics of patient cohort | Responders (n = 14) | IQR | Non-responders (n = 10) | IQR | p value |
---|---|---|---|---|---|
Female sex | 9 | 3 | 0.24 | ||
Median age, years | 41.0 | 35.0–46.0 | 40.0 | 32.0–44.0 | 0.4 |
Median age at diagnosis, years | 2.8 | 1.9–11.5 | 0.3 | 0.1–0.5 | 0.04 |
BMI (Kg/m2) | 21.6 | 20.9–22.7 | 21.9 | 18.9–24.8 | 0.8 |
Lung disease status | |||||
Mild FEV1 (≥70%) | 1 | 1 | |||
Moderate FEV1 (40–70%) | 7 | 3 | |||
Severe FEV1 (≤40%) | 6 | 6 | |||
aMedian FEV1 (%) predicted at initiation | 46.0 | 33.0–57.0 | 38.5 | 33.0–50.0 | 0.4 |
aMedian FEV1 (%) predicted post-initiation | 45.0 | 35.0–57.0 | 33.5 | 30.0–48.0 | 0.2 |
Genotype | |||||
ΔF508 homozyogous | 8 | 6 | |||
ΔF508 heterozygous | 3 | 3 | |||
CF related co-morbidities | |||||
Pancreatic insufficiency | 12 | 10 | |||
CF-related diabetes | 4 | 4 | |||
Impaired glucose tolerance | 5 | 3 | |||
Liver disease | 1 | 1 | |||
Bone disease | 5 | 3 | |||
Sinus disease | 6 | 3 | |||
Recurrent distal intestinal obstructive syndrome (DIOS) | 4 | 1 | |||
Cultured Pathogens | |||||
P. aeruginosa | 14 | 10 | |||
E. coli | 1 | 0 | |||
Methicillin susceptible S. aureus | 0 | 3 | |||
Group B streptococcus Spp. | 1 | 0 |
aRepresents the median of all spirometry values recorded in the year including those where a pulmonary exacerbation occurred