Table 1.
Sample | CFTR genotype | CFTR mutation class | Neutrophil elastase (nM) | Bronchiectasis (0–12 score) | Respiratory pathogens |
---|---|---|---|---|---|
CF1 | p.Gly85Glu/Unknown | –/– | 100 | 0 | Mixed oral flora |
CF2 | p.Phe508del/p.Phe508del | II/II | 1260 | n/a | None |
CF3 | p.Phe508del/p.Phe508del | II/II | 270 | n/a | Moraxella catarrhalis, Haemophilus influenzae, MRSA, Mixed oral flora |
CF4 | p.Phe508del/p.Asn1303Lys | II/II | 270 | n/a | Aspergillus fumigatus, Stenotrophomonas maltophilia |
CF5 | p.Phe508del/p.Thr966ArgfsX2 | #N/A | 170 | n/a | None |
CF6 | p.Phe508del/Ala455Glu | #N/A | 70 | n/a | H. influenzae |
CF7 | p.Phe508del/p.Phe508del | II/II | 140 | n/a | Streptococcus pneumoniae |
CF8 | p.Phe508del/p.Phe508del | II/II | 60 | n/a | None |
CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; MRSA, methicillin-resistant Staphylococcus aureus.