Clinical features the two groups of CF patients
| Genotype | |||||
|---|---|---|---|---|---|
| 3272-26A>G/any (n=60) | F508del/F508del (n=89) | ||||
| Median (QD) | Mean (SD) | Median (QD) | Mean (SD) | ||
| Sex (male / female) | 35/24 (n=59) | 48/41 (n=89) | |||
| Age at diagnosis (y) | 12.3 (13.3) | 8.0 (20.0) | 3.8 (6.6) | 1.0 (4.7) | |
| (n=55) | (n=88) | ||||
| Sweat test (mEq/l) | 99.4 (22.7) | 99.5 (24.7) | 107.3 (19.4) | 105.0 (24.0) | |
| (n=42) | (n=63) | ||||
| FEV1, % predicted | 79.7 (27.7) | 87.0 (47.0) | 62.7 (30.2) | 63.0 (50.0) | |
| (n=43) | (n=67) | ||||
| FVC, % predicted | 93.0 (24.3) | 88.3 (22.6) | 78.5 (37.0) | 72.5 (26.2) | |
| (n=42) | (n=66) | ||||
| Lung colonisation with bacterial pathogens2-150 | |||||
| Pa (yes/no) | 18/34 (n=52) | 67/18 (n=85) | |||
| Bc (yes/no) | 1/51 (n=52) | 5/80 (n=85) | |||
| Hi (yes/no) | 7/45 (n=52) | 8/77 (n=85) | |||
| Sa (yes/no) | 17/35 (n=52) | 28/57 (n=85) | |||
| Other (yes/no) | 2/50 (n=52) | 8/78 (n=85) | |||
| All-Pa (yes/no) | 22/30 (n=52) | 37/48 (n=85) | |||
| Pancreatic function (PS/PI) | 39/16 (n=55) | 9/79 (n=88) | |||
| Meconium ileus (yes/no) | 0/54 (n=54) | 3/83 (n=86) | |||
| Centile - height | 50.0 (42.9) | 46.6 (30.7) | 25.0 (40.0) | 34.5 (29.3) | |
| (n=22) | (n=36) | ||||
| Centile - weight | 50.0 (59.4) | 47.0 (30.0) | 25.0 (40.0) | 30.9 (26.0) | |
| (n=22) | (n=36) | ||||
| BMI | 22.0 (3.5) | 21.2 (2.6) | 19.0 (4.5) | 19.6 (2.6) | |
| (n=25) | (n=41) | ||||
| CN score | 13.8 (20.0) | 7.0 (12.0) | 11.9 (9.1) | 10.0 (14.8) | |
| (n=19) | (n=34) | ||||
| SK score | 81.8 (17.7) | 90.0 (15.7) | 78.6 (16.1) | 80.0 (18.0) | |
| (n=34) | (n=53) | ||||
| Nasal polyposis (yes/no) | 19/32 (n=51) | 6/62 (n=68) | |||
| Other clinical features (yes/no) | 22/19 (n=41) | 35/30 (n=65) | |||
↵2-150 Bc, Burkholderia cepacia; Hi,Haemophilus influenzae; Pa,Pseudomonas aeruginosa; Sa,Staphylococcus aureus; All-Pa, all bacterial pathogens except for Pa; BMI, body mass index; CK, Chrispin-Norman score; FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; PI, pancreatic insufficient; PS, pancreatic sufficient; SK, Shwachman-Kulczycki score.