Variable | ACT deficiency (n=10) | Normal ACT (n=147) | p value | Alanine (GG) (n=26) | Threonine (AA) (n=8) | Ala/Thr (GA) (n=17) | p value |
Age in years (SD) | 22.4 (9.8) | 23.9 (7.5) | 0.56 | 25.1 (8.3) | 26.8 (6.8) | 25.5 (7.3) | 0.87 |
Sex | |||||||
Male | 6 (60%) | 87 (59%) | 1.0 | 14 (54%) | 5 (63%) | 9 (53%) | 0.89 |
Female | 4 (40%) | 60 (41%) | 12 (46%) | 3 (37%) | 8 (47%) | ||
CF genotype | |||||||
ΔF508/ΔF508 | 7 (70%) | 79 (59%) | 0.75 | 14 (54%) | 7 (87%) | 13 (81%) | 0.11 |
ΔF508/other | 2 (20%) | 42 (32%) | 10 (38%) | 1 (13%) | 1 (6%) | ||
Non ΔF508/non ΔF508 | 1 (10%) | 12 (9%) | 2 (8%) | 0 | 2 (13%) | ||
Liver disease | |||||||
No liver disease | 9 (90%) | 125 (85%) | 1.0 | 23 (88%) | 6 (75%) | 12 (71%) | 0.32 |
Liver disease | 1 (10%) | 22 (15%) | 3 (12%) | 2 (25%) | 5 (29%) | ||
Pancreatic status | |||||||
Insufficient | 9 (90%) | 138 (94%) | 0.49 | 23 (88%) | 8 (100%) | 16 (94%) | 0.53 |
Sufficient | 1 (10%) | 9 (6%) | 3 (12%) | 0 | 1 (6%) | ||
P aeruginosa | |||||||
Not present | 7 (70%) | 28 (19%) | 0.001 | 8 (31%) | 2 (25%) | 3 (18%) | 0.63 |
Present | 3 (30%) | 119 (81%) | 18 (69%) | 6 (75%) | 14 (82%) | ||
B cepacia | |||||||
Not present | 9 (90%) | 122 (83%) | 1.0 | 21 (81%) | 7 (87%) | 12 (71%) | 0.58 |
Present | 1 (10%) | 25 (17%) | 5 (19%) | 1 (13%) | 5 (29%) | ||
Smoking status | |||||||
Non-smoker | 9 (100%) | 134 (91%) | 1.0 | 24 (92%) | 6 (86%) | 14 (82%) | 0.61 |
Current or ex-smoker | 0 | 13 (9%) | 2 (8%) | 1 (14%) | 3 (18%) | ||
Centre | |||||||
Manchester | 4 (40%) | 96 (65%) | 0.17 | 7 (27%) | 2 (25%) | 2 (12%) | 0.48 |
East Anglia | 6 (60%) | 51 (35%) | 19 (73%) | 6 (75%) | 15 (88%) | ||
Body mass index (SD) | 21.5 (1.66) | 20.1 (2.8) | 0.19 | 19.9 (2.8) | 23.1 (3.1) | 21.9 (2.8) | 0.02 |
α1-antitrypsin phenotype | |||||||
Normal | 8 (80%) | 125 (86%) | 0.64 | 22 (85%) | 5 (63%) | 16 (94%) | 0.13 |
Deficient | 2 (20%) | 21 (14%) | 4 (15%) | 3 (37%) | 1 (6%) |
Comparisons were made by one way analysis of variance and Fisher's exact test for frequencies. In the normal ACT group 14 patients did not have CF genotyping, one did not have α1-antitrypsin phenotyping, and in another the smoking status was not known. ACT = α, – antichymotrypsin.