Table 2. Comparison of three groups of patients (those in whom two or more mutations were identified, those in whom one mutation was identified, and those in whom no mutations were identified) in terms of their characteristics. a .
| Variables | No. of mutations | p* | ||||
|---|---|---|---|---|---|---|
| ≥ 2 | 1 | 0 | ||||
| (n = 4) | (n = 7) | (n = 26) | ||||
| Gender | 0.242 | |||||
| Male | 1 (25.0) | 0 (0.0) | 8 (30.8) | |||
| Female | 3 (75.0) | 7 (100.0) | 18 (69.2) | |||
| Age, years b | 30.80 ± 10.90 | 30.00 ± 9.95 | 33.00 ± 14.43 | 0.851 | ||
| Race | ||||||
| White | 4 (100.0) | 7 (100.0) | 25 (96.2) | 0.805 | ||
| Non-White | 0 (0.0) | 0 (0.0) | 1 (3.8) | |||
| BMI, b kg/m2 | 22.9 ± 3.7 | 23.3 ± 2.3 | 22.2 ± 4.3 | 0.801 | ||
| Family history of CF | 3 (75.0) | 2 (28.6) | 11 (42.3) | 0.322 | ||
| Exocrine pancreatic insufficiency | 2 (50.0) | 0 (0.0) | 3 (11.5) | 0.570 | ||
| Digital clubbing | 0 (0.0) | 0 (0.0) | 8 (30.8) | 0.115 | ||
| OGTT | ||||||
| Glucose intolerance | 0 (0.0) | 2 (28.6) | 4 (15.4) | 0.624 | ||
| Diabetes | 0 (0.0) | 1 (14.3) | 2 (7.7) | |||
| Chronic sinus disease | 2 (50.0) | 4 (57.1) | 7 (26.9) | 0.266 | ||
| Sputum bacteriology | ||||||
| Pseudomonas aeruginosa | 1 (25.0) | 5 (71.4) | 10 (38.5) | 0.218 | ||
| Mucoid P. aeruginosa | 1 (25.0) | 3 (42.9) | 3 (11.5) | 0.162 | ||
| Nonmucoid P. aeruginosa | 0 (0.0) | 3 (42.9) | 7 (26.9) | 0.306 | ||
| Staphylococcus aureus | 3 (75.0) | 3 (42.9) | 7 (26.9) | 0.154 | ||
| Methicillin-sensitive S. aureus | 2 (50.0) | 3 (42.9) | 7 (26.9) | 0.530 | ||
| Methicillin-resistant S. aureus | 1 (25.0) | 1 (14.3) | 0 (0.0) | 0.062 | ||
| Burkholderia cepacia | 0 (0.0) | 0 (0.0) | 2 (7.7) | 0.639 | ||
| Haemophilus influenzae | 0 (0.0) | 1 (14.3) | 6 (23.1) | |||
| Shwachman-Kulczycki score c | 80 (32.5) | 75 (20.0) | 70 (31.3) | 0.748 | ||
| Liver score c | 3 (0) | 3 (0) | 3 (0) | 0.399 | ||
| Presence of bronchiectasis | 2 (50.0) | 6 (85.7) | 19 (73.1) | 0.439 | ||
| FEV1, L b | 3.01 ± 0.71 | 2.44 ± 0.70 | 2.20 ± 1.13 | 0.317 | ||
| FEV1, % of predicted b | 89.7 ± 26.0 | 72.2 ± 14.7 | 68.2 ± 29.0 | 0.338 | ||
| FVC, L b | 3.7 ± 0.4 | 3.2 ± 0.5 | 2.9 ± 1.2 | 0.359 | ||
| FVC, % of predicted b | 95.5 ± 16.1 | 81.7 ± 6.2 | 78.5 ± 23.2 | 0.320 | ||
| FEV1/FVC b | 80.3 ± 12.5 | 74.8 ± 14.1 | 73.7 ± 19.0 | 0.785 | ||
| Sweat test b | ||||||
| Sample 1 | ||||||
| Sodium, mEq/L | 56.8 ± 20.3 | 59.14 ± 27.3 | 56.5 ± 28.0 | 0.973 | ||
| Chloride, mEq/L | 53.8 ± 15.7 | 61.3 ± 23.10 | 51.34 ± 27.7 | 0.674 | ||
| Sample 2 | ||||||
| Sodium, mEq/L | 53.3± 10.3 | 56.4 ± 27.8 | 58.1 ± 22.2 | 0.941 | ||
| Chloride, mEq/L | 49.7 ± 10.50 | 52.2 ± 26.0 | 52.2 ± 24.4 | 0.985 | ||
| Sweat test result | ||||||
| Normal | 1 (25.0) | 1 (14.3) | 12 (46.2) | 0.306 | ||
| Borderline | 2 (50.0) | 3 (42.9) | 4 (15.4) | |||
| Abnormal | 1 (25.0) | 3 (42.9) | 10 (38.5) | |||
CF
: cystic fibrosis
BMI
: body mass index
OGTT
: oral glucose tolerance test
a
Values expressed as n (%), except where otherwise indicated.
b
Values expressed as mean ± SD.
c
Values expressed as median (interquartile range). *Oneway ANOVA for continuous variables with normal distribution or Kruskal-Wallis test (ordinal variables or continuous variables without normal distribution); chi-square test (categorical variables), Yates' correction or Fisher's exact test being used when necessary