Online Resource 4. Pancreatic Insufficiency As Clinical Determinant of Cystic Fibrosis

Online resource 4. Pancreatic insufficiency as clinical determinant of cystic fibrosis severity considering IL8 (rs4073, rs2227306 and rs2227307) variants and CFTR genotype.

Table 1.CFTR mutations groups and pancreatic insufficiency status in cystic fibrosis patients.
CFTR genotype / Pancreatic insufficiency / P-value / Odds ratio / 95% confidence interval
Presence / Absence / Total
G2 / 76 / 5 / 81 / < 0.001 / 17.82 / 5.69 to 68.08
G1 / 35 / 4 / 39 / 10.26 / 2.92 to 46.90
G0 / 19 / 23 / 42 / 1* / -
Total / 130 / 32 / 162

CFTR, cystic fibrosis transmembrane regulator G2, CFTR mutations patients with two Class I, II and/or III; G1, patients with one Class I, II or III CFTR mutation, and one non-identified mutation or IV, V or VI; G0, patients without mutation in the CFTR gene, or with one or two Class IV, V or VI. * Reference group. Odds ratio was calculated using the value of Fisher's exact test. Significant data are shown in bold type. Alpha=0.05.

Table 2. P-value for rs4073 polymorphism of the IL8 gene, considering patients with pancreatic insufficiency and pancreatic sufficiency, compared with clinical markers of the disease.
Variables / Co-dominant
AA x AT x TT / Dominant
TT x TA + AA / Recessive
AA x TT + TA / Over-dominant
TA x AA + TT
PI / PS / PI / PS / PI / PS / PI / PS
Gender / 0.401 / 0.101 / 0.706 / 0.691 / 0.333 / 0.062 / 0.215 / 0.166
Ethnicity / 0.899 / 0.950 / 0.697 / 1 / 1 / 1 / 1 / 1
Age / 0.907 / 0.689 / 0.693 / 0.575 / 1 / 1 / 0.855 / 1
Symptoms onset / 0.367 / 0.848 / 0.841 / 1 / 0.304 / 1 / 0.192 / 1
Diagnosis / 0.421 / 0.680 / 0.322 / 1 / 1 / 0.592 / 0.266 / 0.606
Onset of digestive symptoms / 0.928 / 0.267 / 0.845 / 0.318 / 0.840 / 1 / 1 / 0.470
Onset of pulmonary symptoms / 0.373 / 0.338 / 0.310 / 1 / 1 / 0.628 / 0.257 / 0.187
Body mass index / 0.397 / 0.457 / 0.511 / 0.550 / 0.514 / 0.593 / 0.225 / 1
Nasal polyposis / 0.984 / 0.930 / 1 / 1 / 1 / 1 / 1 / 1
Diabetes mellitus / 0.379 / 0.741 / 0.477 / 1 / 0.630 / 0.593 / 0.187 / 0.629
Osteoporosis / 0.788 / 0.214 / 0.590 / 0.296 / 1 / 1 / 0.802 / 0.194
Meconium ileus / 0.984 / 0.470 / 1 / 1 / 1 / 1 / 1 / 0.406
1st Pseudomonas aeruginosa / 0.956 / 0.504 / 0.830 / 1 / 1 / 1 / 1 / 0.438
Mucoid P. aeruginosa / 0.809 / 0.445 / 0.570 / 0.397 / 1 / 1 / 0.597 / 0.450
Non-mucoid P. aeruginosa / 0.453 / 0.559 / 0.693 / 0.453 / 0.422 / 1 / 0.267 / 0.491
Achromobacter xylosoxidans / 0.285 / 0.934 / 0.342 / 1 / 1 / 1 / 0.148 / 1
Burkolderia cepacia / 0.181 / - / 0.085 / - / 0.459 / - / 0.371 / -
Staphylococcus aureus / 0.070 / 0.530 / 0.141 / 0.684 / 0.043 / 0.450 / 0.819 / 0.720
Staphylococcus aureus (corrected) / 0.140 / - / 0.188 / - / 0.140 / - / 0.819 / -
SaO2 c / 0.201 / 0.143 / 0.331 / 0.639 / 0.044 / 0.246 / 0.105 / 0.067
SaO2 c(corrected) / 0.268 / - / 0.331 / - / 0.176 / - / 0.210 / -
SaO2 n / 0.171 / 0.523 / 0.177 / 0.546 / 0.519 / 0.550 / 0.066 / 0.283
Bhalla / 0.538 / 0.986 / 0.879 / 1 / 0.336 / 0.896 / 0.336 / 0.872
Kanga / 0.522 / 0.853 / 0.257 / 0.769 / 0.579 / 0.833 / 0.590 / 0.710
Shwachman-Kulczycki / 0.066 / 0.548 / 0.021 / 0.771 / 0.555 / 0.382 / 0.100 / 0.282
Shwachman-Kulczycki(corrected) / 0.132 / - / 0.084 / - / 0.555 / - / 0.133 / -
FVC / 0.247 / 0.693 / 0.637 / 0.811 / 0.203 / 0.553 / 0.112 / 0.415
FEV1 / 0.085 / 0.900 / 0.257 / 0.701 / 0.197 / 1 / 0.027 / 0.723
FEV1(corrected) / 0.170 / - / 0.257 / - / 0.257 / - / 0.108 / -
FEV1/FVC / 0.087 / 0.121 / 0.057 / 0.042 / 0.794 / 0.276 / 0.044 / 0.521
FEV1/FVC(corrected) / 0.116 / - / 0.114 / - / 0.794 / - / 0.114 / -
FEF25% / 0.302 / 0.797 / 0.165 / 0.600 / 0.932 / 0.681 / 0.166 / 1
FEF50% / 0.051 / 0.697 / 0.045 / 0.600 / 0.615 / 0.470 / 0.020 / 0.758
FEF50%(corrected) / 0.068 / - / 0.068 / - / 0.615 / - / 0.068 / -
FEF75% / 0.061 / 0.535 / 0.037 / 0.462 / 0.829 / 0.534 / 0.031 / 1
FEF75%(corrected) / 0.081 / - / 0.074 / - / 0.829 / - / 0.074 / -
FEF25-75% / 0.030 / 0.688 / 0.049 / 0.441 / 0.410 / 1 / 0.010 / 0.518
FEF25-75%(corrected) / 0.060 / - / 0.653 / - / 0.410 / - / 0.040 / -
FEFMax / 0.400 / 0.425 / 0.381 / 0.333 / 0.538 / 0.639 / 0.176 / 0.933
ERV / 0.353 / 0.207 / 0.183 / 1 / 0.993 / 0.106 / 0.216 / 0.109

PI, pancreatic insufficiency; PS, pancreatic sufficiency; SaO2, transcutaneous arterial hemoglobin oxygen-saturation; FVC, forced vital capacity; FEV1, forced expiratory volume in 1 second of FVC; FEF25, forced expiratory flow at 25% of FVC; FEF50, forced expiratory flow at 50% of FVC; FEF75, forced expiratory flow at 75% of FVC; FEF25-75%, average forced expiratory flow between 25% and 75% of FVC; FEFmax, maximum forced expiratory flow; ERV, expiratory reserve volume. Statistical analysis of categorical data was performed by χ2 test and the Fisher´s exact test. Numerical data were analyzed using the Kruskal-Wallis test and the Mann-Whitney test. The data with positive values are presented in in bold type. The corrected p-value was achieved by false rate discovery test for multiple comparisons.

Table 3. P-value for rs2227306 polymorphism of the IL8 gene, with pancreatic insufficiency and pancreatic sufficiency, compared with clinical markers of the disease.
Variables / Co-dominant
AA x AT x TT / Dominant
TT x TA + AA / Recessive
AA x TT + TA / Over-dominant
TA x AA + TT
PI / PS / PI / PS / PI / PS / PI / PS
Gender / 0.912 / 0.038 / 0.720 / 0.046 / 0.841 / 0.086 / 1 / 0.730
Gender (corrected) / - / 0.092 / - / 0.092 / - / 0.115 / - / 0.730
Ethnicity / 0.715 / 0.470 / 0.474 / 0.532 / 1 / 1 / 0.709 / 0.573
Age / 0.676 / 0.315 / 0.852 / 1 / 0.406 / 0.289 / 0.710 / 0.372
Symptoms onset / 0.254 / 0.465 / 0.186 / 0.558 / 1 / 0.561 / 0.128 / 1
Diagnosis / 0.597 / 0.089 / 0.580 / 0.065 / 0.391 / 0.545 / 0.851 / 0.596
Onset of digestive symptoms / 0.867 / 0.186 / 0.712 / 0.152 / 0.673 / 1 / 1 / 0.455
Onset of pulmonary symptoms / 0.464 / 0.876 / 0.257 / 1 / 0.829 / 1 / 0.339 / 1
Body mass index / 0.673 / 0.072 / 0.538 / 0.069 / 0.493 / 1 / 1 / 0.103
Nasal polyposis / 0.747 / 0.343 / 0.628 / 1 / 0.590 / 0.306 / 1 / 0.399
Diabetes mellitus / 0.705 / 0.868 / 0.501 / 1 / 0.806 / 1 / 0.650 / 1
Osteoporosis / 0.538 / 0.910 / 0.443 / 1 / 1 / 1 / 0.441 / 1
Meconium ileus / 0.617 / 0.554 / 0.472 / 1 / 0.591 / 1 / 1 / 0.467
1st Pseudomonas aeruginosa / 0.423 / 0.668 / 0.229 / 1 / 0.813 / 1 / 0.307 / 1
Mucoid P. aeruginosa / 0.304 / 0.038 / 0.153 / 0.030 / 1 / 0.372 / 0.209 / 0.260
Mucoid P. aeruginosa (corrected) / - / 0.076 / - / 0.076 / - / 0.3720 / - / 0.347
Non-mucoid P. aeruginosa / 0.406 / 0.941 / 0.267 / 1 / 1 / 1 / 0.260 / 1
Achromobacter xylosoxidans / 0.979 / 0.149 / 1 / 0.534 / 1 / 0.128 / 1 / 1
Burkolderia cepacia / 0.945 / - / 1 / - / 0.799 / - / 1 / -
Staphylococcus aureus / 0.191 / 0.372 / 0.363 / 0.687 / 0.313 / 0.215 / 0.100 / 0.707
SaO2 c / 0.230 / 0.061 / 0.103 / 0.033 / 0.417 / 1 / 0.463 / 0.128
SaO2 n / 0.255 / 0.090 / 0.118 / 0.036 / 0.817 / 0.240 / 0.172 / 0.430
SaO2 n (corrected) / - / 0.180 / - / 0.144 / - / 0.320 / - / 0.430
Bhalla / 0.099 / 0.659 / 0.177 / 0.791 / 0.360 / 0.517 / 0.032 / 0.426
Bhalla (corrected) / 0.198 / - / 0.236 / - / 0.360 / - / 0.128 / -
Kanga / 0.966 / 0.471 / 0.796 / 0.570 / 0.947 / 0.485 / 0.846 / 0.240
Shwachman-Kulczycki / 0.034 / 0.604 / 0.027 / 0.841 / 0.810 / 0.335 / 0.016 / 0.579
Shwachman-Kulczycki(corrected) / 0.045 / - / 0.045 / - / 0.810 / - / 0.045 / -
FVC / 0.105 / 0.852 / 0.276 / 0.601 / 0.256 / 0.717 / 0.035 / 0.892
FVC (corrected) / 0.210 / - / 0.276 / - / 0.276 / - / 0.140 / -
FEV1 / 0.067 / 0.920 / 0.442 / 0.823 / 0.110 / 0.717 / 0.026 / 0.892
FEV1(corrected) / 0.134 / - / 0.442 / - / 0.147 / - / 0.104 / -
FEV1/FVC / 0.057 / 0.786 / 0.658 / 0.549 / 0.022 / 0.646 / 0.104 / 0.905
FEV1/FVC(corrected) / 0.114 / - / 0.658 / - / 0.088 / - / 0.139 / -
FEF25% / 0.094 / 0.612 / 0.609 / 0.454 / 0.102 / 0.791 / 0.044 / 0.345
FEF25%(corrected) / 0.136 / - / 0.609 / - / 0.136 / - / 0.136 / -
FEF50% / 0.128 / 0.389 / 0.784 / 0.304 / 0.057 / 0.659 / 0.143 / 0.181
FEF75% / 0.130 / 0.798 / 0.525 / 0.570 / 0.046 / 0.909 / 0.239 / 0.589
FEF75%(corrected) / 0.260 / - / 0.525 / - / 0.184 / - / 0.319 / -
FEF25-75% / 0.071 / 0.960 / 0.888 / 0.823 / 0.048 / 0.836 / 0.054 / 0.964
FEF25-75%(corrected) / 0.095 / - / 0.888 / - / 0.095 / - / 0.095 / -
FEFMax / 0.194 / 0.751 / 0.349 / 1 / 0.346 / 0.582 / 0.071 / 0.662
ERV / 0.398 / 0.122 / 0.916 / 0.073 / 0.192 / 0.218 / 0.276 / 0.537

Table 4.P-value for rs2227307 polymorphism of the IL8 gene, with pancreatic insufficiency and pancreatic sufficiency, compared with clinical markers of the disease.
Variables / Co-dominant
AA x AT x TT / Dominant
TT x TA + AA / Recessive
AA x TT + TA / Over-dominant
TA x AA + TT
PI / PS / PI / PS / PI / PS / PI / PS
Gender / 0.996 / 0.330 / 1 / 1 / 1 / 0.246 / 1 / 0.276
Ethnicity / 0.777 / 0.468 / 1 / 1 / 0.710 / 0.535 / 1 / 1
Age / 0.025 / 0.270 / 0.468 / 0.218 / 0.008 / 0.352 / 0.064 / 1
Age (corrected) / 0.050 / - / 0.468 / - / 0.032 / - / 0.853 / -
Symptoms onset / 0.044 / 0.682 / 0.466 / 0.422 / 0.013 / 1 / 0.089 / 1
Symptoms onset (corrected) / 0.088 / - / 0.466 / - / 0.052 / - / 0.119 / -
Diagnosis / 0.287 / 0.518 / 0.238 / 0.422 / 0.709 / 1 / 0.193 / 0.593
Onset of digestive symptoms / 0.273 / 0.478 / 0.816 / 0.491 / 0.132 / 1 / 0.202 / 0.491
Onset of pulmonary symptoms / 0.451 / 0.529 / 1 / 0.521 / 0.334 / 0.358 / 0.258 / 0.630
Body mass index / 0.092 / 0.806 / 0.121 / 1 / 0.065 / 1 / 0.685 / 1
Nasal polyposis / 0.816 / 0.343 / 0.763 / 0.290 / 0.807 / 1 / 1 / 0.418
Diabetes mellitus / 0.233 / 0.584 / 0.570 / 1 / 0.110 / 1 / 0.269 / 0.560
Osteoporosis / 0.167 / 0.599 / 0.748 / 1 / 0.127 / 1 / 0.076 / 1
Meconium ileus / 0.777 / - / 0.549 / - / 0.631 / - / 1 / -
1st Pseudomonas aeruginosa / 0.285 / 0.532 / 0.609 / 1 / 0.143 / 1 / 0.411 / 0.462
Mucoid P. aeruginosa / 0.251 / 0.242 / 0.374 / 0.626 / 0.142 / 0.125 / 0.470 / 0.434
Non- mucoid P. aeruginosa / 0.652 / 0.330 / 1 / 1 / 0.452 / 0.246 / 0.458 / 0.276
Achromobacter xylosoxidans / 0.067 / 0.519 / 0.121 / 1 / 0.059 / 0.543 / 0.760 / 1
Burkolderia cepacia / 0.337 / - / 0.241 / - / 0.342 / - / 1 / -
Staphylococcus aureus / 0.225 / 0.011 / 0.564 / 0.626 / 0.106 / 0.041 / 0.368 / 0.005
Staphylococcus aureus (corrected) / - / 0.022 / - / 0.626 / - / 0.055 / - / 0.020
SaO2 c / 0.001 / 0.472 / 0.172 / 0.635 / <0.001 / 0.683 / 0.018 / 0.267
SaO2 c(corrected) / 0.002 / - / 0.172 / - / 0.001 / - / 0.024 / -
SaO2 n / 0.002 / 0.615 / 0.205 / 1 / 0.001 / 0.367 / 0.015 / 0.403
SaO2 n(corrected) / 0.004 / - / 0.205 / - / 0.004 / - / 0.002 / -
Bhalla / 0.044 / 0.100 / 0.406 / 0.023 / 0.013 / 0.442 / 0.070 / 0.604
Bhalla(corrected) / 0.088 / 0.200 / 0.406 / 0.092 / 0.052 / 0.589 / 0.093 / 0.604
Kanga / 0.098 / 0.180 / 0.358 / 0.145 / 0.032 / 0.177 / 0.187 / 0.927
Kanga (corrected) / 0.196 / - / 0.358 / - / 0.128 / - / 0.249 / -
Shwachman-Kulczycki / 0.001 / 0.121 / 0.163 / 0.026 / <0.001 / 0.460 / 0.007 / 0.633
Shwachman-Kulczycki(corrected) / 0.002 / 0.242 / 0.163 / 0.104 / 0.001 / 0.613 / 0.009 / 0.633
FVC / 0.008 / 0.089 / 0.444 / 0.049 / 0.002 / 0.856 / 0.015 / 0.087
FVC (corrected) / 0.016 / 0.119 / 0.444 / 0.119 / 0.008 / 0.856 / 0.020 / 0.119
FEV1 / 0.002 / 0.068 / 0.717 / 0.019 / 0.002 / 0.660 / 0.001 / 0.180
FEV1(corrected) / 0.003 / 0.136 / 0.717 / 0.076 / 0.003 / 0.660 / 0.003 / 0.240
FEV1/FVC / 0.003 / 0.022 / 0.055 / 0.012 / 0.049 / 0.026 / 0.001 / 0.807
FEV1/FVC(corrected) / 0.006 / 0.035 / 0.055 / 0.035 / 0.055 / 0.035 / 0.004 / 0.807
FEF25% / 0.151 / 0.120 / 0.475 / 0.333 / 0.159 / 0.048 / 0.053 / 0.214
FEF25%(corrected) / - / 0.240 / - / 0.333 / - / 0.192 / - / 0.285
FEF50% / 0.038 / 0.155 / 0.398 / 0.333 / 0.052 / 0.073 / 0.011 / 0.813
FEF50%(corrected) / 0.069 / - / 0.398 / - / 0.069 / - / 0.044 / -
FEF75% / 0.020 / 0.139 / 0.131 / 0.400 / 0.099 / 0.056 / 0.005 / 0.257
FEF75%(corrected) / 0.040 / - / 0.131 / - / 0.131 / - / 0.020 / -
FEF25-75% / 0.002 / 0.069 / 0.280 / 0.019 / 0.006 / 0.363 / <0.001 / 0.357
FEF25-75%(corrected) / 0.004 / 0.138 / 0.280 / 0.076 / 0.008 / 0.363 / 0.001 / 0.363
FEFMax / 0.104 / 0.233 / 0.858 / 0.400 / 0.041 / 0.151 / 0.072 / 0.476
FEFMax(corrected) / 0.139 / - / 0.858 / - / 0.139 / - / 0.138 / -
ERV / 0.058 / 0.033 / 0.854 / 1 / 0.031 / 0.016 / 0.028 / 0.010
ERV (corrected) / 0.077 / 0.044 / 0.854 / 1 / 0.062 / 0.032 / 0.062 / 0.032

Variables with statistical significance after false rate discovery applied (P<0.05) are presented in Tables 5 and 6 considering pancreatic insufficiency as clinical determinant of cystic fibrosis severity.

Table 5. Association of clinical markers and patient´s age with IL8 gene polymorphisms in patients with cystic fibrosis, taking into account the pancreatic insufficiency status.
Polymorphism / Analysis model / Genotype / Variable / Total / p-value corrected / OR / 95%CI
SaO2 (%)
≤96 >96
rs2227307
+ pancreatic insufficiency / Co-dominant / GG / 10 / 14 / 24 / 0.002 / 0.511 / 0.184 to 1.374
GT / 22a / 30 / 52 / 0.409 / 0.183 to 0.895
TT / 37b / 12 / 47 / 4.189 / 1.801 to 10.29
Total / 69 / 56 / 125
Age (months)
≤ 143 / > 143
rs2227307 + pancreatic insufficiency / Co-dominant / GG / 14 / 7 / 21 / 0.050
GT / 36 / 17 / 53
TTc / 20 / 27 / 47
Total / 70 / 51 / 121
Staphylococcus aureus
rs2227307 + pancreatic sufficiency / Co-dominant / GG / Yes / No
4 / 1 / 5 / 2.495 / 0.201 to 140.5
GTd / 4 / 8 / 12 / 0.022 / 0.081 / 0.006 to 0.615
TTe / 10 / 1 / 11 / 10.35 / 1.168 to 108.4
Total / 18 / 10 / 28

IL8, interleukin 8; SaO2, transcutaneous arterial hemoglobin oxygen-saturation; OR, odds ratio; CI, confidence interval; %, percentage; ≤ smaller and equal; > greater than. a. p-value for analysis in the over-dominant model, 0.024; b. p-value for analysis in the recessive model, 0.001; c. p-value for analysis in the recessive model, 0.032; d. p-value for analysis in the over-dominant model, 0.020; e. p-value for analysis in the recessive model, 0.055. Statistical tests performed included Fisher's exact test for 2 x 2 tables and the χ2 test for the data of co-dominant model. Odds ratio was calculated using the value of Fisher's exact test. Significant data are shown in bold type. Alpha=0.05.

Table 6. Association of clinical markers with IL8 gene polymorphisms in patients with cystic fibrosis, taking into account the pancreatic insufficiency status.
Variable / Polim. / Type / Genotype / N / Mean ± DP / Mean / P-value / Observed difference
SaO2 / rs2227307 / Co-dominant + pancreatic insufficiency / GG / 24 / 95.92 ± 2.48 / 97 / 0.004 / TT ≠ GG and GT
GT1 / 52 / 95.35 ± 5.18 / 97
TT2 / 49 / 93.82 ± 3.88 / 95
SK / rs2227306 / Co-dominant + pancreatic insufficiency / CC3 / 40 / 61.10 ± 15.86 / 60 / 0.045 / CC ≠ CT
CT4 / 43 / 70.46 ± 14.38 / 70
TT / 29 / 64.31 ± 16.94 / 65
rs2227307 / Co-dominant + pancreatic insufficiency / GG / 19 / 70 ± 13.94 / 70 / 0.002 / TT ≠ GG and GT
GT5 / 48 / 70.10 ± 13.35 / 70
TT6 / 41 / 57.17 ± 16.90 / 60
FVC% / rs2227307 / Co-dominant + pancreatic insufficiency / GG / 19 / 75.52 ± 25.90 / 80 / 0.016 / TT ≠ GG and GT
GT7 / 46 / 76.83 ± 22.24 / 80.50
TT8 / 40 / 62.73 ± 22.49 / 61.50
FEV1% / rs2227307 / Co-dominant + pancreatic insufficiency / GG / 19 / 58.58 ± 24.81 / 64 / 0.003 / TT ≠ GT
GT9 / 46 / 68.63 ± 24.13 / 71
TT10 / 39 / 49.72 ± 22.56 / 44
FEV1/FVC / rs2227307 / Co-dominant + pancreatic insufficiency / GG / 19 / 73.53 ± 13.83 / 74 / 0.006 / GT ≠ GG and TT
GT11 / 46 / 84.39 ± 12.56 / 85
TT / 39 / 75.46 ± 15.68 / 78
Co-dominant + pancreatic sufficiency / GG12 / 5 / 95.80 ± 6.10 / 96 / 0.035 / GG ≠ TT
GT / 10 / 83.80 ± 16.23 / 84
TT13 / 10 / 71.50 ± 18.54 / 74.50
FEF50% / rs2227307 / Over-dominant + pancreatic insufficiency / GG+TT / 53 / 39.40 ± 29.88 / 33 / 0.044
GT / 44 / 55.50 ± 31.43 / 48
FEF75% / rs2227307 / Co-dominant + pancreatic insufficiency / GG / 19 / 27.32 ± 22.94 / 21 / 0.040 / GT ≠ GG and TT
GT14 / 44 / 44.21 ± 31.31 / 36
TT / 34 / 30 ± 25.91 / 22
FEF25-75% / rs4073 / Over-dominant + pancreatic insufficiency / AA+TT / 61 / 38.98 ± 28.59 / 35 / 0.040
AT / 45 / 55.22 ± 33.26 / 46
rs2227307 / Co-dominant + pancreatic insufficiency / GG / 19 / 38.74 ± 28.21 / 38 / 0.004 / GT ≠ GG and TT
GT15 / 46 / 58.04 ± 32.08 / 50.50
TT16 / 39 / 36.31 ± 29.63 / 29
ERV / rs2227307 / Co-dominant + pancreatic sufficiency / GG / 1 / - / - / 0.044 / GT ≠ TT
GT17 / 4 / 149.50 ± 45.62 / 157.50
TT18 / 5 / 55.60 ± 32.90 / 70

SaO2, transcutaneous arterial hemoglobin oxygen-saturation; SK, Shwachman-Kulczycki score; pol, polymorphism; N, sample size; SD, standard deviation; med, median; FVC, forced vital capacity; FEV1, forced expiratory volume in 1 second; FEF50, forced expiratory flow at 50% of FVC; FEF75, forced expiratory flow at 75% of FVC; FEF25-75%, average forced expiratory flow between 25% and 75% of FVC; ERV, expiratory reserve volume. Comparative analysis between genetic models which also showed significance correspond to: 1. P-value for the over-dominant model, 0.002; 2. P-value for the recessive model, 0.004; 3. P-value for the dominant model; 0.045; 4. P-value for the over-dominant model, 0.045; 5. P-value for the over-dominant model, 0.009; 6. P-value for the recessive model, 0.001; 7. P-value for the over-dominant model, 0.020; 8. P-value for the recessive model, 0.008; 9. P-value for the over-dominant model, 0.003; 10. P-value for the recessive model, 0.003; 11. P-value for the over-dominant model, 0.004; 12. P-value for the dominant model; 0.035; 13. P-value for the recessive model, 0.035; 14. P-value for the over-dominant model, 0.020; 15. P-value for the over-dominant model, 0.001; 16. P-value for the recessive model, 0.008; 17. P-value for the over-dominant model, 0.032; 18. P-value for the recessive model, 0.032. The statistical analysis of numerical data was conducted by Kruskal-Wallis test (co-dominant model) and the Mann-Whitney test (Models: recessive, dominant and over-dominant). Alpha=0.05.