Nasal Potential Difference in Cystic Fibrosis considering Severe CFTR Mutations

Table 2

Clinical and laboratory characteristics of the cystic fibrosis patients enrolled in the study.

Clinical markers^*	G1	G2	value
Gender (male)	5	2	1
Race (Caucasian)	10	5	—
Age (months)	210.60 ± 132.01; 146 (87–443)	336.40 ± 119.13; 336 (170–480)	0.099
Onset of symptoms (months)	8.20 ± 15.50; 1 (0–39)	3.75 ± 4.86; 1.50 (1–11)	0.454
Onset of pulmonary symptoms (months)	13.60 ± 22.26; 4 (0–69)	4 ± 4.97; 2.50 (0–11)	0.539
Onset of digestive symptoms (months)	23.70 ± 41.51; 1 (0–120)	1 ± 1.41; 1 (0–2)	0.758
Diagnosis (months)	36.10 ± 47.24; 17 (1–144)	99.25 ± 8.50; 185.88 (2–378)	1
Body mass index (normal values)	9	3	0.242
Nasal polyposis (presence)	1	2	0.242
Diabetes mellitus (presence)	2	—	0.524
Osteoporosis (presence)	1	2	0.242
Pancreatic insufficiency (presence)	10	3	0.095
Meconium ileus (presence)	3	—	0.505
Pseudomonas aeruginosa	6	4	0.600
Mucoid P. aeruginosa	4	4	0.282
Achromobacter xylosoxidans	2	—	0.524
Burkholderia cepacia	3	1	1
Staphylococcus aureus	9	3	0.242
Weight (kg)	41.50 ± 17.51; 36 (21–66)	48 ± 16.98; 50 (27–70)	0.513
Height (m)	1.74 ± 0.43; 2 (1-2)	1.50 ± 0.53; 1.50 (1-2)	0.594
Body mass index	18.54 ± 3.18; 19.95 (14.38–21.60)	18.25 ± 2.56; 18.48 (14.34–21.31)	0.768
SpO2	96.10 ± 1.45; 96 (94–98)	96 ± 2.31; 96 (94–98)	1
Bhalla	8 ± 4.74; 7 (0–17)	12.50 ± 3.53; 12.50 (10–15)	0.327
Kanga	19 ± 4.82; 19 (12–25)	30.50 ± 20.17; 23.50 (15–60)	0.260
Shwachman-Kulczycki	68.50 ± 13.34; 65 (50–90)	71.67 ± 10.40; 75 (60–80)	0.811
FVC	91.80 ± 20.02; 87.50 (69–131)	70.20 ± 20.17; 63 (57–106)	0.028
FEV1	85.10 ± 22.41; 75.50 (64–132)	59 ± 25.40; 60 (30–95)	0.055
FEV1/FVC	82.60 ± 18.92; 85.50 (37–100)	67.75 ± 17.58; 71 (46–83)	0.106
%	71.40 ± 30.85; 66 (33–118)	45.50 ± 24.78; 47.50 (17–70)	0.188

The data are shown as (percentage) for the categorical data; the statistical analysis consisted of the test and Fisher’s exact test; the mean ± standard deviation and median (minimum and maximum) values were used for the numerical data that was analyzed by the Mann-Whitney test. : number of patients; SpO₂: blood oxygen saturation; FVC: forced vital capacity; FEV₁: forced expiratory volume in the first second; %: forced expiratory flow between 25 and 75% of the FVC. G1: cystic fibrosis patients with two class I, II, or III CFTR mutations; G2: cystic fibrosis patients with at least one class IV, V, or V CFTR mutation.