Nasal Potential Difference in Cystic Fibrosis considering Severe CFTR Mutations
Table 1
Clinical and laboratory characteristics of the cystic fibrosis patients enrolled in the study∗.
Gender (male)
15
7 (46.7%)
Race (Caucasian)
15
15 (100%)
Age (months)
15
213.13 ± 122.03; 171 (87–443)
Onset of symptoms (months)
14
6.93 ± 13.28; 1 (0–39)
Onset of pulmonary symptoms (months)
14
10.86 ± 19.21; 3 (0–69)
Onset of digestive symptoms (months)
12
19.92 ± 38.57; 1 (0–120)
Diagnosis (months)
14
54.14 ± 101.95; 8,50 (1–378)
Body mass index (normal values)
15
12 (80%)
Nasal polyposis (presence)
15
3 (20%)
Diabetes mellitus (presence)
15
2 (13.3%)
Osteoporosis (presence)
15
3 (20%)
Pancreatic insufficiency (presence)
15
13 (86.7%)
Meconium ileus (presence)
15
3 (20%)
Pseudomonas aeruginosa
15
10 (66.7%)
Mucoid P. aeruginosa
15
8 (53.3%)
Achromobacter xylosoxidans
15
2 (13.3%)
Burkholderia cepacia
15
4 (26.7%)
Staphylococcus aureus
15
12 (80%)
Weight (kg)
15
43.67 ± 17.02; 34 (21–70)
Height (m)
15
1.58 ± 0.50; 1 (1-2)
Body mass index
15
18.35 ± 2.67; 17.75 (14.33–21.60)
SpO2
14
96.07 ± 1.64; 96 (94–98)
Bhalla
11
8.82 ± 4.75; 10 (0–17)
Kanga
13
22.54 ± 12.16; 21 (12–60)
Shwachman-Kulczycki
13
69.23 ± 12.39; 65 (50–90)
FVC
15
84.60 ± 22.02; 82 (57–131)
FEV1
15
76.40 ± 25.84; 72 (30–132)
FEV1/FVC
14
78.36 ± 19.17; 85 (37–100)
%
14
59.50 ± 34.25; 57,50 (70–118)
∗The data are shown as (percentage) for the categorical data and as the mean ± standard deviation and the median (minimum and maximum) values for the numerical data. : 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.
