RESULTS
27 (52.9%) out of 51 patients included in this study were male and the median age was 10 years (min-max: 6-18 years), where the median age of CF diagnosis was 4 months (min-max:1-55 months). Minimal function and residual function CFTR gene mutations were detected in 15 (29.4%) and 16 (31.4%) patients, respectively. Identified in ten patients (19.6%), the Delta F508 mutation was the most prevalent gene mutation. 25 (49%) of the patients had bacterial colonization. Spirometry analysis could not be performed in 10 patients, who were not suitable for the procedure. The FEV1 predicted value was ≥80% in 19 (46.34%), 60-80% in 16 (39.02%), and <60% in 6 (14.63%) patients out of 41 patients. Only 5 (12.19%) patients had early reversibility upon spirometry analysis. The MSK score regarding the severity of the disease was excellent in 7 (13.7%), good in 18 (35.3%), average in 20 (39.2%), and severe in 6 patients (11.8%). As regards the BMI, 20 (39.2%) patients were severely underweight, 16 (31.4%) were underweight, 12 (23.5%) were normal, and 3 (5.9%) were overweight. The 1st degree relatives of six patients (11.8%) had a remarkable history of atopic disease. There were elevated Total IgE and eosinophilia in 19 (37.3%) and 15 (29.4%) patients, respectively (Table 1).
17 (33.3%) patients had allergen susceptibility according to the SPT results. The most prevalent susceptibility were identified against fungi, 10 (19.6%); pollen, 8 (15.7%); animals, 5 (9.8%); and house dust mites, 4 (7.8%), respectively. AF was the most prevalent allergen with a rate of 17.6% (n=9). Three (5.9%) patients had ABPA.
Patients with allergen susceptibility based on the SPT results, had higher Total IgE levels and higher prevalence of eosinophilia (p<0.001, p=0.011, respectively). These patients more frequently used inhaled corticosteroids (ICS) and short acting beta agonists (SABA) (p<0.01). There was no difference between allergen susceptibility and genetic disorder, familial history of atopy, bacterial colonization, spirometry results, MSK scores, BMI, and bronchiectasis (p>0.05). The patients susceptible to AF had higher Total IgE levels and more prevalent eosinophilia (p=0.001, p=0.030, respectively). The prevalence of bacterial colonization and bronchiectasis was higher, and the MSK scores were lower (p=0.010, p=0.001, p=0.007, respectively). Similar differences were not identified in patients with pollen, house dust mite, and animal epithelial susceptibility (p<0.05) (Table 2).
Ten (19.60%) patients had Allergic rhinitis (AR). There was no difference between the patients with and without AR by age (p=0.526), gender (p=0.835), genetic disorder (p=0.364), MSK score (p=0.298), FEV1 predicted value (p=0.270), Total IgE level (p=0.286), and AEC (p=0.116).
The most prevalent bacterial colonization cases include Pseudomonas aeruginosa in 17 (33.3%) patients and Staphylococcus aureus in 11 (21.6%) patients. These patients had significantly higher rates of bronchiectasis compared to non-colonized (NC) patients (p= 0.043, p=0.001, respectively). Patients colonized with Pa had higher rates of positive SPT and AF susceptibility compared to the non-colonized patients (p=0.036, p=0.004, respectively). However, there was no similar difference with Sa colonization (p=1,000, p=0.385, respectively).
There was no difference between the patients’ FEV1 predicted values and the genetic disorders and positive SPT results (p>0.05). Nevertheless, the FEV1 predicted value was lower in patients with bacterial colonization, comorbid bronchiectasis, without elevated Total IgE, and with lower BMI (p<0.05) (Table 3).
There was no relationship between the patients’ genetic disorders and MSK scores, bacterial colonization, bronchiectasis, Total IgE level, AEC, and BMI (p=0.142; p=0.259; p=0.121; p=0.424; p=0.344; p=0.780, respectively). Bronchiectasis was more prevalent in patients with Delta F508 mutation (p=0.026).
There was no correlation between the MSK score and positive SPT results (p=0.133). Nevertheless, the MSK scores were lower in patients susceptible to AF compared to non-susceptible patients (p=0.007) (Table 3). The MSK scores were significantly lower in patients with fungal susceptibility (p= 0.030). There was no similar differences with the susceptibility to pollen, house dust mite, and animal epithelium (p=0.376, p=0.682, p=0.793, respectively). The MSK scores were lower in patients with minimally function mutations, bronchiectasis, bacterial colonization, SABA use, and BMI of ≤18.5 (p<0.05) (Table 4).
The MSK scores of patients were correlated moderately with the FEV1 predicted value and weakly with BMI (r=0.616, p<0.001; r=0.473, p<0.001, respectively), and not correlated with age and Total IgE levels (p>0.05) (Figure 1). FEV1 predicted value was moderately correlated with BMI (r=0.528, p<0.001).
All our patients received Dornase alfa therapy, pancreatic enzyme replacement, and supportive vitamin treatments. Furthermore, 35 (68.62%) patients were treated with SABA and 20 (39.21%) with ICS.
There was no difference by the MSK score (p=0.239), FEV1predicted value (p=0.161), and BMI (p= 0.740) between the patients, who received and not received ICS treatment (p=0.150). Nevertheless, the total IgE level was higher [149 (min-max:2.40-1143) vs 21.1 (min-max:2.11-1030), p= 0.002].
Positive SPT results were more prevalent in patients on ICS compared to those, who did not use ICS (60% vs 25%, p=0.001). There was also no difference by MSK score and FEV1 predicted value between the patients on ICS, with and without allergen susceptibility (p=0.583, p=0.539, respectively).