MATERIALS AND METHODS
The patients, who met the CF diagnostic criteria, followed up at the
Department of Pediatric Respiratory and Allergy Department, Dokuz Eylül
University (DEU) Hospital, between June 2019 and July 2020 were
identified.11 Patients aged between 6 and 18 years,
who attended to control examinations at least 4 times a year, diagnosed
with CF, and underwent a skin prick test (SPT) were included in the
study.
The above skin prick test (SPT) included pollens (Grasses, Artemisia
vulgaris, Alnus glutinosa, Populus alba, Betula, Fagus silvatica,
Parietaria officinalis, Olea europaea); house dust mites
(Dermatophagoides pteronysinus, Dermatophagoides farinae); animal
epithelium (Felis domesticus, Canis familiaris, Blatella germanica);
fungi (Alternaria alternata, Cladosporium herbarum, Aspergillus
fumigatus) commercial extracts Alk-Abello®, Hørsholm, Denmark) . An
induration diameter of 3 mm or above was considered positive for the
purposes of SPT.
The demographic characteristics, CFTR gene mutations, medications in
use, Total IgE, and blood eosinophil levels were retrieved from the file
records of the patients. An eosinophil level of ≥470/uL were considered
eosinophilia, where Total IgE of ≥77.7 IU/ml were considered
high.12,13
The modified Shwachman-Kulczycki (MSK) score was used to rate the
general activity, physical examination, nutritional status, and
radiological findings of the patients based on a 0-25 point scale for
each of the subdomains.14,15 Accordingly, the patients
were classified as excellent (86-100), good (71-85), average (56-70),
poor (41-55) and severe (<40) based on the disease severity
group. The patients were divided into 2 groups, including those with a
MSK score of >70 and those with a MSK score of ≤70, which
were simultaneously rated with SPT.
The spirometry analysis results of the patients, including the forced
expiratory volume in 1 second (FEV1) predicted values,
forced vital capacity (FVC) values, and FEV1/FVC ratio
during their latest presentation were recorded. Spirometry reference
values were based on NHANES III, Hankinson et al., and Wang et
al.16,17
Phlegm cultures collected from the patients were examined. Chronic
infection was investigated for Pseudomonas aeruginosa (Pa),Staphylococcus aureus (Sa), Burkholderia cepacia complex(Bcc), Atypical mycobacteria (Amb), Aspergillus fumigatus (AF),Stenotrophomonas maltophilia (Sm), Escherichia coli (Ec),Haemophilus influenzae (Hi) and Klebsiella pneumoniae (Kp)
microorganisms. In cases where the same microorganism was detected in 2
or more samples in at least 4 samplings during the last year, this was
considered a chronic infection of the microorganism.18
Patients were divided into classes based on the estimated mutation
effect on CFTR function.19 The ’minimal’ function was
defined as only the existence of class I, II or III mutations, while the
’Residual’ function was defined as the existence of at least one
mutation from Class IV or V. In case of at least 1 unknown mutation in
the patients, these were classified as unclassified genotypes.
Allergic Bronchopulmonary Aspergillosis (ABPA) diagnosis was made
pursuant to the Agarwall et al. criteria.20
Allergic rhinitis (AR) was clinically diagnosed based on the presence of
symptoms, including rhinorrhea, nasal congestion, nasal itching, and
sneezing pursuant to the Allergic Rhinitis and its Impact on Asthma
(ARIA) guidelines.21
The patients were classified in accordance with their body mass index
(BMI) during their most recent presentation as severely underweight
(BMI)<16.5), underweight (BMI<18.5), normal weight
(BMI=18.5–24.9,) overweight (BMI≥25), and obese
(BMI≥30).22 Patients were divided into 2 groups
including patients with a BMI of <18.5 and with a BMI of ≥18.5
according to their respective BMI.
Ethics Committee
The required ethics committee approval for this study was obtained from
the Local Ethics Committee of Dokuz Eylül University, School of Medicine
(Approval number: 2020/21-28).
Statistical Analyses
The normality hypothesis was tested by Kolmogorov Smirnov test to decide
the statistical methods to be used. The non-parametric test methods were
used in case any of the groups did not meet the assumption of normality.
Accordingly, the Mann-Whitney U test or Student’s t-test was used in
comparison of the variables obtained upon measurement in two independent
groups, where the Chi-squared and Fisher exact tests were used in the
analysis of the relationships between categorical variables or
differences intergroup between. Pearson Correlation Test analysis was
used to investigate the possible relationship between MSK scores,
FEV1 predictive value and age, Total IgE level, Absolute
eosinophil count (AEC), and BMI. Statistical analyses of the study were
conducted using the IBM Statistical Package for the Social Sciences
(SPSS) for Windows, Version 25 and a p value of ≤ 0.05 was considered
statistically significant.