The study was performed on 47 children with CF aged 0-17 years 11 months 29 days and 54 almost healthy children as control group in Kharkiv region. The CF diagnosis was based on clinical, paraclinical characteristics and positive results of pilocarpine test.
It was found that for the Kharkiv region median age of CF diagnosis was 8.0 (3.0; 24.0) months. CF manifestation was characterized with more frequent gastrointestinal signs. The most common CFTR gene mutation is delF508 – (90.62 ± 5.36) %, which is also specific for most patients with CF of Caucasian races. The incidence of lung fibrosis was found in (76.59 ± 5.42) % of children, bronchiectasis – (40.42 ± 6.58) %, liver lesions – (85.11 ± 4.88) % (including cirrhotic changes – 17.02 ± 15.98) %).
The investigation of regional peculiarities of the respiratory tract biocenosis showed that the most common sputum pathogens are C. albicans ((54.8 ± 8.4) %), S. aureus ((52.3 ± 7.6) %), P. aeruginosa ((40.5 ± 7.5) %); bronchial lavage – P. aeruginosa ((80.0 ± 20.0) %), C. albicans ((60.0 ± 24.0) %), Str. spp. ((60.0 ± 24.0) %); the mucous membrane of the throat and nose – Str. spp. (47.6 ± 8.4) %, S. epidermidis (42.9 ± 7.1) %, S. saprophyticus (28.6 ± 7.4) %. The chronic form of P. aeruginosa occurs in (35.0 ± 11.5) % of children infected by P. aeruginosa. Pathognomonic pathogens sensitivity of respiratory infections to antibiotics was determined.
Elevated levels of the CD3, CD16, CD25, phagocytosis of latex, circulating immune complexes, spontaneous nitroblue tetrazolium test, spontaneous index of activated neutrophils test, IgМ and decrease levels of the leukocytes, neutrophils, CD4, CD8, stimulated nitroblue tetrazolium test, lysosomal cationic proteins, IgА were found during the investigation of immune status in children with CF comparing with the control group that claims literary data about the immune response tension in CF.
Added details about CF phenotype associated with the elevated level of total IgE comparing with patients with the normal level of total IgE were characterized by the predominance within respiratory pathological microorganisms in sputum – S. аureus, Klebsiela pneumoniae and C. albicans in bronchial lavage – S. epidermidis, Str. spp; in the mucous membrane of the throat and nose – Klebsiela pneumoniae. Sensitization to domestic, pollen and food allergens is not typical for children with CF.
Clarification of the features of the course of CF, depending on the level of the general IgE, is a scientific basis for increasing the amount of prophylactic therapy in the CF phenotype, which is associated with elevated total serum IgE level.
The study of the polymorphous variants of the C589T gene of IL-4 and G1082A of the IL-10 gene revealed the association of the pathological allele 589T (comparing with patients without mutation) with S. aureus ((88.89 ± 11.11) % vs (40.00 ± 16.00) %), C. albicans ((77.78 ± 14.23) % vs (10.00 ± 10.00) %) in the sputum and bronchial lavage, the higher incidence of bronchiectasis ((55.56 ± 17.55) % vs 0 %) and lung fibrosis (100 % vs (50.00 ± 17.00) %), cirrhotic changes in the liver parenchyma ((44.44 ± 17.55) % vs 0 %), increase of CD25, circulating immune complexes levels, decrease IgA levels and association of the pathological allele 1082A (comparing with patients without mutation) with P. aeruginosa ((46.15 ± 13.46) % vs 0 %) in the sputum, higher incidence of lung fibrosis ((92.31 ± 8.67) % vs (33.33 ± 20.76) %), cirrhotic changes in liver parenchyma ((30.83 ± 13.26) % vs 0 %), increase of circulating immune complexes levels and decrease of activated neutrophils test levels. These data should be predictors of the CF severity in children.
Detection of the CF course features depending on the presence of C589T polymorphisms of the IL-4 gene and G1082A of the IL-10 gene as adverse predictive factors in the course of the disease and should provide an opportunity to improve the individualization of patients' treatment at the third (high-tech) level of medical care.
Mathematical models of prediction of the risk of development of bronchiectasis and liver cirrhosis in children with CF were created with the method of logistic regression to determine the volume of baseline therapy of the respiratory tract and gastrointestinal system during choosing the treatment algorithm.
