The dissertation is dedicated to the improvement of the effectiveness of inhalation therapy with Hypertonice saline (HS) in children with cystic fibrosis (CF) by way of personalization approaches to their recognition on the basis of nonspecific bronchial reactivity.
To achieve the goal, a retrospective analysis was conducted medical histories of disease 78 children with CF to determine the clinical and anamnestic features of the course of the disease, clinical-laboratory and functional-instrumental study of 59 children with CF aged 1 to 18 years, included in main group dynamically observed. As a comparison group was examined 14 children with chronic bronchitis without CF and 21 children without chronic bronchopulmonary pathology.
Analysis of molecular genetic research showed that in 10 (20,0%) sick children were identified as "mild", and in 40 (80.0%) patients - "severe" mutations The most common mutation among children with CF in Dnipro was F508del (71,2%), which was detected in 17 patients (28,8%) in the homozygous state and in
25 (42,4%) – in the compound heterozygous state. More often among the latter combinations F508del/2184insA (n=7, 11.9%) and F508del/N1303K (n=7, 11,9%), which also refer to "severe" mutations. Children with a mutation F508del/IVS12+2T>C (n=3, 5,1%) were assigned to the " mild" group due to presence of a second "mild" mutation.
Comparison of the phenotypic characteristics of cystic fibrosis in children depending on from the state of mutation F508del did not reveal significant differences in the course diseases, with the exception of the earlier formation of pulmonary hypertension in patients with a homozygous state (47,1% vs. 16,0%, p<0,05).
Based on a multifactorial approach to probability estimation hyperreactivity of the bronchi are the most important prognostic predictors of chronic obstructive pulmonary disease CF patients showed: the presence of ABPA (I=6,18), the appearance of dry whistling rales in lungs during auscultation during the procedure (I=3,76), reduction of FEV1 by 7% or more from the initial level after inhalation of 3% NaCl solution (I=6,18) or by 10% or more -6% NaCl solution (I=7,12), as well as an increase in the FeNO content by ≥ 8% from primary level (I=2,77). At the same time, the presence of ABPA in patients, as well as a decrease
FEV1 by ≥ 7% of the initial level after inhalation of 3% NaCl solution can be independent criteria of BHR.
It was established that CF patients with bronchial hyperreactivity had more
adverse course of the disease than patients without BHR. In patients with BHR exacerbations of the chronic bronchopulmonary process occurred more often (4,0 [2,0; 4,0] vs. 2,0 [0,0; 4,0]; p<0,01) and RF (52,9% vs. 26,1%; p<0,1).
On the basis of the obtained clinical and anamnestic, molecular genetic, functional and instrumental data during the performance of inhalation tests with increasing concentrations of HS, created convenient for use in practical medicine prognostic scales were built, which allow predicting the level of risk of development of BHR syndrome in children, including . preschool age, ill at CF, she can win at various stages of medical assistance for recognition of a personal selection of HS and the choice of preventive broncholitic therapy as required
