FEATURES OF COURSE OF CYSTIC FIBROSIS IN CHILDREN OF TERNOPIL REGION

Abstract

The aim of the study – to determine the peculiarities of the clinical course of cystic fibrosis in children of the Ternopil region.

Materials and Methods. A retrospective analysis of history of the diseases of 15 children of the Ternopil region, patients with cystic fibrosis was conducted. 5 children were examined during their stay in the hospital.

Results and Discussion. Ideally, detection of patients with cystic fibrosis during neonatal screening and initiation of treatment can be considered immediately after diagnosis, even in the absence of clinical manifestations. The diagnosis of cystic fibrosis in all patients is confirmed in the Western Ukrainian Specialized Children's Medical Center. In the study of the genotype, various types of mutations were detected, but the most commonly occurring mutation was F508del / F508del. We determined that most children were 10 years old. The time of diagnosis of cystic fibrosis is different: up to 1 year – in 2 children (13.3 %); from 2 to 5 years – in 5 children (33.3 %); from 5 to 10 years – in 8 children (53.4 %). It is established that the later the patient is diagnosed with the disease, the heavier it is going through and the lower the effectiveness of treatment. In these children, multiple recurrences of inflammatory processes in bronchuses were noted – in 10 (66.6 %) patients, additional nasal sinuses – in 5 (33.3 %), pancreatic dysfunction disorders – in 15 (100.0 %), development of resistance to pathogenic microflora to antibiotics – in 15 (100.0 %). Therefore, in these children, treatment was complicated and, accordingly, its effectiveness was reduced. Nearly half of patients, namely 7 (46.6 %), due to prolonged hypoxia, deformed fingers and nails in the form of "drum sticks" and "clock clocks". Pulmonary hypertension is diagnosed in 4 patients (26.6 %). Deformation of the chest (barrel, enlarged in the anterior-posterior size) was noted in 7 children (46.6 %). Cholestatic hepatitis was detected in 5 patients (33.3 %). Two children (13.3 %) were diagnosed with glucose intolerance.

Conclusions. In the structure of patients with cystic fibrosis children of the Ternopil region dominate the girls and the inhabitants of the countryside. Today, the majority of children with cystic fibrosis (53.3 %) reached the age of over 10 years. The children of our region are dominated by the mutation F508del/ F508del (66.7 %). In most children (53.4 %) the disease was diagnosed after 5 years. In the debut of the disease, protracted obstructive bronchitis (66.7 %) and pancreatic dysfunction (100.0 %) were most often observed. Among the complications, bone deformities, signs of chronic hypoxia and a high incidence of infection with golden staphylococci (73.3 %) and blue-purulent rod (46.7 %) predominated. It is necessary to increase the caution in early diagnosis of cystic fibrosis in order to improve the quality and lengthen the life expectancy of children.