CLINICAL AND EPIDEMIOLOGICAL FEATURES OF CYSTIC FIBROSIS IN CHILDREN DURING NEWBORN SCREENING: THE EXAMPLE OF THE KHANTY-MANSI AUTONOMOUS OKRUG - UGRA IN 2012-2018

Abstract

The aim of the study is to establish the epidemiological features of cystic fibrosis (CF) in children and its clinical pathomorphism in conditions of early disease diagnosis following the newborn screening over a period of 2012-2018 in the Khanty-Mansi Autonomous Okrug - Ugra (KhMAO). Material and methods. A retrospective cohort comparative analysis of CF patients within the regional register of the KhMAO in 2012-2018 is carried out. The comparative study of clinical, laboratory and functional parameters of patients with and without newborn screening is made. Results. The long-term average annual incidence rate is 0.18, the prevalence rate is 3.00, the mortality rate is 0.009 per 100 000 population, the birth rate of children with CF is one per 9 025 newborns. After the newborn screening, an earlier diagnosis of CF during the first year of life and a more favorable clinical course of CF in terms of nutritional status and functional state of the lungs are noted, which required less prolonged courses of antibiotics, bronchodilators and inhaled steroids.