Chronic rhinosinusitis (CRS), including chronic rhinosinusitis with nasal polyps (CRSwNP), is one of the manifestations of cystic fibrosis (CF). The paranasal sinuses (PNS) are considered as a reservoir of pathogenic microflora and a source of descending infection of the entire respiratory tract. Nevertheless, paranasal sinuses pathology is usually assessed in isolation from both the lower respiratory tract and the entire spectrum of multiple organ damage in CF.Aim. To determine the prevalence of CRSwNP and its correlation with other characteristics of CF in children in Russia.Methods. The study included data of 2216 children from 0 to 18 years old from 81 regions of the Russian Federation from the Registry of patients with cystic fibrosis in the Russian Federation for 2018. Children with an established diagnosis of CRSwNP (n = 599) were compared to those without this condition (n = 1 617). The prevalence was assessed in the subgroups by age, sex, respiratory function, nutritional status, microbiological status, complications, the need for various therapies, and the average duration of inpatient treatment during the year. The statistical analysis included parametric and nonparametric tests.Results. According to the Registry, the average prevalence of CRSwNP among children with CF was 27.03% and tended to increase with age. Children with CRSwNP had statistically significant higher incidence of homozygous F508del mutation, more common intermittent and chronic infection with P. aeruginosa, S. aureus (MSSA and MRSA), cystic fibrosis-associated diabetes mellitus with daily use of insulin, liver damage (as with or without cirrhosis), osteoporosis, a more frequent need for systemic antibacterial and inhalation (except for bronchodilators) therapy, as well as a relatively high need for hospital stay. Unexpectedly, the respiratory function indicators (FEV1, %, FVC, %) and body mass index turned out to be higher in the group of patients with nasal polyposis. A likely explanation is that the study included only children, and the lung function was not analyzed in the children and adolescents.Conclusion. The results suggest that CRSwNP is a predictor of adverse course of CF and requires attention from CF specialists and otorhinolaryngologists, as well as interdisciplinary collaborations.
P001 Phenotypic features of cystic fibrosis gene mutations in patients from the Russian Federation