Clinical and epidemiological characteristics of cystic fibrosis patients living in the Siberian Federal District, according to the National Register (2017)

Analysis of data of the National Cystic Fibrosis (CF) Register allows evaluating the quality of medical care for children and adults with CF in the whole country and in a single Federal District. The Siberian Federal District (SFD) has the third-highest number of CF patients in the Russian Federation (RF). The study aimed to determine the clinical and epidemiological characteristics of CF patients in the SFD in 2017 to improve the approaches to medical care in the region.

Methods. The National CF Register’s main sections for 2017 were evaluated: demographic data, diagnostics, genetics, airway microflora, lung function, nutritional status, complications of the disease, and treatment. The SFD data were compared with the data of the All-Russian Register in 2017.

Results. The information was provided on 433 CF patients from the SFD. No differences were found between the SFD and the RF in the incidence of the disease, F508del and CFTRdele2,3(21kb) mutations, the microbiological profile, lung function, and nutritional status. The SFD was characterized by the lower average age, the detection of rare mutations in patients from indigenous groups of the North, the predominance of “severe” genotypes, the noticeable variability in the frequency of chronic Pseudomonas aeruginosa infections, and a significant increase in the Burkholderia cepacia complex detection. An insufficient level of laboratory diagnosis of methicillin-resistant Staphylococcus aureus and non-tuberculous mycobacteriosis was noted.

Conclusion. Strict adherence to sanitary and anti-epidemiological measures is required to control the chronic respiratory infections in the regions of the SFD. Careful implementation of the national consensus recommendations on the diagnosis and treatment of CF is also required.

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