Abstract

The aim of this study was to characterize chronic respiratory infection in cystic fibrosis (CF) patients in Russia according to the National Russian CF Register. Methods. The National Russian CF Register included 2,131 patients from 74 Russian regions (2,092 alive patients and 39 died patients) in 2014 (the median age, 10.2 (15.2) years; 29.2% were adults ≥ 18 лет). Microbiological findings of CF patients (chronic infection caused by Staphylococcus aureus, Pseudomonas aeruginosa, Stenotrophomonas maltophilia, Burkholderia cepacia соmplex, non-tuberculous mycobacteria (NTM), non-Pseudomonas Gram negative pathogens including Achromobacter spp., and frequency of intermittent P. aeruginosa infection) have been analyzed in this article. Results. The tested pathogens were not found in 18.0% of patients. The prevalence of pathogens in descending order was as follows: S. aureus, 54,5%; P. aeruginosa, (chronic infection), 32,1%; P. aeruginosa (intermittent infection), 14,1%; B. cepacia complex, 7,3%; Achromobacter spp., 3,5%; Stenotrophomonas maltophilia, 3,3%; NTM, 0,8%. Other non-Pseudomonas Gram negative pathogens including Achromobacter spp., Escherichia coli, Acinetobacter spp., Klebsiella spp., Citrobacter spp. caused chronic airway infection in 11.8% of patients. Children were infected more frequently by S. aureus and adult patients infected were frequently by P. aeruginosa, В.сepacia complex, Achromobacter spp., and NTM. No geographic prevalence of pathogen distribution was found. Russian CF patients were more often infected by S. aureus and B. cepacia complex compared to European CF patients. P. aeruginosa was yielded with equal rate in Russia and in Europe. S. maltophilia and NTM were found less frequently in Russia compared to Europe. Conclusion. According to the National Russian CF Register, the airways of 18.0% of CF patients in Russia are not colonized by respiratory pathogens. As the patient's age increases, the rate of Gram negative respiratory infection is growing. The most harmful infections are thought to be B. cepacia complex and Achromobacter spp.

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