The aim of this study was to evaluate changes in survival of patients with cystic fibrosis (CF) in Moscow and Moscow region and to determine fac tors influencing the survival. We analyzed outpatient medical records of patients followed up in Moscow CF centers by the 1st of January, 2002, and by the 1 st of January, 2012. Median survival for 2002-2012 was 37.2 years; this was significantly higher that the similar value for the previous decade (25.9 years). A total num ber of CF patients was more than doubled due to increased survival and improved diagnosis of the disease. Improved survival was due to improved work of CF centers and to implementation of effective medications, primarily dornase alfa (Pulmozyme) and some inhaled and systemic antibiotics and macrolides in subinhibitory concentrations, in routine clinical practice. Gram negative infection, espe cially Burkholderia cepacia, was shown to decrease survival in CF patients in contrary to "mild" mutations that are better prognostic factors.
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