The aim is to present data collected in 2012. Methods In 2012 RCFPR included data of 1309 (697 M) patients from 26 regions while in 2011 – 17 regions and 1015 (544 M) CF patients. Results The main ...data of 2012: average age was 12±9, vs 11±9 in 2011; the proportion of adult patients: 26% vs 25% in 2011. Frequency of F508del: 53%, CFTR dele2,3: 6%, E92K: 3%. The treatment regimen was similar to 2011, except hypertonic saline 25% vs 9%, intravenous antibiotics – 62% vs 71% and oral antibiotics – 75% vs 69%. Chronic infection with P. aeruginosa had 31% (2011 – 32%) of patients, with S. aureus – 58% (the same in 2011), B. cepacia – 6% (2011 – 7%) and S. maltophilia – 4% (2011 – 3%). CF complications: diabetes – 4% (2011 – 3%), liver disease – 33% (2011 – 32%), electrolyte disorders – 4.1% (the same in 2011), pneumothorax – 1.2% (1.5% in 2011). During 2012 20 patients died, a mean age of death 17.3±12.8, in 2011 – 15 patients, mean age 16.0±12.8. Conclusion The number of centers and patients participating in RCFPR is increasing. Comparison of similar data collected in 2011 and 2012 don't differ much, though there is a shift in the frequency of some medications used, especially hypertonic saline inhalations.
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