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- W978563957 abstract "Objective To compare the data describing demographic characteristics of the patients, survival rates, pulmonary function, microbiologic data, complication rates and health care utilization of 2010 with similar data collected in 2012. Methods 2010 CFPRMMO included data of 359 (180 male) CF patients, while in 2012 – 436 (224 male). Results The main data of 2010: average age of CF patients 12.5±8.9 (min 0.1, max 43.2), vs. 13.1±10.2 (min 0.2, max 63.0) in 2012; the proportion of patients of 18 years and older – 28.7% and 32.6% in 2012; the median age of diagnosis – 3.7 years (min 0, max 41.0) vs. 3.93 (min 0, max 59.0) in 2012. Frequency of F508del: 53.4% vs. 54.4%, CFTRdele2,3: 9.5% vs. 8.9%, 3849+10kbC→T: 3.1% vs. 3.7%, unknown: 17.0% vs. 8.8%. Spirometry was done in 34.0% vs. 50.7%; 92.8% (94.5% in 2012) of patients were treated by rhDNase, 53.5% (64.7%) – bronchodilators, hypertonic saline 0.2% (27.1%), inhaled antibiotics – 35.9% vs 42.6%, pancreatic enzymes 94.7% vs 89.7%, UDCA: 95.8% vs. 94.7%, 2.2% and 4.1% resp. – oxygen therapy. P. aeruginosa had 30.0% (28.2% in 2012) patients, S. aureus – 67.1% (72.2%), B. cepacia – 8.4% (9.8%) and S. maltophilia – 3.6% (5.7%). CF complications: diabetes – 5.7% (5.5% in 2012), liver disease – 5.9% (7.1%), pneumothorax – 2% (1.4%). During 2010 8 patients died, a mean age of death 18.6±7.6 yrs, in 2012 5 patients died, mean age 17.3±11.9 yrs. Conclusion The number of patients included in CFPRMMO is increasing. Comparison of data collected in 2010 and 2012 show increase of adults, there is a shift in the frequency of spirometry performed and some medications used, especially hypertonic saline inhalations." @default.
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- W978563957 date "2015-06-01" @default.
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- W978563957 title "307 Cystic Fibrosis Patient Registry of Moscow and Moscow Region (CFPRMMO) in 2010–2012" @default.
- W978563957 doi "https://doi.org/10.1016/s1569-1993(15)30481-1" @default.
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