P104 The dynamics of health of children with cystic fibrosis in Russian Federation according to the registry data during last 10 years

Dynamics of the health status children with CF in Russian Federation (RF). Patient Registry RF 2011–2020 Over 10 years, number registry increased from 770 to 2735. The average age 2011 was 11.5 ± 8.9, 2020 13.7 9.7. diagnosed by neonatal screening 24% (28.8% 2011, 52.3% 2020). patients meconium ileus doubled 8.7% 15.7% 2020. total allele frequency 90.1%. Two genetic variants CFTR gene were found 83% children, one 14.3%, none 2.7%. F508del homozygous accounted for 32.29%, 23.55% do not have genotype. “Severe” genotypes amounted 81.8%. seeding S.aureus among 60.9%, 65.2%, P.aeruginosa 27.2% and 28.5%, Bcc 3.8% 3.6%, which is associated an improved laboratory work. In M SD FEV1 FVC 81.8 28.7% 89.1 32.4%, 86.1 22.6 88.8 20.7%. Me (IQR) percentile BMI (2 – 18 years) 25.5(53.5), 30.2(52.0). nasal polyposis 14.7%/31.3%, ABPA 1.2%/1.9%, liver cirrhosis portal hypertension 3.5% 3.9% due diagnosis. electrolyte disorders decreased 6.1% 3.6% osteoporosis 4.6% 3% introduction preventive measures. CFRD did change 1.2%/1.3%. inhaled NaCl 7% 8.6% 74.2%. IV antibiotics decreased: 71.4% versus 26.4%. 0.9% received targeted therapy. a 10-year period, 7 transplants, 2 lungs. During years diagnosis has primarily screening, condition according BMI, FEV1, antibiotics, complications indicates improvement RF.