Age at CFLD diagnosis, years (SD) Splenomegaly at diagnosis of CFLD Diabetes Meconium ileus Death Progression (n = 80) 7.3 (3.5) 61 (76%) 32 (40%) 21 (26%) 26 (33%) No progression (n = 133) 8.9 (4.0) 42 (32%) 36 (27%) 34 (26%) 10 (8%) p 0.004 <0.001 0.05 0.91 <0.001 [Characteristics between patients with and without progression of CFLDv] Conclusion: Splenomegaly was the only significant marker of CFLD which was present in children who had progressive liver disease, indicating established liver disease at the time of diagnosis.There was no clear evidence that UDCA reduced the rate of progression of liver disease in those with established liver disease, but may do so if given prior to the onset of CFLD.

Objective: To assess the dose of enzyme replacement therapy for lipase (U/kg and U/g) in different age groups.Material and Methods: 140 children with CF from 1 to 18 years old were investigated, M (±SD) -6.1 (±4.0) years.Design -cross-sectional study.The entry criterion is elastase less than 200 μg/g.The children were divided into 4 age groups:1 group (1-2 years, n-37); group 2 (3-5 years, n-40); group 3 (6-9 years, n-40); group 4 (10-18 years old, n-23).. Recommendations for enzyme therapy for children with CF ESPEN-ESPGHAN-ECFS (Turk D., 2016) were accepted as the norm.Statistical methods: using Statistica 10, lipase dose was assessed by median -Me (Q1-Q3), Mann -Whitney test.We used the developed computer program "Monitoring of nutritional status, enzyme therapy, diet in cystic fibrosis."Results: In the total group, the dose of lipase was -8700 (5990-11700) U lipase/kg per day (U/kg) and 1500 U lipase/gram dietary fat per day (U/g).It was revealed that the dose of lipase in children with CF is significantly reduced with age from 10620 U/kg to 7000 U/kg.In 1 group -10620 (8700-14192) U/kg, in group 2 -9150 (6446-12612) U/kg ( p 1-2 = 0.3); in group 3 -7450 (5350-9700) U/kg ( p 1-3 = 0.008, p 2-3 = 0.02); in group 4 -7000 (4300-8450) U/kg ( p 1-4 = 0.0003, p 3-4 = 0.3).The following values were obtained for the dose of lipase based on the amount of fat in food: 1 group -1800 (1200-2500) U/g, group 2 -1500 (1200-2000) U/g, 3 group -1500 (1200-2300) U/g, 4 group -1500 (1000-2500) U/g.No significant difference between groups was found.Conclusions: The average daily lipase dose in the calculation of U/kg was within the recommended values (up to 10000 U/kg) and decreased with age from 10620 U/kg to 7000 U/kg ( p < 0.001).The daily dose of lipase U/g of fat in food was below the recommended values of 1500 U/g (2000-4000 U/g).The highest values were in group 1 -1800 U/g, in the 2,3.4 group -1500 U/g ( p > 0.05).

than ≪non-Bcc≫ patients.It may suggest the antifungal effect of Bcc.