ESPE Abstracts (2014) 82 P-D-3-2-739

Cystic Fibrosis-Related Diabetes in Children and Adolescents in the Russian Federation

Elena Kondratyeva, Nicolay Kapranov & Victoria Sherman

Medical Genetics Research Center, Moscow, Russia

Background: Disorders of carbohydrate metabolism in children and adolescents with cystic fibrosis occur from 5 to 12.6%, significantly increasing during the teenage period and reaching 24–50% in adults.

Method: An analysis of 499 medical records was conducted. All the patients were treated in the Russian Children’s Clinical Hospital in 2012. The survey was carried out on carbohydrate metabolism and indications according to children after 12 years (Management of Cystic Fibrosis Diabetes Mellitus, 2011) and as recommended by WHO (1999).

Results: Violation of carbohydrate metabolism was detected in 5.1% (29 children). In addition, the frequency of cystic fibrosis-related diabetes (CFRD) was 2.8% (14 children), while the insulin requirement was observed in 2.2% of children, intermittent postprandial hyperglycemia find impaired glucose tolerance (IGT), CFRD without fasting hyperglycaemia was registered in 3% (15 children). Most often the disorders of carbohydrate metabolism were observed in 27% of adolescents, in the general group of patients with impaired glucose metabolism they was 86%, at that, girls were 83% and overpass the boys were 17%. All children (100%) had a mutation F508del, while 30% in the homozygous state. Underweight was observed in 60% of children, stunting – 40%. 30% of children (all getting insulin) had FEV1 < 50%. 30% of children were receiving systemic glucocorticoids. In 90% of cases, in sputum were detected associations of two to four microorganisms. HbA1 was above 7% in 25% of children.

Conclusion: It is established that glucose intolerance and diabetes are most often observed during adolescence, among girls, with exacerbation of bronchopulmonary process and receiving glucocorticoids.

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