BACKGROUND Diabetes mellitus may be present in patients with cystic fibrosis starting in the second decade of life. The prevalence increases rapidly with increasing age. As life-expectancy increases in cystic fibrosis, cystic fibrosis related diabetes will be diagnosed more frequently in the future.Up to date, no data are available to answer the question if cystic fibrosis related diabetes shou...

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 foun...

Insulin edema is a rare complication of insulin therapy primarily seen with newly diagnosed or uncontrolled diabetes (1–3). Patients at risk are those who are beginning insulin treatment, underweight, or increasing their insulin dose either in the normal course of the disease or after diabetic ketoacidosis (1,4). The prevalence of insulin edema is unknown; a review of the literature revealed fe...

Background: The cystic fibrosis transmembrane conductance regulator (CFTR) mutation I1234V (I1234V, p.Ile1234Val, c.3700A>G), found mainly in the Middle East, is a missense-causing that creates cryptic splice site, with formation of protein lacking 6 amino acids misfolded and misprocessed. vitro effect modulators on human bronchial cell models intestinal organoids has shown controversial result...

BACKGROUND diabetes has become a co-morbidity with a negative impact on nutritional status, lung function and survival in cystic fibrosis. OBJECTIVE To identify any changes in intermediate points after a 2-hour oral glucose tolerance test (OGTT), pancreatic β-cell dysfunction, and insulin resistance in cystic fibrosis-related diabetes. METHODS It was carried out a retrospective analysis in ...