Diabetes mellitus associated with cystic fibrosis. Clinical cases

Abstract

Cystic fibrosis is one of the most prevalent orphan monogenic diseases (one case per 2500 newborns). The disease is characterized by violation of the chlorine transport from a cell and an increase in sodium absorption into a cell, which results in the decreased water content in the secretions of all exocrine glands. The reduced volume of periapical epithelial fluid prevents proper mucociliary clearance, and increased sodium chloride concentration and altered pH of fluid decreases enzymatic activity. The most widespread complications of cystic fibrosis are diabetes mellitus, diseases of kidneys and bones, syndrome of distal intestinal obstruction, gastroesophageal reflux disease and depression. Complication of diabetes mellitus develops in about 80 % of patients with cystic fibrosis under 40 years. Diabetes mellitus associated with cystic fibrosis is one of the diabetes forms that require an approach with involvement of multidisciplinary team.Usually, the manifestation and course of diabetes are asymptomatic. Clinical manifestations of diabetes associated with cystic fibrosis in pediatric patients may include constant feeling of fatigue, loss of body weight in the absence of changes in nutrition, delay in physical or sexual development.The authors present clinical cases of diabetes mellitus in patients with cystic fibrosis and perform a comparison of the onset and subsequent course of diabetes, associated with cystic fibrosis, on the example of two pediatric patients.All patients with cystic fibrosis require conduction of screening tests to reveal hyperglycemia, starting from an early school age. The only method to treat diabetes mellitus associated with cystic fibrosis is insulin therapy with the continuation of hypercaloric nutrition and constant monitoring of not only glycemia indices, but also parameters of physical and sexual development, indicators of the function of external respiration, lipid profile, etc.