Vnitr Lek 2024, 70(4):219-223

Cystic fibrosis related diabetes - current situation and perspective

Kateřina Štechová¹, Libor Fila²

¹ Interní klinika UK 2. LF a FN Motol, Praha

² Pneumologická klinika UK 2. LF a FN Motol, Praha

Cystic fibrosis (CF) is an autosomal recessive inherited disease caused by mutations in the CFTR gene (the Cystic Fibrosis Transmembrane Conductance Regulator). In most patients, the disease manifests itself in the form of chronic sinobronchial disease, pancreatic insufficiency, and increased concentration of chlorides in sweat. With increasing age, other complications appear, one of which is a specific form of diabetes CFRD (Cystic Fibrosis Related Diabetes), which affects up to 50 % of adult CF patients. The introduction of treatment with so-called CFTR modulators has led to a significant extension of the lifespan of patients with CF, and with that, CFRD also becomes more important. Decompensated diabetes leads to more frequent and more severe exacerbations of the pulmonary manifestations of CF and thus to increased patient morbidity and mortality. CFRD is mainly characterized by postprandial hyperglycemia, fasting hyperglycemia develops later. Patients with CF must be regularly screened for the development of CFRD. The method of choice for the treatment of CFRD is insulin therapy. It is advantageous to use continuous glucose concentration monitoring and insulin pump therapy in CFRD patients, which enables better adaptation to the necessary high-calorie diet of CF patients.

Keywords: cystic fibrosis, CFRD (Cystic Fibrosis Related Diabetes), insulin, continuous glucose monitoring.

Accepted: June 5, 2024; Published: June 20, 2024  Show citation