Cystic fibrosis-related diabetes (CFRD) is one of the most common comorbidities affecting Cystic Fibrosis (CF) patients. Increasing incidence with age, the condition affects up to 50% of people with CF. Cystic fibrosis is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). The CFTR channel is located on the apical membrane of secretory epithelial cells and mediates the transport of Cl- and HCO3- ions. Through this action it plays a crucial role in mediating mucous viscosity in multiple systems in the body. The pancreas is one of the most frequently affected organs in patients with CF. Nevertheless, the etiology of CFRD is not entirely understood. It is currently thought of as a multifactorial condition resulting from a combination of beta-cell dysfunction, and secretion obstruction.
Mutations in the CFTR in the ductal epithelial cells result in the lower water content and high viscosity of secretions causing obstructions of the ductal canal and prevention of enzymes reaching the digestive system. This can require people with CFRD to take pancreatic enzyme replacement therapy. Moreover the associated fibrosis causes by the retained enzymes can cause decreases islet cell mass leading to both insulin and glucagon insufficiencies. CFTR is also expressed in pancreatic beta-cells where its exact role remains unknown but mutations in the CFTR gene are thought to cause abnormal beta-cell function and decreased insulin secretion.
Despite current research, the underlying causes still require elucidating. In this Research Topic we hope to highlight new research and perspectives on the causes and progression of the disease to inform future therapy and management approaches.
• How CFTR dysfunction in beta cells contributes to CFRD
• The role of immune cell and inflammation
• Future perspectives for better diagnostics and treatments
We will accept submissions in the form of original research, review and commentary.
Cystic fibrosis-related diabetes (CFRD) is one of the most common comorbidities affecting Cystic Fibrosis (CF) patients. Increasing incidence with age, the condition affects up to 50% of people with CF. Cystic fibrosis is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). The CFTR channel is located on the apical membrane of secretory epithelial cells and mediates the transport of Cl- and HCO3- ions. Through this action it plays a crucial role in mediating mucous viscosity in multiple systems in the body. The pancreas is one of the most frequently affected organs in patients with CF. Nevertheless, the etiology of CFRD is not entirely understood. It is currently thought of as a multifactorial condition resulting from a combination of beta-cell dysfunction, and secretion obstruction.
Mutations in the CFTR in the ductal epithelial cells result in the lower water content and high viscosity of secretions causing obstructions of the ductal canal and prevention of enzymes reaching the digestive system. This can require people with CFRD to take pancreatic enzyme replacement therapy. Moreover the associated fibrosis causes by the retained enzymes can cause decreases islet cell mass leading to both insulin and glucagon insufficiencies. CFTR is also expressed in pancreatic beta-cells where its exact role remains unknown but mutations in the CFTR gene are thought to cause abnormal beta-cell function and decreased insulin secretion.
Despite current research, the underlying causes still require elucidating. In this Research Topic we hope to highlight new research and perspectives on the causes and progression of the disease to inform future therapy and management approaches.
• How CFTR dysfunction in beta cells contributes to CFRD
• The role of immune cell and inflammation
• Future perspectives for better diagnostics and treatments
We will accept submissions in the form of original research, review and commentary.
