Introduction
respiratory infections, death, CF, exacerbations, CF-related diabetes, pancreatic insufficiency, Pancreatic insufficiency, gastroparesis, Lung disease, CFRD, diabetes
RESPIRATORY INFECTIONS, LUNG DISEASE, CYSTIC FIBROSIS, DISEASE, IMPAIRED GLUCOSE TOLERANCE, TYPE 1 DIABETES, COMPLICATION, HYPOGLYCEMIA, PANCREATIC INSUFFICIENCY, PANCREATIC INSUFFICIENCY, GASTROPARESIS, LUNG DISEASE, TYPE 2 DIABETES MELLITUS, GENETIC DISEASE, DIABETES
Cystic fibrosis (CF) is a life-threatening genetic disease caused by mutations of the CF transmembrane conductance regulator (CFTR) gene. The gene encodes a protein channel expressed in many organs including the pancreas and the lung. The main clinical manifestations of the disease are in fact pancreatic insufficiency and progressive lung disease. Pancreatic insufficiency is present since early childhood in ~80% of this population and requires high energy-density diet with no restrictions on fat and carbohydrate content and pancreatic enzyme replacement therapy. Lung disease is sustained by recurrent respiratory infections and represents the main cause of death in these individuals. However, the recent availability of highly-effective CFTR modulators is going to radically change the natural history of CF.CF also affects the endocrine function of the pancreas and patients develop a unique form of diabetes, defined as CF-related diabetes (CFRD), which differs from both type 1 and type 2 diabetes mellitus. It is the most common extra-pulmonary CF complication, with a prevalence that is strictly age-related, raising from ~5% in the pediatric population to ~50% in adults, with a further 35% of people having impaired glucose tolerance (IGT) (Dysregulation of glucose metabolism in CF is caused by a progressive decline in insulin secretion, but β-cell loss due to self-digestion of endocrine tissue by trapped pancreatic enzymes is not the only pathological mechanism (Conversely, other studies suggest that CFTR may not be expressed in human pancreatic endocrine cells (Currently, the only recommended treatment for CFRD is insulin therapy, with an early start being associated with a significant improvement in disease prognosis (However, insulin treatment may contribute to the multidrug burden of care for people with CF and, our clinical experience has been that, in many cases, multiple daily injection (MDI) regimens may not be optimal for the management of the multifactorial complexity of CFRD. This includes minimal insulin requirements, high risk of hypoglycemia, exacerbations and concomitant steroid therapies, gastroparesis and intestinal and nutritional recommendations.Sensor augmented pump (SAP) therapy is increasingly accepted as gold standard therapy for people with type 1 diabetes (This study was designed to evaluate the potential impact of insulin therapy optimization with SAP over MDI therapy on glycemic control and body composition in individuals with CFRD requiring insulin treatment.