Cystic fibrosis diabetes in adult

Cystic fibrosis is an autosomal recessive disorder affecting about 1/3500 case in France. The disease, that affects all epithelia, is responsible for pulmonary tract infections but also pancreas, gut, liver and genital tract abnormalities. It is linked to CFTR gene mutations, inducing unusually high increase of sodium chloride in sweat, used to track down the illness. deltaF508 CFTR mutation, encountered in 70% of cases, is nearly always associated to pancreatic insufficiency with early-onset lung attack. Around 10% of cystic fibrosis cases, whatever the age, are complicated with partially insulinopenic diabetes, favored by pancreatic fibrosis, while one third of patients shows glucose intolerance. After 20 years old, one third of patients suffers from diabetes and one half after 30 years. Diabetes diagnosis is difficult, and requires the fulfillment of oral glucose tolerance test (OGTT). One glycemia greater or equal to 2 g/l, two hours after a 75 g glucose load, established diabetes diagnosis. Indeed, fasting blood glucose and glycated hemoglobin appear as poor diagnosis markers. Despite histological arguments in favor of the mainly mechanical islet disturbances, an increased prevalence of anti-islets auto-antibodies and an increased frequency of HLA DR3/DR4 have been reported in cystic fibrosis population with glucose tolerance troubles. Also, glucose metabolism is influenced by specific factors linked to cystic fibrosis (infection, malnutrition, steroids...). In reason of the silent phase of diabetes, systematic tracking down of diabetes with a yearly OGTT is recommended, all the more so that hyperglycemia appears as a worsening factor of cystic fibrosis. The efficacy of oral anti-diabetic drugs has not been evaluated on large studies. By contrast, some studies argue for insulin therapy as soon as diabetes appears, insulin improving respiratory and nutritional prognosis. In conclusion, the aim of treatment of cystic fibrosis is to prevent the lung function decline by controlling inflammation and infection, to implement endo- and exo-crine pancreas insufficiency, and to improve nutritional status.