Since the early 1990s the management of children with cystic fibrosis (CF) has come a long way and advances in both nutritional and medical care have resulted in a median age of survival of 30-35 years, as compared with a life expectancy of <1 year in the 1950s. The first definitive reports of glucose intolerance or diabetes in CF are from 1955. The combination of CF and related diabetes (CFRD)...

Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian populations. Individuals with CF have seen significant increases in life expectancy in the last 60 years. As a result, previously rare complications are now coming to light. The most common of these is cystic fibrosis-related diabetes (CFRD), which affects 40-50% of CF adults. CFRD significantly impacts the pulmon...

Cystic fibrosis–related diabetes (CFRD) is the most common comorbidity in patients with cystic fibrosis (CF) (1). Consensus guidelines state that all patients above the age of 10 years should be screened annually for CFRD with a 2-h 75-g oral glucose tolerance test (OGTT) (2). There are four categories of glucose tolerance based on OGTT fasting and 75-g 2-h glucose levels: normal glucose tolera...

BACKGROUND Cystic fibrosis related diabetes (CFRD) with fasting hyperglycemia is found in 15% of adult and 11% of adolescent CF patients. Because of concerns about hypoglycemia, it is not common practice to treat CFRD with 24-hour basal insulin therapy, despite evidence that insulin deficiency may contribute to protein catabolism and have an adverse effect on weight, muscle mass, pulmonary func...

OBJECTIVE Longer survival of patients with cystic fibrosis has increased the occurrence of cystic fibrosis-related diabetes (CFRD). In this study we documented the incidence of CFRD and evaluated the association between mutations responsible for cystic fibrosis and incident CFRD, while identifying potential risk factors. RESEARCH DESIGN AND METHODS This was a population-based longitudinal stu...

D iabetes is the most common co-morbidity in individuals with cystic fibrosis. The etiology is poorly understood. Data on the presence of diabetes autoantibodies are conflicting, and little is known about type 1 diabetes gene associations. Our goal was to determine the prevalence of antibodies and HLA haplotypes known to be associated with type 1 diabetes in cystic fibrosis–related diabetes (CF...

CFRD — Cystic fibrosis-related diabetes — is the most prevalent extrapulmonary complication of cystic fibrosis and is associated with decline in pulmonary function, poor nutritional status, and greater mortality. Although our general understanding of CFRD has increased, difficult areas remain in its pathophysiology, screening, diagnosis, and management that require further research and developm...

OBJECTIVE Cystic fibrosis (CF)-related diabetes (CFRD) is associated with declining pulmonary function and increased mortality. During oral glucose tolerance testing (OGTT), CFRD is defined by 2-h plasma glucose (PG2). We hypothesized PG elevations during OGTT resolving by 2 h, not meeting CFRD criteria, influence pulmonary function in CF. Thus we investigated the frequency of elevated 1-h OGTT...

C ystic fibrosis (CF) is one of the most common life-threatening inherited disorders in Caucasian populations, affecting 30,000 children and adults in the U.S. and 70,000 worldwide. Due to advances in pulmonary care and nutrition, the life expectancy for CF has increased from less than 5 years in the 1950s to greater than 37 years at present, with many patients living into their fifth and sixth...

Cystic Fibrosis (CF) is often accompanied by diabetes leading to worsening lung function, the reason for which is unclear. The receptor for advanced-glycation-end-products (RAGE) regulates immune responses and inflammation and has been linked to diabetes and possibly CF. We performed a pilot study to determine if CF and CF-related diabetes (CFRD) are associated with enhanced RAGE expression. Fu...