Abstract
BackgroundPublished estimates on age-dependent frequency of diabetes in cystic fibrosis (CF) vary widely, and are based mostly on older data. However, CF treatment and prevention of comorbidities changed over recent years. In many studies, definition of cystic fibrosis-related diabetes (CFRD) is not in line with current guideline recommendations. Therefore, we evaluated age-dependent occurrence of glucose abnormalities and associated risk factors in CF patients who participated in a multicenter screening program using oral glucose tolerance tests (OGTT).MethodsBetween 2001 and 2010, 43 specialized CF centers from Germany and Austria serially performed 5,179 standardized OGTTs in 1,658 clinically stable, non-pregnant CF patients with no prior steroid medication or lung transplantation. Age-dependent occurrence of impaired fasting glucose (IFG), impaired glucose tolerance (IGT), IFG+IGT, one (DGT) or two consecutive (CFRD) diabetic OGTTs was analyzed, using Kaplan Meier curves. Cox proportional-hazards models were created to elucidate the influence of sex or underweight.ResultsAt baseline/last OGTT, median age was 15.9 years/18.2 years and 30.6%/31.8% of patients were underweight. 25% of patients showed IFG at age 14.3 years; IGT at age 16.3 years; IFG+IGT combined at age 17.7 years. DGT was observed in 25% of patients at age 22.6 years; CFRD at age 34.5 years. Females had a 3.54 [95% CI 1.23–10.18] times higher risk for CFRD; risk for DGT was 2.21 [1.22–3.98] times higher. Underweight was a risk factor for IGT (HR [95% CI]: 1.38 [1.11–1.71]) and IFG+IGT (1.43 [1.11–1.83]), and in males also for DGT (1.49 [1.09–2.04]).Conclusions/SignificanceIf confirmation of diabetes by a second test is required, as recommended in current guidelines, age at CFRD diagnosis was higher compared to most previous studies. However, known risk factors for glucose abnormalities in CF were confirmed. Confirmation of diabetic OGT by a repeat test is important for a consistent diagnosis of CFRD.
Highlights
Cystic fibrosis (CF) is the most frequent autosomal recessive disease in Caucasians
2.2 Study Subjects Patients were recruited during a longitudinal, prospective, multicenter study on ‘Early Diagnosis of Diabetes Mellitus in Patients with Cystic Fibrosis’ (Trial No NCT00662714) carried out between 2001 and 2010. 43 specialized centers (40 German, 3 Austrian) performed standardized oral glucose tolerance tests (OGTT) to screen serially for cystic fibrosis-related diabetes (CFRD) starting at age 10
In cystic fibrosis (CF) patients screened by OGTT, an early onset of abnormalities in glucose metabolism that became more frequent with age was observed
Summary
Cystic fibrosis (CF) is the most frequent autosomal recessive disease in Caucasians. Due to improvements in medical and nutritional therapy, life expectancy in CF increased over the last decades. The most common comorbidity is cystic fibrosis-related diabetes (CFRD). It occurs in about 2% of children, 19% of adolescents and 40–50% of adults [1]. Contrary to type 1 diabetes mellitus, insulin secretion is never totally absent in CFRD because destruction of beta cells is incomplete. As in type 2 diabetes mellitus, insulin resistance plays a role in CFRD, it is usually mild and its degree varies with infection status and steroid therapy. Published estimates on age-dependent frequency of diabetes in cystic fibrosis (CF) vary widely, and are based mostly on older data. Definition of cystic fibrosis-related diabetes (CFRD) is not in line with current guideline recommendations. We evaluated age-dependent occurrence of glucose abnormalities and associated risk factors in CF patients who participated in a multicenter screening program using oral glucose tolerance tests (OGTT)
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