Abstract

Objectives In recent years, CF-related diabetes (CFRD) became more prevalent and is associated with a negative effect on CF outcome. To study demographics and clinical characteristics of CFRD patients documented in the standardized, multicenter German/Austrian DPV registry. Methods Until September 2014, data on 777 CFRD patients were included in the registry. Since 2013, the participating centers document lung function and type of CFTR mutation (n = 105) additionally to diabetes-related parameters. Descriptive statistics were carried out using SAS 9.4. χ 2 -test was applied for group comparisons. Results Patients aged on average 19.3 [IQR: 16.5–26.1] years and median age at CFRD diagnosis was 16.0 [13.4–20.6] years. Females were predominantly affected (58 vs. 42%, p 2 ) was present in 49.2% of patients. Glycemic control assessed by HbA1c was 6.5 [5.7–7.8]%. 75.4% of patients were treated with insulin or in combination with other glucose-lowering agents. Oral antidiabetic drugs only were used in 6.7% of patients and 17.9% received no glucose-lowering pharmacological therapy. The most common CF mutation ‘F508del homozygote' was present in 76.2% of patients. Compared to the latest national CF benchmarking report from 2012, this mutation was more common in CFRD patients (p Conclusion The study presents current data on CFRD from daily routine care. Diabetes onset is mostly around transition from pediatric care to internal medicine. Therefore, a successful and planned transition is important to provide optimal care. The previously described association between CFRD and ‘F508del homozygote' is confirmed by our data.

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