Cystic fibrosis related diabetes in Europe: Prevalence, risk factors and outcome; Olesen et al

Abstract

Background: Cystic fibrosis related diabetes (CFRD) has implications for morbidity and mortality with several risk factors identified. We studied the epidemiology of CFRD in the large dataset of the European Cystic Fibrosis Society Patient registry.

Methods: Data on CF patients were investigated for the prevalence of CFRD as well as for any association with suggested risk factors and effects.

Results: CFRD increased by approximately ten percentage points every decade from ten years of age. Prevalence was higher in females in the younger age groups. CFRD was associated with severe CF genotypes (OR = 3.11, 95%CI: 2.77-3.48), pancreatic insufficiency (OR = 1.46, 95%CI: 1.39-1.53) and female gender (OR = 1.28, 95%CI: 1.21-1.34). Patients with CFRD had higher odds of being chronically infected with Pseudomonas aeruginosa, Burkholderia cepacia complex and Stenotrophomonas maltophilia than patients without CFRD, higher odds of having FEV1% of predicted <40% (OR = 1.82, 95%CI: 1.70-1.94) and higher odds of having BMI SDS ≤-2 than patients without CFRD (OR = 1.24, 95%CI: 1.15-1.34).

Conclusions: Severe genotype, pancreatic insufficiency and female gender remain considerable intrinsic risk factors for early acquisition of CFRD. CFRD is associated with infections, lower lung function and poor nutritional status. Early diagnosis and aggressive treatment of CFRD are more important than ever with increasing life span.

Keywords: Body mass index; Cystic fibrosis; Diabetes; Gender; Lung function.