In recent decades, two concepts have grown to increasing prominence in the field of cystic fibrosis related diabetes (CFRD). The first notion is that glucose abnormalities can be detected well before the diagnosis of CFRD can be made using current diagnostic criteria. The second concept is that it is biologically plausible that these glucose abnormalities may be of clinical importance to people with cystic fibrosis (via mechanisms that include increasing glucose in airway secretions [
], thereby promoting respiratory infections, as well as through catabolism from reduced secretion of insulin, a potent anabolic hormone [
- Brennan AL
- Gyi KM
- Wood DM
- Johnson J
- Holliman R
- Baines DL
- et al.
Airway glucose concentrations and effect on growth of respiratory pathogens in cystic fibrosis.
J Cyst Fibros. 2007; 6: 101-109
]. However, as these concepts have gained increasing acceptance, they have prompted further questions which presently remain unanswered.
- Ripa P
- Robertson I
- Cowley D
- Harris M
- Masters IB
- Cotterill AM.
The relationship between insulin secretion, the insulin-like growth factor axis and growth in children with cystic fibrosis.
Clin Endocrinol (Oxf). 2002; 56: 383-389
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Accepted: January 28, 2023
Received: January 19, 2023
Crown Copyright © 2023 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. All rights reserved.
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- Glycemic indices at night measured by CGM are predictive for a lower pulmonary function in adults but not in children with cystic fibrosisJournal of Cystic FibrosisVol. 22Issue 1
- PreviewAbnormalities in glucose metabolism were already described in the first publication on cystic fibrosis (CF), by Andersen . Due to the increasing life expectancy, CF-related diabetes (CFRD) has become a major comorbidity in people living with CF . Reported registry prevalence of CFRD varies depending on the applied screening frequency and definition but is rather high [2–4]. Glucose metabolism abnormalities are present in approximately 40% of infants and children [5,6], and up to 50% of adults with CF are reported to have CFRD .
- Insulinogenic index and early phase insulin secretion predict increased risk of worsening glucose tolerance and of cystic fibrosis-related diabetesJournal of Cystic FibrosisVol. 22Issue 1
- PreviewCystic fibrosis-related diabetes (CFRD) has emerged as a major co-morbidity of cystic fibrosis (CF) . Its occurrence is associated with a higher risk of accelerated pulmonary function degradation and/or weight loss exposing patients to a higher risk of early death . A defect in insulin secretion is the primary pathology underlying cystic fibrosis-related diabetes (CFRD) [2–5]. Cross-sectional studies of CF patients demonstrate both quantitative (e.g. reduced early phase and total insulin secretion and altered insulinogenic index) and qualitative (e.g.
- Continuous glucose monitoring versus self-monitoring of blood glucose in the management of cystic fibrosis related diabetes: A systematic review and meta-analysisJournal of Cystic FibrosisVol. 22Issue 1
- PreviewDue to the improved life expectancy of individuals diagnosed with cystic fibrosis (CF), there has been a rise in co-morbidities such as cystic fibrosis related diabetes (CFRD) [1,2]. Up to half of all adults with CF may have CFRD which is associated with adverse pulmonary outcomes [3–6]. This is of great consequence given respiratory disease remains the commonest cause of mortality. Studies have demonstrated that individuals with CFRD have lower baseline lung function , faster rate of pulmonary decline , altered lung microbiome , prolonged pulmonary exacerbations  and increased mortality  when compared to individuals without diabetes.