Cystic-fibrosis related-diabetes (CFRD) is preceded by and associated with growth failure and deteriorating lung
Insights
Children with cystic fibrosis (CF) and impending cystic fibrosis-related diabetes (CFRD) show poor growth and declining lung function years before diagnosis. Early detection of CFRD is crucial for managing CF complications.
Area of Science:
- Pediatric Endocrinology
- Pulmonology
- Metabolic Disorders
Background:
- Cystic Fibrosis (CF)-related diabetes (CFRD) is linked to poor growth and lung function in pediatric CF patients.
- The causal relationship between CFRD development and impaired growth in children with CF remains unclear.
Purpose of the Study:
- To investigate if the onset of CFRD contributes to poor growth in children and adolescents with CF.
- To analyze growth parameters and lung function in relation to CFRD development.
Main Methods:
- A retrospective case-control study involving 32 CF patients (with or without diabetes), matched for age, gender, and pancreatic insufficiency.
- Analysis of height, weight, BMI, and lung function (FVC, FEV1, FEF75) standard deviation scores (SDS) over a mean 13-year observation period.
Main Results:
- CF patients who developed CFRD exhibited reduced height and weight SDS years before diabetes diagnosis compared to those without CFRD.
- A significant decline in height and weight SDS was observed in CFRD patients post-diagnosis, with a widening gap compared to non-diabetic CF patients.
- Lung function parameters significantly declined in CFRD patients starting one year prior to diabetes diagnosis.
Conclusions:
- Impaired growth, reduced weight, and diminished lung function precede the clinical diagnosis of CFRD in pediatric CF patients.
- These findings suggest that metabolic changes leading to CFRD impact growth and lung health several years before overt diagnosis.
Background:
Impaired glucose metabolism and cystic fibrosis (CF)-related diabetes (CFRD) are associated with insufficient weight gain and impaired lung function in children and adolescents with CF. We have asked whether imminent CFRD may be a cause of poor growth in children and adolescents.
Methods:
A retrospective case control study including 32 patients with CF with or without diabetes was conducted. Sixteen pairs, matched according to age, gender and exocrine pancreatic insufficiency, were analysed. Standard deviation scores (SDS) of height, growth, weight, body mass index (BMI), forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and forced expiratory flow at 75% of expired FVC (FEF75) were recorded during a mean observation period of 13 years per patient.
Results:
SDS of height and weight were reduced in CF patients with diabetes compared to those without, not only at the point of diagnosis (both p<0.05) but years before the evidence of diabetes. Afterwards there was a significant decline in height (p<0.001) and weight (p<0.01) SDS in CFRD patients and an increasing difference between the height and weight of CF patients with or without diabetes. In contrast, no significant reduction of BMI-SDS was observed in CFRD patients. All analysed lung function parameters showed a marked decline in CFRD patients starting 1 year prior to the diagnosis of diabetes.
Conclusions:
Deteriorating growth, reduced weight and impaired lung function are related to the development of CFRD and are obvious several years before the actual diagnosis of diabetes.
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