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Cystic fibrosis-related diabetes in children--gaps in the evidence?
Malay Rana1, Craig F Munns, Hiran Selvadurai
1Department of Endocrinology and Diabetes, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, 2145 NSW, Australia.
Insights
Cystic fibrosis-related diabetes mellitus is increasing, but screening and management lack standardized guidelines. More research is needed to address treatment and complications for this growing patient population.
Area of Science:
- Endocrinology
- Pulmonology
- Metabolic Disorders
Background:
- Increasing life expectancy in cystic fibrosis (CF) patients has led to a higher prevalence of CF-related diabetes mellitus (CFRDM).
- Current screening practices for CFRDM are inconsistent, hindering accurate assessment of its health burden.
- Effective management strategies for CFRDM, including prediabetes and hyperglycemia, are lacking.
Purpose of the Study:
- To highlight the growing need for evidence-based guidelines in screening and management of CFRDM.
- To identify gaps in current research regarding CFRDM treatment and complication prevention.
- To emphasize the necessity of a robust evidence base for clinical practice.
Main Methods:
- Review of existing literature on CFRDM screening, treatment, and complications.
- Analysis of current management practices and their limitations.
- Identification of areas requiring further research and intervention studies.
Main Results:
- Wide variations in CFRDM screening practices exist, impacting prevalence estimates.
- Limited evidence supports insulin use for impaired glucose tolerance in CF patients.
- Nutritional management for CFRDM lacks specific evidence-based recommendations.
- Microvascular complications are increasingly prevalent, with no intervention studies on prevention or management.
Conclusions:
- There is a critical need for standardized screening protocols for CFRDM.
- Further research is essential to establish effective insulin and nutritional therapies for CFRDM.
- Intervention studies are required to address the prevention and management of diabetic complications in CF patients.
Abstract:
As the life span of patients with cystic fibrosis has increased, so has the prevalence of cystic fibrosis-related diabetes mellitus. However, screening practices for cystic fibrosis-related diabetes mellitus vary widely, which affects accurate estimates of the health burden of this comorbidity. The management of prediabetes and hyperglycemia is an increasingly important aspect of care in patients with cystic fibrosis, but few studies have specifically addressed the management of cystic fibrosis-related diabetes mellitus. Previous studies support the use of insulin for the treatment of patients with this disorder, but the evidence for its use in patients with cystic fibrosis and impaired glucose tolerance is poor. Nutritional management is currently guided by dietary recommendations for individuals with cystic fibrosis, with little evidence specific to the dietary management of patients with cystic fibrosis-related diabetes mellitus. Additionally, microvascular complications have become more frequent as a result of the rise in life expectancy of these patients, yet to date no intervention studies have addressed prevention or management of diabetic complications in patients with cystic fibrosis. A strong evidence base is needed to guide the management of patients with cystic fibrosis-related diabetes mellitus and its complications.
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