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Published on: July 5, 2022
Diabetes and prediabetes in children with cystic fibrosis
Riccardo Schiaffini1, Valentina Pampanini
1Endocrine and Diabetes Unit - Bambino Gesù Childrens' Hospital, Rome, Italy.
Insights
Cystic fibrosis-related diabetes (CFRD) diagnosis and management are evolving. Current recommendations emphasize personalized insulin therapy and highlight the benefits of CFTR modulators for glucose control in cystic fibrosis patients.
Area of Science:
- Endocrinology
- Pulmonology
- Metabolic Disorders
Background:
- Glucose metabolism abnormalities, including cystic fibrosis-related diabetes (CFRD), prediabetes, and glucose intolerance, are common in cystic fibrosis (CF).
- Early and accurate classification of these glucose abnormalities is crucial for effective management.
- This review addresses recent advancements in CFRD diagnosis and therapy.
Approach:
- Review of current literature on CFRD diagnosis and treatment.
- Evaluation of the role of Oral Glucose Tolerance Test (OGTT) versus Continuous Glucose Monitoring (CGM).
- Assessment of therapeutic strategies including insulin therapy, nutritional intervention, oral hypoglycemic agents, and CFTR modulators.
Key Points:
- Oral Glucose Tolerance Test remains the gold standard for CFRD diagnosis.
- Continuous Glucose Monitoring (CGM) is valuable for therapy management but not yet diagnostic.
- Personalized insulin therapy is the recommended treatment for pediatric CFRD.
- Nutritional intervention and oral hypoglycemic agents are also effective.
- CFTR modulators improve life expectancy, pulmonary function, nutritional status, and glucose control in CF patients.
Conclusions:
- Accurate diagnosis and timely intervention are critical for managing glucose metabolism alterations in cystic fibrosis.
- Personalized therapeutic approaches, including insulin therapy and CFTR modulators, significantly improve outcomes.
- Ongoing research and technological advancements, such as CGM, will further refine CFRD management.
Purpose Of Review:
Glucose metabolism alterations in cystic fibrosis range from the classic cystic fibrosis-related diabetes (CFRD) to forms of glucose intolerance and prediabetes. The aim of the present work is to review the most up-to-date novelties in terms of CFRD diagnosis and therapy. This review is timely and relevant because it allows an update for the early and correct classification of glucose abnormalities in cystic fibrosis and because it favours an appropriate therapeutic approach.
Recent Findings:
Confirm that Oral Glucose Tolerance Test is still the diagnostic gold standard despite the advent of continuous glucose monitoring (CGM) systems; this latter is spreading very rapidly, however, to date, there is still no strong evidence to hypothesize the use of CGM for diagnostic purposes. CGM has indeed proven to be very useful in managing and guiding CFRD therapy.
Summary:
Tailored and personalized insulin therapy is still the recommended therapy for children and adolescents with CFRD, although nutritional intervention and oral hypoglycaemic treatment are equally important and efficacious. Finally CFTR modulators have allowed the increase of the life expectancy of cystic fibrosis patients and have proven effective not only in improving the pulmonary function and the nutritional status but also the glucose control.
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