Abnormal glucose metabolism in cystic fibrosis☆,☆☆,★,★★,♢
Section snippets
Definitions
Type 1 diabetes, caused by autoimmune destruction of pancreatic beta cells that eventually leads to complete absence of insulin secretion, is associated with specific HLA genotypes and the presence of islet cell autoantibodies. Individuals with type 1 diabetes are initially insulin sensitive; insulin resistance may develop once hyperglycemia is present.5 Patients may have microvascular complications, including retinopathy, nephropathy, and neuropathy. Type 1 diabetes probably occurs in CF with
Frequency
A multicenter European study of 1348 patients with CF of all ages found that 4.9% of patients had diabetes,10 defined as FH (glucose >7.8 mmol/L) requiring medical therapy. Patients were excluded if they had only intermittent hyperglycemia, such as during glucocorticoid treatment. Lanng et al.16 included patients without FH and reported that 32% of Danish patients with CF who were older than 25 years had diabetes. They recommended insulin therapy for all these patients. The Cystic Fibrosis
Abnormal Pancreatic Hormone Secretion
Intravenous studies have shown that first-phase insulin and C-peptide secretion in response to glucose and other stimulatory agents is impaired in adult patients with CF who are exocrine insufficient.22, 23, 25, 28, 30 We found diminished secretion of the islet hormones insulin, glucagon, and pancreatic polypeptide in response to physiologic stimuli in 19 exocrine-insufficient patients with CF, which suggests destruction of entire islets.25 These hormonal responses were normal in 10
Clinical Significance of Hyperglycemia in CF
A wide spectrum of glucose abnormalities is seen in CF, depending on both the number and function of the remaining islets and the insulin sensitivity (and thus the insulin needs) of the individual. Glucose metabolism is further influenced by clinical factors distinctive to CF, including undernutrition, malabsorption, abnormal intestinal transit time, liver dysfunction, chronic infection, elevated energy expenditure, and increased work of breathing. Acute illness commonly causes insulin
Future Directions
The decline in weight and CF pulmonary status that has been reported to occur before the onset of clinically apparent diabetes is an important issue that deserves further attention. Insulin deficiency in CF is usually thought of in terms of its effect on blood glucose levels. Insulin, however, has important functions, independent of carbohydrate metabolism, that have scarcely been studied in CF. As a potent anabolic hormone, it plays a pivotal role in the stimulation of protein synthesis,
Acknowledgements
We thank Drs. David M. Brown, Elizabeth Seaquist, Warren Warwick, Dana Hardin, and Erica Eugster for their critical review of the manuscript.
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2021, Nutrition, Metabolism and Cardiovascular DiseasesCitation Excerpt :Although cystic fibrosis-related diabetes (CFRD) is generally recognized as a complication occurring during adulthood, it affects up to 26% of individuals under the age of 18 [1–3].
Cystic fibrosis and pregnancy: Outcome, prognostic factors and obstetrical management
2020, Gynecologie Obstetrique Fertilite et SenologiePeak OGTT glucose is associated with lower lung function in young children with cystic fibrosis
2020, Journal of Cystic FibrosisCystic fibrosis related diabetes: Medical management
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From the Departments of Pediatrics and Biostatistics, University of Minnesota, Minneapolis.
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Supported by grants from the Cystic Fibrosis Foundation, NIH-M01-RR-00400 (General Clinical Research Center) and the Minnesota Viking Children’s Fund.
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Reprint requests: Antoinette M. Moran, MD, University of Minnesota Hospital and Clinics, Pediatrics Department Box 404, 516 Delaware St. SE, Minneapolis, MN 55455.
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J Pediatr 1998;133:10-7.
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0022-3476/98/$5.00 + 0 9/19/85888