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Epidemiology, pathophysiology, and prognostic implications of cystic fibrosis-related diabetes: a technical review.

Moran A, Becker D, Casella SJ, Gottlieb PA, Kirkman MS, Marshall BC, Slovis B, CFRD Consensus Conference Committ - Diabetes Care (2010)

View Article: PubMed Central - PubMed

Affiliation: University of Minnesota Medical School, Minneapolis, Minnesota, USA. moran001@umn.edu

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As in type 1 diabetes, individuals with CFRD are not obese, it often occurs in young people, insulin insufficiency is the primary defect, and metabolic syndrome features (hyperlipidemia, hypertension, visceral adiposity) are not usually present... The most surprising clamp finding is that liver insulin resistance with elevated hepatic glucose production (both in the fasting state and in response to insulin infusion) occurs not only in CF patients with diabetes, but also in those with completely normal fasting glucose levels... The earliest suggestion that CFRD might be related to type 2 diabetes was a finding of islet amyloid deposition in individuals with CFRD, similar to type 2 but not type 1 diabetes or chronic pancreatitis... More recently, a family history of type 2 diabetes was found to increase the risk of CFRD... Variation in a type 2 diabetes susceptibility gene, transcription factor 7-like 2 (TCF7L2), was shown to be associated with diabetes in CF and decreased the mean age of diabetes diagnosis by 7 years... An association was also found between CFRD and a genetic polymorphism in calpain-10, which has been reported in type 2 diabetes... Those patients who are pancreatic sufficient because of milder defects in CFTR experience less islet destruction, although chronic pancreatitis may damage and destroy islets over time... Despite significantly reduced β-cell mass, many people with CF have only mild glucose tolerance abnormalities because their peripheral insulin sensitivity is normal and their remaining β-cells are competent enough to compensate... More severe glucose tolerance abnormalities develop in those who either have worse inflammation and thus greater insulin resistance, or who have intrinsic β-cell dysfunction related to type 2 diabetes–associated genetic variations... Delayed gastric emptying is found in about half of CFRD patients, but it is also common in CF patients who do not have diabetes; diabetes may exacerbate gastrointestinal motility problems intrinsic to CF... Recently, a multicenter, randomized, placebo-controlled trial demonstrated that insulin therapy was able to reverse chronic weight loss in adult patients with CFRD FH−, ending the controversy about whether insulin should be prescribed for this “milder” form of diabetes... Thus, high blood glucose levels may contribute to CF lung disease by creating a proinflammatory, pro-bacteria environment in the airways... The presence of fasting hyperglycemia does not appear to be a critical determinant since lung function and nutritional status do not differ between CFRD FH− and CFRD FH+... However, early detection combined with aggressive insulin therapy has been shown to reduce the mortality gap between CF patients with and without diabetes and to eliminate the sex disparity in survival.

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Prevalence of CFRD based on 2005–2008 data for all patients within each age-group at the University of Minnesota. Data are presented for all patients with CFRD (CFRD total) and separately by fasting hyperglycemia status (ref. 7).
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Figure 2: Prevalence of CFRD based on 2005–2008 data for all patients within each age-group at the University of Minnesota. Data are presented for all patients with CFRD (CFRD total) and separately by fasting hyperglycemia status (ref. 7).

Mentions: CFRD is present in about 20% of adolescents and 40–50% of adults with CF (7) (Fig. 2). It is rare in childhood but has been described in children of all ages including infants (8–11). Beginning in the teenage years, the incidence is ∼3%, with some but not all centers reporting an overall female predominance (7,12). In younger individuals CFRD FH− predominates, but the prevalence of fasting hyperglycemia rises with age (7). Diabetes is associated with more severe CF gene mutations, increasing age, worse pulmonary function, undernutrition, liver dysfunction, pancreatic insufficiency, and corticosteroid use (12–15).


Epidemiology, pathophysiology, and prognostic implications of cystic fibrosis-related diabetes: a technical review.

Moran A, Becker D, Casella SJ, Gottlieb PA, Kirkman MS, Marshall BC, Slovis B, CFRD Consensus Conference Committ - Diabetes Care (2010)

Prevalence of CFRD based on 2005–2008 data for all patients within each age-group at the University of Minnesota. Data are presented for all patients with CFRD (CFRD total) and separately by fasting hyperglycemia status (ref. 7).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2992212&req=5

Figure 2: Prevalence of CFRD based on 2005–2008 data for all patients within each age-group at the University of Minnesota. Data are presented for all patients with CFRD (CFRD total) and separately by fasting hyperglycemia status (ref. 7).
Mentions: CFRD is present in about 20% of adolescents and 40–50% of adults with CF (7) (Fig. 2). It is rare in childhood but has been described in children of all ages including infants (8–11). Beginning in the teenage years, the incidence is ∼3%, with some but not all centers reporting an overall female predominance (7,12). In younger individuals CFRD FH− predominates, but the prevalence of fasting hyperglycemia rises with age (7). Diabetes is associated with more severe CF gene mutations, increasing age, worse pulmonary function, undernutrition, liver dysfunction, pancreatic insufficiency, and corticosteroid use (12–15).

View Article: PubMed Central - PubMed

Affiliation: University of Minnesota Medical School, Minneapolis, Minnesota, USA. moran001@umn.edu

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

As in type 1 diabetes, individuals with CFRD are not obese, it often occurs in young people, insulin insufficiency is the primary defect, and metabolic syndrome features (hyperlipidemia, hypertension, visceral adiposity) are not usually present... The most surprising clamp finding is that liver insulin resistance with elevated hepatic glucose production (both in the fasting state and in response to insulin infusion) occurs not only in CF patients with diabetes, but also in those with completely normal fasting glucose levels... The earliest suggestion that CFRD might be related to type 2 diabetes was a finding of islet amyloid deposition in individuals with CFRD, similar to type 2 but not type 1 diabetes or chronic pancreatitis... More recently, a family history of type 2 diabetes was found to increase the risk of CFRD... Variation in a type 2 diabetes susceptibility gene, transcription factor 7-like 2 (TCF7L2), was shown to be associated with diabetes in CF and decreased the mean age of diabetes diagnosis by 7 years... An association was also found between CFRD and a genetic polymorphism in calpain-10, which has been reported in type 2 diabetes... Those patients who are pancreatic sufficient because of milder defects in CFTR experience less islet destruction, although chronic pancreatitis may damage and destroy islets over time... Despite significantly reduced β-cell mass, many people with CF have only mild glucose tolerance abnormalities because their peripheral insulin sensitivity is normal and their remaining β-cells are competent enough to compensate... More severe glucose tolerance abnormalities develop in those who either have worse inflammation and thus greater insulin resistance, or who have intrinsic β-cell dysfunction related to type 2 diabetes–associated genetic variations... Delayed gastric emptying is found in about half of CFRD patients, but it is also common in CF patients who do not have diabetes; diabetes may exacerbate gastrointestinal motility problems intrinsic to CF... Recently, a multicenter, randomized, placebo-controlled trial demonstrated that insulin therapy was able to reverse chronic weight loss in adult patients with CFRD FH−, ending the controversy about whether insulin should be prescribed for this “milder” form of diabetes... Thus, high blood glucose levels may contribute to CF lung disease by creating a proinflammatory, pro-bacteria environment in the airways... The presence of fasting hyperglycemia does not appear to be a critical determinant since lung function and nutritional status do not differ between CFRD FH− and CFRD FH+... However, early detection combined with aggressive insulin therapy has been shown to reduce the mortality gap between CF patients with and without diabetes and to eliminate the sex disparity in survival.

Show MeSH
Related in: MedlinePlus