Identification and Treatment of Cystic Fibrosis-Related Diabetes: A survey of current medical practice in the U.S

Allen, Holley; Klingensmith, Georgeanna J.; Hamman, Richard F.

doi:10.2337/diacare.21.6.943

Cited by 29 publications

(28 citation statements)

References 4 publications

(4 reference statements)

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“…In our study, both CFRD and CF-ITG patients scored significantly lower than CF-NGT patients with respect to all clinical parameters evaluated. Whereas onset of CFRD is associated with deterioration of pulmonary function and weight loss (complications that can reverse under insulin therapy (14,20)), reports on the possible impact of IGT in clinical deterioration of CF patients are scarce in the literature (38,39). In this study, we found that the severity of impairment of glucose metabolism is inversely correlated with all indices for CF clinical status.…”

Section: Discussionsupporting

confidence: 61%

“…The well-established benefit of insulin replacement on reversion of clinical deterioration in CF patients (38,40) is currently not well documented for the pre-diabetic state. Recent studies have demonstrated that early insulin therapy improves anabolism and clinical status in CF patients with IGT, providing good glycemic control with few episodes of severe hypoglycemia (38,41).…”

Section: Discussionmentioning

confidence: 99%

“…Recent studies have demonstrated that early insulin therapy improves anabolism and clinical status in CF patients with IGT, providing good glycemic control with few episodes of severe hypoglycemia (38,41). From the perspective of clinical practice, in a cross-sectional survey performed in USA up to 61% of physicians reported the use of insulin for IGT patients (36).…”

Section: Discussionmentioning

confidence: 99%

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Insulin-secretion abnormalities and clinical deterioration related to impaired glucose tolerance in cystic fibrosis

et al. 2005

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Objective: To evaluate insulin-secretion kinetics and insulin sensitivity in cystic fibrosis (CF) patients with normal glucose tolerance (CF-NGT), impaired glucose tolerance (CF-IGT) or CF-related diabetes (CFRD), and the potential effects of moderate hyperglycemia on clinical and nutritional status. Design and methods: Cross-sectional study including 50 outpatients with CF. Patients underwent both oral (OGGT) and intravenous (IVGTT) glucose tolerance tests in order to assess insulin secretion and peripheral insulin sensitivity. Homeostasis assessment model and OGGT were used to investigate insulin sensitivity. Forced expiratory volume in the first second (FEV 1 ) and forced vital capacity (FVC) were measured to evaluate pulmonary function. Body mass index (BMI) was determined to assess nutritional status. Results: Insulin secretion was significantly decreased (and delayed at OGTT) in the CFRD group (n ¼ 9) versus the CF-IGT group (n ¼ 10) and the CF-IGT versus the CF-NGT group (n ¼ 31). Insulin sensitivity was significantly different in the CF-IGT and CFRD groups versus the CF-NGT group. FEV 1 , FVC and BMI presented a significant linear correlation with plasma glucose value at 120 min at OGTT and were significantly lower in both CF-IGT and CFRD versus the CF-NGT group, whereas no differences were found between the CF-IGT and CFRD groups. Conclusions: CF patients with IGT present diminished insulin secretion and increased peripheral insulin resistance, correlating with a worse clinical status, undernutrition and impaired pulmonary function. These findings open the question of whether early treatment of mild alterations of glucose metabolism with insulin secretagogues or short-action insulin may lead to improvement of clinical status in CF patients.European Journal of Endocrinology 152 241-247

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Section: Discussionsupporting

confidence: 61%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Insulin-secretion abnormalities and clinical deterioration related to impaired glucose tolerance in cystic fibrosis

et al. 2005

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“…20 In Canada, similar improvements have been reported, with (rounded) median survival being 24 years in 1982, and 29, 34, 33 and 37 years in 1987, 1992, 1997 and 2002, respectively, reaching 48 years in 2007. 27 The severity of CF can be assessed by the Shwachman clinical score (SS), which allocates points for general activity, physical examination, nutritional status and radiographic findings with a score out of 100, with severe disease having a score of < 40.…”

Section: Pathologysupporting

confidence: 56%

“…A survey in the USA by Allen et al 80 found a wide range of screening practices and tests, with random PG the most common, followed by HbA 1c , and urinary glucose. 80 Very few used the OGTT.…”

Section: Decision Analysismentioning

confidence: 99%

Screening for cystic fibrosis-related diabetes: a systematic review.

Waugh

Royle²,

Craigie³

et al. 2012

Health Technol Assess

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Cystic Fibrosis Related Diabetes (CFRD)—The End Stage of Progressive Insulin Deficiency

Hameed

Jaffé

Verge

2011

Pediatric Pulmonology

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In cystic fibrosis, gradual pancreatic destruction causes progressive insulin deficiency, culminating in cystic fibrosis related diabetes (CFRD). As a consequence of insulin deficiency, elevated glucose levels can be detected (well before the diagnosis of CFRD), by continuous ambulatory subcutaneous interstitial fluid glucose monitoring or 30-min sampled oral glucose tolerance test (OGTT). Current diagnostic criteria for CFRD (based on 0 and 120-min OGTT blood glucose levels) were originally designed to forecast microvascular disease in type 2 diabetes, rather than CF-specific outcomes such as declining weight or lung function. In CF, decline in either weight or lung function predicts early mortality. Both may precede the diagnosis of CFRD by several years. Insulin, a potent anabolic hormone, is recommended treatment for CFRD, but use in earlier stages of insulin deficiency is not established. Conventional dosing (with four or more insulin injections per day) is burdensome and carries substantial risk of hypoglycemia. However, recent uncontrolled trials suggest that once-daily injection of intermediate or long-acting insulin improves weight and lung function, with minimal hypoglycemia risk, in CFRD and also in early insulin deficiency. It is plausible that insulin may be of greater benefit to respiratory function when given prior to the diagnosis of CFRD, after which structural lung disease may be irreversible. It is also plausible that early insulin treatment may prolong the lifespan of the remaining insulin-secreting β-cells. Randomized controlled trials are now needed to determine whether or not current clinical practice should be altered toward the earlier commencement of insulin in CF.

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Identification and Treatment of Cystic Fibrosis-Related Diabetes: A survey of current medical practice in the U.S

Abstract: Screening for IGT is not routinely done in CF patients and screening tests vary. Greater agreement exists on methods of treating patients with persistent IGT or CFRD, although goals and aggressiveness of treatment vary with the provider's background. A consensus conference is recommended.

Cited by 29 publications

References 4 publications

Insulin-secretion abnormalities and clinical deterioration related to impaired glucose tolerance in cystic fibrosis

Insulin-secretion abnormalities and clinical deterioration related to impaired glucose tolerance in cystic fibrosis

Screening for cystic fibrosis-related diabetes: a systematic review.

Cystic Fibrosis Related Diabetes (CFRD)—The End Stage of Progressive Insulin Deficiency

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