Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications

Lanng, S; Thorsteinsson, Birger; C, Lund-Andersen; Nerup, Jørn; Po, Schiøtz; Koch, Christian

doi:10.1111/j.1651-2227.1994.tb12956.x

Cited by 132 publications

(93 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…These findings reflect the high risk of glucose metabolism impairment associated with this mutation, as well documented in the literature (6,9). No differences were found for sex and age distribution among groups, and mean age at CFRD diagnosis (20.4 years) was within the reported range for CFRD onset (18 -21 years) (6,8,16). According to these data, we consider our cohort of patients representative of the CF general population.…”

Section: Discussionmentioning

confidence: 58%

“…In the USA, 5 -6% of CF patients are reported to have CFRD (4,6), with increasing prevalence as patients become older (7). In Denmark, where oral glucose tolerance tests (OGGTs) are performed on a yearly basis among the CF population, up to 50% of patients older than 30 years are reported to have CFRD (8). Similarly, impaired glucose tolerance is found in 18 -47% of CF patients, showing an agerelated increase in prevalence (7,9).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

et al. 2005

View full text Add to dashboard Cite

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

show abstract

Section: Discussionmentioning

confidence: 58%

Section: Introductionmentioning

confidence: 99%

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

et al. 2005

View full text Add to dashboard Cite

show abstract

“…was recorded: age, sex, duration of diabetes, HbA 1c (A1C), BMI, sense of vibration at the index toe, pulsation in dorsalis pedis artery, blood pressure, use of antihypertensives, 24-h urinary albumine excretion, serum creatinine, and cholesterols, as described previously (4). When measurement of diurnal excretion of albumin was not possible, albumin-tocreatinine excretion ratio was measured (n ϭ 6).…”

Section: Research Design Andmentioning

confidence: 99%

“…However, ϳ20 cases of retinopathy, nephropathy, and neuropathy in CF patients with diabetes have been reported worldwide (1)(2)(3)(4)(5)(6). Background and proliferative retinopathy in CF patients with diabetes was first reported in 1986 (1,2).…”

mentioning

confidence: 99%

Cystic Fibrosis–Related Diabetes

et al. 2006

View full text Add to dashboard Cite

OBJECTIVE -Cystic fibrosis (CF)-related diabetes has been regarded as a mild form of diabetes with a low risk of severe diabetes complications. The prevalence of CF-related diabetes increases with age, resulting in a 50% prevalence of diabetes at age 30 years. We sought to investigate whether microvascular complications in CF-related diabetes appear with a relevant frequency.RESEARCH DESIGN AND METHODS -Thirty-eight patients aged 30 (range 18 -55) years with CF-related diabetes for 20 (0 -31) years were screened for diabetes complications. Because of chronic pulmonary infections, the majority of patients were regularly treated with aminoglycoside and cyclosporine given frequently.RESULTS -Since the pharmacological treatment of lung transplant patients could influence metabolical regulation and renal function, the results are given separately for nontransplanted (n ϭ 29) and transplanted (n ϭ 9) CF patients. Nine patients (27%) had retinopathy, two of which had proliferative retinopathy and needed laser treatment. Lung transplantation did not affect the prevalence of retinopathy. In nontransplanted patients, nine had hypertension, three microalbuminuria, and one elevated creatinine. None had macroalbuminuria. In transplanted patients, eight of nine had hypertension, two had microalbuminuria, and none had macroalbuminuria. Seven of nine lung transplant patients had elevated plasma creatinine, and severely reduced glomerular filtration rate was significantly more frequent.CONCLUSIONS -A high frequency of diabetic retinopathy was found in patients with insulin-treated CF-related diabetes, stressing the need for a regular screening program as in type 1 diabetes. Severely impaired kidney function was common in lung transplant patients, probably secondary to cyclosporine treatment. Diabetes Care 29:2660 -2663, 2006C ystic fibrosis (CF) is the most frequent autosomal recessive inheritable disease in the Caucasian population in Denmark, affecting 1 in 4,700 children. The disease is characterized by recurring lung infections, reduced function of the exocrine pancreas, intestinal obstruction, and liver diseases. Median life expectancy is increasing, presently ϳ30 years. Diabetes occurs with increasing frequency as the patient grows older; ϳ50% have developed diabetes at age 30 years. The pathogenesis of CF-related diabetes is unknown.Previously, treatment of patients with CF-related diabetes did not aim for strict metabolic control due to the short life expectancy. However, ϳ20 cases of retinopathy, nephropathy, and neuropathy in CF patients with diabetes have been reported worldwide (1-6). Background and proliferative retinopathy in CF patients with diabetes was first reported in 1986 (1,2). Since then, at least four cases of proliferative retinopathy have been reported, with two cases leading to blindness (3). All cases had a poor metabolic control and associated nephropathy (1-3).Patients with CF are at risk of developing secondary renal disease as a result of nephrotoxic medications, primarily aminoglycosides and cyclo...

show abstract

“…A recent study suggests that metabolic derangements in CFRD originate from an islet dysfunction inherent to the cystic fibrosis transmembrane conductance regulator (CFTR) state (6), but this hypothesis remains to be extensively demonstrated in humans. CFRD has a burden of complications, chiefly the deterioration of pulmonary function (7)(8)(9), but also classic diabetes complications, as recently shown (10,11). Overall, CFRD increases the mortality risk, particularly in females (12).…”

Section: Introductionmentioning

confidence: 99%

Spontaneous hypoglycemia in patients with cystic fibrosis

Battezzati¹,

Battezzati²,

Costantini³

et al. 2007

eur j endocrinol

View full text Add to dashboard Cite

Objective: Diabetes frequently complicates cystic fibrosis (CF) without fasting hyperglycemia or despite spontaneous hypoglycemia (anecdotally ascribed to malnutrition), whose prevalence, clinical meaning, and relationship with glucose tolerance and clinical/nutritional status were not previously investigated. The relationship of CF genotype with insulin secretion control is also unclear. Design and methods: A total of 129 CF patients without stable diabetes received 188 oral glucose tolerance tests. Distribution of fasting plasma glucose (FPG), glucose, insulin and C-peptide responses, clinical/nutritional variables, and their relationships were analyzed. Results: FPG!60 mg/dl (3.3 mmo/l) was detected in 14% of studies and reactive hypoglycemia (PG!50 mg/dl (2.8 mmo/l)) in 15%. OGTT-based diabetes frequency was similar in the lowest quartile (Q1) and Q2-3 for FPG (10 and 8%), with higher glucose increment and area under the curve in Q1. Insulin and C-peptide levels were similar among FPG quartiles. Class I cystic fibrosis transmembrane conductance regulator mutation carriers had higher insulin concentrations than class II, especially in Q1 for FPG. Age, sex, nutritional, and anthropometric parameters including fat and lean body mass were unrelated to FPG. Lower FPG was associated with more frequent hospitalization rates (PZ0.002) and lower Shwachman scores (PZ0.041). Steroids weaning was accurately evaluated but then excluded as a possible cause of hypoglycemia. Conclusions/interpretation: Fasting asymptomatic hypoglycemia is frequent and possibly related to inappropriate insulin secretion control in class I mutation carriers. Low FPG does not exclude impaired glucose tolerance (IGT) and diabetes in CF and reflects worse clinical status.

show abstract

Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications

Cited by 132 publications

References 28 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

Cystic Fibrosis–Related Diabetes

Spontaneous hypoglycemia in patients with cystic fibrosis

Contact Info

Product

Resources

About