Cystic Fibrosis–Related Diabetes

Abstract

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...

Update on diagnosis and monitoring of cystic fibrosis-related diabetes mellitus (CFRD)

“…In Denmark, 36% of the patients who have had diabetes for over 10 years present retinopathy (55). A study that evaluated 285 patients with CFRD did not find complications in patients who presented normal fasting glycemia.…”

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

“…retinopathy, neuropathy and nephropathy) occur in patients with CFRD. 66,67 Yung et al, 63 albeit in a small study, reported a prevalence of retinopathy among patients with CFRD who had been diagnosed for 5 years or more of 16% (5 out of 31 patients) and among those who had been diagnosed for 10 years or more of 23% (3 out of 13 patients). The prevalence of nephropathy was between 3% and 16% and of peripheral neuropathy between 5% and 21%.…”

Clinical and Laboratory Findings of Infants with Cystic Fibrosis

“…3,19,36 KF'de hipoalbüminemi karaciğer tutulumuna, pankreas etkilenmesi sonucu malabsorbiyona ve malnütrisyona bağlı gelişebilir. İlk başvuruda hipoalbüminemi %4-50 oranında bulunmuş ve hipoalbümineminin ciddi solunum sistemi semptomları ile korele olduğu bildirilmiştir.…”