Background Cystic fibrosis related diabetes (CFRD) is a combination between reduced insulin secretion and peripheral insulin resistance that only people with cystic fibrosis can get. CFRD is associated with a decline in lung function, poor nutritional status and high mortality rate.
Aim Study of the clinical course and therapeutic management in adolescents with CFRD.
Methods We present 2 cases of CFRD in a female patient (15 years old) and a male patient (17 years old) hospitalised at the CF Centre. The diagnosis of CF was confirmed by positive sweat test (Macroduct USA), identification of CFTR mutation (F508del/F508del), small amounts of elastase in stool. Confirmation of diabetes was achieved by a blood glucose test, blood glucose profile, glycosylated Hb, C peptide, glucose and ketones in urine.
Results On the background of pulmonary exacerbation, both patients had hyperglycemia (9.8/14.1 mmol/l). Subsequently, it triggered clinical syndromes suggestive for diabetes - polydipsia, polyuria, weight loss. Glycemic profile variations 7.8–15.8 mmol/l in boy and 10.4–21.0 mmol/l in girl. Glycosylated Hb values were high (7.3/14.1%) and C-peptide values were low (0.624/0.513). Glucose concentration in urine was 7.3/37.1 g/l and ketones was not detected. Diabetes treatment was performed with Insulin (Glargine, Aspart, Human), which produced clinical benefits by achieving glycemic and clinical syndromes control.
Conclusions Patients with CFRD shows a specific clinical framework and require a strict medical diet control and surveillance of the insulin therapy, case that differs from the other types of diabetes. If early introduced, it significantly improves life expectancy towards these patients.