ESPE Abstracts

Case presentation: We report two cases of patients with Cystic fibrosis-related diabetes (CFRD) Patient 1. At 5 years of age, a genetic diagnosis of cystic fibrosis with homozygous delta F508 variant was made in relation to a brother diagnosed in the newborn period. At 12 years old FEV1 91%. Initial screening at age 14 with impaired fasting glucosa and HbA1c of 5.9%; She was overweight and had acanthosis. Hospitalizations for recurrent pneumonia. At 17 years of age with exocrine pancreatic insufficiency. Poor adherence to controls. At 22 years of age OGTT (>200 mg/dl) and fasting blood glucosa (>126 mg/dl) HbA1c 6.52%. Treatment was started with an intensified insulin regimen of 0.2 IU/kg/day with FEV1 86%. Maintains irregular controls. Patient 2. Diagnosis at 8 months of age due to edematous syndrome, bronchitis and recurrent pneumonia associated with malnutrition. Genetic study of compound heterozygous variants, one of them delta F508. Exocrine pancreatic insufficiency since 8 months. Carrier of MRSA from an early age. Poor adherence to controls and treatment. With malnutrition and short stature. At 14 years of age, the first fasting blood glucose and OGTT are requested. At 18 years of age, diagnosis of diabetes by OGTT (>200 mg/dl) and fasting blood glucosa (>126 mg/dl) with HbA1c 6.1%, with FEV1 54%. Intensified insulin regimen begins at 0.15 U/kg/day until reaching 0.7 Ui/kg/day. HbA1c 7.14%. Progressive deterioration of lung function FEV1 14% with recurrent respiratory infections, severe malnutrition. He died at the age of 22.

Discussion: The development of cystic fibrosis -related diabetes (CFRD) has been associated with a worse prognosis, which is why early screening is necessary. In both patients, poor adherence to controls was observed, with late screening and diagnoses. Currently, there is only insulin therapy treatment for CFRD, but there is no recommended therapy in previous stages (impaired fasting glucose and impaired glucose tolerance). None of these patients had an indication for modulatory therapy; It must be evaluated in those, if there is a change in the evolution of this disease.