D2 MTE4 Case 12: A case of neonatal diabetes…

A 2-month-old baby girl (DOB 11/6/21) was diagnosed to have diabetes and was referred for further management. She was born out of non-consanguineous marriage, full term normal vaginal delivery with uneventful antenatal and perinatal period. Her birth weight was 2.3 kgs, small for gestational age, with catch up growth postnatally. Baby was apparently normal till the age of 1 month when she was hospitalised for fever and dehydration. Investigations revealed high blood glucose (450 mg/dl), HbA1c -10.5 %, serum acetone 80 mg/dl, HCO3 was 3 mEq/L. Child was discharged on regular insulin 1-0-1 and NPH 1-0-1and had multiple hypoglycaemic episodes as per SMBG. There was a history of suspicious seizure like activity as per parents. In the last 1 week, despite continuing same dose of insulin SMBG readings were 300-400 mg/dl. Child was admitted and was started on insulin infusion at 0.02 ml/kg/hr. Glibenclamide was started at a dose of 0.1 mg/kg. However, the response to Glibenclamide was partial with blood glucose in the range of 300-400 mg/dl without insulin. Child was discharged on Lispro 1-1- 0.5 and detemir 1-0-0. Genetic analysis revealed two variants of uncertain significance in KCNJ11 gene and INSR gene. She was restarted on Glibenclamide 1 mg at follow up and insulin was tapered and stopped. Currently she is on Glibenclamide 2 mg without insulin with blood glucose levels < 250 mg/dL and no hypoglycaemic episodes. The child has occasional hyperglycemic episodes with glucose values of 400 to 450 mg/dl.