Case 2: Recurrent Hypoglycemia in Early Neonatal Period

Presentation

A female neonate is born at 40 weeks of gestation to a primigravida mother via caesarean section for a failed induction. History included second-degree consanguinity, with the mother having hypothyroidism (receiving thyroxin supplementation) and gestational diabetes mellitus (controlled on diet) antenatally. The neonate weighs 2.7 kg at birth, appropriate for gestational age. The postnatal transition is uneventful and she has no gross dysmorphic features. She continues to be exclusively breastfed and is hemodynamically stable throughout. On routine blood glucose monitoring (in view of the mother having gestational diabetes), she is found to have asymptomatic hypoglycemia with a blood glucose level of 36 mg/dL (2 mmol/L). On reexamination, she looks hyperpigmented compared to both her parents (Figs 1 and 2) without any virilization of genitals or hairy pinna. Neurologically she remains normal and abdominal examination does not reveal any hepatosplenomegaly. Her blood glucose remains less than 40 mg/dL (2.2 mmol/L) on multiple occasions in spite of supplemental formula feeds. Therefore she is admitted to the NICU, and is given glucose at a rate of 6 mg/kg per minute. Repeat blood glucose after 1 hour of starting intravenous 10% glucose is 58 mg/dL (3.2 mmol/L) and remains well within the normal range …