Hypoglycaemia due to inherited metabolic disease — branching viva

Emergency department: a 16-month-old previously well boy, found unresponsive in the morning after 36 hours of a viral illness with poor intake, seizing on arrival with a glucose of 1.0 mmol/L and mild hepatomegaly. The examiner asks: what is the emergency principle and why is the critical sample drawn before treatment, how does the insulin–ketone–free-fatty-acid pattern localise the cause, what is the fasting-tolerance and glucagon-response logic, and what is the disease-specific long-term management — then branches to a neonate with persistent high-glucose-requirement hypoglycaemia and a positive glucagon response, and asks you to discuss congenital hyperinsulinism genetics, the diazoxide–sirolimus–surgery ladder, and the diffuse-versus-focal surgical decision.