Early brain atrophy in persistent hyperinsulinemic hypoglycemia of infancy

We present two siblings with persistent hyperinsulinemic hypoglycemia of infancy, accelerated intrauterine growth and early neonatal brain atropky. Fetal plasma glucose and insulin levels in the second sibling revealed normo-glycemia despite hyperinsulinemia. The absence of intrauterine hypoglycemia suggests that the brain damage is not secondary to hypoglycemia and other etiologies must be considered.