Hypoglycemia in neonates, infants and children should be considered a medical emergency that can cause seizures, permanent neurological injury, and in rare cases, death, if inadequately treated. Under normal conditions, glucose is the primary fuel for brain metabolism. Due to the metabolic demands of the developing brain, infants and children have increased rates of glucose utilization as compared to adults. Normal regulation of glucose during fasting requires integration of glycogenolysis, gluconeogenesis, and fatty acid oxidation coordinated by various hormones. In the first days of life, the time course of the physiologic transitional changes to autonomous glucose regulation may overlap with presentation of inherited and acquired pathologic forms of hypoglycemia, introducing inherent challenges and controversy in addressing neonatal hypoglycemia. Therefore, a careful approach to the neonate with hypoglycemia is critical to determine the precise etiology so that rapid and appropriate interventions can be implemented to avoid permanent neurological injury. After infancy, hypoglycemia is uncommon, but, up to 10% of children older than one year of age presenting to emergency rooms with previously undiagnosed hypoglycemia have a serious underlying condition requiring long-term treatment. An important caveat in the pediatric population is that the classical definition of hypoglycemia, Whipple’s triad, is of limited use as young children are unable to reliably communicate symptoms. At all ages, determining the cause of the hypoglycemia is paramount for establishing specific and effective treatment to prevent further episodes of hypoglycemia and long-term neurological sequelae. The majority of hypoglycemic events in infants and children with hypoglycemic disorders occur during periods of fasting. Evaluation of key metabolic fuels and hormones (the critical sample) during a supervised fast, or at the time of spontaneous hypoglycemia, thus permits classification and relevant treatment of hypoglycemia disorders. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text,
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