Effect of glucagon on glucose production, lipolysis, and gluconeogenesis in familial hyperinsulinism.

We present an infant with severe familial hyperinsulinism in whom glucose production rate, lipolysis, and gluconeogenesis from glycerol were measured by use of glucose and glycerol labelled with stable isotopes. Administration of a single dose of glucagon (0.1 mg/kg) caused an increase in glucose production rate by near 140% from 4.