The diagnosis of congenital hyperinsulinemia is based on the child’s clinical presentation and the biochemical markers of a hypoglycemic episode. There are 3 main aspects: non-ketotic hypoglycemia, excessive insulin secretion that is incompatible with hypoglycemia, and the need to increase the amount of glucose to prevent hypoglycemia. The specific diagnostic indicators of CHI were formulated for the blood tests performed in the child at the time of the crisis as follows: 1. Hyperinsulinemia (plasma insulin: >2 mU/I.); it is worth noting that the absence of hyperinsulinemia does not exclude the diagnosis of congenital hyperinsulinemia. 2. Hypofatty acidemia (plasma free fatty acids <1.5 mmol/l). Hypoketonemia (plasma free fatty acids <1.5 mmol/l). -Hydroxybutyric acid＜2.0mmol/l). 4, lmg intravenous glucagon response: blood glucose changes > 30mg / dl. If necessary, the feasibility of starvation test induced hypoglycemia to help confirm the diagnosis. Additional tests for specific types of CHI include: 1, GDH-HI: blood ammonia levels, characterized by elevated blood ammonia and leucine-sensitive hypoglycemia; 2, SCHADHI: plasma acylcarnitine concentration distribution, plasma 3 a hydroxybutyric acid a carnitine increase; urinary organic acid 3 a hydroxyglutarate increase.