The goals of treatment for congenital hyperinsulinemia (CHI) are: to prevent brain damage due to recurrent hypoglycemic episodes; to establish a specific protocol for diagnosis and treatment; and to encourage normal eating habits in children while ensuring safe fasting tolerance. Treatment modalities include medical and surgical treatment. Successful treatment should enable the child to maintain a blood glucose concentration of 3.9 mmol/L or higher under age-appropriate eating habits. 1. Internal treatment, including feeding and medication, etc. (1) Intravenous glucose sedation: acute high dose of glucose: 0.2g/kg, such as 10% glucose 2ml/kg, to correct severe hypoglycemia; maintain the rate of glucose sedation at 6-8mg/kg/min, and increase the dose of glucose sedation if necessary to maintain the blood glucose above 3.9mmol/L. (2) Feeding: Give the child oral sugar or other carbohydrates according to a certain schedule to prevent the occurrence of hypoglycemia. When the child cannot eat normally, nasal feeding can be used. (3) Drug treatment: ① Diazoxide: Once the diagnosis of CHI is established, experimental treatment with diazoxide should be started for the child. Diazoxide is a potassium channel opener and is the main and preferred treatment for CHI. It is able to bind to the SURl subunit of the KATP-sensitive potassium channel, leaving the potassium channel open and inhibiting insulin secretion from the I-side. Common side effects include sodium and water storage. The starting dose of diazoxide is 5mg/kg/d (5-15mg/kg/d) orally once or twice daily, and the dose is gradually increased according to the condition of the child; at the same time, dihydroketorolac tide should be added to strengthen the effect of diazoxide and reduce the occurrence of edema. The half-life of diazoxide is long, so the evaluation of the efficacy of diazoxide should be done at least after 5 days of treatment. GDH-HI, GcK-HI, SCHAD-HI and KATP-HI due to rare ABCC8 dominant mutations are usually treated with diazoxide with good results. The vast majority of patients with KATP-HI caused by ABCC8 and KCNJll genetic mutations are ineffective in diazoxide treatment because their potassium channels are non-functional. Octreotide: Patients with KKFP-HI who are not treated with diazoxide can be further treated with octreotide. Octreotide is a long-acting injectable growth hormone inhibitory flash analog that is a potential inhibitor of insulin release. The starting dose is 5ug/kg/d subcutaneously three to four times daily. If the glucose concentration is lower than 3.9 mmol/L, the dose can be gradually increased to 20ug?kg-1?d-1. Octreotide has a good initial therapeutic effect in most children, but it can downregulate the level of bovine growth hormone inhibitory factor receptor in B cells, which makes the drug less sensitive, so it often triggers rapid tolerance soon after administration, which limits its long-term application. (3) Glucagon: It can mobilize liver glycogen to release glucose and raise blood glucose level. When the child’s blood glucose level is extremely low but cannot eat, this drug can be used to rapidly raise the blood glucose level. 2.Surgical treatment Many children with KATP-HI do not respond to medical treatment and require different degrees of pancreatic resection to maintain blood glucose at normal levels. The surgical procedure chosen depends on the histological class of the child’s congenital hyperinsulinemia. Patients with diffuse KATP-HI usually require subtotal pancreatectomy, but this procedure does not result in a cure in all children. In children with focal KATP-HI, only selective resection of the part of the pancreas containing the lesion (selective partial pancreatectomy) is required to cure the child.