Treatment of severe hypoglycemia due to congenital hyperinsulinemia

Congenital hyperinsulinemia is defined as excessive insulin secretion despite low blood sugar. Children with hyperinsulinemia will be at risk of neurological sequelae, even death if not diagnosed and treated promptly.

Principles of disease treatment include:
Maintaining a hypertonic glucose solution intravenously and providing it through the gastrointestinal tract; Using drugs that increase blood sugar; Surgical treatment of total pancreatectomy in case of diffuse pancreatic islet damage and local resection; The goal of treatment is to maintain blood sugar > 70 mg/dl (3.8 mmol/l) to avoid hypoglycemia and reduce the risk of convulsions causing brain damage; The treatment of hypoglycemia must be considered as a medical emergency. The priority is to establish an intravenous line immediately, in cases where a large rate of infusion and high concentrations of glucose are required, a central intravenous line should be established.

Establish a continuous intravenous glucose line. If the infusion rate is high and the glucose concentration is high, a central intravenous infusion should be ordered. Glucagon can also be used urgently to maintain blood sugar. It has the effect of raising blood sugar levels. At the same time, glucagon increases the hydrolysis of glycogen to glucose in the liver. Glucagon is used in emergency treatment as follows:
Children under 20 kg should use glucagon subcutaneously or intravenously with a dose of 0.5mg. Children from 20kg or more, intravenous injection or subcutaneous injection with a dose of 1mg. The main drugs commonly used in maintaining blood sugar due to congenital hyperinsulinemia are Diazoxide, Nifedipine, Octreotide.

Surgical treatment or surgical treatment is indicated if medical treatment is not effective or in cases where an isolated lesion is identified.
The distinction between focal and diffuse lesions is very important in choosing surgical intervention. If a focal lesion is found before or during surgery, it can be resected locally without the need for pancreatectomy.
About the procedure, first, the doctor will order many biopsy samples from different parts of the pancreas. The finding of abnormal beta-cell nuclei in all specimens suggests that this is a diffuse lesion. In this case, surgical removal of the pancreas is indicated. On the other hand, if only one sample contains an abnormal beta cell nucleus, this may be a focal lesion.
Common pancreatic biopsy abnormalities include: Significant increase in size or irregularities in the shape of the beta cells. However, these abnormalities can also be seen in patients without hypoglycemia, so a clinical diagnosis of hyperinsulinemia and hypoglycemia should be obtained prior to surgery.
If no localized lesion is identified, the surgeon will perform a partial pancreatectomy.
For children with congenital hyperinsulinemia, surgical intervention is usually performed within the first 2 months of life. Therefore, if the child is suspected of showing signs of hyperinsulinemia, parents should take the child to a reputable medical facility for timely diagnosis and treatment, to avoid dangerous complications.

Currently, at the Pediatrics Department of Vinmec International General Hospital system, many parents trust and choose to examine and treat diseases that babies and children have. Vinmec receives the trust of customers and is highly appreciated by industry experts by:
Gathering leading doctors in the field of Pediatrics: including leading experts with professional qualifications. Highly experienced (professor, associate professor, doctorate, master), experienced, worked at major hospitals such as Bach Mai, National Children's Hospital, 108... Besides, in terms of treatment, there are the participation of foreign experts (Japan, Singapore, Australia, USA) are always pioneers in applying the latest and most effective treatment regimens. Intensive treatment techniques: Vinmec has been successfully implementing many specialized techniques to help effectively treat difficult diseases in Pediatrics more effectively, including hypoglycemia caused by congenital hyperinsulinemia. Professional care: In addition to understanding children's psychology, Vinmec also pays special attention to the children's play space. This helps the children have comfortable playing conditions and familiarize themselves with the hospital's environment, cooperate in the treatment process and improve the efficiency of medical examination and treatment.