Hyperinsulinism

What is Hyperinsulinism (HI)?

Hyperinsulinism (HI) is a genetic disorder in which the insulin cells of the pancreas, called beta cells, secrete too much insulin. Excess insulin causes low blood sugar (hypoglycemia).

This condition affects children and newborns. 

What are the symptoms of Hyperinsulinism (HI)? 

Congenital hyperinsulinism causes low blood sugar (hypoglycemia). Common symptoms of hypoglycemia include:

• irritability
• sleepiness
• lethargy
• excessive hunger
• rapid heart rate 

More severe symptoms, such as seizures and coma, can occur with a prolonged low blood sugar or an extremely low blood sugar. Common symptoms of hypoglycemia in older children include feelings of shakiness, weakness, tiredness, confusion, and rapid heart rate.

What causes Hyperinsulinism (HI)? 

Congenital hyperinsulinism is caused by genetic mutations that result in inappropriate and excess insulin secretion from the beta cells of the pancreas.

How is Hyperinsulinism (HI) diagnosed?

The diagnosis of congenital hyperinsulinism is based on history, laboratory findings, and genetic testing. Prompt diagnosis and establishment of effective treatment are essential to avoid neurologic damage. The diagnosis is made by evaluating the child’s medical history, and the result of laboratory and genetic tests.

What are the treatments for Hyperinsulinism (HI)?

Because congenital HI causes dangerously low blood sugars as a result of excess insulin, the treatment for this condition is to try and maintain blood sugars greater than 70 mg/dL. There are two options for treatment of congenital HI, medical therapy and surgical intervention. About 50 percent of children respond to medical therapy, while the other half require surgery for a partial or near total pancreatectomy.

Where can I find out more about Hyperinsulinism (HI)? 

• NIH
• National Organization for Rare Disorders (NORD)