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Congenital Hyperinsulinism

What is congenital hyperinsulinism?

Congenital hyperinsulinism is a condition in which the pancreas secretes too much insulin, resulting in low blood sugar. Low blood sugar can be dangerous if the brain is not getting an adequate amount; affected individuals can experience seizures and brain damage. 

One of every 25,000-50,000 babies are born with this condition, and they can have one of eight forms of congenital hyperinsulinism. 

What are the symptoms of congenital hyperinsulinism?

Symptoms of this condition include:

  • Irritability 
  • Lethargy
  • Excessive hunger
  • A rapid heart rate
  • Sleepiness
  • Confusion
  • Weakness
  • Feeling shaky

If one is experiencing prolonged levels of very low blood sugar, they may see serious symptoms, such as seizures, comas, and even death. 

What causes congenital hyperinsulinism?

Because there are a number of forms of this condition, there are also a number of causes. Some are genetic, meaning that there are mutated genes that result in the excess of insulin being secreted. Others can be caused by a number of things, including the mother’s unmanaged diabetes and being born prematurely. 

How is congenital hyperinsulinism diagnosed?

Doctors will look at a patient’s medical history, characteristic symptoms, and test results in order to diagnose this condition. Tests that are used include blood tests and genetic testing. 

What are the treatments for congenital hyperinsulinism?

Maintaining blood sugar is an important part of treatment. This can be accomplished through surgery or medical therapy. About half of affected individuals respond to medical therapy, but the other half require a partial pancreatectomy. 

Where can I find out more about congenital hyperinsulinism?

Congenital Hyperinsulinism Articles