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Congenital Hyperinsulinism—Child

(CHI; Neonatal Hyperinsulinism)

Conn-JENN-it-ULL Hi-PERR-in-SULL-in-IZ-um

Definition

CHI is a high level of insulin. The condition is present from birth. High insulin causes low levels of glucose in the blood. Glucose is important fuel for all organs in the body. Each episode can cause low energy and general ill feeling. Repeated low levels can cause serious health issues and slow growth. It may also leave to brain damage, since the brain is very sensitive to glucose levels.

Causes

Insulin is made and released by the pancreas. The level of glucose in the blood should determine how much insulin is released. With CHI, insulin is released even when it is not needed. It is caused by a defect in certain genes. There are different types of CHI based on which gene is affected.

The Pancreas

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Risk Factors

The risk of CHI is higher if there is a family history of CHI.

Symptoms

Some symptoms of CHI in newborns may include:

  • Irritability
  • Weak or high-pitched cry
  • Sleepiness
  • Trouble feeding
  • Tremors
  • Seizures

Symptoms in children may include:

  • Shaking
  • Weakness
  • Fatigue
  • Confusion
  • Loss of consciousness

Diagnosis

You will be asked about your child’s symptoms and health past. A physical exam will be done. Tests may include:

  • Blood tests—to check glucose levels.
  • A glucagon stimulation test—blocks insulin and causes release of glucose. CHI may be present if there is an increase in glucose.
  • Genetic testing—to find which gene is affected. Can be done with blood or spit sample.

Treatment

Early treatment is important. It can help to decrease the risk of complications.

Low glusoe levels can cause damage to growing brains. If CHI is present, treatment will help to keep blood glucose at safe levels. Steps may include:

  • Feeding regularly to keep blood glucose up
  • High glucose tabs or drink or glucose given through IV
  • Frequent blood glucose testing
  • Medicine to:
    • Make the liver release glucose
    • Stop the release of excess insulin

Some types of CHI may go away on its own. Further care will not be needed. Other types of CHI may be severe or permanent. Surgery may be needed to help control these types. With surgery, the pancreas will be partly removed. This will reduce the amount of insulin the pancreas can make. For some, surgery may be a cure. For others, it should decrease episodes of low blood glucose.

Prevention

There is no known way to prevent CHI.

RESOURCES:

Congenital Hyperinsulinism International
http://www.congenitalhi.org

National Organization for Rare Disorders
https://rarediseases.org

CANADIAN RESOURCES:

Canadian Organization for Rare Disorders
https://www.raredisorders.ca

Health Canada
http://www.hc-sc.gc.ca

REFERENCES:

Congenital hyperinsulinism. Congenital Hyperinsulinism International website. Available at: http://congenitalhi.org/congenital-hyperinsulinism/#diagnosis. Accessed January 11, 2019.

Congenital hyperinsulinism. Genetics Home Reference website. Available at: https://ghr.nlm.nih.gov/condition/congenital-hyperinsulinism. Published January 8, 2019. Accessed January 11, 2019.

Congenital hyperinsulinism. National Organization for Rare Disorders website. Available at: https://rarediseases.org/rare-diseases/congenital-hyperinsulinism/#investigational-therapies. Published 2016. Accessed January 11, 2019.

Neonatal hypoglycemia. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T114886/Neonatal-hypoglycemia. Updated August 20, 2018. Accessed January 11, 2019.

Last reviewed September 2018 by EBSCO Medical Review Board Michael Woods, MD, FAAP  Last Updated: 1/8/2019