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Congenital Hyperinsulinism—Child

Authors:
  • Cynthia M. Johnson, MA
Publication Type:

Condition

Congenital Hyperinsulinism—Child

(CHI; Neonatal Hyperinsulinism)

Definition

Congenital Hyperinsulinism (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 of CHI can cause low energy and general ill feeling. Repeated low levels can cause serious health issues and slow growth. It may also lead to brain damage. This is because the brain is very sensitive to glucose levels.

Causes

CHI is caused by a problem in certain genes. There are different types of CHI based on which gene is affected. The genes control how much insulin is released. Insulin should only be released to balance glucose levels in the blood. With CHI, insulin is released even when it is not needed.

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

The doctor will ask about a child’s symptoms and past health. A physical exam will be done. Tests may include:

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

Treatment

Early treatment is important. It can help decrease the risk that CHI will cause other problems.

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

  • Feeding regularly to keep blood glucose up
  • High glucose tabs or drink
  • Giving glucose through an IV
  • Testing blood glucose often
  • Medicine to:
    • Make the liver release glucose
    • Stop the release of excess insulin

Some types of CHI may go away on their own. Further care will not be needed.

Other types of CHI may be severe or lasting. Surgery may be needed to help control these types. The pancreas will be removed. The pancreas makes insulin. For some, surgery may be a cure. For others, it should decrease episodes of low blood glucose.

Prevention

There are no steps to prevent CHI.

RESOURCES:

CANADIAN RESOURCES:

CANADIAN RESOURCES:

References

  • Congenital hyperinsulinism. Congenital Hyperinsulinism International website. Available at: https://congenitalhi.org/congenital-hyperinsulinism/#diagnosis.
  • Congenital hyperinsulinism. National Organization for Rare Disorders website. Available at: https://rarediseases.org/rare-diseases/congenital-hyperinsulinism.
  • Demirbilek, H. and Hussain, K. Congenital hyperinsulinism: diagnosis and treatment update. Journal of Clinical Research in Pediatric Endocrinology, 2017; 9 (Suppl 2): 69-87.
  • Neonatal hypoglycemia. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/neonatal-hypoglycemia-22.

Contributors

  • Chelsea Skucek, MSN, BS, RNC-NIC
Last Updated:
2022-08-01

(C) Copyright 2022 EBSCO Information Services

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.