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Congenital Hyperinsulinism—Child

(CHI; Neonatal Hyperinsulinism)

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

Definition

When an infant’s pancreas releases too much insulin, it is known as congenital hyperinsulinism (CHI). This rare condition presents after a child’s birth. It can result in low blood glucose levels known as hypoglycemia. Low blood glucose levels mean the body does not have enough fuel to function or grow.

The body may compensate for most types of hypoglycemia by releasing more glucose from storage or switching to other fuel sources if they are available. However, CHI blocks these other forms of fuel. If the hypoglycemia is not reversed it can cause problems in the developing brain of infants and children.

The Pancreas

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Causes    TOP

Some forms of CHI are caused by premature birth, lack of oxygen to the brain, diabetes in the mother, or a baby with large birthweight. This is known as transient hyperinsulinism. It will usually pass on its own within 1-2 days.

Other forms of CHI are caused by a specific gene defect. The gene affects cells of the pancreas that detect glucose. This type of CHI tends to be more severe and long term.

Risk Factors    TOP

Having a family member with CHI increases a child’s risk of having the disorder.

Symptoms    TOP

Some symptoms of CHI in newborns may include:

  • Irritability
  • Sleepiness
  • Excess hunger
  • Muscle spasms
  • Loss of consciousness

Sometimes symptoms do not appear at birth. Symptoms in children may include:

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

Diagnosis    TOP

You will be asked about your child’s symptoms and medical history. A physical exam will be done. CHI can be difficult to catch because insulin levels change constantly. It may be suspected if your child has hypoglycemia even while receiving glucose.

Tests that may help with the diagnosis include:

  • Blood tests—to check your child’s insulin levels, glucose, and ketones.
  • A glucagon stimulation test—glucagon blocks insulin and stimulates the release of glucose. If there is an increase in glucose, CHI may be present.

Genetic testing may be done to find out if your child has the genetic change responsible for CHI. Since there are several forms of CHI, it will also identify which form your child has. Genetic testing can be done through a blood or saliva sample.

Treatment    TOP

For anyone with CHI, controlling episodes of hypoglycemia is the most important step. Each episode of hypoglycemia can not only make your child feel ill but can impact the health of growing organs. The brain in particular is very sensitive to low glucose levels. Regular management may include:

  • Frequent feedings to keep blood glucose up
  • Glucose given through IV
  • Regular blood testing
  • Medication to:
    • Stimulate the release of glucose from the liver, such as a glucagon injection
    • Prevent the release of excess insulin

Treatment of CHI itself will depend on the type of CHI your child has. Your child’s CHI may resolve on its own. More severe or permanent types may need surgery for better management or a possible cure.

Talk with your child’s doctor about the best treatment plan for your child. Early treatment will help decrease the risk of complications such as seizures or developmental delays. The earlier treatment is started, the better the outcomes

Surgery

The pancreas releases insulin. In rare cases of CHI, the pancreas may need to be partially or almost fully removed. This will help to reduce insulin in the body. For some, surgery may be a cure. For others, it may simply decrease the number and severity of hypoglycemia episodes.

Prevention    TOP

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

References:

Congenital hyperinsulinism. Children’s Hospital of Philadelphia website. Available at:
...(Click grey area to select URL)
Accessed May 8, 2017.
Congenital hyperinsulinism. Congenital Hyperinsulinism International website. Available at:
...(Click grey area to select URL)
Accessed May 8, 2017.
Congenital hyperinsulinism. Genetics Home Reference website. Available at: https://ghr.nlm.nih.gov/condition/congenital-hyperinsulinism. Published May 2, 2017. Accessed May 8, 2017.
Congenital hyperinsulinism. National Organization for Rare Disorders website. Available at: https://rarediseases.org/rare-diseases/congenital-hyperinsulinism/#investigational-therapies. Published 2016. Accessed May 8, 2017.
Neonatal hypoglycemia. EBSCO DynaMed Plus website. Available at: http://www.dynamed.... Updated March 12, 2017. Accessed June 29, 2017.
Last reviewed September 2017 by EBSCO Medical Review Board Michael Woods, MD, FAAP

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