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Understanding Type A Insulin Resistance Syndrome 

Type A insulin resistance syndrome is a rare disease that causes your body to not process blood sugar well. Your insulin can’t be used normally by your body’s cells. The condition also causes other problems. These include skin changes and cysts on the ovaries. It can also lead to ongoing high blood sugar (diabetes).

What is insulin resistance?

Insulin is a hormone made by the pancreas. It helps your cells use sugar (glucose) that’s in your blood. Your pancreas makes insulin when your blood sugar level goes up. Insulin resistance is when cells don’t respond to insulin normally.

What causes type A insulin resistance syndrome?

The condition is present at birth. It’s caused by a change (mutation) in 1 or more genes. This gene change causes cells to not use insulin normally. Experts aren’t sure why the gene changes. You may have a parent with the condition. If so, you can inherit the gene. Or you may be the only person in your family with the gene change. 

Symptoms of type A insulin resistance syndrome

Women often have more symptoms. These can include:

  • Skin in body folds that turns dark, thicker, and velvety (acanthosis nigricans)

  • Extra body hair on face, chest, and back

  • Acne

  • Light and irregular periods

  • No periods

  • Cysts and more scar tissue on the ovaries

  • Problems with fertility

  • High levels of the hormone testosterone

  • Low blood sugar after eating 

Men have fewer symptoms. These can include:

  • Acanthosis nigricans

  • Low blood sugar after eating

  • Adult-onset diabetes 

Diagnosing type A insulin resistance syndrome

Your healthcare provider will ask about your symptoms and give you a physical exam. You may also have blood tests. These are done to check your blood sugar levels. 

Treatment for type A insulin resistance syndrome

Researchers are looking for the best ways to treat this condition. Your healthcare provider will talk with you about a treatment plan. You may take a medicine to help lower your blood sugar levels, such as metformin. Ask your healthcare provider about clinical trials that you can join. 

Living with type A insulin resistance syndrome

You’ll need to keep regular healthcare visits for life to watch your health. The condition can lead to diabetes that needs to be treated with high doses of insulin. Over time, high blood sugar can cause diseases of the blood vessels and heart.

When to call your healthcare provider 

Call your healthcare provider if you have any of the following:

  • Symptoms that don’t get better, or get worse

  • New symptoms

Online Medical Reviewer: Fetterman, Anne, RN, BSN
Online Medical Reviewer: Hurd, Robert, MD
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