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Underactive Adrenal Glands/Addison's Disease in Children

What is Addison's disease?

Addison's disease occurs when the two adrenal glands don't make enough steroid hormones, specifically cortisol and aldosterone. Cortisol controls the body's metabolism, suppresses inflammatory reactions, and affects immune system functions. Aldosterone regulates sodium and potassium levels. Addison's disease is relatively rare and may first appear at any age.

What causes Addison's disease or inadequate corticosteroid production?

Destruction of the adrenal glands due to an autoimmune disease is the most common cause. Some cases of Addison's disease are caused by the actual destruction of the adrenal glands through cancer, infection (tuberculosis, for instance), an autoimmune process, or rare genetic diseases. Other causes of inadequate corticosteroid production may include the following:

  • Corticosteroid  therapy  (such as prednisone), which slows production of natural corticosteroids by the adrenal glands

  • Certain medications used to treat fungal infections may block production of corticosteroids in the adrenal glands

Rarely, Addison's disease is inherited.

What results from inadequate corticosteroid production?

Lack of adrenal hormones may cause:

  • Elevated blood levels of potassium, which affect the water and sodium balance

  • Extreme sensitivity to the hormone insulin, which may lead to low blood sugar levels

  • Increased risk of deterioration during stressful periods, such as surgery, infection, or injury. Corticosteroids play an important role in helping the body fight infection and promote health during physical stress.

What are the symptoms of Addison's disease?

Mild symptoms of Addison's disease may only be apparent when the child is under physical stress. Symptoms may include:

  • Muscle weakness

  • Fatigue

  • Dizziness

  • Rapid pulse

  • Dark skin (first noted on hands and face)

  • Black freckles

  • Bluish-black discoloration around the nipples, mouth, rectum, scrotum, or vagina

  • Weight loss

  • Dehydration

  • Loss of appetite

  • Intense salt craving

  • Muscle aches

  • Nausea

  • Vomiting

  • Diarrhea

  • Intolerance to cold

If left untreated, Addison's disease may lead to severe abdominal pain, extreme weakness, low blood pressure, kidney failure, and shock from dehydration. Severe complications are most likely to occur when the child is experiencing physical stress. The symptoms of Addison's disease may look like other problems or medical conditions. Always consult your child's health care provider for a diagnosis.

How is Addison's disease diagnosed?

In addition to a complete medical history and physical exam, other tests may include blood tests to measure corticosteroid hormone and potassium levels.

How is Addison's disease treated?

The goal of treatment is to replace the deficient hormones and to relieve the symptoms of hormone deficiency. Since Addison's disease can be life-threatening, treatment often begins with prompt administration of corticosteroids. Corticosteroids, such as cortisol, may be taken orally or intravenously, depending on your child's condition. Usually the child must continue taking the corticosteroids for the rest of his or her life. Treatment may also include taking a medication that helps restore and maintain the body's levels of sodium and potassium.