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Addison

Michael S. Kappy, M.D., Ph.D.
Chief, Pediatric Endocrinology, The Children's Hospital
Denver, Colorado

What is Addison's disease?

Addison's disease is a condition where the adrenal glands fail to secrete the normal amounts of two hormones-cortisol and aldosterone. These hormones are vital to maintain the balance of salt, blood sugar, and blood pressure in an individual. An English physician, Thomas Addison, first described this disease almost 150 years ago.

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What causes Addison's disease?

When Thomas Addison first described it, the disease primarily was caused by a tuberculosis infection that resulted from consuming the milk of tuberculous cows. In modern times, the cause of Addison's disease is the production of antibodies against the adrenal glands for reasons unknown. It is known that "autoimmune" diseases of the endocrine glands can run in families, and more than one gland can be involved. Thus, individuals with Addison's disease may have problems with their thyroid glands, or they may develop diabetes.

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Who gets Addison's disease?

This condition is sporadic. That is, although Addison's disease may run in families, there is no clear mechanism of inheritance. Infected milk or any other contaminated food no longer causes the disease.

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How does it cause disease?

The two major hormones of the adrenal gland-cortisol and aldosterone-are extremely important in regulating the balance of salts and blood sugar in the body. In addition, cortisol is a very important hormone in maintaining a normal blood pressure and a normal reaction to physical stresses, such as infections, surgeries, or trauma. The absence of these hormones during times of physical stresses can lead to shock; a serious metabolic imbalance; and, if not treated rapidly, death.

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What are the common findings?

In the untreated state, Addison's disease may be accompanied by weakness, weight loss from decreased appetite, nausea and vomiting, and salt craving. Low blood pressure and metabolic imbalance also may result in a decreased level of consciousness or a coma. Addison's disease is rare in childhood, but children who are affected with it may not have a normal growth pattern.

A hormone that darkens the skin often is elevated in untreated Addison's disease. Thus, a person with this condition may show a tanning of the skin out of proportion to sunlight exposure, and without a tan line. There also may be darkening in the creases of the palms, soles, and fingers; along the gum line; and on the nipples.

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How is Addison's disease diagnosed?

Aside from the clinical signs and the signs found upon physical examination, including low blood pressure, a diagnosis of Addison's disease is usually made using laboratory tests. Typically, a patient has low blood sodium, high potassium (occasionally to life-threatening levels), and low blood sugar. The antibodies against the adrenal glands also can be measured; however, this test is just to confirm the diagnosis, since the results may not be available for many days.

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How is Addison's disease treated?

Fortunately, Addison's disease is treatable with oral forms of the missing hormones. Cortisol is available in tablet form, and it is given 2 to 3 times a day. Aldosterone is not available, but a substitute drug, Florinef, is available in tablet form, and it is given 1 to 2 times daily. In emergencies, such as physical stresses (e.g., a fever of over 101 degrees or trauma), the daily dose of cortisol is tripled.

If the patient has an illness that is accompanied by vomiting, an intramuscular injection of cortisol (Solu-Cortef) must be given at home. Then, the patient must be taken to an emergency room for further treatment. All patients or their parents must have a dose of Solu-Cortef at home and be instructed on the proper technique to administer it.

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What are the complications?

The complications of untreated Addison's disease include cardiovascular collapse, coma, and death.

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How is Addison's disease prevented?

Since there is no clear cause for the development of the antibodies against the adrenal glands, Addison's disease cannot be prevented at this time.

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What research is being done?

Research is being conducted to clarify the relationship between Addison's disease and other autoimmune diseases of the endocrine glands and other organs. Early recognition and treatment of this condition, especially during physical stresses, are key in preventing the complications caused by Addison's disease.

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References

Gotlin RW, Kappy MS, Slover RH, et al. Endocrine disorders. In: Hay WW, Jr., et al. Current pediatric diagnosis and treatment. 14th ed. Stamford, CT: Appleton and Lange, 1999:841-3.

About the Author

Dr. Kappy is a professor of pediatrics at the University of Colorado Health Sciences Center and the Chief of the Pediatric Endocrinology Department at The Children's Hospital in Denver, Colorado.

He was a recipient of the Johns Hopkins University Distinguished Alumnus Award in 1996. His research interest include the treatment of precocious puberty and the effects of growth hormone in growth hormone-deficient individuals.

Copyright 2012 Michael S. Kappy, M.D., Ph.D., All Rights Reserved

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