What is Addison's disease?
What causes Addison's disease?
Who gets Addison's disease?
How does it cause disease?
What are the common findings?
How is Addison's disease diagnosed?
How is Addison's disease treated?
What are the complications?
How is Addison's disease prevented?
What research is being done?
Michael S. Kappy, M.D., Ph.D.
Chief, Pediatric Endocrinology, The Children's Hospital
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.
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
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.
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.
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.
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.
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.
The complications of untreated Addison's disease include cardiovascular collapse,
coma, and death.
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.
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.
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
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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