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Addison's Disease


Description

Addison's disease is a severe or total deficiency of the hormones made in the adrenal cortex, caused by a destruction of the adrenal cortex.

The inner part of the adrenal (the medulla) produces epinephrine (adrenaline) which is produced at times of stress and helps the body respond to "fight or flight" situations.

The outer portion of the adrenal, the cortex, makes two important steroid hormones, cortisol and aldosterone. Cortisol production is regulated by adrenocorticotrophic hormone (ACTH), made in the pituitary gland. Aldosterone regulates salt and water levels which affects blood volume and blood pressure.

Causes

When Dr. Thomas Addison first described this disease in London in 1855, the most common cause was tuberculosis.

Currently, the major cause of Addison's disease results from an auto-immune reaction in which the body's immune system erroneously makes antibodies against the cells of the adrenal cortex and slowly destroys them. That process takes months to years.

There are also several less common causes of Addison's disease: other chronic infections besides tuberculosis, especially certain fungal infections, invasion of the adrenal by cancer cells that have spread from another part of the body, especially the breast; CMV virus in association with AIDS; rarely, hemorrhage into the adrenals during shock; and the surgical removal of both adrenals.

Conventional Labs

The ACTH Stimulation Test is the most specific test for diagnosing Addison's disease. In this test, blood cortisol, urine cortisol, or both are measured before and after a synthetic form of ACTH is given by injection. In the so-called short, or rapid, ACTH test, measurement of cortisol in blood is repeated 30 to 60 minutes after an intravenous ACTH injection. The normal response after an injection of ACTH is a rise in blood and urine cortisol levels. Patients with either form of adrenal insufficiency respond poorly or do not respond at all.



When the response to the short ACTH test is abnormal, a "long" CRH stimulation test is required to determine the cause of adrenal insufficiency. In this test, synthetic CRH is injected intravenously and blood cortisol is measured before and 30, 60, 90, and 120 minutes after the injection. Patients with primary adrenal insufficiency have high ACTHs but do not produce cortisol. Patients with secondary adrenal insufficiency have deficient cortisol responses but absent or delayed ACTH responses. Absent ACTH response points to the pituitary as the cause; a delayed ACTH response points to the hypothalamus as the cause.

 

 

 

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