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General: The adrenal glands are located just above each of the two kidneys. These glands secrete cortisol, also called hydrocortisone. Cortisol, often called the stress hormone, regulates the body's response to stress. It regulates proper glucose metabolism, blood pressure, and insulin release for blood sugar maintenance. It is also involved in the inflammatory response. The adrenal glands also secrete aldosterone, which belongs to a class of hormones called mineralocorticoids. This hormone helps the kidneys retain sodium and expel potassium. As a result, the hormone helps regulate blood pressure and the water and salt balance in the body. Addison's disease: Addison's disease, also called adrenal insufficiency or hypocortisolism, is a rare hormonal disorder that is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and occasional darkening of the skin. Addison's disease develops when the adrenal glands do not produce enough cortisol. Some patients with the disease may also have low levels of aldosterone. When aldosterone production falls too low, the kidneys are unable to regulate the salt and water balance, subsequently causing blood volume and blood pressure to drop. Addison's disease can be caused by a primary adrenal insufficiency. This type of deficiency occurs when the outer layer of the adrenal glands (called the adrenal cortex) is damaged and does not produce enough cortisol. Adrenal insufficiency occurs when at least 90% of the adrenal cortex has been destroyed. Several conditions, including autoimmunity, polyendocrine deficiency syndrome, and tuberculosis infection, may lead to primary adrenal insufficiency. Secondary adrenal insufficiency occurs when the adrenal glands are healthy, but factors outside of the adrenal glands cause them to produce less cortisol. The most common cause of secondary adrenal insufficiency occurs when patients who are taking cortisol-like drugs (corticosteroids) suddenly stop taking the medication. These drugs, which can be inhaled, taken by mouth, or injected into a vein, suppress the hypothalamic corticotropin releasing hormone (CRH) and adrenocorticotropic hormone (ACTH). CRH stimulates the release of ACTH, which then stimulates the adrenal glands to release cortisol hormones. Prolonged suppression of the adrenal glands may lead to shrinkage (atrophy), and it may take several weeks or months for the patient to recover to full function after the drug is discontinued. Therefore, patients should slowly taper off mediations under the supervision of their healthcare providers. Secondary adrenal insufficiency may also occur if the pituitary gland does not produce adequate amounts of ACTH, the chemical that signals the adrenal glands to release cortisol. Consequently, individuals who have low levels of ACTH will also have low levels of cortisol, even if the adrenal glands are healthy. Symptoms of Addison's disease typically develop slowly, often over several months. Physical stress, such as injury, infection, or illness, may cause symptoms to appear suddenly. Common symptoms include muscle weakness and fatigue, decreased appetite (which may cause weight loss), darkening of the skin, low blood pressure, fainting, cravings for salt, low blood sugar levels, irritability, depression, diarrhea, nausea, and vomiting. Patients who have untreated Addison's disease may experience Addisonian crisis, also known as acute adrenal failure. This typically occurs in response to physical stress, such as an injury, infection, or illness. Addisonian crisis is a serious condition that can be life threatening. Symptoms of Addisonian crisis may include lower back pain, abdominal pain, leg pain, low blood pressure, loss of consciousness, and severe vomiting and diarrhea that may lead to dehydration. Addison's disease is diagnosed after the adrenocorticotropic hormone (ACTH) test is performed. This test measures the level of cortisol in the blood and urine before and 30-60 minutes after an injection of tetracosactide (man-made ACTH). Individuals who do not have adrenal insufficiency will begin producing cortisol in response to the injection. Patients with Addison's disease will produce minimal or no cortisol in response to the injection. If the patient tests positive to the ACTH test, a corticotropin releasing hormone (CRH) stimulation test is conducted to determine the cause of adrenal insufficiency. The patient's cortisol levels are measured in the urine and blood before the test. Then, a man-made CRH is injected into the patient's vein. Cortisol levels in the blood and urine are then measured 30, 60, 90, and 120 minutes after the injection. Patients with primary adrenal insufficiency have high levels of ACTH but do not produce cortisol. Patients with secondary adrenal insufficiency have will have very low cortisol levels in response to the injection and absent or delayed ACTH responses. Absent ACTH responses indicate that the pituitary gland is causing the disease. A delayed ACTH response indicates that the hypothalamus (part of the brain that controls the release of hormones from the pituitary gland) is the cause. Since patients with Addison's disease do not produce enough hormones, treatment may include one or more hormones to replace the deficiency. Corticosteroids, such as hydrocortisone (Cortef ® or Hydrocortone ®), prednisone, cortisone, and dexamethasone (Decadron ® Baldex ®, or Dexone ®), are used to replace cortisol. Mineralocorticoids, such as fludrocortisone (Florinef ®), are used to replace aldosterone. Addisonian crisis (acute adrenal failure) is a life-threatening condition that requires immediate medical care. Treatment generally includes intravenous injections of hydrocortisone, saline solution, or sugar (dextrose). Cushing's syndrome: Cushing's syndrome, or hypercortisolism, is a condition that is characterized by a fatty hump between the shoulders (buffalo hump), a rounded face (moon face), and pink or purple stretch marks on the skin. The condition is caused by long-term exposure to high levels of cortisol, a hormone that reduces inflammation (swelling) in the body. Excessive cortisol levels may be caused by factors outside of the body (exogenous), such as medications. It may also be caused by factors inside the body (endogenous). Cushing's syndrome is most often caused by high doses of cortisol-like medications (corticosteroids), which are often prescribed to treat chronic inflammation. Cushing's syndrome may also develop if the adrenal glands make too much cortisol or if the pituitary gland releases too much adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands to release cortisol. When excessive secretions of ACTH cause the condition, it is called Cushing's disease. Tumors may also cause these glands to produce too many hormones. Common symptoms of Cushing's syndrome include weight gain (especially around the midsection and upper back), buffalo hump (fatty hump between the shoulders), fatigue, muscle weakness, rounded face (often called moon face), facial flushing, thin and fragile skin that bruises easily, depression, anxiety, irritability, hirsutism (thicker or more visible body and facial hair), irregular or absent menstrual periods in females, erectile dysfunction in males, high blood pressure, acne, and pink or purple stretch marks on the skin of the abdomen, thighs, breasts, and arms. Cushing's syndrome may be fatal if left untreated. Severe symptoms may include high blood pressure, bone loss, kidney stones, infections, and sometimes, diabetes. Urine, blood, and saliva tests are conducted to determine if the patient has elevated levels of cortisol in the body. Patients who have excessive cortisol levels will then undergo body scans that produce pictures of the internal organs to determine if a tumor inside the body is causing the condition. If the patient is taking corticosteroids, it is usually suspected that the medication is causing the condition. These tests may also help the healthcare provider rule out other medical conditions with similar symptoms. Treatments for Cushing's syndrome are designed to lower the level of cortisol in the body. Treatment options vary, depending on the cause of the syndrome. Patients should tell their healthcare providers if they are taking any other drugs (prescription or over-the-counter), herbs, or supplements because they may interact with treatment. Patients may be advised to reduce or discontinue corticosteroid use if the condition is drug-induced. Many conditions that are treated with corticosteroids can also be treated with non-corticosteroid drugs. This allows the patient to either reduce or eliminate the use of corticosteroids altogether. However, patients should not stop taking medications until they consult their healthcare providers. If a tumor is causing the condition, treatment may include surgery and/or radiation therapy. In some instances, patients with Cushing's syndrome never experience a resumption of normal adrenal function and lifelong hormone replacement therapy is necessary. Medications that are commonly used to control excessive production of cortisol include ketoconazole (Nizoral ®), mitotane (Lysodren ®), and metyrapone (Metopirone ®).
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