Adrenal disorders

The adrenal glands are a pair of small organs located just above the kidney.These glands contain two types of tissue. The adrenal cortex produces hormones that affect water balance and metabolism in the body. The adrenal medulla produces adrenaline and noradrenaline (also called epinepherine and norepinepherine).

Release of some of these hormones is stimulated by adrenocorticotropic hormone (ACTH). ACTH is produced in the pituitary gland. ACTH levels rise in response to stress, emotions, injury, infection, burns, surgery, and decreased blood pressure. ACTH causes the release of the hormones hydrocortisone (cortisol), aldosterone, and androgen.

If the body produces too much or too little of the adrenal hormones, it couldbe an indication of a disease. Cushing's syndrome is caused by an abnormallyhigh level of hydrocortisone. The high level may be the result of an adrenalgland tumor; enlargement of both adrenal glands due to a pituitary tumor; orthe result of taking corticosteroid drugs for a long time.

Addison's disease is a rare disorder in which symptoms are caused by a deficiency of hydrocortisone and aldosterone. The most common cause of this diseaseis an autoimmune disorder, in which the immune system attacks the adrenal gland. Addison's disease generally progresses slowly. However, acute episodes,called Addisonian crises, are brought on by infection, injury, or other stresses.

Adrenal virilism is the development or premature development of male secondary sexual characteristics caused by male sex hormones (androgens) excessivelyproduced by the adrenal gland. This disorder can occur before birth and can lead to sexual abnormalities in newborns. It can also occur in girls and womenlater in life. The cause is usually genetic, but in rare cases adrenal virilism is caused by an adrenal gland tumor.

Adrenal gland cancers are rare cancers occuring in the endocrine tissue of the adrenals. A cancer that arises in the adrenal cortex is called an adrenocortical carcinoma and can produce high blood pressure, weight gain, excess bodyhair, weakening of the bones and diabetes. A cancer in the adrenal medulla is called a pheochromocytoma and can cause high blood pressure, headache, palpitations, and excessive perspiration. Although these cancers can happen at any age, most occur in young adults.

Adrenoleukodystrophy (ALD) is a rare genetic disease characterized by a lossof myelin, a substance that surrounds and insulates nerve cells in the brain.Progressive adrenal gland dysfunction is another symptom of the disease. Thechildhood form of the disease is the classical form and is the most severe.It is progressive and usually leads to total disability or death. It affectsonly boys because the genetic defect is sex-linked (carried on the X chromosome).

Diagnosis of adrenal gland disorders involves performing laboratory tests tomeasure the amounts of hormones in a patient's blood. ALD diagnosis is made based on observed symptoms, a biochemical test, and a family history. An adrenal gland scan is done when too much adrenaline and noradrenaline are producedin the body and a tumor in the adrenal gland is suspected. One such situation in which a tumor might be suspected is when high blood pressure (hypertension) does not respond to medication.

The adrenal gland scan takes several days. On the first day, a radiopharmaceutical is injected intravenously into the patient. On the second, third, and fourth day the patient is positioned under the camera for imaging for approximately 30 minutes each day. The area scanned extends from the hips to the lower chest. Sometimes the upper legs and head are also included. Follow-up testsmight include a nuclear scan of the bones or kidney, a computed tomography scan (CT) of the adrenals, or an ultrasound of the pelvic area.

If a tumor is causing the disorder, the treatment will depend on the type andlocation of the tumor. If the tumor is benign, then surgically removing thetumor may be the best option. Surgery may be done by laparoscopy in some cases. It may be followed by chemotherapy and/or radiation therapy.

Sometimes the doctor must remove the adrenal gland and the surrounding tissues. Adrenalectomy is the surgical removal of one or both glands. It is usuallyperformed by conventional (open) surgery, but in selected patients surgeonsmay use laparoscopy.

Adrenal virilism may be treated with daily doses of a glucocorticoid, but there are no drug treatments for ALD. Treatment for ALD consists of treating thesymptoms and supporting the patient with physical therapy, psychological counseling, and special education in some cases. There is no cure for this disease. Death usually occurs between one and ten years after onset of symptoms.