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Cushing's Syndrome

What is this condition?

Cushing's syndrome is a cluster of abnormalities caused when the adrenal glands secrete excessive steroid hormones, Its unmistakable signs include rapidly developing fatty tissue in the face, neck, and trunk and purple streaks on the skin.

Cushing's syndrome is most common in women. The prognosis depends on the underlying cause of the problem .

What causes it?

In most cases, Cushing's syndrome results from an overproduction of a hormone called corticotropin. This hormone stimulates the adrenal gland to produce the hormone cortisol and, to a lesser extent, the hormones called androgens and aldosterone.

Too much corticotropin may stem from excessive production of the hormone (a phenomenon called Cushing's disease), a tumor in another organ that produces corticotropin, or the consumption of large amounts of prescribed steroids.

In other people, the disorder results from a tumor in the adrenal gland that secretes cortisol and that's usually benign. In infants, the usual cause of Cushing's syndrome is cancer of the adrenal gland.

What are its symptoms?

Like most hormonal disorders, Cushing's syndrome induces changes throughout the body. Signs and symptoms may include the following:

  • Endocrine and metabolic systems: diabetes with decreased sugar tolerance, fasting high blood sugar, and glucose in the urine

  • Musculoskeletal system: muscle weakness, pathologic fractures, skeletal growth retardation in children

  • Skin: purplish streaks; bruises; fat pads above the clavicles, over the upper back ("buffalo hump"), on the face ("moon face"), and throughout the trunk, with slender arms and legs; poor wound healing; and acne and abnormal hairiness in women.

  • Digestive system: peptic ulcer and decreased stomach mucus

  • Nervous system: irritability and moodiness, ranging from euphoric behavior to depression or psychosis; insomnia

  • Heart and blood vessels: high blood pressure, an enlarged heart, bleeding

  • Immune system: increased susceptibility to infection, decreased resistance to stress

  • Kidneys and bladder: sodium and fluid retention, increased potassium excretion, inhibited antidiuretic hormone secretion, kidney stones

  • Reproductive system: increased androgen production, with clitoral enlargement, mild virilism, and absent or decreased menstruation in females, sexual dysfunction.

How is it diagnosed?

Lab tests measuring levels of hormones in the blood are crucial to diagnosing Cushing's syndrome. If results indicate the need for further tests, your doctor may order another lab test, called the dexamethasone suppression test, to confirm the diagnosis.

Another dexamethasone suppression test may be administered to determine if Cushing's syndrome results from a pituitary problem (Cushing's disease). Ultrasound, a computed tomography scan (commonly called a CAT scan), or angiography can help to localize tumors in the adrenal gland. A CAT scan or magnetic resonance imaging (also called MRI) of the head may help identify pituitary tumors.

How is it treated?

Treatment to restore hormone balance and reverse Cushing's syndrome may include radiation, drug therapy, or surgery. For example, people with poorly controlled diabetes, osteoporosis, and severe pathologic fractures may require radiation or surgical removal of the pituitary gland. If the person fails to respond, his or her adrenal glands may be removed.

The drugs Cytadren and Nizoral may reduce cortisol levels and are beneficial in relieving symptoms of Cushing's syndrome. Cytadren alone, or in combination with Metopirone, may also be useful in treating adrenal cancer that has spread.

Cortisol therapy is essential during and after surgery to help the person tolerate the physiologic stress imposed by removal of the pituitary or adrenal glands. If normal cortisol production resumes, steroid therapy may be gradually tapered and eventually discontinued. However, removal of both adrenal glands or of the entire pituitary gland requires lifelong steroid replacement therapy to correct hormonal deficiencies.

What can a person with Cushing's syndrome do?

Following surgical removal of the adrenal or pituitary glands, the doctor will prescribe replacement steroids. Follow necessary precautions for undergoing this type of drug therapy:

  • Be aware that you'll need lifelong drug therapy.

  • Take prescribed steroids with antacids or meals to minimize stomach upset. (Usually, it helps to take two-thirds of the dose in the morning and the remainder in the early afternoon.)

  • Carry a medical identification card.

  • Immediately report stressful situations, such as infections, which call for an increase in the steroid dosage.

  • Watch closely for signs of an inadequate steroid dosage (fatigue, weakness, dizziness) and steroid overdose (severe swelling, weight gain). Never abruptly discontinue taking steroids - this may cause a fatal adrenal crisis.

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