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Cushing's syndrome

Cushing's syndrome or hypercortisolism is an endocrine disorder caused by excessive levels of the endogenous corticosteroid hormone cortisol. It may also be induced iatrogenically by treatment with exogenous corticosteroids for other medical conditions. It was discovered by American physician, surgeon and endocrinologist Harvey Cushing (1869-1939) and reported by him in 1932. more...

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Signs and symptoms

Symptoms include rapid weight gain, particularly of the trunk and face with sparing of the limbs (central obesity), "moon face", excess sweating, telangiectasia (dilation of capillaries), atrophy of the skin (which gets thin and bruises easily) and other mucous membranes, purple or red striae on the trunk, buttocks, arms, legs or breasts, proximal muscle weakness (hips, shoulders), and hirsutism (facial male-pattern hair growth). A common sign is the growth of fat pads along the collar bone and on the back of the neck (known as a buffalo hump). The excess cortisol may also affect other endocrine systems and cause, for example, reduced libido, impotence, amenorrhoea and infertility. Patients frequently suffer various psychological disturbances, ranging from euphoria to frank psychosis. Depression and anxiety, including panic attacks, are common.

Other signs include persistent hypertension (due to the aldosterone-like effects) and insulin resistance, leading to hyperglycemia (high blood sugars); many develop frank diabetes. Untreated Cushing's syndrome can lead to heart disease and increased mortality.

Diagnosis

When Cushing's is suspected, a dexamethasone suppression test (administration of dexamethasone and frequent determination of cortisol and ACTH levels) and 24-hour urinary measurement for cortisol have equal detection rates (Raff & Findling 2003). A novel approach is sampling cortisol in saliva over 24 hours, which may be equally sensitive. Other pituitary hormones may need to be determined, and performing physical examination directed for any visual field defect may be necessary if a pituitary lesion is suspected (which may compress the optic chiasm causing typical bitemporal hemianopia).

When these tests are positive, CT scanning of the adrenal gland and MRI of the pituitary gland are performed. These should be performed when other tests are positive, to decrease likelihood of incidentalomas (incidental discovery of harmless lesions in both organs). Scintigraphy of the adrenal gland with iodocholesterol scan is occasionally necessary. Very rarely, determining the cortisol levels in various veins in the body by venous catheterisation working towards the pituitary (petrosal sinus sampling) is necessary.

Pathophysiology

Cortisol is secreted by the adrenal glands under regulation by the pituitary gland and hypothalamus. Strictly, Cushing's syndrome refers to excess cortisol of any etiology. Cushing's disease refers only to hypercortisolism secondary to excess production of adrenocorticotropin (ACTH) from a pituitary gland adenoma.

Therapy

If an adrenal adenoma is identified it may be removed by surgery. Pituitary ACTH producing adenoma should be removed after diagnosis. Regardless of the adenoma's location, most patients will require steroid replacement postoperatively at least in the interim as long-term suppression of pituitary ACTH and normal adrenal tissue does not recover immediately. Clearly, if both adrenals are removed replacement with hydrocortisone or prednisolone is imperative.

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Cushing's Syndrome and Cushing's Disease
From American Family Physician, 9/1/00

What is Cushing's syndrome?

"Cushing's syndrome" is the name for what happens when your body makes too much cortisol. Cortisol is a natural steroid hormone that's like the "cortisone" in some medicines. Your body might make too much cortisol or you might take too much cortisone-like drugs and end up with Cushing's syndrome.

What causes Cushing's syndrome?

The most common cause of Cushing's syndrome is taking cortisone-like medicines orally (by mouth) every day for weeks to months. Prednisone is the most common medicine that's taken this way.

Inhaled steroid medicines for asthma and steroid skin creams for eczema and other skin conditions don't cause Cushing's syndrome. Even oral medicines taken every day for short periods of time or every other day for longer periods don't often cause a problem.

Be sure to tell your doctor if you're taking any other medicine or herbal product.

The next most common cause of Cushing's syndrome is Cushing's disease. Other causes are tumors in the adrenal glands or somewhere else in the body.

What is Cushing's disease?

"Cushing's disease" is the name doctors use when Cushing's syndrome is caused by a tumor in the pituitary gland. The pituitary gland is on the bottom of the brain. This gland controls the body's production of cortisol. These small tumors can cause the adrenal glands, which are near the kidneys, to make too much cortisol.

The pituitary gland tumors in Cushing's disease aren't usually cancerous. However, if these tumors are allowed to get too big, they can cause problems with your eyesight.

What are the signs and symptoms of Cushing's syndrome and disease?

One common symptom is weight gain. Fat deposits can form around your stomach and upper back. Arms and legs stay thin and don't usually get fatter.

Your skin gets thinner and is easily bruised. Cuts, scratches and insect bites take a long time to heal. Pink or purple stretch marks may form on your skin. Your face gets round and puffy.

You may feel tired and have weak muscles. You may feel depressed. Women usually have irregular menstrual periods and may grow thick or more visible body hair.

High blood pressure, osteoporosis and diabetes can be signs of Cushing's syndrome or disease.

Infections take longer to heal. If you often get skin infections or other infections, especially if the infections are unusual, you may have Cushing's syndrome or disease.

How does my doctor find out if I have Cushing's syndrome or disease?

Your doctor may start by asking you questions and doing a physical exam. If the cause is a medicine you are taking, no tests are needed. If your doctor thinks that you have Cushing's syndrome or disease, but no medicines are causing it, you may need to have some blood and urine tests.

Your doctor may ask you to collect your urine for 24 hours. Be sure to do what the doctor tells you to do for this test. Your doctor may have you take a medicine called dexamethasone before your blood or urine is collected. This tests your body's response to steroids.

At some point, you may need a CT scan or an MRI. These tests show a "picture" of your insides.

How are Cushing's disease and syndrome treated?

If you have Cushing's disease, a doctor will remove the tumor from your pituitary gland. The surgery is usually a success. Radiation treatments are sometimes used after surgery. You'll need to take cortisone-like medicines for several months after the tumor is removed. You'll need to follow your treatment plan very carefully.

COPYRIGHT 2000 American Academy of Family Physicians
COPYRIGHT 2000 Gale Group

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