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Wednesday, March 16, 2011

Cushings Disease

Cushing's syndrome or hypercortisolism or hyperadrenocorticism is an endocrine disorder caused by excessive levels of the endogenous corticosteroid hormone cortisol . Sometimes called "hypercortisolism," it is relatively rare and most commonly affects adults aged 20 to 50. An estimated 10 to 15 of every million people are affected each year. It was discovered by American physician, surgeon and endocrinologist Harvey Cushing (1869-1939) and reported by him in 1932. Cushing's syndrome occurs when the body's tissues are exposed to excessive levels of cortisol for long periods of time. Exposure to too much cortisol can occur from long-term use of synthetic glucocorticoid hormones to treat inflammatory illnesses. Another adrenal cortex hormone, aldosterone, regulates salt and water levels which affects blood volume and blood pressure. Hyperadrenocorticism occurs for two reasons a tumor of the adrenal gland that produces adrenal hormones or stimulation of the normal adrenal glands from the hormones that control it. The primary reason for this to occur is a pituitary gland tumor that produces excessive ACTH, which stimulates the adrenal gland to produce corticosteroids. Common symptoms of Cushing's syndrome include upper body obesity, severe fatigue and muscle weakness, high blood pressure, backache, elevated blood sugar, easy bruising, and bluish-red stretch marks on the skin. In women, there may be increased growth of facial and body hair, and menstrual periods may become irregular or stop completely.
Animal bodies are a marvel of interactions between organs and systems kept in balance by the production of enzymes that aid in metabolic processes and hormones that regulate body functions. When the balance is disturbed by illness, injury, or advancing age, the body goes awry: Cortisol affects the function of many organs in the body, so the signs of Cushing's disease may be varied. Hair loss caused by Cushing's disease occurs primarily on the body, sparing the head and legs. The skin is not usually itchy as it is with other skin diseases. People who have been diagnosed with depression, alcoholism, malnutrition and panic attacks tend to have higher cortisol levels as well. These types of Cushing's may be called Pseudo-Cushing's. With inflammation, the enzymes are excreted into the bloodstream and abdominal cavity instead of the gastrointestinal tract, resulting in a severe illness associated with vomiting, abdominal pain, peritonitis (inflammation of the abdominal cavity), and loss of protein into the abdominal cavity. When too much cortisol is produced in the adrenal glands, or an excess is taken in treating other diseases, significant changes occur in all of the tissues and organs of the body. All of these effects together are called Cushing's Syndrome.

Causes of Cushings Disease

The common Cause of Cushings Disease :
  • A benign or malignant growth within the adrenal gland itself, which produces cortisol;
  • Production within another part of the body (ectopic production) of a hormone that directly or indirectly stimulates the adrenal gland to make cortisol.
  • Adrenal tumors
  • An abnormal growth of the pituitary gland associated with an excessive production of one of its hormones, adrenocorticotropic hormone (ACTH), which stimulates the adrenal gland .
  • Familial Cushing's syndrome.
  • Symptoms of glucocorticoid excess generally occur with the administration of oral steroids; however, occasionally injections of steroids into joints and the use of steroid inhalers can cause Cushing syndrome.
  • Patients who have undergone organ transplants also are at risk for developing Cushing syndrome due to exogenous steroids required as part of graft antirejection medication regimens.
  • Long-term use of medication resembling cortisol (corticosteroids).

Symptoms of Cushings Disease

Some common Symptoms of Cushings Disease :
  • Absence of menstruation (amenorrhea).
  • You often get skin infections or other infections, and they take longer to heal. 
  • High blood pressure ( hypertension ).
  • Fatigue.
  • Pink or purple stretch marks may form on your skin.
  • Women usually have irregular menstrual periods and may grow thick or more visible body hair.
  • Fever
  • Sensation of abdominal "fullness".
  • Seizures.
  • Vomiting.

Treatmnent of Cushings Disease

  • The best treatment for ectopic Cushing's syndrome is surgical removal of the tumor.
  • The treatment for exogenous Cushing syndrome is gradual withdrawal of glucocorticoid.
  • Gradual withdrawal of cortisone-type drugs (under close medical supervision)
  • Bilateral adrenalectomy is an option if transsphenoidal surgery, pituitary irradiation, and medical therapy fail or if rapid normalization of cortisol levels is required. The patient then requires lifelong glucocorticoid and mineralocorticoid therapy.
  • Patients receiving these medications may require glucocorticoid replacement to avoid adrenal insufficiency. Patients should be counseled on the signs and symptoms of adrenal insufficiency when starting these drugs.
  • Drugs that inhibit cortisol synthesis - metyrapone, ketoconazole, aminoglutethimide. Indicated when case is not amenable to surgery.
  • External beam irraidation of the pituitary - slow response and restricted when surgery unsuccessful, contraindicated or not acceptable to patient.

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