Cushing's Syndrome
Cushing's syndrome is the result of chronic exposure to high concentrations of circulating cortisol (a glucocorticosteroid produced in the adrenal gland), either due to exogenous administration of ACTH or glucocorticoids or due to endogenous increased secretion of ACTH or cortisol. About 85% of endogenous Cushing's syndrome is caused by excessive ACTH. About 80% of the ACTH dependent Cushing's syndromes are caused by pituitary oversecretion of ACTH (Cushing's disease) and 20% caused by ectopic ACTH. ACTH independent Cushing's syndrome can be due to a benign cortisol secreting adrenal adenoma or an adrenocortical carcinoma.

The clinical appearance of Cushing's syndrome is a result of the increased circulating levels of the steroid hormone cortisol. The Cushingoid appearance includes

  1. * rounded face (moon facies), truncal obesity (fat especially around the waist), "buffalo hump" (increased fatty tissue in the supraclavicular region)
  2. * muscle weakness (especially involving the proximal muscles that makes it difficult to stand from a squat) and fatigue
  3. * purple striae (linear streaks) on the skin
  4. * easy bruisability and low fracture threshold for the ribs and vertebrae
  5. * carbohydrate intolerance or frank diabetes mellitus
  6. * hypertension
  7. * amenorrhea

The diagnostic tests to confirm hypercortisolism may include either the 24 hour urine free cortisol concentration or the single 1 mg overnight dexamethasone suppression test. The 24 hour urine test is generally the preferred screening test since there are virtually no false negative results. With either test, positive results must be confirmed and then the cause for the excess cortisol determined. There is a complex multitest regimen that is suggested to determine the cause whenever the initial testing is positive. Identification of this syndrome and treatment is important to avoid some of the clinical consequences of chronic exposure to excess glucocorticoids.

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Eric Daiter, M.D. - Edison, NJ - E-Mail: info@drdaiter.com - Phone: (908)226-0250

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