ACTH-independent CS is due to unilateral adrenocortical tumors, either benign (adrenocortical adenoma: 60%) or malignant (adrenocortical carcinoma: 40%) or to bilateral adrenocortical hyperplasia, including ACTH-independent macronodular adrenal hyperplasia (AIMAH) and primary pigmented nodular adrenocortical disease (PPNAD) (see these terms). ACTH-dependent CS is due to pituitary adenomas (80%) or to ectopic ACTH secretion (20%). Endogenous CS includes adrenocorticotropic hormone (ACTH) dependent CS (75-80%) and ACTH-independent CS (20-25%). EtiologyĮxogenous CS is due to anti-inflammatory steroid medications, ritonavir co-administration in HIV-infected patients or high-dose megestrol. Mild CS (termed subclinical or occult) is more common than previously thought and has been identified while investigating for diabetes, osteoporosis, hypertension or neuropsychological disturbances. Other less specific features include fatigue, high blood pressure, glucose intolerance, hypokalemia, acne, hirsutism, menstrual irregularity, diminished libido, erectile dysfunction, neuropsychological disturbances (including depression, emotional irritability, sleep disturbances, cognitive deficits), increased susceptibility to infections and urolithiasis. Typical clinical features are truncal and facial obesity, signs of hypercatabolism (thinned skin, purple striae, ecchymosis, bruising with no obvious trauma, proximal muscle weakness with amyotrophy, unexplained osteoporosis) and, in children, weight gain with decreasing growth velocity.
Prevalence of endogenous CS is 1/26,000 and, in the EU, it has an annual incidence of 1/1,400,000-1/400,000, with a peak incidence at 25-40 years of age.
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