Epidemiology


Etiology


Pathophysiology


Clinical features

Mnemonic

诱发三高和溃疡,伤口感染不好长,骨松眼青人发狂 三高 is related to Permissive action of corticosteroids

  • ↑ Hematocrit
    • Steroids, particularly anabolic steroids, stimulate the production of erythropoietin

Diagnostics

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  • High-dose dexamethasone suppression test
    • Used to differ ectopic ACTH secretion vs Cushing disease
    • This test is similar to the low-dose dexamethasone suppression test but uses 8 mg of dexamethasone. Most pituitary adenomas retain partial sensitivity to feedback inhibition in response to high doses of glucocorticoids, while ectopic tumors are resistant even to high doses.

Treatment

Bilateral adrenalectomy

  • Indications
    • Primary hypercortisolism caused by bilateral adrenal disease (recommended curative treatment)
    • Emergency treatment in severe ACTH-dependent hypercortisolism that cannot be controlled pharmacologically
    • Symptomatic treatment for metastatic or occult ectopic tumors
  • Complication: Nelson syndrome (post adrenalectomy syndrome)
    • Etiology: bilateral adrenalectomy in patients with a previously undetected pituitary adenoma
    • Pathophysiology: bilateral adrenalectomy → no endogenous cortisol production → no negative feedback from cortisol on the hypothalamus → ↑ CRH production → uncontrolled enlargement of preexisting but undetected ACTH-secreting pituitary adenoma → ↑ secretion of ACTH and MSH → manifestation of symptoms due to pituitary adenoma and ↑ MSH
    • Clinical features: headache, bitemporal hemianopia (mass effect), cutaneous hyperpigmentation
    • Diagnostics
    • Treatment: surgery (e.g., transsphenoidal resection) and/or pituitary radiation therapy (e.g., if the tumor cannot be fully resected)