How is glucocorticoid resistance differentiated from hyperaldosteronism?

Updated: Oct 19, 2018
  • Author: George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London); Chief Editor: Robert P Hoffman, MD  more...
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Answer

Glucocorticoid resistance

Glucocorticoid resistance is a rare disorder that has been identified in several patients or members of kindreds. When familial, it is transmitted in both an autosomal recessive and an autosomal dominant fashion. Point mutations and microdeletions of the glucocorticoid receptor have been described.

Affected patients have an absence of cushingoid features, increased cortisol and ACTH levels (compensating for reduced glucocorticoid receptor function), and resistance to dexamethasone suppression of cortisol levels. The clinical manifestations are highly variable, though increased production of adrenal steroidogenic precursors, including deoxycorticosterone and adrenal androgens (eg, δ-4-androstenedione and dehydroepiandrostenedione), can produce hypertension in both sexes and hyperandrogenism in children and women.

Treatment consists of high-dose synthetic glucocorticoids with minimal mineralocorticoid activity (eg, dexamethasone 1-3 mg/day) to suppress plasma levels of ACTH and, ultimately, the secretion of adrenal steroids with androgenic and mineralocorticoid activity.


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