glucocorticoid suppressible hyperaldosteronism (GSH); glucocorticoid remediable aldosteronism (GRA)

Pathology

• regulation of aldosterone secretion by ACTH

Genetics

• autosomal dominant
• chimeric gene duplication:
  • 11-beta-hydroxylase promoter fused to aldosterone synthase coding sequence; therefore aldosterone synthase production becomes regulated by ACTH

Clinical manifestations

• hypertension

Laboratory

• serum K+: hypokalemia may or may not be present
• serum bicarbonate: metabolic acidosis
• plasma renin: low
• plasma aldosterone: moderately high

Management

• aldosterone antagonists, potassium-sparing diuretics
  • spironolactone
  • triamterene
  • amiloride
• glucocorticoid
  • dexamethasone has less mineralocorticoid activity than other glucocorticois

More general terms

• adrenal gland disease
• genetic disease of the endocrine system

Database

• OMIM: https://mirror.omim.org/entry/103900