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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
a) spironolactone
b) triamterene
c) amiloride
- glucocorticoid
- dexamethasone has less mineralocorticoid activity than other glucocorticois [2]
General
adrenal gland disease
genetic disease of the endocrine system
Database Correlations
OMIM 103900
References
- Harrison's Principles of Internal Medicine; McGraw-Hill,
13th ed. 1994; p.1967.
- Medical Knowledge Self Assessment Program (MKSAP) 15, 17, 18.
American College of Physicians, Philadelphia 2009, 2015, 2018.