Abstract

The three major adrenal mineralocorticoid hormones are aldosterone, deoxycorticosterone (DOC) and corticosterone (B). To date, there are four recognized types of primary aldosteronism: (1) aldosterone-producing adenoma (APA), (2) idiopathic hyperaldosteronism (IHA), (3) indeterminate hyperaldosteronism (IndHA) and (4) glucocorticoid-remediable hyperaldosteronism (GRHA). Preoperative distinction between APA and IHA is best achieved by plotting the basal recumbent plasma renin concentration against the level of aldosterone production after administration of deoxycorticosterone acetate (DOCA). Preoperative predictability has been accurate in thirteen cases. Adrenalectomy effects cure or a marked improvement of hypertension in patients with APA, whereas the results are poor in patients with IHA although the number of patients still remains small. Patients with IndHA have all the features of hyperaldosteronism except aldosterone excretion is readily suppressed by the administration of DOCA, and the administration of spironolactone, 200 to 400 mg/day, corrects the hypertension. GRHA is readily correctable by replacement doses of dexamethasone. DOC excess is observed in patients with the virilizing 11β-hydroxylation deficiency syndrome, and both DOC and B levels are elevated in patients with the nonvirilizing 17α-hydroxylation deficiency syndrome (17-OHDS) of congenital adrenal hyperplasia. Hypertension, hypokalemia and reduced plasma renin activity and aldosterone excretion occur in patients with 17-OHDS. Excessive production of DOC and B may also contribute to the hypertension in patients with the “ectopic” ACTH syndrome and other adrenal malignancies. Other unidentified mineralocorticoid hormones may be present in hypertensive patients with low plasma renin activity and normal or low aldosterone levels.

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