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Impaired Potassium-Stimulated Aldosterone Production: A Possible Explanation for Normokalemic Glucocorticoid-Remediable Aldosteronism¹

Department of Medicine (W.R.L., C.C., G.H.W., R.G.D.), Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts 02115; Department of Medicine (P.S.), New England Deaconess Hospital; Howard Hughes Medical Institute (R.P.L.), Boyer Center for Molecular Genetics, Yale University School of Medicine, New Haven, Connecticut 06510; Division of Endocrinology (F.F.), University of Padova, 35122 Padova, Italy

Address all correspondence and requests for reprints to: W. Reid Litchfield, Endocrine/Hypertension Division, Brigham and Women’s Hospital, 221 Longwood Avenue, Boston, Massachusetts 02115-5817.

Abstract

Unlike other forms of primary aldosteronism, recent prospective studies have paradoxically revealed that glucocorticoid-remediable aldosteronism (GRA) is usually characterized by normal potassium (K⁺) levels. To evaluate this paradox we studied 10 GRA subjects and 14 healthy controls in two protocols: 1) the renal K⁺ excretory response to acute oral administration of 50 mmol K⁺ chloride and to fludrocortisone, 0.2 mg po q12 h x 4 doses; and 2) the aldosterone response to administration of 50 mmol K⁺ chloride.

The K⁺ excretion rate (KER) in GRA subjects (n = 6) at baseline (45.6 ± 8.3 µEq/min), after K⁺ (134 ± 34.2 µEq/min), and after fludrocortisone (100 ± 35.0 µEq/min) was not significantly different than that seen in the control (n = 8) subjects (54.9 ± 19.0, 154 ± 35.5, 112 ± 45.8 µEq/min, respectively). Thus the renal kaliuretic response to K⁺ ingestion and exogenous mineralocorticoid is normal in GRA. Serum aldosterone increased from 5.0 ± 3.8 at baseline to a maximum of 13.1 ± 6.6 ng/dL 60 min after K⁺ ingestion in control subjects (n = 7), but failed to increase in GRA subjects (n = 14), going from 8.7 ± 3.8 (baseline) to 8.8 ± 5.4 ng/dL at 60 min (P = 0.004 vs. control). The blunted aldosterone response to K⁺ in GRA in association with the sharp diurnal decline in aldosterone in this ACTH-regulated syndrome probably results in a milder degree of hyperaldosteronism compared with other forms of primary aldosteronism, thereby producing volume expansion with minimal renal K⁺ wasting.

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