A Case of Glucocorticoid-Responsive Hyperaldosteronism

ABSTRACT We have studied a 17-yr-old girl with hypokalemia, increased aldosterone production and suppressed plasma renin activity. Adrenal exploration revealed bilateral marked hyperplasia of the zona fasciculata and reticularis. Extirpation of the left adrenal and half of the light did not affect the hypertension, hyperaldosteronism, or hypokalemia. Dexamethasone, 2 mg/day for 2 weeks, produced normal blood pressure and serum potassium concentration. The hormonal studies showed: 1) increase in aldosterone and corticosterone secretion rates; 2) decrease in cortisol secretion and low-normal urinary excretion of 17-OHCS; 3) increase in urinary pregnanediol excretion in contrast to low-normal pregnanetriol excretion; 4) elevated plasma ACTH and a distinct increase of desoxycorticosterone secretion rate in response to metyrapone compared with a poor response of urinary 17-OHCS. These results suggest that the patient may have a partial defect of 17α-hydroxylation in the adrenal.