Hypertension, increased aldosterone secretion and low plasma renin activity relieved by dexamethasone.

TLDR

A father and son exhibit benign hypertension, hypokalaemia, increased aldosterone secretion, raised plasma volume, and suppressed plasma renin activity. The authors propose that patients with hypertension, elevated aldosterone secretion, and low renin activity receive a trial of dexamethasone before adrenal surgery. Surgical exploration of the father revealed no adrenal tumour but nodular cortical hyperplasia in a 7‑gram left adrenal gland, with minimal juxtaglomerular granules. The patients showed intermittent urinary 17‑ketosteroid and 17‑hydroxycorticoid elevations, normal 17‑OHCS circadian rhythm and dexamethasone response, normal plasma ACTH and corticosterone, increased pregnanediol, and complete resolution of hypertension and aldosterone excess with daily dexamethasone, with relapse after cessation and no effect in controls or Conn’s syndrome.

Abstract

A father and son are described with a condition characterized by benign hypertension, potassium deficiency, increased aldosterone secretion rate (ASR), raised plasma volume and suppressed plasma renin activity (PRA). There were intermittent elevations of urine 17-ketosteroids and 17-hydroxycorticoids (17-OHCS) but no increase in urine THS, normal circadian rhythm of plasma 17-OHCS, and normal urine 17-OHCS response to dexamethasone and intravenous ACTH. Plasma ACTH and corticosterone secretion were not elevated. Pregnanetriol excretion was normal but urine pregnanediol was increased. At operation on the father no adrenal tumour was found; the excised left adrenal weighed 7 g. and showed nodular cortical hyperplasia; juxtaglomerular cells showed only occasional granules. Following operation hypertension persisted and ASR was half the preoperative value. All abnormalities in father and son were relieved by dexamethasone (DM) 2 mg. daily. The condition recurred following cessation of DM but was relieved by a second course of treatment. No such response to DM was seen in a normal subject or in a patient with Conn's syndrome. For a number of reasons it is suggested that patients with hypertension, increased ASR and low PRA be given a trial of dexamethasone treatment before undergoing adrenal surgery.