GLUCOCORTICOID-REMEDIABLE ALDOSTERONISM - A RARE HEREDITARY FORM OF ADRENOCORTICOTROPIC HORMONE-REGULATED MINERALOCORTICOID HYPERTENSION

We report on a patient with glucocorticoid remediable aldosteronism wh o required bilateral adrenalectomy after medical therapy failed. Gluco corticoid remediable aldosteronism is an autosomal dominant form of mi neralocorticoid-induced hypertension in which aldosterone is solely re gulated by adrenocorticotropic hormone.(1) Normally, the renin-angiote nsin system controls aldosterone production by the zona glomerulosa of the adrenal cortex. In glucocorticoid remediable aldosteronism minera locorticoids are overproduced with resultant suppression of plasma ren in activity.(2,3) The genetic defect responsible for this condition ha s recently been elucidated. A gene duplication on chromosome 8 codes f or a chimeric 11 beta-hydroxylase/aldosterone synthase enzyme, which l eads to ectopic expression of aldosterone synthase in the fasciculata zone.(4) This disease is usually treated with antihypertensives and lo w dose dexamethasone, which suppresses adrenocorticotropic hormone and the subsequent production of mineralocorticoids.(5) Our patient could not tolerate either treatment and required bilateral adrenalectomy. T he literature on this rare type of hereditary hypertension is reviewed .