Pathologic hypofunction of the renin-angiotensin-aldosterone system

The literature now contains reports of many cases of aldosterone deficiency. Usually, the presenting features are persistent hyperkalemia and tendencies to serious urinary sodium loss and hypotension. A classification of defects leading to pathologic hypofunction of the renin-angiotensin-aldosterone system (RAAS hypofunction) is presented, with emphasis on idiopathic hyporeninemia (probably the most common). Criteria for diagnosis are (1) normal adrenal glucocorticoid function, (2) low aldosterone excretion under basal and stimulated conditions, and (3) renal tubular responsiveness to exogenous mineralocorticoid. The condition is effectively managed with mineralocorticoid replacement therapy and should be considered in the presence of unexplained persistent hyperkalemia, hyponatremia, or postural hypotension.