An abnormality in steroid reductive metabolism in a hypertensive syndrome

Studies in a juvenile hypertensive syndrome associated with suppressed plasma renin activity and hypokalemic alkalosis failed to reveal overproduction of aldosterone or any other known steroid. There was however an abnormal increase in the fraction of unconjugated urinary steroids. Analysis of this fraction following the administration of labeled cortisol revealed that it was largely composed of dihydro metabolites reduced either at 4,5 or at C-20 and that the 4,5-dihydro fraction contained an abnormal increase in 5alpha- relative to 5beta-metabolites. There was, however, no absolute defect in the complete reduction of ring A to form tetrahydro derivatives. These findings, together with observations by Marver and Edelman that 5alpha-dihydrocortisol may be an effective mineralcorticoid, suggest the possibility of an etiologic relationship between the metabolic abnormality and the patient's hypertensive disorder.