UNILATERAL ADRENAL HYPERSECRETION OF BOTH ALDOSTERONE AND CORTISOL IN2 FIRST COUSINS WITH A SYNDROME OF MINERALOCORTICOID EXCESS BUT WITHOUT SIGNS OF HYPERCORTISOLISM

A 38 years old woman and her first cousin, a 41 year old man, presente d both with hypertension, hypokalemia, hyperaldosteronism, and low pla sma renin activity in our Hospital, In both patients, plasma and urine aldosterone were constantly above the normal range, even on a high Na Cl diet (250 mEq/day), while the plasma aldosterone response to postur al changes was normal. In the female patient abdominal ultrasonic scan , CT scan, MRI, and adrenal gland phlebography were normal, but blood from the left adrenal vein contained 1002 pg/ml of aldosterone, versus 91 pg/ml in the contralateral one, Interestingly, the secretion of co rtisol was also lateralized (plasma cortisol levels being of 28.8 mcg% in the left, 2.3 mcg% in the right adrenal gland), although neither c linical nor laboratory signs of hypercortisolism were present, Spirono lactone treatment (100 mg/daily) completely reversed the syndrome of m ineralocorticoid excess, After 2 years, patient has normal blood press ure and serum K+ levels. In the male patient MRI showed the picture of marked hyperplasia of the left adrenal gland. Also in this case, bloo d from the left adrenal vein contained more aldosterone and cortisol ( 1304 pg/ml and 119.1 mcg% respectively) than the right one (564 pg/ml of aldosterone, and 14.6 mcg% of cartisol, respectively), without clin ical or laboratory signs of hypercortisolism, When the left gland was excised, microscopic and macroscopic subcapsular nodules were revealed . After 6 months from surgery, patient is still normotensives, with no rmal serum K+ levels and urinary Na+/K+ ratio.