Hypertension, Vol 6, 75-84, Copyright © 1984 by American Heart Association
SE Oberfield, LS Levine, A Firpo, Sr Lawrence D, E Stoner, DJ Levy, S Sen and MI New
We present the first report of primary hyperaldosteronism in childhood due
to unilateral macronodular hyperplasia. A 10-year-old white boy with severe
hypertension (150/100 mm Hg), hypokalemia (1.4 mEq/liter), and suppressed
plasma renin activity (PRA) (less than 0.1 ng/ml/hr) demonstrated fixed PRA
and aldosterone (aldo) levels that did not change with alteration of
dietary sodium. The paradoxical decrease in serum aldo on assumption of
upright posture suggested a tumor. Prolonged ACTH administration produced a
continuous rise in blood pressure, but a transient rise in aldo. A minimal
decrease in urinary aldo during dexamethasone administration was noted,
excluding dexamethasone-suppressible hyperaldosteronism. Blood pressure
normalized with spironolactone. Computerized transaxial tomography,
iodocholesterol scanning, and adrenal venography were not diagnostic of a
discrete adrenal lesion. Although hyperplasia is more common than an
adenoma as a cause of hyperaldosteronism in childhood, a tumor was
predicted, since adrenal vein hormone sampling with ACTH stimulation
lateralized aldosterone secretion unequivocally to the left adrenal gland.
However, left adrenalectomy revealed macronodular hyperplasia.
Postoperatively, there was reversal of hypertension, hypokalemia, and
hyperaldosteronism. Thus, in childhood, unilateral hypersecretion of
aldosterone may result from nodular hyperplasia, rather than a discrete
adenoma.
ARTICLES
Primary hyperaldosteronism in childhood due to unilateral macronodular hyperplasia. Case report
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