Primary hyperaldosteronism in childhood due to unilateral macronodular hyperplasia: Case report

Sharon E. Oberfield, Lenore S. Levine, Adolfo Firpo, David Lawrence, Elizabeth Stoner, Donna Jean Levy, Subha Sen, Maria I. New

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27 Scopus citations

Abstract

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) (< 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.

Original languageEnglish
Pages (from-to)75-84
Number of pages10
JournalHypertension
Volume6
Issue number1
DOIs
StatePublished - Jan 1984
Externally publishedYes

Keywords

  • Adenoma
  • Adrenal gland
  • Adrenalectomy
  • Aldosterone
  • Children
  • Hyperaldosteronism
  • Hypokalemia
  • Renin-angiotensin system
  • Unilateral hyperplasia

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