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Primary hyperaldosteronism in childhood due to unilateral macronodular hyperplasia. Case report
Insights
This study details a rare case of childhood primary hyperaldosteronism caused by unilateral macronodular hyperplasia. Surgical removal resolved severe hypertension and hypokalemia, highlighting hyperplasia as a cause of unilateral aldosterone hypersecretion in children.
Area of Science:
- Pediatric Endocrinology
- Nephrology
- Surgical Endocrinology
Background:
- Primary hyperaldosteronism is rare in childhood, often linked to adrenal adenomas or bilateral hyperplasia.
- Unilateral macronodular adrenal hyperplasia is an uncommon cause of primary hyperaldosteronism.
Observation:
- A 10-year-old boy presented with severe hypertension, hypokalemia, and suppressed plasma renin activity.
- Diagnostic tests suggested a unilateral adrenal tumor, but imaging was inconclusive.
- Adrenal vein sampling indicated lateralized aldosterone hypersecretion.
Findings:
- Left adrenalectomy revealed macronodular hyperplasia, not an adenoma.
- Post-surgery, the patient's hypertension, hypokalemia, and hyperaldosteronism resolved.
- This case demonstrates unilateral aldosterone hypersecretion from macronodular hyperplasia in a child.
Implications:
- Unilateral macronodular hyperplasia should be considered in pediatric primary hyperaldosteronism, even with imaging suggestive of a tumor.
- Accurate diagnosis and surgical intervention can lead to complete recovery.
- This finding expands the differential diagnosis for pediatric hyperaldosteronism.
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) (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.