Oberfield S E, Levine L S, Firpo A, Lawrence D, Stoner E, Levy D J, Sen S, New M I
Hypertension. 1984 Jan-Feb;6(1):75-84. doi: 10.1161/01.hyp.6.1.75.
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.
我们报告首例因单侧大结节性增生导致的儿童原发性醛固酮增多症。一名10岁白人男孩，患有严重高血压（150/100 mmHg）、低钾血症（1.4 mEq/升），血浆肾素活性（PRA）受抑制（低于0.1 ng/ml/小时），其PRA和醛固酮（aldo）水平固定，不随饮食钠的改变而变化。站立姿势时血清aldo反常下降提示有肿瘤。长时间给予促肾上腺皮质激素（ACTH）使血压持续升高，但aldo短暂升高。地塞米松给药期间尿aldo有轻微下降，可排除地塞米松可抑制性醛固酮增多症。使用螺内酯后血压恢复正常。计算机断层扫描、碘胆固醇扫描和肾上腺静脉造影均未诊断出明确的肾上腺病变。虽然增生作为儿童醛固酮增多症的病因比腺瘤更常见，但由于ACTH刺激下肾上腺静脉激素采样明确显示醛固酮分泌定位于左肾上腺，故推测为肿瘤。然而，左肾上腺切除术显示为大结节性增生。术后，高血压、低钾血症和醛固酮增多症均得到逆转。因此，在儿童中，单侧醛固酮分泌过多可能是由结节性增生引起，而非单个腺瘤。
