Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M
Department of Pediatrics, Kenritsu Shinjo Hospital.
Tohoku J Exp Med. 1988 May;155(1):57-70. doi: 10.1620/tjem.155.57.
We present an unusual case of primary aldosteronism in childhood. A 9-year-old boy had hypertension, hypokalemia, hyporeninemia and hyperaldosteronism. Dexamethasone administration decreased plasma aldosterone transiently but failed to correct the hyperaldosteronism, excluding dexamethasone-suppressible hyperaldosteronism. Plasma aldosterone decreased with upright posture and showed a circadian rhythm. Spironolactone treatment normalized blood pressure and serum potassium and lowered aldosterone secretion. During the studies, plasma aldosterone correlated with serum cortisol but not with plasma renin. Preoperative results indicated that this patient presented the functional features of aldosteronoma. Adrenal computed tomography, scintigraphy and left venography were not diagnostic of adrenal lesions. The left adrenal venous sampling showed hypersecretion of aldosterone from the left adrenal gland. The left adrenalectomy revealed micronodular hyperplasia but resulted in a prompt and sustained reversal of hypertension and hyperaldosteronism. These findings suggest that primary aldosteronism in this patient resulted from primary adrenal hyperplasia. Thus, adrenal hyperplasia is a heterogenous group of disorders and carefully selected studies allow prospective selection of appropriate treatment.
我们报告一例儿童原发性醛固酮增多症的罕见病例。一名9岁男孩患有高血压、低钾血症、低肾素血症和醛固酮增多症。给予地塞米松后，血浆醛固酮短暂下降，但未能纠正醛固酮增多症，排除了地塞米松可抑制性醛固酮增多症。血浆醛固酮随直立姿势下降，并呈现昼夜节律。螺内酯治疗使血压和血钾正常化，并降低了醛固酮分泌。在研究过程中，血浆醛固酮与血清皮质醇相关，但与血浆肾素无关。术前结果表明该患者具有醛固酮瘤的功能特征。肾上腺计算机断层扫描、闪烁扫描和左静脉造影均未诊断出肾上腺病变。左肾上腺静脉采血显示左肾上腺醛固酮分泌过多。左肾上腺切除术显示为微结节性增生，但导致高血压和醛固酮增多症迅速且持续逆转。这些发现表明该患者的原发性醛固酮增多症是由原发性肾上腺增生引起的。因此，肾上腺增生是一组异质性疾病，精心选择的研究有助于前瞻性地选择合适的治疗方法。
