Vetter W, Vetter H, Nussberger J, Witassek F, Beckerhoff R, Pouliadis G, Braun K P, Sobbe A, Furrer J, Siegenthaler W
Schweiz Med Wochenschr. 1976 Apr 3;106(14):469-74.
The syndrome of primary aldosteronism is caused either by an aldosterone-producing adenoma or by idiopathic bilateral adrenal hyperplasia. Hypokalemic hypertension is the leading symptome of the disease. Diagnosis is by the combination of abnormally high and non-suppressible aldosterone values with undetectable or low renin values unresponsive to postural changes or salt restriction. Patients with aldosterone-producing adenoma normally show a fall in plasma aldosterone in response to posture and ACTH-dependent circadian rhythm of aldosterone, whereas bilateral hyperplasia is characterized by postural increases in plasma aldosterone and an ACTH-independent diurnal aldosterone rhythm. These creteria serve to differentiate between adenoma and hyperplasia. An aldosterone-producing adenoma can be localized by veinography, determination of aldosterone concentration in both adrenal veins and by 131I-cholesterol scintigraphy. In our hands the determination of aldosterone in blood from both adrenal veins is the most efficient procedure. In interpreting the results, however, rhythmic and sudden changes in adrenal hormone secretion should be considered. In cases where no adrenal venous blood is obtained, 131I-cholesterol scintigraphy may be used to localize adenoma. In patients with aldosterone-producing adenomas unilateral adrenalectomy should be performed, whereas patients with idiopathic bilateral hyperplasia should receive antihypertensive therapy. As rare instances of primary aldosteronism, a case of aldosterone-producing carcinoma of the adrenal cortex and a case of presumably unilateral adrenal hyperplasia are reported.
原发性醛固酮增多症综合征是由醛固酮分泌性腺瘤或特发性双侧肾上腺增生引起的。低钾性高血压是该疾病的主要症状。诊断依据是醛固酮值异常升高且不可抑制,同时肾素值检测不到或降低,对体位改变或限盐无反应。醛固酮分泌性腺瘤患者通常在体位改变时血浆醛固酮下降,且醛固酮有依赖促肾上腺皮质激素的昼夜节律,而双侧增生的特征是血浆醛固酮随体位增加,且醛固酮有不依赖促肾上腺皮质激素的昼夜节律。这些标准有助于区分腺瘤和增生。醛固酮分泌性腺瘤可通过静脉造影、测定双侧肾上腺静脉中的醛固酮浓度以及131I -胆固醇闪烁扫描来定位。在我们手中,测定双侧肾上腺静脉血中的醛固酮是最有效的方法。然而,在解释结果时,应考虑肾上腺激素分泌的节律性和突然变化。在无法获取肾上腺静脉血的情况下,可使用131I -胆固醇闪烁扫描来定位腺瘤。对于醛固酮分泌性腺瘤患者应进行单侧肾上腺切除术,而特发性双侧增生患者应接受抗高血压治疗。作为原发性醛固酮增多症的罕见病例,报告了一例肾上腺皮质醛固酮分泌癌和一例可能为单侧肾上腺增生的病例。