Abdelhamid S, Müller-Lobeck H, Pahl S, Remberger K, Bönhof J A, Walb D, Röckel A
First Department of Medicine, University Hospital, Mainz, Germany.
Arch Intern Med. 1996 Jun 10;156(11):1190-5.
Primary aldosteronism (PA) is caused by an adrenal aldosterone-producing tumor (A-APT) or adrenal hyperplasia. An extra-adrenal APT (E-APT) as a cause of PA has been reported in 5 cases. Autopsy studies show a high incidence of ectopic adrenocortical tissue. We did a prospective study of the prevalence of A-APTs and E-APTs and the biochemical features of E-APTs in patients with PA.
Hypertensive patients (N = 3900) referred to our unit were screened for PA by measuring renin activity, urinary aldosterone-18-glucuronide, tetrahydroaldosterone, and 18-hydroxycorticosterone (18-OH-B). Primary aldosteronism was found in 257 cases. The differentiation between A-APTs and adrenal hyperplasia was based on the results of postural response of renin, plasma aldosterone, 18-OH-B, computed tomography, isotope scanning, or adrenal venous aldosterone. Ultrasound examination of the abdomen was used to screen for E-APT.
The cause of PA was bilateral adrenal hyperplasia in 101 cases, unilateral adrenal hyperplasia in 2, an A-APT in 146, and an E-APT in 1. The site of aldosterone production was uncertain in 7 patients who had normal adrenal glands on computed tomography but refused to undergo isotopic scanning and adrenal venous catheterization. Ultrasound examination disclosed normal retroperitoneum in 4 of the 7 cases but could not rule out E-APT in 3 cases. The biochemical features of the patient with the E-APT were similar to classic A-APT, with low renin, high aldosterone, and high 18-OH-B values without appropriate response to posture or to short-term volume expansion. The excision of the E-APT in the right kidney resulted in normalization of blood pressure and renin, aldosterone, and 18-OH-B levels.
Although E-APT is rare, it should be considered in the interests of specific therapy for PA because aldosterone-secreting malignant ovarian tumors also have been reported.
原发性醛固酮增多症（PA）由肾上腺醛固酮分泌瘤（A - APT）或肾上腺增生引起。已有5例报告称肾上腺外A - APT（E - APT）是PA的病因。尸检研究显示异位肾上腺皮质组织的发生率很高。我们对PA患者中A - APT和E - APT的患病率以及E - APT的生化特征进行了一项前瞻性研究。
转诊至我科的高血压患者（N = 3900）通过测量肾素活性、尿醛固酮 - 18 - 葡糖苷酸、四氢醛固酮和18 - 羟皮质酮（18 - OH - B）来筛查PA。共发现257例原发性醛固酮增多症患者。A - APT与肾上腺增生的鉴别基于肾素、血浆醛固酮、18 - OH - B的体位反应结果、计算机断层扫描、同位素扫描或肾上腺静脉醛固酮。腹部超声检查用于筛查E - APT。
PA的病因是双侧肾上腺增生101例，单侧肾上腺增生2例，A - APT 146例，E - APT 1例。7例计算机断层扫描显示肾上腺正常但拒绝接受同位素扫描和肾上腺静脉插管的患者，醛固酮产生部位不确定。超声检查显示7例中有4例腹膜后正常，但3例不能排除E - APT。E - APT患者的生化特征与典型A - APT相似，肾素低、醛固酮高、18 - OH - B值高，对体位或短期容量扩张无适当反应。右肾E - APT切除后血压及肾素、醛固酮和18 - OH - B水平恢复正常。
尽管E - APT罕见，但鉴于也有分泌醛固酮的恶性卵巢肿瘤的报告，为了PA的特异性治疗，应考虑到E - APT。
