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[双侧功能性肾上腺皮质腺瘤所致原发性醛固酮增多症1例罕见病例]

[A rare case of primary aldosteronism due to bilateral functioning adrenocortical adenomas].

作者信息

Yamamoto H, Sato T, Kato Y

机构信息

Department of Medicine, Shimane Medical University, Izumo, Japan.

出版信息

Nihon Naibunpi Gakkai Zasshi. 1993 Mar 20;69(3):125-34. doi: 10.1507/endocrine1927.69.3_125.

Abstract

A 43-year-old man was admitted to our hospital in January, 1991 for further examination of polydipsia, polyuria and hypertension. He had had a personal history of hypertension since 1976 and of diabetes mellitus since 1982. Physical examination and routine laboratory studies showed that the patient was characterized by asymptomatic hypertension in the presence of hypokalemia and increased urinary potassium excretion. Plasma aldosterone concentrations (PAC) were elevated and plasma renin activity (PRA) was suppressed, resulting in a considerable increase in the ratio of PAC to PRA. PAC was not normally suppressed by saline infusion (2 1/2h, iv). PRA remained suppressed and PAC did not rise after stimulation with iv injection of furosemide (40 mg) in combination with walking for 60 min. PAC was increased in response to ACTH injection (0.25 mg, iv) but not suppressed by dexamethasone administration (2 and 8 mg/day, po). PAC did not rise after iv infusion of angiotensin II (20 ng/kg/min for 30 min). Venous sampling showed that PAC was considerably elevated in the bilateral adrenal vein. CT and MRI demonstrated tumor mass in the bilateral adrenal gland and the remaining normal portion in the left adrenal gland. Scintigraphic imaging with 133I-aldosterol during dexamethasone suppression provided bilateral uptake in the adrenals. Oral administration of spironolactone (375 mg/day) suppressed blood pressure and elevated PRA and serum potassium. Elevated PCA and PRA levels as well as hypertension were corrected by right-total and left-subtotal adrenalectomy performed in March, 1991. However, impaired glucose tolerance was not changed after surgery, and plasma glucose levels were well controlled with a small dose of insulin (9U/day). Pathological studies revealed adrenocortical adenoma cells of clear cell type with spironolactone bodies in the bilateral adrenal tumors. These findings indicate that this is a very rare case of primary aldosteronism due to bilateral functioning adrenocortical adenomas, which is accompanied by diabetes mellitus.

摘要

一名43岁男性于1991年1月入院,以便对其烦渴、多尿和高血压进行进一步检查。他自1976年起有高血压个人史,自1982年起患有糖尿病。体格检查和常规实验室检查显示,该患者的特点是存在低钾血症和尿钾排泄增加的无症状高血压。血浆醛固酮浓度(PAC)升高,血浆肾素活性(PRA)受到抑制,导致PAC与PRA的比值显著增加。静脉输注生理盐水(2.5小时,静脉注射)后,PAC未正常被抑制。静脉注射速尿(40毫克)并步行60分钟刺激后,PRA仍受抑制,PAC未升高。注射促肾上腺皮质激素(0.25毫克,静脉注射)后PAC升高,但地塞米松给药(2毫克和8毫克/天,口服)未使其受到抑制。静脉输注血管紧张素II(20纳克/千克/分钟,持续30分钟)后PAC未升高。静脉采血显示双侧肾上腺静脉中的PAC显著升高。CT和MRI显示双侧肾上腺有肿瘤肿块,左肾上腺有剩余正常部分。地塞米松抑制期间用133I - 醛固酮进行闪烁显像显示双侧肾上腺摄取。口服螺内酯(375毫克/天)可降低血压,升高PRA和血清钾。1991年3月进行的右肾上腺全切和左肾上腺次全切手术纠正了升高的PCA和PRA水平以及高血压。然而,术后糖耐量受损未改变,小剂量胰岛素(9单位/天)可很好地控制血糖水平。病理研究显示双侧肾上腺肿瘤中有透明细胞型肾上腺皮质腺瘤细胞及螺内酯体。这些发现表明,这是一例非常罕见的由双侧功能性肾上腺皮质腺瘤引起的原发性醛固酮增多症病例,且伴有糖尿病。

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