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一例罕见的由双侧肾上腺皮质腺瘤引起的库欣综合征。

A rare case of Cushing's syndrome due to bilateral adrenocortical adenomas.

作者信息

Makino S, Chikazawa H, Yorimitsu A, Suehiro T, Hashimoto K, Ohashi Y, Morioka M, Sasano H

机构信息

Second Department of Internal Medicine, Kochi Medical School, Japan.

出版信息

Endocr J. 1997 Aug;44(4):533-40. doi: 10.1507/endocrj.44.533.

Abstract

We report a rare case of Cushing's syndrome due to bilateral adrenocortical adenomas in a 45-year-old female. She suffered from diabetes mellitus and hypertension for a decade, but her appearance was not Cushingoid. The plasma cortisol level in the morning was at the upper limit of the normal range, but did not show a diurnal rhythm or was suppressed by 1 mg of dexamethasone. The plasma level of ACTH was undetectable, and it failed to respond to human CRH (hCRH). Plasma cortisol responded well to synthetic ACTH. The urinary 17-OHCS level was high, and was not suppressed by 4 mg of dexamethasone. While these findings were consistent with a diagnosis of adrenocortical adenoma, computed tomography showed several nodules in both adrenal glands that suggested the presence of huge nodular adrenocortical hyperplasia or bilateral adrenocortical adenomas. Bilateral adrenalectomy demonstrated the presence of three adenomas, two in the right and one in the left adrenal. Analysis of the extract from each adenoma revealed that two of the three produced an excess amount of cortisol. Magnetic resonance imaging (MRI) of the brain suggested the presence of pituitary adenoma. Prior to adrenalectomy, TSH, GH or LH showed a low response to TRH, GHRH or LHRH, respectively. Since normal responses were restored after bilateral adrenalectomy, these abnormalities were attributed to hypercortisolemia.

摘要

我们报告了一例45岁女性因双侧肾上腺皮质腺瘤导致库欣综合征的罕见病例。她患有糖尿病和高血压十年,但外观并无库欣样表现。早晨血浆皮质醇水平处于正常范围上限,但未显示出昼夜节律,且未被1毫克地塞米松抑制。促肾上腺皮质激素(ACTH)血浆水平检测不到,且对人促肾上腺皮质激素释放激素(hCRH)无反应。血浆皮质醇对合成ACTH反应良好。尿17-羟皮质类固醇(17-OHCS)水平升高,且未被4毫克地塞米松抑制。虽然这些发现与肾上腺皮质腺瘤的诊断相符,但计算机断层扫描显示双侧肾上腺有多个结节,提示存在巨大结节性肾上腺皮质增生或双侧肾上腺皮质腺瘤。双侧肾上腺切除术证实存在三个腺瘤,右侧两个,左侧一个。对每个腺瘤提取物的分析显示,三个腺瘤中有两个产生过量皮质醇。脑部磁共振成像(MRI)提示存在垂体腺瘤。在肾上腺切除术之前,促甲状腺激素(TSH)、生长激素(GH)或促黄体生成素(LH)分别对促甲状腺激素释放激素(TRH)、生长激素释放激素(GHRH)或促黄体生成素释放激素(LHRH)反应低下。由于双侧肾上腺切除术后恢复了正常反应,这些异常归因于高皮质醇血症。

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