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原发性醛固酮增多症合并亚临床库欣综合征:一例报告及文献复习

Primary aldosteronism concurrent with subclinical Cushing's syndrome: a case report and review of the literature.

作者信息

Zhang Yingxiao, Tan Jianyu, Yang Qin, Du Zhipeng, Yang Shumin, He Wenwen, Song Ying, Hu Jinbo, Yang Yi, Li Qifu, Zhang Yao, He Yunfeng, Cheng Qingfeng

机构信息

Department of Endocrinology, The First Affiliated Hospital of Chongqing Medical University, No.1 Youyi St., Chongqing, 400016, China.

Department of Urology, The First Affiliated Hospital of Chongqing Medical University, No.1 Youyi St., Chongqing, 400016, China.

出版信息

J Med Case Rep. 2020 Feb 20;14(1):32. doi: 10.1186/s13256-020-2353-8.

Abstract

BACKGROUND

The prevalence of primary aldosteronism concurrent with subclinical Cushing's syndrome was higher than previously thought. Through analyzing a rare clinical case, we summarized the diagnosis and management of primary aldosteronism with subclinical Cushing's syndrome.

CASE PRESENTATION

A 54-year-old Chinese man of Han nationality was diagnosed as having primary aldosteronism with subclinical Cushing's syndrome. An abdominal computed tomography scan revealed a mass in his left adrenal gland and a mass in his right adrenal gland. After finishing sequential adrenal venous sampling without adrenocorticotropic hormone, the result reminded us that the left and right nodules were responsible for hypercortisolism and aldosterone hypersecretion, respectively. Right and left adrenalectomy were performed successively. The pathological diagnosis was adrenocortical adenoma for both. Histological findings revealed that the right one had positive immunostaining for CYP11B2 and the left one had positive immunostaining for CYP11B1. The immunohistochemistry result helped us to confirm the diagnosis. Somatic KCNJ5 mutation (Leu168Arg) was found in the right tumor; there was no KCNJ5 mutation in the left adrenal tumor.

CONCLUSIONS

We suggest that patients with primary aldosteronism should have a low-dose overnight dexamethasone suppression test to screen for hypercortisolism. It can help avoid misdiagnoses and contribute to proper understanding of the adrenal vein sampling result. Making sure of the nidus of aldosterone and cortisol secretion is crucial for the therapy of patients with primary aldosteronism and subclinical Cushing's syndrome.

摘要

背景

原发性醛固酮增多症合并亚临床库欣综合征的患病率高于以往认为的水平。通过分析一例罕见临床病例,我们总结了原发性醛固酮增多症合并亚临床库欣综合征的诊断与治疗。

病例介绍

一名54岁的中国汉族男性被诊断为原发性醛固酮增多症合并亚临床库欣综合征。腹部计算机断层扫描显示其左肾上腺有一个肿块,右肾上腺也有一个肿块。在完成无促肾上腺皮质激素的序贯肾上腺静脉采血后,结果提示左右结节分别是导致皮质醇增多症和醛固酮分泌过多的原因。先后进行了右侧和左侧肾上腺切除术。病理诊断均为肾上腺皮质腺瘤。组织学检查结果显示,右侧腺瘤对CYP11B2免疫染色呈阳性,左侧腺瘤对CYP11B1免疫染色呈阳性。免疫组化结果有助于我们确诊。右侧肿瘤发现体细胞KCNJ5突变(Leu168Arg);左侧肾上腺肿瘤未发现KCNJ5突变。

结论

我们建议原发性醛固酮增多症患者应进行小剂量过夜地塞米松抑制试验以筛查皮质醇增多症。这有助于避免误诊,并有助于正确理解肾上腺静脉采血结果。确定醛固酮和皮质醇分泌的病灶对于原发性醛固酮增多症合并亚临床库欣综合征患者的治疗至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c112/7031945/82ceb32719d4/13256_2020_2353_Fig1_HTML.jpg

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