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一例罕见的与醛固酮分泌性腺瘤相关的非促肾上腺皮质激素依赖性大结节性肾上腺增生。

A rare case of ACTH-independent macronodular adrenal hyperplasia associated with aldosterone-producing adenoma.

作者信息

Hayakawa Eri, Yoshimoto Takanobu, Hiraishi Kiichiro, Kato Masako, Izumiyama Hajime, Sasano Hironobu, Hirata Yukio

机构信息

Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Tokyo, Japan.

出版信息

Intern Med. 2011;50(3):227-32. doi: 10.2169/internalmedicine.50.4351. Epub 2011 Feb 1.

DOI:10.2169/internalmedicine.50.4351
PMID:21297325
Abstract

A 52-year-old man was evaluated for incidentally discovered bilateral adrenal masses. He had drug-resistant hypertension but lacked Cushingoid features. Endocrinological tests revealed autonomous secretion of cortisol and aldosterone with suppressed plasma ACTH and renin activity. A selective adrenal venous sampling demonstrated aldosterone hypersecretion from the left adrenal vein. The clinical diagnosis of subclinical Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia (AIMAH) associated with primary aldosteronism was made, and he underwent left adrenalectomy; the resected adrenal lesion was consistent with the pathological diagnosis of AIMAH coexistent with aldosterone-producing adenoma (APA). This is a very rare case of AIMAH with concomitant unilateral APA, whose hypertension improved after surgery.

摘要

一名52岁男性因偶然发现双侧肾上腺肿块而接受评估。他患有耐药性高血压,但缺乏库欣样特征。内分泌检查显示皮质醇和醛固酮自主分泌,同时血浆促肾上腺皮质激素(ACTH)和肾素活性受到抑制。选择性肾上腺静脉采血显示左肾上腺静脉醛固酮分泌过多。临床诊断为非促肾上腺皮质激素依赖性大结节性肾上腺增生(AIMAH)伴原发性醛固酮增多症所致的亚临床库欣综合征,他接受了左肾上腺切除术;切除的肾上腺病变与AIMAH合并醛固酮瘤(APA)的病理诊断一致。这是一例非常罕见的AIMAH合并单侧APA病例,术后其高血压病情有所改善。

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A case of Adrenocoricotrophic hormone -independent bilateral adrenocortical macronodular hyperplasia concomitant with primary aldosteronism.一例促肾上腺皮质激素非依赖性双侧肾上腺皮质大结节性增生伴原发性醛固酮增多症。
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