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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

Yasuda Atsushi, Seki Toshiro, Ito Kazuko, Takagi Atsushi, Watanabe Daisuke, Nakamura Naoya, Hanai Kazuya, Terachi Toshiro, Maekawa Takashi, Sasano Hironobu, Fukagawa Masafumi

机构信息

Division of Nephrology, Endocrinology and Metabolism, Department of Internal Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa 259-1193, Japan.

出版信息

Tokai J Exp Clin Med. 2014 Dec 20;39(4):158-65.

Abstract

We report a rare case of Cushing's syndrome caused by bilateral cortisol-secreting adenomas in a 63-year-old man. Our preoperative diagnosis was based on endocrinological results and imaging findings. Laparoscopic adrenalectomy has become a standard technique for adrenal tumors; however, bilateral adrenalectomy results in postoperative adrenal insufficiency, necessitating lifelong steroid replacement. To preserve adrenal function, the left adrenal gland was completely resected, whereas the right adrenal gland was partially resected laparoscopically. Hydrocortisone supplementation was initiated at a dose of 30 mg/day and was slowly tapered. However, symptoms of adrenal insufficiency developed, and adrenal steroid secretion did not respond to exogenous adrenocorticotropic hormone. Bilateral cortisol-secreting tumors rarely cause Cushing's syndrome. The present study comprised few patients, and the utilized surgical procedures (i.e., total/partial adrenalectomy or bilateral total adrenalectomy) were not uniform. Few cases of bilateral adrenal-preserving surgery have been reported. However, our patient developed adrenal insufficiency after the oral cortisone supplementation was tapered. This report demonstrates that partial adrenalectomy does not necessarily preserve normal adrenocortical function. Therefore, careful postoperative observation is necessary for patients undergoing a partial adrenalectomy.

摘要

我们报告了一例63岁男性因双侧分泌皮质醇腺瘤导致库欣综合征的罕见病例。我们的术前诊断基于内分泌检查结果和影像学表现。腹腔镜肾上腺切除术已成为治疗肾上腺肿瘤的标准技术;然而,双侧肾上腺切除术会导致术后肾上腺功能不全,需要终身补充类固醇。为保留肾上腺功能,左侧肾上腺被完全切除,而右侧肾上腺通过腹腔镜进行了部分切除。氢化可的松补充剂开始剂量为每日30毫克,并逐渐减量。然而,肾上腺功能不全的症状仍出现,且肾上腺类固醇分泌对外源性促肾上腺皮质激素无反应。双侧分泌皮质醇的肿瘤很少引起库欣综合征。本研究病例数较少,且所采用的手术方式(即全/部分肾上腺切除术或双侧全肾上腺切除术)并不统一。双侧保留肾上腺手术的病例报道较少。然而,我们的患者在口服可的松补充剂减量后出现了肾上腺功能不全。本报告表明,部分肾上腺切除术不一定能保留正常肾上腺皮质功能。因此,对于接受部分肾上腺切除术的患者,术后需要仔细观察。

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