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重症肌无力合并原发性醛固酮增多症及低钾性肌病。

Myasthenia gravis complicated with primary aldosteronism and hypokalemic myopathy.

作者信息

Yamashita Shu-ichi, Tsuchimochi Wakaba, Yonekawa Tadato, Yamaguchi Hideki, Kyoraku Itaru, Shiomi Kazutaka, Nakazato Masamitsu

机构信息

Neurology, Respirology, Endocrinology and Metabolism, Dapartment of Internal Medicine, Faculty of Medicine, University of Miyazaki.

出版信息

Intern Med. 2009;48(16):1465-9. doi: 10.2169/internalmedicine.48.2311. Epub 2009 Aug 17.

Abstract

A 34-year-old Japanese man was admitted to an outside hospital with an elevated creatinine kinase level and suspected myositis. He was treated with high dose methylprednisolone, which caused severe aggravation of muscle weakness. He was transferred to our hospital at that time. On admission, chest computed tomography (CT) and the presence of anti-acetylcholine receptor antibodies supported a diagnosis of myasthenia gravis and thymoma. Laboratory findings showed hypokalemia, low plasma renin activity and high serum aldosterone. Further studies, including abdominal CT and adrenal venous sampling suggested primary aldosteronism. At first, thymectomy was performed, and one month later, he was treated with laparoscopic adrenalectomy. Immediately after this procedure, he suffered from myasthenic crisis, which was successfully managed with mechanical ventilation and steroid pulse therapy followed by oral prednisolone. This case presented a serious difficulty in differentiating from various myopathies and giving proper treatment because of a rare combination of independent diseases and their masquerading clinical features.

摘要

一名34岁的日本男性因肌酸激酶水平升高和疑似肌炎入住外院。他接受了大剂量甲泼尼龙治疗,这导致肌无力严重加重。当时他被转至我院。入院时,胸部计算机断层扫描(CT)及抗乙酰胆碱受体抗体的存在支持重症肌无力和胸腺瘤的诊断。实验室检查结果显示低钾血症、低血浆肾素活性和高血清醛固酮。包括腹部CT和肾上腺静脉采血在内的进一步检查提示原发性醛固酮增多症。起初,进行了胸腺切除术,一个月后,他接受了腹腔镜肾上腺切除术。此手术后不久,他发生了重症肌无力危象,通过机械通气和类固醇冲击疗法,随后口服泼尼松龙成功处理。由于多种独立疾病罕见的组合及其伪装的临床特征,该病例在与各种肌病鉴别及给予恰当治疗方面存在严重困难。

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