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巨大肾上腺皮质多结节性增生所致库欣综合征

Cushing's syndrome due to huge adrenocortical multinodular hyperplasia.

作者信息

Hidai H, Fujii H, Otsuka K, Abe K, Shimizu N

出版信息

Endocrinol Jpn. 1975 Dec;22(6):555-60. doi: 10.1507/endocrj1954.22.555.

Abstract

A case of Cushing's syndrome due to huge adrenocortial multinodular hyperplasia who was shown to be hyperresponsive to ACTH administration, unresponsive to metyrapone administration and resistant to dexamethasone high dose suppression was reported. After two years' duration of his symptoms, the multinodular adrenals weighing 161 g in total were removed by bilateral adrenalectomy which abolished his symptoms. Postoperatively, plasma ACTH rose gradually to above normal levels, suggesting the presence of primary disorder in the hypothalamus-pituitary axis.

摘要

报告了一例因巨大肾上腺皮质多结节增生导致的库欣综合征患者,该患者对促肾上腺皮质激素(ACTH)给药反应过度,对甲吡酮给药无反应,对高剂量地塞米松抑制有抵抗性。在出现症状两年后,通过双侧肾上腺切除术切除了总重161克的多结节肾上腺,症状得以消除。术后,血浆ACTH逐渐升至正常水平以上,提示下丘脑 - 垂体轴存在原发性疾病。

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