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肾上腺髓质肿瘤伴异位促肾上腺皮质激素综合征所致男性化(作者译)

[Virilism due to an adrenal medullary tumor with ectopic ACTH syndrome (author's transl)].

作者信息

Kley H K, Blessing M H, Nieschlag E, Wiegelmann W, Solbach H G, Krüskemper H L

出版信息

Klin Wochenschr. 1975 Apr 1;53(7):321-7. doi: 10.1007/BF01469059.

Abstract

The case of a 14-year-old girl with hirsutism and virilism due to the secretion of ectopic ACTH by an adrenal medullary tumor is described. At the age of 5 years changes in appearance had begun with masculinization. The effect of ACTH-like material, measured by radioimmunoassay in plasma and in tumor tissue, was compensated partially by the hypothalamo-pituitary-adrenal feedback mechanism. Increased concentrations of dehydroepiandrosterone, estrone and testosterone in plasma and of 17-ketosteroids and free cortisol in urine originated in the adrenals. After operation of the tumor menarche began spontaneously, hirsutism disappeared and testosterone plasma concentrations returned to normal. An adrenogenital syndrome was excluded.

摘要

本文描述了一名14岁女孩的病例,其因肾上腺髓质肿瘤分泌异位促肾上腺皮质激素(ACTH)而出现多毛症和男性化。5岁时外貌开始出现男性化变化。通过放射免疫测定法在血浆和肿瘤组织中测得的ACTH样物质的作用,部分被下丘脑-垂体-肾上腺反馈机制所代偿。血浆中脱氢表雄酮、雌酮和睾酮以及尿中17-酮类固醇和游离皮质醇浓度的升高源自肾上腺。肿瘤切除术后,月经初潮自然出现,多毛症消失,血浆睾酮浓度恢复正常。排除了肾上腺生殖器综合征。

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