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[肾上腺疾病中的性功能和性腺功能障碍]

[Sexual and gonadal dysfunction in adrenal disorders].

作者信息

Horiba N

机构信息

Department of Medicine, Hirosaki University School of Medicine.

出版信息

Nihon Rinsho. 1997 Nov;55(11):2979-84.

PMID:9396299
Abstract

Among various diseases of the adrenals, major disorders that cause sexual and gonadal disturbances are congenital adrenal hyperplasia(CAH) and Cushing's syndrome, and the others include virilizing or feminizing adrenocortical tumors. CAH was reviewed based on the recent advances in molecular genetics. The most striking discovery was steroidogenic acute regulatory protein, mutations of which produce lipoid adrenal hyperplasia that was previously attributed to P-450scc deficiency. Reversible amenorrhea or impotence is found in patients with Cushing's syndrome. Low plasma estrogen and testosterone levels are associated with female and male patients, respectively. Elevated adrenal androgen accounts for mild virilization in female patients with ACTH-dependent subtypes. The sites of action at which hypercortisolemia suppresses the hypothalamic-pituitary-gonadal axis were discussed.

摘要

在各种肾上腺疾病中,引起性和性腺功能紊乱的主要病症是先天性肾上腺增生(CAH)和库欣综合征,其他病症包括男性化或女性化肾上腺皮质肿瘤。基于分子遗传学的最新进展对先天性肾上腺增生进行了综述。最显著的发现是类固醇生成急性调节蛋白,其突变会导致类脂性肾上腺增生,此前该病被归因于P - 450scc缺乏。库欣综合征患者会出现可逆性闭经或阳痿。女性和男性患者的血浆雌激素和睾酮水平分别降低。肾上腺雄激素升高导致促肾上腺皮质激素依赖型亚型女性患者出现轻度男性化。讨论了高皮质醇血症抑制下丘脑 - 垂体 - 性腺轴的作用位点。

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