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先天性肾上腺皮质增生症患者促性腺激素的分泌及对促黄体生成素释放激素给药的反应

Gonadotropin output and response to LRH administration in congenital virilizing adrenal hyperplasia.

作者信息

Wentz A C, Garcia S C, Klingensmith G J, Migeon C J, Jones G S

出版信息

J Clin Endocrinol Metab. 1976 Feb;42(2):239-46. doi: 10.1210/jcem-42-2-239.

Abstract

Three premenarchial, 2 primary amenorrheic, and 5 post-menarchial patients with congenital virilizing adrenal hyperplasia (CAH) were examined, utilizing frequent blood sampling techniques and the administration of synthetic luteinizing hormone-releasing hormone (LHRH) to determine whether a normal pattern of gonadotropin output occurs in CAH. Pulsatile gonadotropin output was not observed in premenarchial patients, but was seen in all who had had spontaneous menses. Bone age did not correlate with baseline or episodic gonadotropin output, or with response to LRH, but did reflect the past or present therapeutic control. The normal developmental progression of gonadotropin output was only documented in those patients who had been maintained at doses of suppressive glucocorticoids appropriate for body surface.

摘要

对3例青春期前、2例原发性闭经和5例青春期后先天性肾上腺皮质增生症(CAH)患者进行了检查,采用频繁采血技术并给予合成促黄体生成素释放激素(LHRH),以确定CAH患者是否存在正常的促性腺激素分泌模式。青春期前患者未观察到促性腺激素脉冲式分泌,但在所有有过自发月经的患者中均有发现。骨龄与基线或间歇性促性腺激素分泌量以及对促性腺激素释放激素(LRH)的反应均无相关性,但确实反映了过去或目前的治疗控制情况。仅在那些使用适合体表面积的抑制性糖皮质激素剂量维持治疗的患者中记录到了促性腺激素分泌的正常发育进程。

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