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中華民國泌尿科醫學會雜誌

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篇名 Acquired Hypothalamus Secretory Dysfunction with Isolated Luteinizing Hormone Deficiency
卷期 9:1
並列篇名 後天性下視丘分泌功能異常合併單純黃體激素缺乏症-病例報告
作者 謝政興黃天祥勞漢信林明中
頁次 36-40
關鍵字 下視丘黃體激素柯羅米芬hypothalamusluteinizing hormoneclomipheneTSCI
出刊日期 199803

中文摘要

單純黃體激素缺乏症經常是先天性,且具有以下特徵:(一)類宦官表徵,兩側睪丸略小(二)睪丸有精子生成能力,晊缺乏萊狄什氏細胞(三)睪丸對於人類胎盤絨毛性激素的刺激反應正常。本文報告一位34歲男性病人結婚七魁,太太曾一度懷孕,後來因故要中止懷孕,實施人工流產,之後六年未曾懷孕;身體健康且性生活正常,直到三年前,性慾開始降低,性功能異常。除施人工流產,之後六年未曾懷孕;身體健康且性生活正常,直而三年前,性慾開始降低,性功能異常。除了左側精索靜脈曲張及兩側睪玉略小外,身體理學檢查正常。睪丸活體組織切片可發現睪丸有活躍的精子生成能力,但萊狄什氏細胞數目明顯減少。荷爾蒙分析顯示血清濾泡刺激激素及泌乳激素濃度正常,黃體激素及睪固酮濃度低於正常值。頭部電腦斷層攝影檢查發現腦下垂體發育不全,但無可見的腫瘤。對於性腺釋放激素,甲狀腺釋放激素及胰島素耐性試驗反應尹常。柯羅米芬(Clomiphene)試驗反應亦正常。荷爾蒙波動分析沒有觀察到黃體激素的波動,並且黃體激素濃度持續低於正常值;濾泡刺激激素的波動分析則是正常。發生於本例的單純九體激素缺乏症是後天性的,產生病變的部位可能在下視丘,其分泌功能異常,無法正常加強性腺釋放激素的分泌。柯羅米芬(Clomiphene)是一種可以刺激下視丘的化合物,這位病人在服用柯羅米芬一個月後,臨床上反應良好,性慾及性功能恢復正常。

英文摘要

A 34-year-old male was seen for evaluation of a barren marriage of 7 years. He had been healthy with a normal sexual life until 3 years ago when the libido decreased with defective in-tercourse. He had testes of subnormal size and preserved spermatogenesis with mild decrease in the number of the Leydig cells. Hormonal assay revealed FSH 10.9 mIU/ml. LH 0.6 mIU/ml and testosterone 0.94 ng/ml. Normal response was demonstrated in GnRH test, TRH test and ITT test. Pulse study by determining basal FSH and LH every 10 minutes revealed no observable LH pulse and sustained low level of LH within 8 hours. He responded well to clomiphene admini-stration clinically and biochemically, and regained sexual drive and function after clomiphene 100 mg daily was administered for one month. The manifestation corresponded to the entity characterized by fertile eunuch syndrome, which was generally believed to be congenital and a less severe form of idiopathic hypogonadotropic hypogonadism. However, the hypothalamus se-cretory dysfunction with isolated LH deficiency in this case was characterized as acquired. (J Urol R.O.C., 9:36-40,1998)

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