Testicular endocrine function in a pubertal boy with 3beta-hydroxysteroid dehydrogenase deficiency.
Testicular endocrine function in a pubertal boy with 3beta-hydroxysteroid dehydrogenase deficiency.
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3β-羟基类固醇脱氢酶缺乏症青春期男孩的睾丸内分泌功能。
DOI：
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发表时间：
1974
影响因子：
5.8
通讯作者：
R. Vihko
中科院分区：
文献类型：
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作者：
Olli A. Jänne;Jaakko Perheentupa;L. Viinikka;R. Vihko
本文对1例青春期3β-羟类固醇脱氢酶缺乏症男性患者的外周血和精索静脉血进行了血浆孕酮、孕酮、17-羟孕酮、睾酮和5α-双氢睾酮的测定，以评价其睾丸内分泌功能。外周睾酮浓度为0.28 ng/ml，并仅上升至0.60 ng/ml，以响应人绒毛膜促性腺激素（HCG）管理。然而，精索静脉睾酮浓度约为外周血睾酮浓度的15倍，这是该患者睾丸睾酮分泌的明确指示。此外，睾丸分泌17-羟孕酮和5α-二氢睾酮也很明显，因此睾丸具有一定的3β-羟类固醇脱氢酶活性。除了对HCG给药的弱反应外，与正常男性相比，精子静脉中的双烯醇酮浓度非常高，血浆促黄体生成素（LH）浓度略有升高。
ABSTRACT Plasma pregnenolone, progesterone, 17-hydroxyprogesterone, testosterone and 5α-dihydrotestosterone were determined in the peripheral and spermatic venous blood of a pubertal boy with 3β-hydroxysteroid dehydrogenase deficiency in order to evaluate his testicular endocrine function. The peripheral testosterone concentration was 0.28 ng/ml, and rose only to 0.60 ng/ml in response to human chorionic gonadotropin (HCG) administration. The spermatic venous testosterone concentration, however, was about 15 times greater than in peripheral blood, which is a clear indication of testicular testosterone secretion in this patient. In addition, testicular secretion of 17-hydroxyprogesterone and 5α-dihydrotestosterone was also evident, thus, the testis has some 3β-hydroxysteroid dehydrogenase activity. In addition to the weak response to HCG administration, a very high spermatic venous pregnenolone concentration as compared to normal males and a slightly elevated plasma luteinizing hormone (LH) concentration s...