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3α,17α - 二羟基 - 5β - 孕烷 - 20 - 酮在先天性肾上腺皮质增生症诊断中的意义

Significance of 3alpha, 17 alpha-dihydroxy-5beta-pregnane-20-one in the diagnosis of congenital adrenal hyperplasia.

作者信息

Kecskés L, Tényi I, Németh M, Juricskay Z, Temesi M

出版信息

Endokrinologie. 1975 Sep;66(1):94-9.

PMID:1225520
Abstract

The 3alpha-17alpha-dihydroxy-5beta-pregnane-20-one (17-hydroxy-pregnanolone) excretion was found by gas chromatography to be as high as the pregnanetriol in urine of 4 virilising CAH female and 2 male patients. According to our results the significance of the determination of 17-hydroxy-pregnanolone is equal to that of the pregnanetriol in the diagnosis of adrenal hyperplasia. As the 17-hydroxy-pregnanolone is demonstrable after mild acid hydrolysis, too, it can be applied independently from the pregnanetriol determination. In five out of the six patients we found the excretion of the pregnanediol to be high, too. Its value reached, irrespective of sex, the characteristic quantity of the woman's luteal phase, so it undoubtedly originated from the adrenal cortex. We dealt shortly with the explanation of high pregnanediol excretion.

摘要

通过气相色谱法发现,在4例男性化先天性肾上腺皮质增生症(CAH)女性患者和2例男性患者的尿液中,3α-17α-二羟基-5β-孕烷-20-酮(17-羟孕烷醇酮)的排泄量与孕三醇一样高。根据我们的结果,在肾上腺增生的诊断中,17-羟孕烷醇酮测定的意义与孕三醇相当。由于17-羟孕烷醇酮在轻度酸水解后也可检测到,因此它可以独立于孕三醇测定进行应用。在6例患者中的5例中,我们还发现孕二醇的排泄量也很高。无论性别如何,其值都达到了女性黄体期的特征量,因此它无疑源自肾上腺皮质。我们简要讨论了孕二醇排泄量高的原因。

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