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17α-羟化酶缺乏症患者中无显著19-去甲-脱氧皮质酮生成的证据。

Evidence against significant 19-nor-deoxycorticosterone production in patients with 17 alpha-hydroxylase deficiency.

作者信息

Shackleton C H

出版信息

Clin Exp Hypertens A. 1982;4(9-10):1529-39. doi: 10.3109/10641968209061623.

DOI:10.3109/10641968209061623
PMID:6982794
Abstract

Tetrahydro-19-nor-deoxycorticosterone (3 alpha, 21-dihydroxy-19-nor-5 beta-pregnan-20-one, a presumed metabolite of 19-nor-DOC was sought in the conjugated steroid fractions of urine from patients with 17 alpha-hydroxylase deficiency syndrome. The reference material was prepared by microbial reduction (Clostridium paraputrificum) of 19-nor-DOC. Urinary steroid fractions of appropriate polarity were examined by high resolution gas chromatography and gas chromatography/mass spectrometry (GC/MS) but no tetrahydro-19-nor-DOC was found. The high selectivity of the GC/MS technique ensured that the excretion of this compound could not exceed about 1% of the excretion of tetrahydroDOC. Assuming that tetrahydro-19-nor-DOC is a major metabolite of 19-nor-DOC, it can be stated that 19-nor-DOC production is not a significant feature of 17 alpha-hydroxylase deficiency. This assumption may not be valid if 19-nor-DOC is formed from DOC in the kidney and is excreted unmetabolized soon after synthesis.

摘要

在17α-羟化酶缺乏综合征患者尿液的结合类固醇组分中寻找四氢-19-去甲-脱氧皮质酮(3α,21-二羟基-19-去甲-5β-孕烷-20-酮,一种假定的19-去甲-DOC代谢产物)。参考物质通过19-去甲-DOC的微生物还原(副腐败梭菌)制备。通过高分辨率气相色谱和气相色谱/质谱联用(GC/MS)对具有适当极性的尿类固醇组分进行检测,但未发现四氢-19-去甲-DOC。GC/MS技术的高选择性确保该化合物的排泄量不超过四氢DOC排泄量的约1%。假设四氢-19-去甲-DOC是19-去甲-DOC的主要代谢产物,则可以说19-去甲-DOC的产生不是17α-羟化酶缺乏的显著特征。如果19-去甲-DOC在肾脏中由DOC形成并在合成后不久未经代谢就被排泄,那么这个假设可能不成立。

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Evidence against significant 19-nor-deoxycorticosterone production in patients with 17 alpha-hydroxylase deficiency.17α-羟化酶缺乏症患者中无显著19-去甲-脱氧皮质酮生成的证据。
Clin Exp Hypertens A. 1982;4(9-10):1529-39. doi: 10.3109/10641968209061623.
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引用本文的文献

1
Origin of urinary nonconjugated 19-nor-deoxycorticosterone and metabolism of infused radiolabeled 19-nor-deoxycorticosterone in men and women.男性和女性尿液中非结合型19-去甲-脱氧皮质酮的来源及注入放射性标记的19-去甲-脱氧皮质酮的代谢情况。
J Clin Invest. 1985 Apr;75(4):1335-8. doi: 10.1172/JCI111834.