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肾上腺肿瘤、促肾上腺皮质激素分泌过多或特发性多毛症患者的血浆孕烯醇酮和17-羟基孕烯醇酮。

Plasma pregnenolone and 17-OH-pregnenolone in patients with adrenal tumors, ACTH excess, or idiopathic hirsutism.

作者信息

McKenna T J, Miller R B, Liddle G W

出版信息

J Clin Endocrinol Metab. 1977 Feb;44(2):231-6. doi: 10.1210/jcem-44-2-231.

Abstract

Plasma levels of the delta5-pregnenes, pregenolone and 17-OH-pregnenolone, were measured in patients with disordered steroidogenesis. While 17-OH-pregnenolone was within the normal range in patients with hypercortisolemia due to Cushing's disease, ectopic ACTH or adrenal adenrenal adenoma, 4 of 6 patients with an adrenal carcinoma had elevated levels of this precursor. Thus, elevated plasma 17-OH-pregnenolone levels in patients with Cushing's syndrome indicate adrenal carcinoma, although a normal value does not exclude this diagnosis. Abnormal resistance of delta5-pregnenes to suppression with dexamethasone proved useful in detecting the presence of residual tumor in the post-operative evaluation of adrenal carcinoma. Basal plasma pregnenolone was within the normal range in 19 of 20 patients with Cushing's disease and was invariably normal in patients with other varieties of hypercortisolism. Since acute administration of ACTH causes marked elevation of delta5-pregnene levels while patients with chronic ACTH excess (Cushing's disease and ectopic ACTH production) have normal levels, it is suggested that ACTH has a chronic influence on the intraadrenal utilization of delta5-pregnenes in addition to stimulating their formation. In pre-menopausal women with idiopathic hirsutism, basal levels of both delta5-pregnenes were elevated (P less than 0.001). Following dexamethasone administration the absolute decrease in delta5-pregnenes levels was greater than that seen in normal subjects. This observation indicates that the metabolism of delta5-pregnenes is abnormal in patients with idiopathic hirsutism.

摘要

对类固醇生成紊乱患者测定了δ5-孕烯类、孕烯醇酮和17-羟基孕烯醇酮的血浆水平。虽然在因库欣病、异位促肾上腺皮质激素(ACTH)或肾上腺腺瘤导致皮质醇增多症的患者中,17-羟基孕烯醇酮水平在正常范围内,但6例肾上腺皮质癌患者中有4例该前体水平升高。因此,库欣综合征患者血浆17-羟基孕烯醇酮水平升高提示肾上腺皮质癌,尽管正常水平不能排除该诊断。δ5-孕烯类对地塞米松抑制的异常抵抗在肾上腺皮质癌术后评估中检测残留肿瘤的存在方面被证明是有用的。20例库欣病患者中有19例基础血浆孕烯醇酮水平在正常范围内,其他类型皮质醇增多症患者的该水平始终正常。由于急性给予ACTH会导致δ5-孕烯水平显著升高,而慢性ACTH过量(库欣病和异位ACTH分泌)患者的水平正常,提示ACTH除了刺激δ5-孕烯的形成外,还对肾上腺内δ5-孕烯的利用有慢性影响。在特发性多毛症的绝经前女性中,两种δ5-孕烯的基础水平均升高(P<0.001)。给予地塞米松后,δ5-孕烯水平的绝对下降幅度大于正常受试者。该观察结果表明特发性多毛症患者中δ5-孕烯的代谢异常。

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