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一名女婴的男性化肾上腺皮质肿瘤:体内和体外生化特征

A virilizing adrenocortical tumor in a female infant: in vivo and in vitro biochemical characteristics.

作者信息

Cahen L A, Villee D B, Powers M L, Crigler J F

出版信息

J Clin Endocrinol Metab. 1978 Aug;47(2):300-6. doi: 10.1210/jcem-47-2-300.

Abstract

Normal adrenal and adrenal tumor cells from a female infant with a virilizing adrenal tumor were grown in tissue culture as monolayers for a period of 7 weeks. Half of the cultures were exposed to ACTH (0.1 U/ml). The cells grew well and continued to produce steroid hormones over the entire period in culture. Production of steroid hormones was measured by RIA of individual steroids in the culture medium. Unstimulated normal and tumor cells produced equivalent amounts of cortisol, 11 beta, 18, 21-trihydroxy-4-pregnene-3,20-dione, and 18,21-dihydroxy-4-pregnene-3,20-dione, but tumor cells produced lesser amounts of dehydroepiandrosterone (DHA), androstenedione, testosterone, and progesterone. Normal cells exposed to ACTH showed an increase in all steroids measured, whereas ACTH-exposed tumor cells showed an increase principally in DHA consistent with a deficiency in 3 beta-hydroxysteroid dehydrogenase activity. Circulating levels of DHA, androstenedione, and testosterone were elevated in the patient before removal of the adrenal tumor. The production of androgens by tumor cells in vitro resembled the pattern of circulating steroids in vivo. These studies demonstrate that tissue culture of human adrenal cells provides a means both to determine their biochemical characteristics and to investigate their responses to exogenous hormones.

摘要

取自一名患有肾上腺雄激素增多症肿瘤的女婴的正常肾上腺细胞和肾上腺肿瘤细胞,以单层形式在组织培养中生长了7周。一半培养物暴露于促肾上腺皮质激素(0.1 U/ml)。细胞生长良好,并在整个培养期间持续产生甾体激素。通过对培养基中单个甾体进行放射免疫分析来测定甾体激素的产生。未受刺激的正常细胞和肿瘤细胞产生等量的皮质醇、11β,18,21-三羟基-4-孕烯-3,20-二酮和18,21-二羟基-4-孕烯-3,20-二酮,但肿瘤细胞产生的脱氢表雄酮(DHA)、雄烯二酮、睾酮和孕酮量较少。暴露于促肾上腺皮质激素的正常细胞所检测的所有甾体均增加,而暴露于促肾上腺皮质激素的肿瘤细胞主要是DHA增加,这与3β-羟基甾体脱氢酶活性缺乏一致。在切除肾上腺肿瘤之前,患者体内循环的DHA、雄烯二酮和睾酮水平升高。肿瘤细胞在体外产生雄激素的模式类似于体内循环甾体的模式。这些研究表明,人肾上腺细胞的组织培养为确定其生化特性和研究其对外源激素的反应提供了一种方法。

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