Horio S, Funahashi H, Mizuno S
Nihon Naibunpi Gakkai Zasshi. 1981 May 20;57(5):807-25. doi: 10.1507/endocrine1927.57.5_807.
For the purpose of studying the relation of diversities of basic lesion as well as clinical manifestation to hormonal abnormality in Cushing's syndrome, 12 kinds of steroid hormones was simultaneously measured in plasma by using 2 types of Sephadex LH-20 column chromatography in a total of 30 patients comprosing 28 cases of Cushing's syndrome and 2 cases of adrenocortical carcinoma which had abnormal plasma steroid hormone levels without sign or symptom of Cushing's syndrome. In the group of Cushing's syndrome were included cases of pituitary ACTH-dependent hyperplasia (Hp.), adrenocortical adenoma(Ad.), bilateral nodular hyperplasia, ectopic ACTH syndrome as well as recurrent Cushing's syndrome following subtotal adrenalectomy. Twelve steroids measured in plasma were pregnenolone(Preg)., 17-OH pregnenolone(17Preg.), progesterone(Prog.), 17-OH progesterone(17Prog.), 11-deoxycorticosterone (DOC), corticosterone(B), aldosterone(Ald.), 11-deoxycortisol(S), cortisol(F), dehydroepiandrosterone(DHA), androstendione(A-dione) and testosterone(T), including precursors (Prec.: Preg., 17-preg., Prog., 17-prog.) as well as hormones belonging to the 3 systems in the biosynthetic pathways of steroid; i.e., glucocorticoids(Glu.C.'s: S,F), mineralocorticoids(Min.C.'s: DOC, B, Ald.) and sex steroids(And.'s: DHA, A-dione, T). In addition, steroidogenesis in isolated adrenal cells obtained surgically from patients with Cushing's syndrome due to Hp. and Ad, was observed. The results were as follows: (1) In cases due to Hp., plasma levels of Glu.C's and And.'s were slightly elevated, while levels of Min.C.'s were within the normal range. On the whole, however, the 3 systems were well balanced. (2) In cases due to Ad., elevated secretion of Glu.C.'s and Min.C.'s was observed, while secretion of And.'s was depressed. Among the 3 fractions of And.'s, depression of DHA and A-dione was characteristic of Ad.. (3) Elevation of And.'s in Hp. and Min.C.'s in Ad. in addition to elevation of Glu.C.'s was in fair correlation with moderate virilism in Hp. and with hypertension and hypokalemia in Ad. respectively. (4) In vitro experiments revealed that Ad. produces not only F but all 12 steroids hormones and that increased DOC and depressed DHA secretion reflected in their plasma levels were the characteristics of Ad. in steroidogenesis. Furthermore, isolated adenoma cells were found to produce Ald. at a higher rate than normal or hyperplasia adrenal cells. This finding may suggest that an intermediate type between Cushing's syndrome and primary hyperaldosteronism can exist in cases of adenoma. (5) In ectopic ACTH syndrome, plasma levels of Glu.C.'s, Min.C.'s and And.'s were equally but more markedly elevated as compared with Hp.. The increase of B was characteristic of this disorder and, coupled with a marked increase of F, seems to be the main cause of hypokalemic alkalosis frequently associated with this syndrome. (6) Nodular hyperplasia was accompanied by elevated Min.C...
为研究库欣综合征基本病变及临床表现的多样性与激素异常的关系,采用两种葡聚糖凝胶LH - 20柱色谱法同时测定了30例患者血浆中的12种甾体激素,其中包括28例库欣综合征患者和2例肾上腺皮质癌患者,后者血浆甾体激素水平异常,但无库欣综合征的体征或症状。库欣综合征组包括垂体促肾上腺皮质激素(ACTH)依赖性增生(Hp.)、肾上腺皮质腺瘤(Ad.)、双侧结节性增生、异位ACTH综合征以及肾上腺次全切除术后复发性库欣综合征的病例。血浆中检测的12种甾体激素为孕烯醇酮(Preg.)、17 - 羟基孕烯醇酮(17Preg.)、孕酮(Prog.)、17 - 羟基孕酮(17Prog.)、11 - 脱氧皮质酮(DOC)、皮质酮(B)、醛固酮(Ald.)、11 - 脱氧皮质醇(S)、皮质醇(F)、脱氢表雄酮(DHA)、雄烯二酮(A - dione)和睾酮(T),包括前体物质(Prec.:Preg.、17 - preg.、Prog.、17 - prog.)以及甾体生物合成途径中3个系统的激素,即糖皮质激素(Glu.C.:S、F)、盐皮质激素(Min.C.:DOC、B、Ald.)和性激素(And.:DHA、A - dione、T)。此外,观察了因Hp.和Ad导致的库欣综合征患者手术获取的分离肾上腺细胞中的甾体激素生成情况。结果如下:(1)在Hp.导致的病例中,糖皮质激素和性激素的血浆水平略有升高,而盐皮质激素水平在正常范围内。然而,总体而言,这3个系统平衡良好。(2)在Ad.导致的病例中,观察到糖皮质激素和盐皮质激素的分泌增加,而性激素的分泌受到抑制。在性激素的3个组分中,DHA和A - dione的抑制是Ad.的特征。(3)Hp.中性激素的升高以及Ad中的盐皮质激素升高,除了糖皮质激素升高外,分别与Hp.中的中度男性化以及Ad.中的高血压和低钾血症有较好的相关性。(4)体外实验表明,Ad.不仅产生F,还产生所有12种甾体激素,血浆水平中DOC增加和DHA分泌受抑制反映了Ad.在甾体激素生成中的特征。此外,发现分离的腺瘤细胞产生醛固酮的速率高于正常或增生的肾上腺细胞。这一发现可能表明腺瘤病例中可能存在库欣综合征和原发性醛固酮增多症之间的中间类型。(5)在异位ACTH综合征中,与Hp.相比,糖皮质激素、盐皮质激素和性激素的血浆水平均同等但更显著地升高。B的增加是该疾病的特征,与F的显著增加一起,似乎是该综合征常伴发的低钾性碱中毒的主要原因。(6)结节性增生伴有盐皮质激素升高。