Cushing症候群各型における血中ステロイドホルモンの検討
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概要
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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 were simultaneously measured in plasma by using 2 types of Sephadex LH-20 column chromatography in a total of 30 patients comprising 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. <BR>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), dehydro-epiandrosterone (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). <BR>In addition, steroidogenesis in isolated adrenal cells obtained surgically from patients with Cushing's syndrome due to Hp. and Ad. was observed. <BR>The results were as follows : <BR>(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. <BR>(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.. <BR>(3) Elevation of And.'s in Hp. and of 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. <BR>(4) In vitro experiments revealed that Ad. produces not only F but all 12 steroid 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 hyperplastic adrenal cells. This finding may suggest that an intermediate type between Cushing's syndrome and primary hyperaldosteronism can exist in cases of adenoma. <BR>(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. <BR>(6) Nodular hyperplasia was accompanied by elevated Min.C.'s secretion and appeared to be similar to adenoma in secretory imbalance of steroid hormones. <BR>(7) The balance of steroid hormone levels in plasma was found to be upset in cases of recurrent Cushing's syndrome in that DOC, S and A-dione levels were elevated while DHA level was depressed. This finding may suggest that the basic lesion causing Cushing's syndrome in this particular case was adenoma-like neoplasm associated with depressed enzymatic activity of 11β-hydroxylase. <BR>(8) In one case of adrenocortical carcinoma present with hypertension and virilism were observed extraordinarily high levels of DOC and S which might suggest defective 11β-hydroxylase activity. In the other case of adrenal carcinoma which was hormonally inactive, an increased production of intermediate metabolites in the early stage of steroidogenesis was all that was observed.
- 日本内分泌学会の論文
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