GONADAL AND ADRENAL CATHETERIZATION DURING ADRENAL SUPPRESSION AND GONADAL STIMULATION IN A PATIENT WITH BILATERAL TESTICULAR-TUMORS AND CONGENITAL ADRENAL-HYPERPLASIA
GONADAL AND ADRENAL CATHETERIZATION DURING ADRENAL SUPPRESSION AND GONADAL STIMULATION IN A PATIENT WITH BILATERAL TESTICULAR-TUMORS AND CONGENITAL ADRENAL-HYPERPLASIA
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DOI：
10.1210/jc.79.5.1390
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发表时间：
1994-11-01
影响因子：
5.8
通讯作者：
ATTALI, JR
中科院分区：
文献类型：
--
作者：
COMBESMOUKHOVSKY, ME;KOTTLER, ML;ATTALI, JR
我们报告一例因α-羟化酶缺乏而患有双侧睾丸肿瘤和先天性肾上腺增生的患者。对左睾丸和肾上腺静脉进行导管插入术。精索静脉中 11 β-羟基化类固醇的存在证实了继发于肾上腺休息细胞的睾丸肿瘤的存在。在用地塞米松抑制肾上腺并结合用 hCG 刺激性腺后，观察到外周血中雄激素和肾上腺类固醇的急剧减少。与外周相比，性腺静脉中的21-脱氧皮质醇和11β-羟基-Delta(4)-雄烯二酮水平仍然高于21-脱氧皮质酮水平，但肾上腺静脉中则不然，这似乎表明这种异位组织的性质不寻常，并且其对地塞米松的敏感性取决于肾上腺皮质区。 hCG 刺激后雌二醇或睾酮没有升高，表明所有睾丸组织均不活跃或被破坏。这一发现得到了组织学检查的证实。
We report the case of a patient with bilateral testicular tumors and congenital adrenal hyperplasia due to al-hydroxylase deficiency. Catheterization of the left testicular and adrenal veins was performed. The presence of 11 beta-hydroxylated steroids in the spermatic veins confirmed the presence of testicular tumor secondary to adrenal rest cells. After adrenal suppression by dexamethasone combined with gonadal stimulation with hCG, a dramatic decrease in androgens and adrenal steroids was observed in the peripheral blood. Compared to the periphery, 21-deoxycortisol and 11 beta-hydroxy-Delta(4)-androstenedione levels remained higher than that of 21-deoxycorticosterone in the gonadal vein, but not in the adrenal vein, which seems to indicate that the nature of this ectopic tissue is unusual and that its sensitivity to dexamethasone depends on the adrenocortical zones. No rise in estradiol or testosterone was obtained after hCG stimulation, suggesting that all of the testicular tissue was inactive or destroyed. This finding was confirmed by histological examination.