Wada N, Kubo M, Kijima H, Ishizuka T, Saeki T, Koike T, Sasano H
Department of Medicine II, Hokkaido University School of Medicine, Sapporo, Japan.
Eur J Endocrinol. 1996 May;134(5):583-7. doi: 10.1530/eje.0.1340583.
We treated two men with Cushing's syndrome due to adrenocorticotropin (ACTH)-independent bilateral macronodular adrenocortical hyperplasia (AIMAH). In both patients, plasma ACTH was low and plasma cortisol was not suppressed by a high dose of dexamethasone (8 mg) but was remarkably responsive to exogenous ACTH. The adrenal glands were extremely enlarged and contained multiple nodules composed of large clear cells and small compact cells. The immunoreactivity of P-450(17) alpha was predominant in the small compact cells, while that of 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) was observed exclusively in the large clear cells. Among various adrenocortical disorders, differential expression of 3 beta-HSD and P-450(17) alpha in clear and compact cells has heretofore been demonstrated only in AIMAH. Total adrenalectomy was done for one patient, and partial adrenalectomy for the other. In the former patient, the normal diurnal rhythm of plasma ACTH was restored 11 months postoperatively. In the latter patient, the normal dynamics in the hypothalamic-pituitary-adrenal axis became evident 15 months after surgery. Thus AIMAH is apparently a primary adrenocortical disorder and is not due to abnormalities of the hypothalamus or pituitary.
我们治疗了两名因促肾上腺皮质激素（ACTH）非依赖性双侧大结节性肾上腺皮质增生（AIMAH）导致库欣综合征的男性患者。两名患者的血浆促肾上腺皮质激素水平均较低，高剂量地塞米松（8毫克）未能抑制血浆皮质醇水平，但对外源性促肾上腺皮质激素有显著反应。肾上腺极度增大，包含多个由大透明细胞和小致密细胞组成的结节。P-450（17）α的免疫反应性在小致密细胞中占主导，而3β-羟类固醇脱氢酶（3β-HSD）仅在大透明细胞中观察到。在各种肾上腺皮质疾病中，迄今为止，仅在AIMAH中证实了3β-HSD和P-450（17）α在透明细胞和致密细胞中的差异表达。一名患者接受了全肾上腺切除术，另一名患者接受了部分肾上腺切除术。在前一名患者中，术后11个月血浆促肾上腺皮质激素的正常昼夜节律得以恢复。在后一名患者中，下丘脑-垂体-肾上腺轴的正常动态在术后15个月变得明显。因此，AIMAH显然是一种原发性肾上腺皮质疾病，并非由下丘脑或垂体异常引起。
