Miura M, Matsukado Y, Kodama T, Hiramatsu R
No Shinkei Geka. 1984 May;12(6):689-96.
Six cases of Cushing's disease, in which perplex diagnostic problem was encountered, were reported. In the endocrinological examination two cases were not suppressed by a high dose of dexamethasone (8 mg), and the other four cases showed complicated clinical findings due to adrenocortical nodular hyperplasia. Among those four cases with adrenocortical nodular hyperplasia two of them were of functionally autonomic and showed different attitude in responding endocrine assay. When the serum cortisol is not suppressed by a high dose of dexamethasone, differential diagnosis from other causes, such as Cushing's syndrome due to adrenocortical macronodular hyperplasia, becomes quite important for surgical treatment. Usually plasma ACTH in the case of Cushing's disease is slightly increased over the normal range and, on the other hand, it is lower than normal value in the case of Cushing's syndrome due to autonomic adrenocortical nodular hyperplasia because of negative feed back phenomenon. However, in some cases of Cushing's disease plasma ACTH is within normal range or even lower than normal level, and, furthermore, it is not extremely unusual to find a case of adrenocortical macronodular hyperplasia with slightly increased plasma ACTH level over normal range. Those cases offer particularly complex problem in making diagnosis and therapeutic indication. According to our clinical experiences with those six cases, selective venous sampling is found quite useful in differentiating Cushing's disease from other causes and ACTH value in selective venous sampling from inferior petrosal vein is remarkably increased in the cases due to pituitary ACTH secreting adenoma. Cushing's disease with adrenocortical nodular hyperplasia reduces the ACTH value in the sampling to the lower normal range as the adrenocortical nodular hyperplasia progresses.(ABSTRACT TRUNCATED AT 250 WORDS)
报告了6例库欣病患者,这些病例遇到了复杂的诊断问题。在内分泌检查中,2例患者未被高剂量地塞米松(8毫克)抑制,另外4例因肾上腺皮质结节性增生出现复杂的临床表现。在这4例肾上腺皮质结节性增生患者中,2例具有功能自主性,在应对内分泌检测时表现出不同的反应。当血清皮质醇未被高剂量地塞米松抑制时,与其他病因(如肾上腺皮质大结节性增生所致库欣综合征)进行鉴别诊断对于手术治疗非常重要。通常,库欣病患者的血浆促肾上腺皮质激素(ACTH)略高于正常范围,而另一方面,由于负反馈现象,自主性肾上腺皮质结节性增生所致库欣综合征患者的血浆ACTH低于正常值。然而,在某些库欣病病例中,血浆ACTH在正常范围内或甚至低于正常水平,此外,发现肾上腺皮质大结节性增生患者血浆ACTH水平略高于正常范围的情况也并不罕见。这些病例在诊断和治疗指征方面存在特别复杂的问题。根据我们对这6例病例的临床经验,发现选择性静脉采血在鉴别库欣病与其他病因方面非常有用,并且垂体ACTH分泌腺瘤所致病例的岩下窦选择性静脉采血中的ACTH值显著升高。随着肾上腺皮质结节性增生的进展,伴有肾上腺皮质结节性增生的库欣病患者在采血中的ACTH值降至较低的正常范围。(摘要截短于250字)