Suda Toshihiro, Kageyama Kazunori, Nigawara Takeshi, Sakihara Satoru
Department of Endocrinology and Metabolism, Hirosaki University Graduate School of Medicine, Aomori, Japan.
Endocr J. 2009;56(3):469-76. doi: 10.1507/endocrj.k08e-353. Epub 2009 Feb 18.
We evaluated the usefulness and accuracy of diagnostic tests for adrenocorticotropic hormone (ACTH)- dependent Cushing's syndrome, based on our experience of 88 cases, including 73 cases with Cushing's disease, and 15 cases with ectopic ACTH syndrome (EAS). In our study, 0.5 mg of dexamethasone failed to suppress the morning cortisol secretion in 100% of cases with Cushing's disease and EAS. Plasma ACTH levels were significantly increased by desmopressin (DDAVP) in 86% of cases with Cushing's disease, especially in microadenomas (90%), while these levels were not affected in normal subjects. In EAS, 44% responded to DDAVP. Plasma ACTH levels were increased in response to the human corticotropin-releasing hormone (CRH) test in 100% of microadenomas and 73% of macroadenomas with Cushing's disease, but only in 27% of cases with EAS. A high dose (8 mg) of dexamethasone suppressed the morning cortisol secretion in 89% of microadenomas with Cushing's disease, and in 82% of all cases with Cushing's disease, while it did in only 20% of cases with EAS. Taken together, the 0.5 mg dexamethasone suppression test (DST) and DDAVP test are considerably useful for the screening of ACTH-dependent Cushing's syndrome. The CRH test and 8 mg DST would be effective for the diagnosis of Cushing's diseases, because our study shows a sensitivity of 81% in cases with Cushing's disease when these tests are considered together. These data were submitted to prepare the diagnostic criteria for Cushing's disease, suggested by the working group of the Ministry of Health, Labour, and Welfare of Japan.
基于我们对88例促肾上腺皮质激素（ACTH）依赖性库欣综合征患者的经验，包括73例库欣病患者和15例异位ACTH综合征（EAS）患者，我们评估了诊断性检查对于此类疾病的实用性和准确性。在我们的研究中，0.5毫克地塞米松未能抑制100%的库欣病和EAS患者早晨的皮质醇分泌。86%的库欣病患者，尤其是微腺瘤患者（90%），血浆ACTH水平因去氨加压素（DDAVP）而显著升高，而正常受试者的这些水平未受影响。在EAS中，44%的患者对DDAVP有反应。100%的库欣病微腺瘤和73%的大腺瘤患者，其血浆ACTH水平在人促肾上腺皮质激素释放激素（CRH）试验后升高，但EAS患者中只有27%出现这种情况。高剂量（8毫克）地塞米松抑制了89%的库欣病微腺瘤患者以及82%的所有库欣病患者早晨的皮质醇分泌，而EAS患者中只有20%出现这种情况。综上所述，0.5毫克地塞米松抑制试验（DST）和DDAVP试验对于ACTH依赖性库欣综合征的筛查相当有用。CRH试验和8毫克DST试验对于库欣病的诊断有效，因为我们的研究表明，综合考虑这些试验时，库欣病患者的敏感性为81%。这些数据已提交，用于制定日本厚生劳动省工作组建议的库欣病诊断标准。
