[Pre-clinical Cushing's syndrome: report of a case and the review of the literature].

I Miyamori, K Iki, R Takeda
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引用次数: 4

Abstract

Among 838 consecutive patients who underwent abdominal CT scan, adrenal incidentalloma was detected in 41 cases (4.7%). We performed high (8 mg/day) and low dose (2 mg/day) dexamethasone (DXM) suppression test and diurnal rhythm of plasma cortisol in these patients. Autonomic cortisol production was observed in one hypertensive patient in whom Cushigoid appearance was absent, suggesting pre-clinical Cushing's syndrome. We reviewed the endocrine results of the 65 reported cases of pre-clinical or non-Cushigoid Cushing's syndrome in world and Japanese literature. Urinary cortisol excretion was normal in 77%, plasma cortisol rhythm was absent in 70%, and high dose DXM failed to suppress plasma cortisol in 95% of cases. Plasma ACTH and cortisol showed subnormal to low responses to CRF administration similar to overt Cushing's syndrome. Therefore, CRF-test alone was considered insufficient to differentiate the two disorders. Hypertension and glucose intolerance were present in approximately 50% of cases in otherwise asymptomatic patients. Post-surgical steroid replacement was required in 55% of cases in Japan and in 75% of the cases world-wide. Women were affected more with pre-Cushing's syndrome than men. Peak incidence of the pre-Cushing's syndrome in women was in the forties which was older than that of overt Cushing's syndrome, suggesting that pre-Cushing's syndrome is not the predisposing condition to clinically symptomatic Cushing's syndrome. We consider that endocrine test is necessary in incidentally discovered adrenal tumors to exclude the presence of pre-clinical Cushing's syndrome.

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【临床前库欣综合征1例报告及文献复习】。
在838例连续接受腹部CT扫描的患者中,有41例(4.7%)发现肾上腺偶发瘤。我们对这些患者进行了高剂量(8mg /天)和低剂量(2mg /天)地塞米松(DXM)抑制试验和血浆皮质醇的日节律。在一名高血压患者中观察到自主皮质醇的产生,该患者没有库欣样症状,提示临床前库欣综合征。我们回顾了世界和日本文献中报道的65例临床前或非库什样库欣综合征的内分泌结果。77%的尿皮质醇排泄正常,70%的血浆皮质醇节律缺失,95%的患者大剂量地的松未能抑制血浆皮质醇。血浆ACTH和皮质醇对CRF治疗表现出亚正常到低的反应,类似于明显的库欣综合征。因此,单独的crf测试被认为不足以区分两种疾病。在其他无症状的患者中,约50%的病例存在高血压和葡萄糖耐受不良。日本手术后需要类固醇替代的病例占55%,全世界占75%。女性比男性更容易患库欣综合征。女性库欣综合征前期发病高峰在40多岁,高于显性库欣综合征的发病高峰,提示库欣综合征前期不是临床症状库欣综合征的易感条件。我们认为对偶然发现的肾上腺肿瘤进行内分泌检查是必要的,以排除临床前库欣综合征的存在。
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