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[临床前库欣综合征:一例报告及文献复习]

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

作者信息

Miyamori I, Iki K, Takeda R

机构信息

Second Department of Internal Medicine, School of Medicine, Kanazawa University.

出版信息

Nihon Naibunpi Gakkai Zasshi. 1994 Jan 20;70(1):25-30. doi: 10.1507/endocrine1927.70.1_25.

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.

摘要

在838例连续接受腹部CT扫描的患者中,发现肾上腺偶发瘤41例(4.7%)。我们对这些患者进行了高剂量(8毫克/天)和低剂量(2毫克/天)地塞米松(DXM)抑制试验以及血浆皮质醇昼夜节律检测。在1例无库欣样外观的高血压患者中观察到自主性皮质醇分泌,提示亚临床库欣综合征。我们回顾了世界及日本文献中报道的65例亚临床或非库欣样库欣综合征的内分泌检查结果。77%的患者尿皮质醇排泄正常,70%的患者血浆皮质醇节律消失,95%的患者高剂量DXM未能抑制血浆皮质醇。血浆促肾上腺皮质激素(ACTH)和皮质醇对促肾上腺皮质激素释放因子(CRF)给药的反应低于正常或呈低反应,与显性库欣综合征相似。因此,仅靠CRF试验被认为不足以区分这两种疾病。在无症状患者中,约50%的病例存在高血压和糖耐量异常。在日本,55%的病例术后需要类固醇替代治疗,在全球范围内这一比例为75%。亚临床库欣综合征女性患者多于男性。女性亚临床库欣综合征的发病高峰在40多岁,高于显性库欣综合征,提示亚临床库欣综合征并非临床症状性库欣综合征的易感状态。我们认为,对于偶然发现的肾上腺肿瘤,有必要进行内分泌检查以排除亚临床库欣综合征的存在。

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