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[库欣综合征的诊断。20例病因各异的库欣综合征患者的诊断评估结果(1979 - 1989年)]

[The diagnosis of Cushing's syndrome. Results of diagnostic assessment of 20 patients with Cushing's syndrome of variable etiology (1979-1989)].

作者信息

Caduff F, Staub J J, Nordmann A, Radü E W, Landolt H

机构信息

Abteilung für Endokrinologie und Stoffwechsel, Departement Innere Medizin, Kantonsspital Basel.

出版信息

Schweiz Med Wochenschr. 1991 Jan 9;121(1-2):10-20.

PMID:1990418
Abstract

In the light of 20 cases of Cushing's syndrome the currently used diagnostic strategies are presented. For initial screening the classical dexamethasone suppression test using 1 mg dexamethasone p.o. and 24-hour urinary extraction of free cortisol were used, and diagnosis was confirmed using the 2 mg dexamethasone suppression test for two days. All three tests had a sensitivity of 100% in these 20 patients. Etiological evaluation was performed using basal ACTH plasma concentration measurement and the more recently available stimulation tests using corticotropin-releasing factor (CRF): in cases with suspected pituitary-hypothalamic etiology, selective catheterisation of the sinus petrous inferior with simultaneous blood sampling for measurement of plasma ACTH concentration was performed. Detailed etiological diagnosis is important in Cushing's syndrome since therapeutic measures differ widely: the etiologies in the 20 patients with Cushing's syndrome were: central Cushing's disease (microadenoma in 11, pituitary carcinoma in 1), adrenal carcinoma (3), adrenal adenoma (1), adrenal hyperplasia (1), ectopic ACTH production (2) and alcohol-induced Cushing's syndrome (1). The stepwise procedure in screening and diagnosis of the various etiologies of Cushing's syndrome are presented and the results of the 20 patients compared with the literature.

摘要

根据20例库欣综合征患者的情况,介绍了目前使用的诊断策略。初始筛查采用口服1mg地塞米松的经典地塞米松抑制试验及24小时尿游离皮质醇提取法,确诊则采用2mg地塞米松抑制试验,连续进行两天。这三项检查在这20例患者中的敏感性均为100%。病因评估采用基础血浆促肾上腺皮质激素(ACTH)浓度测定以及最近可用的使用促肾上腺皮质激素释放因子(CRF)的刺激试验:对于怀疑有垂体-下丘脑病因的病例,进行岩下窦选择性插管并同时采集血样以测定血浆ACTH浓度。详细的病因诊断在库欣综合征中很重要,因为治疗措施差异很大:20例库欣综合征患者的病因如下:中枢性库欣病(11例为微腺瘤,1例为垂体癌)、肾上腺皮质癌(3例)、肾上腺腺瘤(1例)、肾上腺增生(1例)、异位ACTH分泌(2例)以及酒精性库欣综合征(1例)。文中介绍了库欣综合征各种病因的筛查和诊断的逐步流程,并将这20例患者的结果与文献进行了比较。

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