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跨越算法的另一端:一例具有挑战性的肾上腺库欣综合征病例。

Crossing the other side of the algorithm: a challenging case of adrenal Cushing's syndrome.

作者信息

Antonio Imelda Digna Soberano, Sandoval Mark Anthony Santiago, Lantion-Ang Frances Lina

机构信息

Department of Medicine, Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.

出版信息

BMJ Case Rep. 2011 Dec 1;2011:bcr0820114694. doi: 10.1136/bcr.08.2011.4694.

Abstract

The diagnosis of endogenous Cushing's syndrome and its aetiology involved documenting the hypercotisolism and then determining whether that hypercortisolism is adrenocorticotropic hormone-dependent (ACTH-dependent) or not. Hence, following the algorithm, an undetected ACTH level points to an adrenal Cushing's while a detectable or elevated ACTH level points to either a pituitary or ectopic Cushing's syndrome. The authors present a case of florid adrenal Cushing's syndrome initially presenting with a normal ACTH level, which led to the investigation for an ACTH-secreting tumour. Adding to the confusion, a MRI done showed an intrasellar focus. Knowledge of how ACTH-dependent (versus ACTH-independent) Cushing's syndrome manifests clinically, supported by results of repeat laboratory tests, led to the true diagnosis. This case illustrates that a detectable ACTH does not rule out an adrenal Cushing's syndrome nor does a positive pituitary imaging confirm Cushing's disease.

摘要

内源性库欣综合征的诊断及其病因涉及记录皮质醇增多症,然后确定该皮质醇增多症是否依赖促肾上腺皮质激素(ACTH依赖型)。因此,按照该算法,未检测到的ACTH水平提示肾上腺性库欣综合征,而可检测到或升高的ACTH水平提示垂体性或异位性库欣综合征。作者报告了一例典型的肾上腺性库欣综合征病例,该病例最初表现为ACTH水平正常,这导致了对分泌ACTH肿瘤的调查。更令人困惑的是,MRI检查显示鞍内有病灶。在重复实验室检查结果的支持下,了解ACTH依赖型(与ACTH非依赖型)库欣综合征的临床症状表现,从而得出了正确的诊断。该病例表明,可检测到的ACTH并不排除肾上腺性库欣综合征,垂体成像阳性也不能确诊库欣病。

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Endocrinol Metab Clin North Am. 2008 Mar;37(1):135-49, ix. doi: 10.1016/j.ecl.2007.10.010.
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Cushing's Syndrome: important issues in diagnosis and management.库欣综合征:诊断与管理中的重要问题
J Clin Endocrinol Metab. 2006 Oct;91(10):3746-53. doi: 10.1210/jc.2006-0997. Epub 2006 Jul 25.
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Adrenocorticotrophic hormone; stability studies.
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