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原发性醛固酮增多症与库欣综合征合并存在:肾上腺腺瘤的一种罕见表现。

Combined Conn's and Cushing's syndrome: an unusual presentation of adrenal adenoma.

作者信息

Baert D, Nobels F, Van Crombrugge P

机构信息

Department of Endocrinology, Onze Lieve Vrouw Hospital, Aalst, Belgium.

出版信息

Acta Clin Belg. 1995;50(5):310-3. doi: 10.1080/17843286.1995.11718468.

DOI:10.1080/17843286.1995.11718468
PMID:8533534
Abstract

In most aldosterone-producing adenomas (APA) dedifferentiation occurs with formation of transitional cells, bearing characteristics of both glomerulosa and fasciculata cells. These cells are able to produce cortisol, and their aldosterone production follows the circadian rhythm of ACTH. Usually, no clinical signs of cortisol excess develop, since the cortisol production remains under ACTH feedback control. Only a few cases have been described with autonomous cortisol secretion, not suppressible by low dose dexamethasone. We present a patient with an APA, synthesizing enough cortisol to cause the typical clinical expression of Cushing's syndrome. Possible etiopathological mechanisms are discussed.

摘要

在大多数醛固酮分泌性腺瘤(APA)中,会出现去分化现象,并形成具有球状带细胞和束状带细胞特征的过渡细胞。这些细胞能够产生皮质醇,且其醛固酮分泌遵循促肾上腺皮质激素(ACTH)的昼夜节律。通常,不会出现皮质醇过量的临床症状,因为皮质醇的分泌仍受ACTH反馈控制。仅有少数病例被描述为存在自主性皮质醇分泌,且不受低剂量地塞米松抑制。我们报告一名患有APA的患者,其合成的皮质醇足以导致库欣综合征的典型临床表现。文中还讨论了可能的病因病理机制。

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引用本文的文献

1
Primary aldosteronism and hypercortisolism due to bilateral functioning adrenocortical adenomas.双侧功能性肾上腺皮质腺瘤所致原发性醛固酮增多症和皮质醇增多症
Clin Exp Nephrol. 2008 Oct;12(5):382-387. doi: 10.1007/s10157-008-0064-3. Epub 2008 Jun 10.
2
An unusual case of bilateral virilizing adrenal adenoma co-secreting androgens and cortisol.一例罕见的双侧分泌雄激素和皮质醇的男性化肾上腺腺瘤病例。
J Endocrinol Invest. 1996 Jun;19(6):377-81. doi: 10.1007/BF03344973.