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[异位促肾上腺皮质激素分泌所致库欣综合征:低钾血症的病理生理机制分析。两例报告]

[Cushing syndrome by ectopic ACTH secretion: analysis of the physiopathologic mechanism of hypokalemia. Report of two cases].

作者信息

Campusano C, Arteaga E, Fardella C, Cárdenas I, Martínez P

机构信息

Departamento de Endocrinología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Chile.

出版信息

Rev Med Chil. 1999 Mar;127(3):332-6.

Abstract

We report a 42 years old male and a 66 years old female with a Cushing syndrome caused by ectopic ACTH secretion secondary to a carcinoid tumor. These patients had both severe hypokalemia, resistant to medical treatment and that subsided with bilateral adrenalectomy and supplementation with dexametasone. Cushing syndrome caused by ectopic ACTH secretion is characterized by a severe and rapidly evolving hypercortisolism. Hypokalemia is present in 90% of cases and is probably caused by a defect in 11 beta hydroxysteroid dehydrogenase, that limits the binding of cortisol to aldosterone receptor, metabolizing it to cortisone. Therefore, this alteration will increase the mineralocorticoid action of cortisol.

摘要

我们报告了一名42岁男性和一名66岁女性,他们患有由类癌肿瘤继发的异位促肾上腺皮质激素(ACTH)分泌引起的库欣综合征。这些患者均患有严重低钾血症,药物治疗无效,双侧肾上腺切除并补充地塞米松后低钾血症缓解。由异位ACTH分泌引起的库欣综合征的特征是严重且迅速发展的皮质醇增多症。90%的病例存在低钾血症,可能是由于11β-羟类固醇脱氢酶缺陷所致,该缺陷限制了皮质醇与醛固酮受体的结合,将其代谢为可的松。因此,这种改变会增加皮质醇的盐皮质激素作用。

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