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氟康唑和乙酰唑胺治疗伴有严重代谢性碱中毒的异位库欣综合征

Fluconazole and acetazolamide in the treatment of ectopic Cushing's syndrome with severe metabolic alkalosis.

作者信息

Schwetz Verena, Aberer Felix, Stiegler Claudia, R Pieber Thomas, Obermayer-Pietsch Barbara, Pilz Stefan

机构信息

Clinical Division of Endocrinology and Metabolism, Department of Internal Medicine , Medical University of Graz , Graz , Austria.

出版信息

Endocrinol Diabetes Metab Case Rep. 2015;2015:150027. doi: 10.1530/EDM-15-0027. Epub 2015 May 21.

Abstract

UNLABELLED

Cushing's syndrome (CS) due to ectopic ACTH production accounts for about 10% of all types of CS and is frequently associated with metabolic alkalosis. Treatment of CS involves surgical resection and/or medical therapy to control hypercortisolism. We present the case of an 80-year-old woman affected by CS due to an unknown cause. The patient had severe metabolic alkalosis with refractory hypokalemia. To treat the underlying CS, fluconazole was initiated due to unavailability of ketoconazole. In spite of markedly decreasing cortisol levels, metabolic alkalosis persisted. Treatment of metabolic alkalosis with acetazolamide was thus initiated and pH levels successfully lowered. This case report shows that hypercortisolism can be effectively treated with fluconazole in cases where ketoconazole is unavailable or not tolerated and that persistent severe metabolic alkalosis caused by glucocorticoid excess can be safely and successfully treated with acetazolamide.

LEARNING POINTS

Hypercortisolism can be effectively treated with fluconazole where ketoconazole is unavailable or not tolerated.Glucocorticoid excess can cause severe metabolic alkalosis.Persistent severe metabolic alkalosis can be safely and successfully treated with acetazolamide.

摘要

未标注

由异位促肾上腺皮质激素(ACTH)分泌引起的库欣综合征(CS)约占所有类型CS的10%,且常伴有代谢性碱中毒。CS的治疗包括手术切除和/或药物治疗以控制高皮质醇血症。我们报告一例80岁因不明原因患CS的女性病例。该患者有严重的代谢性碱中毒及难治性低钾血症。由于无法获得酮康唑,开始使用氟康唑治疗潜在的CS。尽管皮质醇水平显著下降,但代谢性碱中毒仍持续存在。因此开始用乙酰唑胺治疗代谢性碱中毒,pH值成功降低。本病例报告表明,在无法获得酮康唑或不耐受酮康唑的情况下,氟康唑可有效治疗高皮质醇血症,且由糖皮质激素过量引起的持续性严重代谢性碱中毒可用乙酰唑胺安全、成功地治疗。

学习要点

在无法获得酮康唑或不耐受酮康唑的情况下,氟康唑可有效治疗高皮质醇血症。糖皮质激素过量可导致严重的代谢性碱中毒。持续性严重代谢性碱中毒可用乙酰唑胺安全、成功地治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/608f/4570129/60b6c7f2a95d/edmcr-2015-150027-g001.jpg

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