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医学治疗在肾上腺依赖型库欣综合征中的作用是什么?

What is the role of medical therapy in adrenal-dependent Cushing's syndrome?

机构信息

Medizinische Klinik und Poliklinik IV, Department for Endocrinology, Ludwig-Maximilians-University, Munich, Germany.

出版信息

Best Pract Res Clin Endocrinol Metab. 2020 May;34(3):101376. doi: 10.1016/j.beem.2020.101376. Epub 2020 Jan 31.

DOI:10.1016/j.beem.2020.101376
PMID:32063487
Abstract

Medical therapy to control hypercortisolism in adrenal Cushing's syndrome is currently not the first-line therapy. However, in many clinical scenarios like pre-surgical treatment, in patients who are not suitable candidates for surgery or in patients with bilateral hyperplasia, medical therapy can be important representing the only viable treatment option. Adrenal steroidogenesis inhibitors and glucocorticoid receptor blockers have been used for many years: metyrapone, ketoconazole and mifepristone are in current use and effective. Mitotane can be used as well but is considered second-line therapy because of its high toxicity. Etomidate has a special position as emergency medication in severe hypercortisolism. New drugs are tested in prospective trials (levoketoconazole, osilidrostat and relacorilant) and might become effective alternatives to common drugs. Oher drugs - adrenal steroidogenesis inhibitors as well as glucocorticoid receptor antagonists - are currently tested in vitro.

摘要

目前,控制肾上腺库欣综合征的皮质醇过多症的医学治疗并不是一线治疗方法。然而,在许多临床情况下,如术前治疗、不适合手术的患者或双侧增生的患者,医学治疗可能很重要,是唯一可行的治疗选择。肾上腺类固醇生成抑制剂和糖皮质激素受体阻滞剂已经使用了多年:美替拉酮、酮康唑和米非司酮目前正在使用并有效。密妥坦也可以使用,但由于其高毒性,被认为是二线治疗药物。依托咪酯作为严重皮质醇过多症的急救药物具有特殊地位。新的药物正在前瞻性试验中进行测试(左酮康唑、奥西利德罗司他和雷拉科利兰),可能成为常用药物的有效替代品。其他药物——肾上腺类固醇生成抑制剂和糖皮质激素受体拮抗剂——目前正在体外进行测试。

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Advances in pharmacological treatment of Cushings disease.库欣病药物治疗的进展
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Levoketoconazole.左酮康唑
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The effects of selected inhibitors on human fetal adrenal steroidogenesis differs under basal and ACTH-stimulated conditions.所选抑制剂对人胎儿肾上腺甾体生成的影响在基础状态和 ACTH 刺激条件下不同。
BMC Med. 2021 Sep 8;19(1):204. doi: 10.1186/s12916-021-02080-8.