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异胎ACTH综合征的困难病例:即使没有精确的定位,治疗也可能吗?
Adam I Kaplan1,2, Liam Clifford1, Ammar Wakil3
1Department of Endocrinology, Gosford Hospital, Gosford, New South Wales, Australia.
Journal of the ASEAN Federation of Endocrine Societies
|December 8, 2025
概括
隐藏的宫外 adrenocorticotropic 激素 (ACTH) 综合征 (EAS) 是罕见的. 凯托可纳在患有症状库辛综合征 (CS) 的患者中有效抑制皮质醇,当广泛测试后ACTH来源仍未确定时.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
背景情况:
- 隐形外阴上腺皮质激素 (ACTH) 综合征 (EAS) 是一种罕见的ACTH依赖的库辛综合征 (CS) 的原因.
- 由于无法定位子宫外ACTH源,诊断可能具有挑战性.
研究的目的:
- 报告由于隐性EAS引起的症状性CS病例.
- 突出基托可纳在治疗高皮质醇症时的治疗效果,当子宫外来源难以捉摸时.
主要方法:
- 一名中年女性患者患有症状的CS经历了全面的诊断工作.
- 调查包括激素抑制测试,多种成像方式和劣质状鼻采样 (IPSS).
- 开启了基托可纳治疗,以控制皮质醇过度产生的情况.
主要成果:
- 尽管进行了广泛的调查,但无法定位子宫外ACTH来源.
- 凯托可纳在抑制皮质醇产生,缓解CS症状方面表现出有效性.
结论:
- 凯托可纳作为一种可行的治疗选择,用于抑制隐性EAS中的皮质醇,即使异胎源仍未确定.
- 对于因隐性EAS而接受基托可纳治疗的患者来说,长期监测至关重要.
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