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Updated: Jun 17, 2025

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A Novel Method: Super-selective Adrenal Venous Sampling
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宫外丘辛综合征二次扩散性异常神经内分泌细胞增生-一报告2例病例
Raul Lopez Fanas1, Travis Goettemoeller1, Keerthi Cedeno2
1Division of Endocrinology, Diabetes, and Metabolism, Montefiore Medical Center, Bronx, NY 10467, USA.
JCEM case reports
|August 7, 2024
概括
扩散性肺内神经内分泌细胞增生 (DIPNECH) 可以导致外阴ACTH分泌 (EAS),导致库辛综合征. 手术可能无法治愈DIPNECH的EAS,因为它具有扩散性,需要医疗管理.
科学领域:
- 内分泌学 在内分泌学.
- 肺部病理学 肺部病理学
- 在瘤学瘤学.
背景情况:
- 宫外ACTH分泌 (EAS) 导致库希综合征,而扩散性肺内神经内分泌细胞增生 (DIPNECH) 是一个罕见的原因.
- DIPNECH涉及异常的支气管神经内分泌细胞增殖,可能是肺癌前瘤.
研究的目的:
- 突出DIPNECH作为EAS和库辛综合征的重要原因.
- 讨论对DIPNECH次要的EAS管理的挑战.
主要方法:
- 一系列病例呈现了两名患有DIPNECH和库辛综合征的患者.
- 进行了临床,放射和病理评估.
- 治疗包括手术切除和医疗治疗.
主要成果:
- 这两位患者都因EAS出现了严重的库希综合征.
- 最初的治疗包括肺切除和药物治疗并没有完全解决高皮质醇症.
- DIPNECH的分散性质有助于持续的EAS.
结论:
- 在EAS诱导的库辛综合征的差异诊断中应考虑DIPNECH.
- 来自DIPNECH的EAS的手术治疗可能无法治愈.
- 当手术失败时,及时的医疗治疗,预防和双边上腺切除术至关重要.
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