在库希氏病中引起的葡萄糖皮质激素的积极反:西班牙多中心队列的见解
Betina Biagetti1, Andres Cardona-Arias1, Alfonso Soto-Moreno2
1Endocrinology & Nutrition Department. Hospital Universitario Vall de Hebrón. and Vall d'Hebron Research Institute (VHIR), Department of Medicine, Autonomous University of Barcelona, Reference Networks (ERN), 08035. Barcelona, Spain.
概括
葡萄糖皮质体诱导的正反 (GIPF) 发生在近10%的库辛病患者中. 这种独特的皮质腺瘤亚型与更差的手术结果和更低的缓解率有关,需要更密切的监测.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 神经外科 神经外科
背景情况:
- 库希氏病 (CD) 是由于负面反受损而导致皮质醇过量的结果.
- 一部分CD患者表现出葡萄糖皮质激素诱导的正反 (GIPF),这表明一种独特的皮质腺瘤表型.
研究的目的:
- 确定GIPF在一个大的库辛病队列中的流行率.
- 分析与GIPF相关的临床,生化和放射性特征.
- 评估GIPF患者的手术结果,包括缓解和复发率.
主要方法:
- 从SPAIN-CUSHING注册表中对139名库辛病患者的回顾性分析.
- GIPF通过增加血清皮质醇后德克萨米他抑制试验来定义.
- 在GIPF和非GIPF组之间比较临床,生化,放射学数据和手术结果.
主要成果:
- 在9.3%的患者中发现了GIPF.
- GIPF组的BMI较低,尿液自由皮质醇较高,对德斯摩辛的ACTH反应更大.
- 在GIPF患者中,手术缓解率显著降低 (28.6%vs70.2%),无复发存活率减少.
结论:
- 在大约9%的库希病病例中,GIPF是一种可识别的表型.
- 这种亚型与不良的外科治疗结果和更高的复发风险有关.
- GIPF可能代表了一个独特的皮质腺瘤亚型,需要量身定制的管理和进一步的分子研究.
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