儿科库辛病:长期结果和复发的预测因素
Martin O Savage1, Rosario Ferrigno2
1Centre for Endocrinology, William Harvey Research Institute, Barts and the London School for Medicine & Dentistry, Queen Mary, University of London, London, United Kingdom.
Frontiers in endocrinology
|February 6, 2024
概括
儿科库辛病 (CD) 是一种罕见的疾病,由垂体腺瘤引起,导致皮质醇过多. 转形手术提供了高缓解率,复发与术后激素水平有关.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 神经外科 神经外科
- 在瘤学瘤学.
背景情况:
- 儿科库辛病 (CD) 是由脑垂体腺瘤引起的,导致过多的ACTH和高皮质醇.
- 它在儿童中很少见,具有明显的表现特征,如面部外观改变,体重增加,生长减缓和男性化.
研究的目的:
- 概述了儿童库辛病的诊断和治疗方法.
- 强调专业中心对于最佳患者治疗结果的重要性.
主要方法:
- 诊断包括证明高皮质醇和确定ACTH依赖.
- 治疗共识有利于选择性腺瘤切除的跨形手术 (TSS).
- 第二线选择包括放射治疗和非响应者的上腺切除术.
主要成果:
- 脑膜外科手术 (TSS) 在70-100%的儿科CD病例中实现了缓解.
- 复发率较低,由TSS后更高的皮质醇/ACTH水平和HPA轴的快速恢复预测.
- 完整的微腺瘤切除与组织学和生物化学确认预测低复发.
结论:
- 及时诊断和管理对于预防长期高皮质醇症至关重要.
- 建议儿童CD患者转诊于具有多学科专业知识的高等大学中心.
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