儿科和青少年子宫外丘辛综合征:系统性审查
Chethan Yami Channaiah1, Manjiri Karlekar1, Vijaya Sarathi2
1Department of Endocrinology, Seth GS Medical College and KEM Hospital, Mumbai 400012, India.
European journal of endocrinology
|October 6, 2023
概括
在儿童中异位上腺皮质激素综合征 (EAS) 是罕见的. 支气管神经内分泌瘤 (NET) 提供了更好的生存率,而转移和瘤大小预测了儿科EAS的不良结果.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 在瘤学瘤学.
- 内部医学 内部医学
背景情况:
- 宫外 adrenocorticotropic 激素综合征 (EAS) 是一种罕见的内分泌疾病.
- 关于儿科EAS的临床,生化,放射性特征和结果的数据有限.
- 这项研究通过报告新病例和进行系统审查来解决儿科EAS的罕见性.
研究的目的:
- 描述儿科EAS的临床,生化,放射性特征和结果.
- 系统地审查有关儿科EAS的现有文献.
- 为了比较儿科EAS与成人病例的病因概况和结果.
主要方法:
- 从1997年到2021年,对儿科和青少年EAS患者 (≤20岁) 的回顾性评估.
- 在1970-2022年间发表的儿科EAS系统文献综述.
- 对161名确诊病人的分析,包括3例新病例.
主要成果:
- 支气管神经内分泌瘤 (NET) (28.5%) 和胸膜NET (22.9%) 是常见的原因.
- 原始细胞衍生的瘤在第一个十年中更频繁,而支气管NET在第二个十年中占主导地位.
- 计算机断层扫描在92.9%的病例中发现了瘤; 77.9%的病例得到了缓解,但30.4%的病例复发. 远程转移和瘤大小预测了生存率不佳.
结论:
- 儿科EAS的病因概况不同于成人EAS.
- 支气管NET与儿科EAS的最佳长期存活率有关.
- 远程转移和较大的瘤大小是儿科EAS生存的独立负面预测因素.
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