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获得的下丘脑功能障碍的许多不同的临床面孔:荷兰的一项回顾性队列研究
Ichelle M A A van Roessel1,2, Sanne C Hulsmann1,2, Antoinette Y N Schouten-van Meeteren1
1Princess Máxima Center, Heidelberglaan 25, 3584 CS, Utrecht, the Netherlands.
EClinicalMedicine
|July 21, 2025
概括
在患有脑瘤的儿童中,获得的下丘脑功能障碍除了肥胖之外还表现出各种症状,影响超过80%的患者. 识别这些不同的临床"面孔"对于及时诊断和个性化治疗至关重要.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 儿科瘤学 儿科瘤学
- 神经瘤学神经瘤学
背景情况:
- 获得的下丘脑功能障碍 (HD) 是治疗 (超细胞) 脑瘤的儿童的一种严重并发症.
- 虽然肥胖和垂体功能障碍是公认的,但其他HD症状,如低温,睡眠和行为问题往往被忽视.
- 疾病的各种表现可能会阻碍诊断和治疗的有效性.
研究的目的:
- 系统地描述获得的下丘脑功能障碍在患有 (超细胞) 脑瘤的儿科患者中的各种临床表现.
- 调查这些表现与患者,瘤或治疗相关因素之间的关联.
主要方法:
- 对336名儿科患者进行了回顾性队列研究,这些患者患有超门中线低级质瘤 (LGG),膜瘤 (cCP) 或超细胞生殖细胞瘤 (GCT).
- 患者使用诊断性下丘脑评分进行评估,报告了六个领域的症状流行率.
- 分析的重点是确定HD的不同临床面孔和相关因素.
主要成果:
- 在81.3%的患者中存在下丘脑功能障碍;38.1%的患者符合下丘脑综合征 (HS) 标准,呈现42种不同的临床表现.
- 最常见的HS组合包括过,下丘脑肥胖和垂体功能障碍.
- 神经外科切除,向治疗和更长的随访与HS存在有关.
结论:
- 儿科脑瘤幸存者的下丘脑功能障碍是多方面的,超越肥胖.
- 肥胖可以发生在完整的垂体功能,和HD可以存在没有肥胖.
- 识别HD的各种临床表现对于减少诊断延迟和个性化患者管理至关重要.
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