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Updated: Jan 11, 2026

Measuring Cardiac Autonomic Nervous System ANS Activity in Children

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Published on: April 29, 2013

在患有下丘脑综合征的儿童中获得了新的自主失调评分

Nathalie J Doelman-Oldenburger^1,2, Hermann L Müller³, Hanneke M van Santen^1,2

¹Princess Máxima Center, Utrecht, The Netherlands.

Endocrine connections

|November 18, 2025

概括

一个新的得分有助于诊断罕见的快速发作的肥胖与下丘脑功能障碍,低通风和自主失调 (ROHHAD) 综合征. 这种工具有助于在患有ROHHAD综合征的儿童中早期识别自主功能障碍.

科学领域:

儿科内分泌学儿科内分泌学
神经学神经学
遗传学遗传学是一个

背景情况:

快速发作的肥胖与下丘脑功能障碍,低通风和自主失调 (ROHHAD) 综合征是罕见的儿童疾病.
由于症状的多样化和演变,特别是自主功能障碍,诊断是具有挑战性的.
目前的2023年下丘脑综合征 (HS) 诊断标准缺乏足够的自主失调评估.

研究的目的:

开发和验证自主失调的附加分数,以提高2023年HS诊断标准.
为了支持早期和更准确的ROHHAD综合征诊断.

主要方法:

根据现有仪器,开发了一个额外的自主失调评分 (0-10) 根据ROHHAD症状量身定制.
在四名ROHHAD患者中追溯测试了组合的HS标准和附加分数.

主要成果:

所有四名被评估的ROHHAD患者都患有HS,并在至少三个领域表现出自主失调.
中位数的自主性失调得分是10分之9的.
附加评分证明了在评估这些患者自主功能障碍时的临床实用性.

结论:

仅仅2023年HS诊断标准就不足以诊断ROHHAD综合征中的自主功能障碍.
建议的附加分数是实用的临床使用,有助于早期识别和跟踪ROHHAD和其他HS原因.

关键词:

罗哈德罗哈德是什么意思诊断标准诊断标准诊断标准诊断标准下丘脑肥胖症下丘脑肥胖症下丘脑下垂体综合征的发生

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