遗传性转甲状腺素淀粉样变性病的颈三角神经扩张与自主神经、心脏和胃肠道系统的变化相关。

IF 9 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Journal of Internal Medicine Pub Date : 2024-10-22 DOI:10.1111/joim.20019
Hsueh-Wen Hsueh, Chi-Chao Chao, Yen-Hung Lin, Ping-Huei Tseng, Mao-Yuan Su, Sung-Tsang Hsieh
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引用次数: 0

摘要

背景:遗传性转甲状腺素淀粉样变性(ATTRv)是一种影响多个身体系统的遗传性疾病。虽然声像图通常显示四肢神经、臂丛神经和迷走神经增大,但这些发现的临床意义仍不明确:我们对 ATTRv 患者和健康对照组的正中神经、C5-C7 颈椎神经和迷走神经进行了声像图测量。我们还收集了临床概况、心脏和胃肠道检查结果,用于线性回归分析:我们招募了 47 名 ATTRv 患者(男性/女性:34/13,年龄:65.6 ± 5.3 岁)。取样的节段均明显大于对照组。在临床资料中,颈部三角神经(颈脊神经和迷走神经)和所有神经(颈脊神经、迷走神经和腕部正中神经)的 Z 评分总和与家族性淀粉样变性多神经病分期、自律神经系统(ANS)症状发作和自律神经症状评分有明显相关性。在心脏检查中,发现一些超声波和磁共振成像参数(主要是那些反映心脏容积的参数)与颈脊神经的 Z 评分总和有明显相关性,但与迷走神经的 Z 评分无明显相关性。在胃肠道评估中,迷走神经的横截面积与闪烁扫描的胃排空时间参数相关:结论:超声检查中颈部三角神经的增大与自律神经系统功能障碍的相关参数有关,表明超声检查中观察到的神经增大可作为ATTRv的潜在替代生物标志物。
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Neck triangle nerve enlargement in hereditary transthyretin amyloidosis correlates with changes in the autonomic, cardiac, and gastrointestinal systems.

Background: Hereditary transthyretin amyloidosis (ATTRv) is a hereditary disease that affects multiple bodily systems. Although sonography generally reveals enlargement of nerves in the limbs, the brachial plexus, and vagus nerve, the clinical significance of these findings remains unclear.

Methods: We performed sonographic measurements of the median nerve, cervical spinal nerves at the C5-C7 level, and the vagus nerve in patients with ATTRv and healthy controls. Clinical profiles and cardiac and gastrointestinal examination results were also collected for linear regression analysis.

Results: We recruited 47 patients with ATTRv (males/females: 34/13, age: 65.6 ± 5.3 years). The sampled segments were all significantly larger than those of the controls. In the clinical profiles, the sum of the Z scores of the neck triangle nerves (cervical spinal nerves and vagus nerve) and of all nerves (cervical spinal nerves, vagus nerve, and median nerve at the wrist) significantly correlated with the familial amyloid polyneuropathy stage, onset of autonomic nervous system (ANS) symptoms, and autonomic symptom scores. On cardiac examinations, several ultrasonography and magnetic resonance imaging parameters (primarily those that reflect heart volume) were found to be significantly correlated with the sum of the Z scores of the cervical spinal nerves but not with the Z score of the vagus nerve. In gastrointestinal evaluation, the cross-sectional area of the vagus nerve was correlated with gastric emptying time parameters on scintigraphy.

Conclusions: Neck triangle nerve enlargement on sonography correlated with parameters related to ANS dysfunction, indicating that nerve enlargement observed on ultrasonography may serve as a potential surrogate biomarker of ATTRv.

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来源期刊
Journal of Internal Medicine
Journal of Internal Medicine 医学-医学:内科
CiteScore
22.00
自引率
0.90%
发文量
176
审稿时长
4-8 weeks
期刊介绍: JIM – The Journal of Internal Medicine, in continuous publication since 1863, is an international, peer-reviewed scientific journal. It publishes original work in clinical science, spanning from bench to bedside, encompassing a wide range of internal medicine and its subspecialties. JIM showcases original articles, reviews, brief reports, and research letters in the field of internal medicine.
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