Autonomic dysregulation as an early pathologic feature of Huntington Disease.
Autonomic dysregulation as an early pathologic feature of Huntington Disease.
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DOI：
10.1016/j.autneu.2021.102775
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发表时间：
2021-03
期刊：
影响因子：
--
通讯作者：
Nopoulos PC
中科院分区：
文献类型：
--
作者：
Schultz JL;Harshman LA;Kamholz JA;Nopoulos PC
患有运动明显亨廷顿病 (HD) 或接近预测运动发作的成年人存在自主神经系统 (ANS) 功能障碍。目前尚不清楚 ANS 功能障碍是否在 HD 运动症状出现前数年就已存在。为了弥合这一知识差距，我们比较了具有导致 HD 的基因扩展的儿童（GE 组）和未扩展基因的儿童（GNE 组）之间的 ANS 功能的粗略标记，这些基因扩展的儿童距离预测的运动发作有数十年的时间。我们纳入了 Kids-HD 研究中年龄<18 岁的参与者。构建了线性混合效应回归模型，控制了性别、年龄和体重指数，并包括每个参与者和每个家庭的随机效应。我们比较了 GE (n=84) 和 GNE (n=238) 组之间的静息心率 (rHR)、核心体温 (CBT)、收缩压 (SBP) 和舒张压 (DBP)。然后，我们根据预测的发病年份 (YTO) 对 GE 组的参与者进行分组，并将他们的生命体征与 GNE 组进行比较。 GE组具有较高的rHR（Δ=3.83，p=0.0064）、SBP（Δ=2.38，p=0.032）和CBT（Δ=0.16，t=2.92，p=0.007）。与 GNE 组相比，具有 15-25 YTO 和 < 15 YTO 的参与者的平均 rHR 和 CBT 显着升高。与 GNE 组相比，YTO 为 25-35 的参与者的平均 SBP 显着升高。 HD 中的 ANS 功能障碍似乎发生在 HD 运动症状预计出现之前大约 20 年。
Autonomic nervous system (ANS) dysfunction has been described in adults with motor-manifest Huntington’s Disease (HD) or those who are near their predicted motor onset. It is unclear if ANS dysfunction is present years prior to the onset of motor symptoms of HD. To bridge this gap in knowledge, we compared crude markers of ANS function between children with the gene-expansion that causes HD (GE group) who were decades from their predicted motor onset and gene-non-expanded children (GNE group). We included participants from the Kids-HD study who were <18 years old. Linear mixed effects regression models were constructed that controlled for sex, age, and BMI, and included a random effect per participant and per family. We compared resting heart rate (rHR), core body temperature (CBT), systolic blood pressure (SBP), and diastolic blood pressure (DBP) between the GE (n=84) and GNE (n=238) groups. We then grouped participants from the GE group based on their predicted years to onset (YTO) and compared their vital signs to the GNE group. The GE group had higher rHR (Δ=3.83, p=0.0064), SBP (Δ=2.38, p=0.032), and CBT (Δ=0.16, t=2.92, p=0.007). The mean rHR and CBT became significantly elevated compared to the GNE group in participants who had 15-25 YTO and those who had < 15 YTO. The mean SBP of participants who had 25-35 YTO was significantly elevated compared to the GNE group. ANS dysfunction in HD seems to occur approximately 20 years prior to the predicted onset of motor symptoms of HD.
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影响因子：
--
作者：
DENHEIJER, JC;BOLLEN, WLEM;BURUMA, OJS
通讯作者：
BURUMA, OJS
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Strucl, Martin
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通讯作者：
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