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特纳综合征青少年的主动脉血管功能受损。

Impaired Vascular Function of the Aorta in Adolescents with Turner Syndrome.

作者信息

An Hyo Soon, Baek Jae Suk, Kim Gi Beom, Lee Young Ah, Song Mi Kyoung, Kwon Bo Sang, Bae Eun Jung, Noh Chung Il

机构信息

Department of Pediatrics, Seoul National University Children's Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, South Korea.

Department of Pediatrics, SMG-SNU Boramae Medical Center, Seoul, South Korea.

出版信息

Pediatr Cardiol. 2017 Jan;38(1):20-26. doi: 10.1007/s00246-016-1478-4. Epub 2016 Sep 15.

DOI:10.1007/s00246-016-1478-4
PMID:27628435
Abstract

To investigate intrinsic vascular abnormalities of the ascending aorta before adulthood in Turner syndrome patients (TS), we compared 25 adolescent TS patients (mean age 14.6 ± 3.4 years) and 16 healthy controls from a university hospital. Blood pressure and other vascular indices were evaluated using echocardiography such as aortic strain, stiffness index, distensibility, and pulse wave velocity. Aortic strain (0.1237 vs. 0.1865, respectively; P = 0.003) and aortic distensibility (0.0049 vs. 0.0081, P = 0.002) were significantly lower in the TS group than in the controls. The fractional area change on velocity vector imaging was also lower in TS patients (29.05 vs. 36.19, P = 0.002). These findings were still observed after adjustment for age, body mass index, and systolic blood pressure. The aortic stiffness index was greater in the TS patients than in the control group (6.79 vs. 3.34, P = 0.02). The pulse wave velocity and ascending aorta diameter did not significantly differ between the TS and control groups. Blood pressures were significantly higher in the TS patients than in the controls (systolic, 120.4 vs. 108.4 mmHg, P = 0.001; diastolic 71.5 vs. 61.7 mmHg, P < 0.001). Although the dimensional changes in the aorta were not clearly observed in adolescents with TS, the elastic properties of the aorta were significantly decreased in TS patients as compared to control subjects.

摘要

为了研究特纳综合征(TS)患者成年前升主动脉的内在血管异常情况,我们比较了来自一家大学医院的25例青少年TS患者(平均年龄14.6±3.4岁)和16例健康对照者。使用超声心动图评估血压和其他血管指标,如主动脉应变、硬度指数、扩张性和脉搏波速度。TS组的主动脉应变(分别为0.1237和0.1865;P = 0.003)和主动脉扩张性(0.0049和0.0081,P = 0.002)显著低于对照组。TS患者速度向量成像上的面积变化分数也较低(29.05和36.19,P = 0.002)。在对年龄、体重指数和收缩压进行调整后,这些结果仍然存在。TS患者的主动脉硬度指数高于对照组(6.79和3.34,P = 0.02)。TS组和对照组之间的脉搏波速度和升主动脉直径没有显著差异。TS患者的血压显著高于对照组(收缩压,120.4对108.4 mmHg,P = 0.001;舒张压71.5对61.7 mmHg,P < 0.001)。虽然在患有TS的青少年中未清楚观察到主动脉的尺寸变化,但与对照受试者相比,TS患者主动脉的弹性特性显著降低。

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本文引用的文献

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Increased aortic stiffness in prepubertal girls with Turner syndrome.患有特纳综合征的青春期前女孩的主动脉僵硬度增加。
J Cardiol. 2017 Jan;69(1):201-207. doi: 10.1016/j.jjcc.2016.03.006. Epub 2016 Apr 5.
2
Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations.特纳综合征患儿及青少年的主动脉夹层:危险因素及管理建议
Arch Dis Child. 2015 Jul;100(7):662-6. doi: 10.1136/archdischild-2014-307080. Epub 2015 Jan 8.
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儿童特纳综合征中高血压的患病率:系统评价和荟萃分析。
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MicroRNA-126-3p/5p and Aortic Stiffness in Patients with Turner Syndrome.Turner综合征患者中的微小RNA-126-3p/5p与主动脉僵硬度
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Left atrial remodelling among Turner syndrome patients: novel insights from non-invasive 3D echocardiography.特纳综合征患者的左心房重塑:来自非侵入性三维超声心动图的新见解。
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Cardiovascular Care of Turner Syndrome Women in Germany: Where Do We Stand?-Results from an Online Patient Survey.德国特纳综合征女性的心血管护理:我们目前的状况如何?——一项在线患者调查的结果
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