In patients with pseudoxanthoma elasticum a thicker and more elastic carotid artery is associated with elastin fragmentation and proteoglycans accumulation

In patients with pseudoxanthoma elasticum a thicker and more elastic carotid artery is associated with elastin fragmentation and proteoglycans accumulation
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DOI:
10.1016/j.ultrasmedbio.2004.06.004
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发表时间:
2004-08-01
影响因子:
2.9
通讯作者:
Reneman, RS
Reneman, RS
中科院分区:
医学3区
文献类型:
--
作者:
Kornet, L;Bergen, AAB;Reneman, RS

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弹性假性黄瘤(PXE)患者的皮肤活检显示弹性纤维断裂,钙和蛋白聚糖积聚。假设这种改变也存在于动脉壁,我们研究了这种改变对人类颈总动脉(CCA)功能和结构的影响。事实上,两名PXE患者的动脉中存在弹性蛋白断裂和钙和蛋白聚糖含量增加。超声(US)测定PXE患者(n = 19)和对照组(n = 39) CCA内径、扩张和内膜-中膜厚度(IMT)。评估肱动脉脉压。计算了膨胀系数、柔度系数和杨氏模量。PXE患者与对照组的直径和脉压无显著差异。老年PXE患者的扩张性和顺应性系数明显高于老年对照组。PXE患者和对照组的扩张系数随年龄的增长而下降。与对照组不同,PXE患者的顺应系数并没有随着年龄的增长而降低,杨氏模量也几乎没有增加。PXE患者的IMT在年轻和老年时都明显大于对照组,而老年时的杨氏模量明显小于对照组。PXE患者颈动脉较对照组更厚、更有弹性;这种差异在年龄较大时最为明显。这些改变可能是由在这些患者中观察到的弹性蛋白断裂和蛋白聚糖积累来解释的。(E-mail: Reneman@Fys.unimaas.nl) (C) 2004年世界超声医学生物学联合会。
Skin biopsies in patients with pseudoxanthoma elasticum (PXE) show elastic fiber fragmentation and calcium and proteoglycans accumulation. Assuming such changes to be present in the artery wall as well, we studied the influence of such alterations on function and structure of the human common carotid artery (CCA). Indeed, elastin fragmentation and increased calcium and proteoglycans content were present in the arteries of the two PXE patients examined. Internal diameter, distension and intima-media thickness (IMT) in the CCA of PXE patients (n = 19) and controls (n = 39) were determined by ultrasound (US). Pulse pressure was assessed in the brachial artery. The distensibility and compliance coefficients as well as the Young's modulus were calculated. Diameter and pulse pressure were not significantly different in PXE patients and controls. The distensibility and compliance coefficients were significantly greater in older PXE patients than in older controls. The distensibility coefficient decreased with age in both PXE patients and in controls. Unlike in controls, the compliance coefficient did not decrease and the Young's modulus barely increased with age in PXE patients. IMT was significantly greater at both younger and older ages and the Young's modulus was significantly smaller at older ages in PXE patients than in controls. The carotid artery is thicker and more elastic in PXE patients than in control subjects; differences are most pronounced at older ages. These alterations might be explained by the elastin fragmentation and proteoglycans accumulation as observed in these patients. (E-mail: Reneman@Fys.unimaas.nl) (C) 2004 World Federation for Ultrasound in Medicine Biology.