The contribution of arterial calcification to peripheral arterial disease in pseudoxanthoma elasticum.

The contribution of arterial calcification to peripheral arterial disease in pseudoxanthoma elasticum.
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动脉钙化对弹性假黄瘤周围动脉疾病的贡献。

DOI:
10.1371/journal.pone.0096003
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发表时间:
2014
期刊:
影响因子:
3.7
通讯作者:
Martin L
Martin L
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Leftheriotis G;Kauffenstein G;Hamel JF;Abraham P;Le Saux O;Willoteaux S;Henrion D;Martin L

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动脉钙化(AC)在外周动脉疾病(PAD)和动脉壁可压缩性中的作用是一个有争议的问题。弹性假黄瘤(PXE),一种遗传性代谢疾病,由于ABCC 6基因突变,结合弹性纤维断裂和钙化的各种软组织,包括动脉壁。由于AC与PAD相关,PAD是PXE的常见并发症,我们试图确定AC在该组患者的PAD和动脉壁压缩性中的作用。通过踝臂压力指数(ABI)测定了71例PXE患者(平均年龄48±SD 14岁,45名女性)的动脉可压缩性和通畅性,并与30例无PAD的对照组进行了比较。下肢动脉钙化(LLAC)通过非增强螺旋CT扫描确定。计算双腿股动脉、腘动脉和腘下动脉段的钙化评分(Ca评分)。40例PXE患者的ABI为<0.90 and none had an ABI>1.40。LLAC随着年龄的增长而增加,PXE组明显高于对照组。LLAC和ABI之间存在负相关(r =-0.363,p = 0.002)。    LLAC与PXE和年龄独立相关,ABI与心血管危险因素无关。AC的存在与PAD和PXE相关,但不影响动脉可压缩性。PXE患者的PAD可能是由于近端阻塞性病变的发展独立于心血管危险因素。
The contribution of arterial calcification (AC) in peripheral arterial disease (PAD) and arterial wall compressibility is a matter of debate. Pseudoxanthoma elasticum (PXE), an inherited metabolic disease due to ABCC6 gene mutations, combines elastic fiber fragmentation and calcification in various soft tissues including the arterial wall. Since AC is associated with PAD, a frequent complication of PXE, we sought to determine the role of AC in PAD and arterial wall compressibility in this group of patients. Arterial compressibility and patency were determined by ankle-brachial pressure index (ABI) in a cohort of 71 PXE patients (mean age 48±SD 14 yrs, 45 women) and compared to 30 controls without PAD. Lower limb arterial calcification (LLAC) was determined by non-contrast enhanced helicoidal CT-scan. A calcification score (Ca-score) was computed for the femoral, popliteal and sub-popliteal artery segments of both legs. Forty patients with PXE had an ABI<0.90 and none had an ABI>1.40. LLAC increased with age, significantly more in PXE subjects than controls. A negative association was found between LLAC and ABI (r = −0.363, p = 0.002). The LLAC was independently associated with PXE and age, and ABI was not linked to cardiovascular risk factors. The presence of AC was associated with PAD and PXE without affecting arterial compressibility. PAD in PXE patients is probably due to proximal obstructive lesions developing independently from cardiovascular risk factors.
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