Cardiovascular abnormalities in cutis laxa.
Cardiovascular abnormalities in cutis laxa.
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皮肤松弛的心血管异常。
DOI：
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发表时间：
1977
期刊：
影响因子：
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通讯作者：
P. Beighton
中科院分区：
文献类型：
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作者：
E. Weir;H. S. Joffe;A. Blaufuss;P. Beighton
拉克萨在组织学上表现为全身弹性组织的碎裂。主动脉和其他全身血管表现出扩张、伸长甚至动脉瘤形成。我们报告一位有皮肤松弛拉克萨的儿童，并发多个肺动脉分支狭窄。肺动脉狭窄可能是在患有这种疾病的患者中观察到的肺动脉高压和右心衰竭的原因。
Cutis laxa is manifest histologically by fragmentation of elastic tissue throughout the body. The aorta and other systemic vessels show dilatation, elongation and even aneurysm formation. We report multiple pulmonary artery branch stenoses in a child who had cutis laxa. Pulmonary artery stenoses may be the cause of the pulmonary hypertension and right heart failure observed in patients with this condition.