Weir E K, Joffe H S, Blaufuss A H, Beighton P
Eur J Cardiol. 1977 May;5(3):255-61.
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.
皮肤松弛症在组织学上表现为全身弹性组织断裂。主动脉和其他体循环血管出现扩张、伸长甚至动脉瘤形成。我们报告了一名患有皮肤松弛症的儿童出现多处肺动脉分支狭窄。肺动脉狭窄可能是患有这种疾病的患者出现肺动脉高压和右心衰竭的原因。
