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胸腹主动脉夹层动脉瘤内侧胶原纤维的分布与成熟度:马凡综合征患者与非马凡综合征患者的比较研究

Distribution and Maturity of Medial Collagen Fibers in Thoracoabdominal Post-Dissection Aortic Aneurysms: A Comparative Study of Marfan and Non-Marfan Patients.

作者信息

Doukas Panagiotis, Hruschka Bernhard, Bassett Cathryn, Buhl Eva Miriam, Simon Florian, Saraber Pepijn, Jacobs Michael Johan, Uhl Christian, Schurgers Leon J, Gombert Alexander

机构信息

Department of Vascular Surgery, RWTH Aachen University Hospital, 52074 Aachen, Germany.

Institute of Pathology, Electron Microscopy Facility, RWTH Aachen University Hospital, 52074 Aachen, Germany.

出版信息

Int J Mol Sci. 2024 Dec 24;26(1):14. doi: 10.3390/ijms26010014.

Abstract

Thoracoabdominal aortic aneurysms (TAAAs) are rare but serious conditions characterized by dilation of the aorta characterized by remodeling of the vessel wall, with changes in the elastin and collagen content. Individuals with Marfan syndrome have a genetic predisposition for elastic fiber fragmentation and elastin degradation and are prone to early aneurysm formation and progression. Our objective was to analyze the medial collagen characteristics through histological, polarized light microscopy, and electron microscopy methods across the thoracic and abdominal aorta in twenty-five patients undergoing open surgical repair, including nine with Marfan syndrome. While age at surgery differed significantly between the groups, maximum aortic diameter and aneurysm extent did not. Collagen content increased from thoracic to infrarenal segments in both cohorts, with non-Marfan patients exhibiting higher collagen percentages, notably in the infrarenal aorta (729.3 nm vs. 1068.3 nm, = 0.02). Both groups predominantly displayed mature collagen fibers, with the suprarenal segment containing the highest proportion of less mature fibers. Electron microscopy revealed comparable collagen fibril diameters across segments irrespective of Marfan status. Our findings underscore non-uniform histological patterns in TAAAs and suggest that ECM remodeling involves mature collagen deposition, albeit with lower collagen content observed in the infrarenal aorta of Marfan patients.

摘要

胸腹主动脉瘤(TAAAs)较为罕见但病情严重,其特征为主动脉扩张,伴有血管壁重塑,弹性蛋白和胶原蛋白含量发生变化。患有马凡综合征的个体具有弹性纤维断裂和弹性蛋白降解的遗传易感性,易于早期形成动脉瘤并使其进展。我们的目的是通过组织学、偏光显微镜和电子显微镜方法,分析25例接受开放手术修复患者的胸主动脉和腹主动脉的中膜胶原特征,其中包括9例马凡综合征患者。虽然两组患者的手术年龄差异显著,但主动脉最大直径和动脉瘤范围并无差异。两个队列中,胶原蛋白含量从胸段到肾下段均增加,非马凡综合征患者的胶原蛋白百分比更高,尤其是在肾下段主动脉(729.3纳米对1068.3纳米,P = 0.02)。两组均主要显示成熟的胶原纤维,肾上腺上段含比例最高的不太成熟的纤维。电子显微镜显示,无论马凡综合征状态如何,各节段的胶原纤维直径相当。我们的研究结果强调了胸腹主动脉瘤中组织学模式的不均匀性,并表明细胞外基质重塑涉及成熟胶原沉积,尽管在马凡综合征患者的肾下段主动脉中观察到较低的胶原蛋白含量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0edd/11720456/6aa6c6df1b26/ijms-26-00014-g001.jpg

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