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[埃尔德海姆-格塞尔中膜坏死及中膜黏液样变性作为主-动脉动脉瘤的病因。对150条切除血管的病理解剖分析]

[Erdheim-Gsell medial necrosis and mucoid degeneration of the media as a cause of aorto-arterial aneurysm. Pathologico-anatomical analysis of 150 excised vessels].

作者信息

Leu H J

机构信息

Institut für Pathologie der Universität Zürich.

出版信息

Schweiz Med Wochenschr. 1988 May 7;118(18):687-91.

PMID:3387965
Abstract

150 cases of true and dissecting aneurysms due to degenerative, non-arteriosclerotic lesions of the media were analyzed. Histologically, two types of alterations may be distinguished. The classical medionecrosis of Erdheim and Gsell is characterized by focal destructions of elastic fibers, while so-called mucoid degeneration of the media shows mucoid deposits between the rarified and atrophic elastic fibers but no focal destruction of the latter. These two lesions are clearly distinguishable in the majority of cases. Average age and distribution among age groups are identical in both types. In both groups the male patients predominate but with a different ratio. True and dissecting types of aneurysm are equally distributed in medionecrosis of Erdheim and Gsell (including the cases with Marfan's syndrome), but dissecting aneurysms predominate by a ratio of 8:1 in mucoid degeneration. Several arguments strengthen the opinion that the two types of lesion represent different disease entities. The etiology of the disorder is still unknown; our findings do not show a relation to arteriosclerosis or to a simple aging process of the vessel wall.

摘要

对150例因中膜退行性、非动脉硬化性病变所致的真性和夹层动脉瘤进行了分析。组织学上,可区分出两种类型的改变。经典的埃尔代姆和格塞尔中膜坏死的特征是弹性纤维局灶性破坏,而所谓的中膜黏液样变性表现为在稀疏和萎缩的弹性纤维之间有黏液样沉积物,但后者无局灶性破坏。在大多数病例中,这两种病变明显可区分。两种类型的平均年龄和年龄组分布相同。两组中男性患者均占多数,但比例不同。真性和夹层型动脉瘤在埃尔代姆和格塞尔中膜坏死(包括马方综合征病例)中分布相同,但在黏液样变性中,夹层动脉瘤以8:1的比例占优势。有几个论据支持这两种病变代表不同疾病实体的观点。该疾病的病因仍不清楚;我们的研究结果未显示与动脉硬化或血管壁的简单老化过程有关。

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1
[Erdheim-Gsell medial necrosis and mucoid degeneration of the media as a cause of aorto-arterial aneurysm. Pathologico-anatomical analysis of 150 excised vessels].[埃尔德海姆-格塞尔中膜坏死及中膜黏液样变性作为主-动脉动脉瘤的病因。对150条切除血管的病理解剖分析]
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[Gsell-Erdheim syndrome: main pathomorphological alterations].[格塞尔-厄尔德海姆综合征:主要病理形态学改变]
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[Dissecting aneurysm of the aorta due to cystic media necrosis (Erdheim-Gsell)].[因囊性中层坏死导致的主动脉夹层动脉瘤(埃尔德海姆-格塞尔病)]
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Two cases of unexpected sudden death due to cystic medionecrosis of the aorta associated with bloodless aortic dissection.两例因主动脉囊性中层坏死伴无血主动脉夹层导致的意外猝死。
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