Zerbino Dmytrij, Kusik Julia, Havrilyuk Elena
Institute of Clinical Pathology, Danylo Halytsky Lviv State Medical University, Lviv, Ukraine.
Pol J Pathol. 2005;56(2):75-9.
Twenty four cases of Gsell-Erdheim syndrome observed in 1999-2001 have been examined. There were 20 men (83.4%) and 4 women (16.6%). The age ranged from 22 to 69 years but 6 men (25%) died at age under 50. The following variants of aortic lesions were revealed in autopsy material: intimal tear with the following dissection which extends along the aorta to variable distances--in most cases into iliac arteries. The morphologic changes, associated with were: anuclear zones, elastolysis, thinning, dyschromia and fragmentation of elastic fibers, focal hyperelastosis. Such changes were revealed in all parts of the aortic wall, independently of the site of rupture.
对1999年至2001年间观察到的24例格塞尔 - 厄尔德海姆综合征病例进行了检查。其中男性20例（83.4%），女性4例（16.6%）。年龄范围为22至69岁，但有6名男性（25%）在50岁以下死亡。尸检材料中发现了以下主动脉病变变体：内膜撕裂并伴有随后的夹层分离，夹层沿主动脉延伸至不同距离，大多数情况下延伸至髂动脉。与之相关的形态学变化有：无核区、弹性纤维溶解、变薄、色素异常和弹性纤维断裂、局灶性弹性组织增生。这些变化在主动脉壁的所有部位均有发现，与破裂部位无关。
