Zerbino D D, Kuzyk Iu I, Gavryliuk E M
Lvov State Medical University, 79010, Lvov, Ukraine.
Arkh Patol. 2003 Nov-Dec;65(6):36-40.
24 cases of Gsell-Erdheim syndrome observed in 1999-2001 have been analysed. There were 20 men (83.4%) and 4 women (16.6%) aged 22-69 years. Six men (25%) died at the age under 50 years. At necropsies the following characteristic variants of stratification were observed: a total damage with stratification of the aorta at all length or, less often, local damage. Destruction of an elastic skeleton of the media is the basis of aorta stratification: zones free of the nuclei, foci of elastolysis (medianecrosis), thinning, dyschromia and fragmentation of elastic fibers, focal hyperelastosis. The pathological process involves all the aortic length not only the area of aneurysm and rupture. Manifestations of variable severity of the same process were seen in different parts of the aortic wall.
对1999年至2001年观察到的24例格塞尔-厄尔德海姆综合征病例进行了分析。患者年龄在22至69岁之间，其中男性20例（83.4%），女性4例（16.6%）。6名男性（25%）在50岁以下死亡。尸检时观察到以下分层特征性变异：主动脉全长完全损伤伴分层，或较少见的局部损伤。中膜弹性骨架的破坏是主动脉分层的基础：无细胞核区域、弹性溶解灶（中层坏死）、弹性纤维变薄、变色和断裂、局灶性弹性组织增生。病理过程累及主动脉全长，不仅是动脉瘤和破裂区域。同一过程不同严重程度的表现可见于主动脉壁的不同部位。
