主动脉瘤，家族性胸 4
基因病名称(英)
Aortic aneurysm, familial thoracic 4
别名(英)
Aortic aneurysm/aortic dissection and patent ductus arteriosus,FAA4,Familial aortic aneurysm 4,Non-syndromic thoracic aortic aneurysms and dissection,TAAD,Thoracic aortic aneurysms and dissection
别名
主动脉瘤/主动脉夹层和动脉导管未闭，FAA4，家族性主动脉瘤 4，非综合征性胸主动脉瘤和夹层，TAAD，胸主动脉瘤和夹层
统计
reviewed:1; annotated:0
交叉引用
132900,C1851504,D017545
描述(英)
A disease characterized by permanent dilation of the thoracic aorta usually due to degenerative changes in the aortic wall. It is primarily associated with a characteristic histologic appearance known as 'medial necrosis' or 'Erdheim cystic medial necrosis' in which there is degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and an accumulation of basophilic ground substance.
描述
一种以胸主动脉永久扩张为特征的疾病，通常是由于主动脉壁的退行性变化。它主要与称为“中层坏死”或“Erdheim 囊性中层坏死”的特征性组织学表现有关，其中有弹性纤维的退化和断裂、平滑肌细胞的损失和嗜碱性基质的积累。