Ascending aortic aneurysm and histopathology in Alport syndrome: a case report.
Ascending aortic aneurysm and histopathology in Alport syndrome: a case report.
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DOI：
10.1186/s12882-023-03345-5
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发表时间：
2023-10-12
期刊：
影响因子：
2.3
通讯作者：
Shehadeh, Lina A.
中科院分区：
文献类型：
--
作者：
Kamiar, Ali;Alitter, Qusai;Capcha, Jose M. C.;Saad, Ali;Webster, Keith A.;Shehadeh, Lina A.
关键词：
Alport综合征（AS）是由IV型胶原基因突变引起的，该基因通常靶向并损害肾、眼和耳蜗感觉神经组织基底膜的完整性。IV型和V型胶原也是动脉壁的组成部分，尽管包括AS在内的胶原病变与主动脉疾病有关，但AS中主动脉瘤的发病率尚不清楚，可能是因为报告不足。因此，AS目前不被认为是动脉瘤的独立危险因素，需要更详细的病例研究，包括基底膜异常的组织学证据来确定这种可能的联系。在这里，我们提出了一个独特的组织病理学发现的上升主动脉瘤收集在手术时从一个AS患者，其中高血压是唯一的其他已知的危险因素。研究揭示了主动脉瘤的典型组织学特征，包括动脉粥样硬化、淋巴细胞浸润、弹性蛋白破坏和粘液样变性，可能与AS有关。
Alport syndrome (AS) is caused by mutations in type IV collagen genes that typically target and compromise the integrity of basement membranes in kidney, ocular, and sensorineural cochlear tissues. Type IV and V collagens are also integral components of arterial walls, and whereas collagenopathies including AS are implicated in aortic disease, the incidence of aortic aneurysm in AS is unknown probably because of underreporting. Consequently, AS is not presently considered an independent risk factor for aortic aneurysm and more detailed case studies including histological evidence of basement membrane abnormalities are needed to determine such a possible linkage. Here, we present unique histopathological findings of an ascending aortic aneurysm collected at the time of surgery from an AS patient wherein hypertension was the only other known risk factor. The studies reveal classical histological features of aortic aneurysm, including atheroma, lymphocytic infiltration, elastin disruption, and myxoid degeneration with probable AS association.
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影响因子：
168.9
作者：
Libby, P
通讯作者：
Libby, P
影响因子：
--
作者：
Vaicys, C;Hunt, CD;Heary, RF
通讯作者：
Heary, RF
与复发性斯坦福大学主动脉解剖相关的ALPORT综合征病例。
影响因子：
0.8
作者：
通讯作者：
--
影响因子：
3.9
作者：
Gunthner, Roman;Knipping, Lea;Jeruschke, Stefanie;Satanoskij, Robin;Lorenz-Depiereux, Bettina;Hemmer, Clara;Braunisch, Matthias C.;Riedhammer, Korbinian M.;Comic, Jasmina;Tonshoff, Burkhard;Tasic, Velibor;Abazi-Emini, Nora;Nushi-Stavileci, Valbona;Buiting, Karin;Gjorgjievski, Nikola;Momirovska, Ana;Patzer, Ludwig;Kirschstein, Martin;Gross, Oliver;Lungu, Adrian;Weber, Stefanie;Renders, Lutz;Heemann, Uwe;Meitinger, Thomas;Buscher, Anja K.;Hoefele, Julia
通讯作者：
Hoefele, Julia
DOI：
10.1097/01.asn.0000090034.71205.74
发表时间：
2003-10-01
影响因子：
13.6
作者：
Jais, JP;Knebelmann, B;Gubler, MC
通讯作者：
Gubler, MC