Lyons O T A, St John E R C, Morales J P, Chan Y C, Taylor P R
Department of Vascular and Endovascular Surgery, Guy's and St. Thomas' NHS Foundation Hospital, London, UK.
Ann Vasc Surg. 2007 Nov;21(6):816-8. doi: 10.1016/j.avsg.2007.06.004. Epub 2007 Aug 13.
Alport's syndrome is a rare genetic disorder of type IV basement membrane collagen synthesis that typically presents with nephropathy, deafness, and ocular abnormalities. To the best of our knowledge, this is the first report in the world's literature of ruptured thoracoabdominal aortic aneurysm in a young patient with Alport's syndrome and a renal transplant. Hypotheses on an association between collagen disease in Alport's syndrome and aortic aneurysms are discussed.
奥尔波特综合征是一种罕见的IV型基底膜胶原蛋白合成的遗传性疾病，通常表现为肾病、耳聋和眼部异常。据我们所知，这是世界文献中首例关于一名患有奥尔波特综合征并接受肾移植的年轻患者发生胸腹主动脉瘤破裂的报告。本文讨论了奥尔波特综合征中的胶原病与主动脉瘤之间关联的假说。
