Hamano K, Kuga T, Takahashi M, Fujioka K, Katoh T, Zempo N, Fujimura Y, Esato K
First Department of Surgery, Yamaguchi University School of Medicine, Ube, Yamaguchi, Japan.
J Vasc Surg. 1998 Dec;28(6):1104-6. doi: 10.1016/s0741-5214(98)70037-0.
We analyzed 79 consecutive patients with aneurysms and found a patient who lacked type III collagen. Collagen was extracted from the skin, and the lack of type III collagen was determined by means of sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). Only 1 of the 79 patients was found to lack type III collagen. This patient was a 55-year-old man who had sustained an abdominal aortic aneurysm and aortic dissection. He did not show any of the typical clinical symptoms of Ehlers-Danlos syndrome type IV, such as hyperextensible skin and joints. He had none of the usual risk factors nor any clear family history of the syndrome. Furthermore, his collagen fibrils demonstrated a homogeneous appearance. This case may represent a new form of type III collagen deficiency.
我们分析了79例连续性动脉瘤患者，发现1例缺乏III型胶原蛋白的患者。从皮肤中提取胶原蛋白，通过十二烷基硫酸钠-聚丙烯酰胺凝胶电泳（SDS-PAGE）确定III型胶原蛋白的缺乏情况。79例患者中仅1例被发现缺乏III型胶原蛋白。该患者为一名55岁男性，患有腹主动脉瘤和主动脉夹层。他没有表现出IV型埃勒斯-当洛综合征的任何典型临床症状，如皮肤和关节过度伸展。他没有常见的危险因素，也没有该综合征的明确家族史。此外，他的胶原纤维呈现出均匀的外观。该病例可能代表了一种新形式的III型胶原蛋白缺乏症。
