马凡综合征151例初步分析  

Preliminary Study of Clinical Features in Marfan's Syndrome of 151 Patients

作　　者：张建强[1] 张颖[1] 惠汝太 浦介麟[1] 

机构地区：[1]北京协和医学院,中国医学科学院,阜外心血管病医院心血管病研究所病理生理中心,北京市100037 [2]中德分子医学研究室

出　　处：《中国循环杂志》2009年第4期289-291,共3页Chinese Circulation Journal

基　　金：国家重点基础研究发展计划项目(NO.2007CB512008)

摘　　要：目的:对马凡综合征患者作回顾性分析。方法:根据Pyeritz诊断标准收集马凡综合征患者151例,初步分析其临床及病理表现。结果:病程小于2天的患者,主动脉夹层检出率(89.1%)显著高于病程大于等于2天者(43.8%)(P<0.01)。术前高敏C反应蛋白水平,急性主动脉夹层者明显高于陈旧性主动脉夹层者、无主动脉夹层者(P<0.01)。主动脉直径大于或等于60mm者(57.5%),主动脉夹层检出率显著高于主动脉直径小于60 mm者(41.7%),差异有统计学意义(P<0.01)。5例无主动脉扩张,伴随主动脉瓣及二尖瓣反流,4例左心室扩张。5例出现主动脉瓣粘液样变性。结论:病史和高敏C反应蛋白的水平对提示是否出现主动脉夹层有价值。大多数患者的主动脉瓣、二尖瓣反流及左心室扩张继发于主动脉扩张;小部分患者的这种血流动力学改变可先于主动脉扩张。主动脉瓣与主动脉壁的病理改变可能同时发生,甚至更早。Objective：To retrospectively analyze the clinical features of Marfan＇ s syndrome（MS）. Methods ：A total of 151 patients were collected according to Pyeritz criteria for preliminary study of MS＇s clinical features and pathological changes. Results ： The patients with acute onset shorter than 2 days had the higher detection rate of artery dissection than those equal to or longer than 2 days（ P 〈 0. 01 ）. The serum high sensitivity C reactive protein（hsCRP） concentration in patients with artery dissection was significantly higher than those without artery dissection（P 〈 0. 01 ）. The detection rate of artery dissection in patients with aneurysm diameter more than 60 mm was higher than those less than 60 mm（ P 〈 0.01 ）. Aortic regurgitation accompanied by mitral regurgitation was found in 5 patients with normal sized aorta diameter. 4 patients presented left ventricular dilata- tion. Mucoid degenerations of aortic valve was found in patients with normal sized aorta. High detection rate of aortic valve degeneration was found in patients with aortic regurgitation. Conclusion ： The acute onset and high level of HsCRP might indicate artery dissection in patients with Marfan＇ s syndrome. In a few cases, aortic regurgitation occurred superior to aortic dilatation, mitral regurgitation and left ventricle dilatation might not be the subsequent change of aortic dilatation. Mucoid degeneration of aortic valve could occur simultaneously with aortic wall degeneration.

关 键 词：马凡综合征 诊断 高敏C反应蛋白 

分 类 号：R54[医药卫生—心血管疾病]