用新标准对76例马方综合征的诊断再分析_谢进生.pdfVIP

2004 2 19 1 ( 125 ) 59 ## 76 * X 谢进生 孙衍庆 周子凡 刘愚勇 侯晓彤 郑斯宏 : , : 1996 , 1986 ~ 1996 76 / 0, , : 561 6% ( 43/ 76) , 321 9% ( 25/ 76) , 101 5% ( 8/ 76) : , New Criteria and Diagnosis of Marfan Syndrome-An Analysis of 76 Cases Xie Jinsheng , Sun Yanqing , Zh u Zifan, et al. Department f Cardi vascular Surgery, Anzhen H spital, Capital University f Medical Sciences, Beijing ( 100029) Abstract Objective: Mindless diagn sis f Marfan syndr me sh uld be av ided, because its clinical management and genetic c nsult a- ti n are different fr m ther diseases. Meth ds: Based n the new ly revised criteria in 1996, data n 76 perated pat ients, w h had been diagn sed as having Marfan syndr me in ur h spital fr m 1986 t 1996, were analyzed with respect t their familial pedigrees, and skeletal, cular and cardi vascular abn rmalities in rder t c nfirm r rule ut t he preri ns diagn sis. Results: The previ ns accurate diagn stic rate was 56. 6% ( 43/ 76) , the false-p sitive rate was 32. 9% ( 25/ 76) , undear cause wasin 10. 5% ( 8/ 76) . C nclusi n: Mindless Marfan syndr me was very p pular in perated patients. Many false-p sitive diagn sis may be av ided if the new criteria is used in clinical practice. Key words Marfan syndr me; Diagn sis; New criteria ( Chinese Circulati n J urnal, 2004, 19: 59. ) , 15 / 0, , , , , [ 1, 2] , 1/ 3 000~ 5 000 1 , ,