摘要
目的 :探讨马凡 (Marfan)氏综合征的临床与X线表现。方法 :对 2 8例马凡氏综合征进行回顾性分析 ,采用X线平片 ,动脉造影 ,辅以心脏彩超和ABDX多型眼部扫描。结果 :在 2 8例中 ,骨骼异常 2 7例 ,X线平片检查表现为双手足指 (趾 )细长、胸廓畸形、脊椎曲度改变 :心血管症状 2 1例 ,X线平片及动脉导管造影检查示升主动脉扩张 :眼部异常 2 1例 ,9例行晶状体摘除术 ;15例有家族遗传史 ,按照Pyeritz等[1] 提出的四项诊断标准 ,具有其中两项者 9例 ,三项者 15例 ,四项者 4例。结论 :马凡氏综合征骨骼改变以掌指 (跖趾 )骨细长为主 ;在心血管病变方面以升主动脉根部扩张为主 ;眼部异常主要为晶状体半脱位。
Objective: To evaluate the clinical and radiographic diagnosis in Marfan's Syndrome.Methods : 28 cases were studied by X-ray plain film and radio-ultrasongraphy . 2 cases were accepted with angiocardiography. Results : The slender tubulder bones in all cases. Cardiovascular lesion and ocular abnormalities in 21 cases separately . With a positive family history in 15 cases . The diagnosis by Pyeritz's four standard , 9 patients with two , 15 with three and 4 with four abnormalities .Conclusion : The slender tubulder bones and dilatation ascending aortic were fundamental X- ray signs . The lens subluxations is main abnormalitis in eyes.
出处
《河北医学》
CAS
2003年第3期222-224,共3页
Hebei Medicine
