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完全型马凡氏综合征临床少见,我们发现一家系五例,现报告如下: 先证者Ⅱ_2:男,43岁。因晶体脱位入院进行手术治疗。体检:瘦长体型,双手指细长,掌骨指数8.41,右眼晶体脱入鼻侧前房,左眼有角巩膜葡萄肿。两肺正常,心尖部可闻及粗糙的收缩期杂音,主动脉瓣听诊区可闻及双期杂音。肝、脾肋下未触及,两下肢无水肿。X线胸片:左心增大。超声心动图:升主动脉瘤样扩张,内径50 mm,左房33 mm,左房与主动脉比率<0.70,
Complete Marfan syndrome clinically rare, we found a family of five cases, are as follows: Proof II_2: male, 43 years old. Due to dislocation of the hospital for surgical treatment. Physical examination: slender body, fingers slender, metacarpal index 8.41, right eye lens detachment of the nasal anterior chamber, the left eye angular scleral staphyloma. Normal lungs, apex can be heard and rough systolic murmur, aortic valve auscultation area can be heard and double noise. Liver, spleen ribs did not touch, two lower extremity edema. X-ray: increased left heart. Echocardiography: Ascending aortic aneurysm-like dilatation, diameter 50 mm, left atrium 33 mm, atrial and aortic ratio <0.70,