摘要
目的探讨儿童马方综合征的临床表现、实验室检查的特点。方法对24例马方综合征患儿的症状、体征及检查进行综合评估,分析各自阳性率。结果24例患儿中,胸廓、脊柱畸形19例(79%),其次为瘦长体形16例(67%),蜘蛛指(趾)15例(63%)。超声心动图主动脉窦增宽16例(67%),左心室增大及二尖瓣返流3例(13%)。结论早期诊断马方综合征,预防心血管并发症,是降低本病死亡率的关键。
Objective To investigate the differences of symptoms, physical signs and examinations in childhood and adult patients with Marfan syndrome. Methods Twenty four children diagnosed as Marfan syndrome were investigated and evaluated synthetically in symptoms, physical signs and examinations. The positive rate of each item was analyzed to find the differences between childhood and adult patients. Results Among 24 patients, thorax and spinal deformity were found in 19 children （79%）, leptosonme type existed in 16 children （67%） ,dolichostenomelia was found in 15 children 163% ）. The dilation of the aortic sinus was detected in 16 children （67%） , and the dilations of left ventricle and mitral regurgitation were found in 3 children（ 13 % ） with echoeardiogram. Conclusions It is critical ascertain the abnormalities in cardiovascular system and give intervention, and it is expected to prolong the patients＇ life by slowing down the changes in aortic walls.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2006年第8期473-474,共2页
Journal of Applied Clinical Pediatrics
