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目的:探讨小儿原发性心内膜弹力纤维增生症(EFE)的误诊原因,评估其疗效及预后。方法:对我院儿科1993年1月~2003年1月24例EFE患儿的临床表现、心电图、X线、超声心动图检查、疗效及随访情况进行分析。结果:本组24例中有21例被误诊,误诊率高达87.5%,13例被误诊为肺炎并心衰占61.9%,5例被误诊为病毒性心肌炎占23.8%。随访18例其中临床治愈12例,好转2例,死亡4例;临床治愈的部分患儿超声心动图检查仍可见心脏组织结构的异常。结论:提高对本病警惕性及重视超声心动图的检查,可提高诊断的正确率。合理的治疗可以改善预后,远期疗效有赖进一步观察。
Objective: To investigate the causes of misdiagnosis of pediatric primary endocardial fibroelastosis (EFE) and to evaluate its efficacy and prognosis. Methods: The clinical manifestations, electrocardiogram, X-ray and echocardiography of 24 pediatric patients with EFE from January 1993 to January 2003 in our hospital were analyzed. The curative effect and follow-up were analyzed. Results: Twenty-one of the 24 cases were misdiagnosed with a misdiagnosis rate of 87.5%. Thirteen cases were misdiagnosed as pneumonia with heart failure (61.9%) and five cases were misdiagnosed as viral myocarditis (23.8%). Follow-up 18 cases, including clinical cure in 12 cases, improved in 2 cases, 4 cases of death; clinical cure echocardiography still visible in some children with abnormal heart tissue structure. Conclusion: To improve the vigilance of this disease and emphasis on echocardiography examination, can improve the diagnostic accuracy. Reasonable treatment can improve the prognosis, long-term efficacy depends on further observation.