摘要
目的探讨儿童心内膜弹力纤维增生症(EFE)的临床特点及预后。方法选取2006年1月—2016年12月广西医科大学第一附属医院确诊的EFE病儿65例,分析其临床特点并密切随访其预后。结果病儿多因呼吸道感染、心功能不全就诊;心电图以左心室增大、左心室高电压、ST-T段改变为主;胸片示心胸比(C/T)增大;心脏彩超检查示左心室射血分数(LVEF)、左心室短轴缩短率(LVFS)、每搏指数(SI)及心脏指数(CI)下降,左心室舒张末期内径(LVDD)增大,左心室心内膜增厚。接受洋地黄、肾上腺皮质激素、扩血管药物等1～3年治疗后,病儿LVEF、LVFS、SI及CI增大,LVDD缩小,左心室心内膜厚度减小,差异有显著性(P<0.05)。65例病儿中52例(80.0%)临床好转,7例(10.7%)病情反复发作,6例(9.2%)死亡,均为诊断后6个月内死亡;生存曲线示EFE病儿1、5年生存率均为88.8%。结论 EFE常见于婴幼儿期病儿,以心内膜增厚、心脏增大为主要特点,易出现呼吸道感染和心力衰竭,起病后前6个月内死亡率高;采用洋地黄、肾上腺皮质激素、扩血管药物等综合治疗可取得良好疗效。
Objective To explore the clinical characteristics and prognosis of pediatric patients with endocardial fibroelastosis(EFE).Methods Totally 65 cases of EFE confirmed in The First Affiliated Hospital of Guangxi Medical University from January 2006 to December 2016 were enrolled in the study.Their clinical characteristics were analyzed,and these patients were followed up.Results Most of the patients presented to the hospital with respiratory tract infection and cardiac insufficiency.An electrocardiogram mainly showed left ventricular enlargement,left ventricular high voltage,and ST-T segment change.A chest radiograph showed an increased cardiothoracic ratio.Color Doppler echocardiography revealed reduced left ventricular ejection fraction(LVEF),left ventricular fractional shortening(LVFS),stroke index(SI),and cardiac index(CI)and increased left ventricular end-diastolic diameter(LVDD)and left ventricular endocardial thickness.After 1-3 years of treatment with digitalis,adrenal corticosteroids,and vasodilators,LVEF,LVFS,SI,and CI significantly increased,and LVDD and left ventricular endocardial thickness significantly decreased(P<0.05).Fifty-two cases(80.0%)had a clinical symptom improvement,7 cases(10.7%)had recurrent attacks,and 6 cases(9.2%)died within 6 months after diagnosis.The survival curve showed that the 1-and 5-year survival rates of EFE patients were both 88.8%.Conclusion EFE is a common disease in infants and young children,characterized by endocardial thickening,cardiac enlargement,respiratory tract infection,and heart failure.The pediatric patients with EFE have high mortality within 6 months after diagnosis.Comprehensive treatment with digitalis,adrenal corticosteroids,and vasodilators has a good clinical effect.
作者
覃素元
罗柳
庞玉生
QIN Suyuan;LUO Liu;PANG Yusheng(Department of Pediatrics,First Affiliated Hospital of Guangxi Medical University,Nanning 530021,China)
出处
《精准医学杂志》
2018年第5期394-397,402,共5页
Journal of Precision Medicine
基金
国家自然科学基金资助项目(81660043)
广西省自然科学基金资助项目(2016GXNSFAA380186)
