摘要
Abstract
Objective To study the clinical efficacy of glucocorticoid in treatment of primary endocardial fibroelastosis (EFE) in children.Methods Fifty-eight primary EFE cases admitted in the Children's Hospital of Chongqing Medical University from Jan.2006 to Dec.2013 were treated and followed-up for more than 3 years.Based on whether the application of glucocorticoid and the length of time of glucocorticoid therapy,cases were divided into three groups:no glucocorticoid group (n=15),glucocorticoid 0.5-1 year group (n=17) and glucocorticoid 1-2 year group (n=26).The following indicators were followed up and observed:the cardiac function indexes before and after treatment [Modified ROSS score,left ventricular ejection fraction (LVEF),left ventricular fractional shortening (LVFS)],cardiac size [cardiothoracic ratio (C/T)] and the proportion of death.The efficacy and safety of glucocorticoid were studied.Results No statistically significant difference of modified ROSS scores,LVEF,LVFS and C/T existed among the 3 groups before treatment (P＞0.05).After treatment,the modified ROSS score,LVEF,LVFS and C/T in glucocorticoid 0.5-1 year group were 2.06 ± 1.78,59.29 ± 8.34,31.24 ± 6.0 and 0.580 ± 0.055,respectively,which were significantly different from those before treatment (5.06 ± 1.92,38.76 ± 6.31,18.47 ± 3.14 and 0.67 ± 0.05,respectively,P＜0.05);the modified ROSS score,LVEF LVFS and C/T in glucocorticoid 1-2 years group were 1.28 ± 1.54,64.64 ± 9.08,35.44 ± 5.44 and 0.57 ± 0.06,which were significantly different from those before treatment (5.28 ± 2.26,37.88 ± 8.48,18.12 ± 4.99 and 0.67 ± 0.04,P＜0.05).After treatment,the modified ROSS score,LVEF,LVFS and C/T in no glucocorticoid group were 6.00 ± 1.48,42.91 ± 14.36,21.55 ± 8.63 and 0.63 ± 0.05,which were significantly different compared with those in glucocorticoid 0.5-1 year group and glucocorticoid 1-2 years group (P＜0.05).However,no significant difference existed between glucocorticoid 0.5-1 year group and 1-2 years group on the modified ROSS score,LVEF and C/T after treatment (P＞0.05).Conclusion The glucocorticoid has good clinical effect on endocardial fibroelastosis,and no significant difference is found on the efficacy between the total course of glucocorticoid therapy 0.5-1 year group and 1-2 years group.关键词
婴儿/原发性心内膜弹力纤维增生症/糖皮质激素/随访研究Key words
children/endocardial fibroelastosis/glucocorticoid/follow-up study分类
医药卫生
