摘要
目的探讨糖皮质激素治疗小儿原发性心内膜弹力纤维增生症(EFE)的临床疗效。方法选取2006年1月-2013年12月于重庆医科大学附属儿童医院心脏内科初次就诊的58例小儿原发性EFE病例,治疗随访3年以上。根据激素治疗时间分3组:未应用激素组(n=15)、激素总疗程0.5~1年组(n=17)及激素总疗程1~2年组(n=26)。随访观察治疗前后心功能指标[改良ROSS评分、左心室射血分数(LVEF)、左室短轴缩短率(LVFS)]、心脏大小[心胸比值(C/T)]变化和死亡人数,分析糖皮质激素治疗EFE的疗效及药物安全性。结果治疗前,3组患儿的改良ROSS评分、LVEF、LVFS、C/T之间差异无统计学意义(P>0.05)。激素总疗程0.5~1年组治疗后改良ROSS评分、LVEF、LVFS和C/T分别为2.06±1.78、59.29±8.34、31.24±6.0、0.580±0.055,与治疗前(5.06±1.92、38.76±6.31、18.47±3.14、0.67±0.05)比较差异均有统计学意义(P<0.05)。激素总疗程1~2年组治疗后改良ROSS评分、LVEF、LVFS和C/T(分别为1.28±1.54、64.64±9.08、35.44±5.44、0.57±0.06)与治疗前(分别为5.28±2.26、37.88±8.48、18.12±4.99、0.67±0.04)比较差异均有统计学意义(P<0.05)。治疗后激素总疗程0.5~1年组和激素总疗程1~2年组改良ROSS评分、LVEF、LVFS和C/T与未应用激素组(分别为6.00±1.48、42.91±14.36、21.55±8.63、0.63±0.05)比较差异均有统计学意义(P<0.05),但治疗后激素总疗程0.5~1年组与激素总疗程1~2年组改良ROSS评分、LVEF、C/T差异无统计学意义(P>0.05)。结论糖皮质激素治疗小儿原发性EFE有良好的临床效果,激素总疗程0.5~1年与1~2年疗效无明显差异。
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 leng th of time of glucocor ticoid therapy, cases were div ided into three groups： no glucocor ticoid 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 gro
作者
李志鑫
钟家蓉
王丹
刘虎
张静
LI Zhi-xin;ZHONG Jia-rong;WANG Dan;LIU Hu;ZHANG Jing(Department of Cardiology, Children＇s Hospital of Chongqing Medical University/Children＇s Development and Disorders Key Laboratory of Ministry of Education/International and National Science and Technology Cooperation Base of Children Development and Critical Disorders/ Chongqing Key Laboratory of Pediatrics, Chongqing 400014, China)
出处
《解放军医学杂志》
CAS
CSCD
北大核心
2018年第4期341-344,共4页
Medical Journal of Chinese People's Liberation Army
