目的:通过对心内膜弹力纤维增生症(endocardial fibroelastosis,EFE)患儿血浆脑钠肽(brain natrium peptide,BNP)、N端脑钠肽前体(N-terminal pro-BNP,NT-pro-BNP)的动态观察以及其与左室射血分数(left ventricular ejection fraction,LVEF)的相关性,来评估其在EFE转归和预后判定中的价值。方法:(1)病例选择:选自中国医科大学附属盛京医院2009年1月初至2013年4末住院的EFE患儿共46例,所有患儿均符合EFE相关诊断标准。(2)药物治疗:所有患儿均在强心、利尿并口服糖皮质激素基础上加用丙种球蛋白治疗,对治疗6个月疗效不佳者,加服环磷酰胺口服。(3)随访项目:血清离子、心肌标记物、肝肾功能和BNP、NT-pro-BNP、心电图、心脏超声加左心功能测定。结果:全组46例患儿,男24例,女22例,平均发病年龄9个月,平均随访时间24个月。全组初诊EFE时血浆BNP、NT-pro-BNP中位数及上下四分位间距分别为:BNP 2037.5(387.2,4489.0) pg·ml-1,NT-pro-BNP 6565.5(3696.5,9992.2)pg·ml-1。随心功能恢复其值逐渐下降,分别于治疗6个月、12个月时其中位数值降至正常范围。全组LVEF中位数于治疗6个月后升高至50%以上,于24个月时恢复正常范围(60%)。血浆BNP及NT-pro-BNP水平与LVEF均呈负相关,回归方程分别为Y=-71.2X+3451,r=-0.46和Y=-136.30X+8509.04,r=-0.70。结论:血浆BNP、NT-pro-BNP水平在EFE治疗前均升高,且与LVEF呈负相关;BNP、NT-pro-BNP检测可结合患儿临床症状及心脏超声、心电图等共同应用于EFE的疗效和预后的评估。 |
Objective:To evaluate the significant of BNP/NT-pro-BNP and the relationship between them and the LVEF in patients with endocardial fibroelastosis (EFE). Methods:(1) Choose patients:From January 2009 to April 2013,total 46 EFE children were selected. (2) Treatment:Gamma globulin intravenous IVIG(intravenous immunoglobulin),at the same time took captopril, prednisone or medrol,digoxin,uragogue.To those patients who had taken medicine regularly over 6 months and LVEF were still less than 30% additionally took cyclophosphamide (CTX). (3) Follow-up:cardiac functional grading, BNP/NT-pro-BNP, the electrolyte concentration, myocardial enzyme, troponin T, liver and kidney function test, ECG, pulse Doppler electrocardiograph(PDE) including LVEF and left ventricular diastolic diameter(LVED) were recorded at each time. Results:The patients were male 24 and female 22, the average age was 9 months.The median of BNP/NT-pro-BNP levels were 2037.5(387.2, 4489.0)pg·ml-1 and 6565.5(3 696.5, 9992.2)pg·ml-1 before treatment. It recovered at 6 and 12 months. The correlation between BNP/NT-pro-BNP and LVEF were negative. The relevant regression equation between BNP and LVEF was Y=-71.2X+3 451, r=-0.46, while between NT-pro-BNP and LVEF was Y=-136.30X+8509.04,r=-0.70. Conclusions:BNP/NT-pro-BNP increase before treatment of EFE. There are negative correlation between BNP/NT-pro-BNP and LVEF. The former and clinical symptoms,PDE,ECG can be used to evaluate the cardiac function in EFE patients. |
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