大剂量丙种球蛋白治疗婴幼儿特发性心内膜弹力纤维增生症临床分析

目的:分析大剂量丙种球蛋白(IVIG)治疗婴幼儿特发性心内膜弹力纤维增生症的临床疗效。方法:回顾性分析1984年8月至2010年2月,在首都医科大学附属北京安贞医院小儿心脏科住院的53例特发性婴幼儿心内膜弹力纤维增生症患儿临床资料,应用大剂量丙种球蛋白治疗组患儿24例(2 g.kg-1.次-1,分2 d使用,每月1次连用3次,以后每3个月重复1次)未使用大剂量丙种球蛋白治疗组患儿29例作为对照。对比两组治疗前后1年的临床疗效。结果:两组患者发病月龄、发病时左心室射血分数、舒张末径之间差异无统计学意义(P>0.05),治疗后1年左心室射血分数、舒张末径的改善情况,两组之间差异无统计学意义(P>0.05),但IVIG组环磷酰胺的使用率明显低于非IVIG组,分别为(33.3%vs.62.1%,χ2=4.339,P=0.037)差异有统计学意义。结论:大剂量丙种球蛋白治疗婴幼儿特发性心内膜弹力纤维增生症有一定临床疗效,并能显著降低环磷酰胺的使用率。

Large doses of gamma globulin treatment of infantile idiopathic the endocardial fibroelastosis clinical analysis

Objective:Analysis of high-dose gamma globulin(IV IG) clinical efficacy for the treatment of infants with idiopathic endocardial fibroelastosis.Methods:A retrospective analysis of the August 1984 to February 2010,the capital medical university,Beijing Anzhen hospital pediatric cardiac inpatient 53 cases of idiopathicinfant endocardial fibroelastosis in children with clinical data.24 cases of children with a high dose of gamma globulin treatment group(2 g/kg/time,two-day use,once a month for 3 times,after every three months repeated every) not to use high-dose gamma globulin treatment group sufferingchildren 29 cases as control.Results:The incidence of two groups of patients months of age,the incidence of left ventricular ejection fraction,end-diastolic diameter was no statistically significant difference between the 1 year after treatment,left ventricular ejection fraction,end-diastolic diameter to improve the situation between the two groups showed no significant difference,Utilization of IVIG group cyclophosphamide significantly lower than non-IVIG group(IVIG group and non-the IVIG group cyclophosphamide utilization rate of 33.3% vs.62.1%,respectively,χ2=4.339,P=0.037) and the difference was statistically significant.Conclusion:Large doses of gamma globulin treatment of infantile idiopathic endocardial fibroelastosis have some clinical efficacy,and can significantly reduce the utilization rate of cyclophosphamide.