儿童继发性扩张型心肌病的临床特征  

Clinical Characteristics of Children with Secondary Dilated Cardiomyopathy

作　　者：张小建 张军毅[1] 冯嵩[1] ZHANG Xiaojian;ZHANG Junyi;FENG Song(Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院儿科,河南郑州450052

出　　处：《河南医学研究》2022年第1期85-89,共5页Henan Medical Research

摘　　要：目的探讨儿童继发性扩张型心肌病的临床特征。方法回顾性分析2010年1月1日至2021年8月9日郑州大学第一附属医院收治的9例儿童继发性扩张型心肌病的临床资料,其中男4例,女5例。记录病史、临床表现、实验室检查、器械检查以及结局(以2021年8月21日为观察结局点)。结果在9例病例中,继发于血液肿瘤3例,继发于X连锁无丙种球蛋白血症、尿毒症、甲基丙二酸血症合并同型半胱氨酸血症、地中海贫血、系统性红斑狼疮、进行性肌营养不良各1例。9例患儿中1例失访,1例家属拒绝随访,3例死亡,4例健康。诊断扩张型心肌病的中位年龄为12.11岁。首次诊断扩张型心肌病时,患儿左室射血分数(LVEF)为(31.00±6.34)%,左室短轴缩短率(LVFS)为(15.13±2.74)%,合并二尖瓣关闭不全、三尖瓣关闭不全等畸形。患儿入院时纽约心脏病学会(NYHA)心功能分级为Ⅱ级5例,Ⅲ级2例,Ⅳ级2例。B型钠尿肽、乳酸脱氢酶水平高于参考值(P<0.05)。结论儿童扩张型心肌病可继发于X连锁无丙种球蛋白血症、甲基丙二酸血症合并同型半胱氨酸血症、地中海贫血。B型钠尿肽水平显著升高但入院时心功能分级以Ⅱ级居多,与B型钠尿肽存在分离现象。Objective To explore the clinical features of children with secondary dilated cardiomyopathy.Methods The clinical data of 9 children with secondary dilated cardiomyopathy admitted to the First Affiliated Hospital of Zhengzhou University from January 1,2010 to August 9,2021 were retrospectively analyzed,including 4 boys and 5 girls.The medical history,clinical manifestations,laboratory examination,instrument examination and outcome(August 21,2021 as the observation outcome point)were recorded.Results Among the 9 children,3 cases were secondary to hematological tumor diseases,and secondary to X-linked agammaglobulinemia,uremia,methylmalonic acidemia combined with homocysteinemia,thalassemia,systemic lupus erythematosus and progressive muscular dystrophy in 1 case each.Among 9 children,1 case was lost to follow-up,and 1 case refused to follow up,and 3 cases died,and 4 cases were healthy.The median age of the diagnosis of dilated cardiomyopathy was 12.11 years old.When dilated cardiomyopathy was diagnosed for the first time,left ventricular ejection fraction(EF)was(31.00±6.34)%,and left ventricular fractional shortening rate(FS)was(15.13±2.74)%,combined with malformations such as mitral regurgitation and tricuspid regurgitation.At the time of admission,the heart function of New York Heart Association(NYHA)was classified as gradeⅡin 5 cases,gradeⅢin 2 cases and gradeⅣin 2 cases.The levels of B-type natriuretic peptide and lactate dehydrogenase were higher than the reference values(P<0.05).Conclusion Children with dilated cardiomyopathy could be secondary to X-linked agammaglobulinemia,methylmalonic acidemia with homocysteinemia and thalassemia.The level of B-type natriuretic peptide is significantly increased,but the cardiac function classification is mostly gradeⅡat admission,which is separated from B-type natriuretic peptide.

关 键 词：扩张型心肌病 儿童 临床特征 

分 类 号：R725.4[医药卫生—儿科]