Clinical Analysis of Cardiac Involvement in Children with Mitochondriopathies
- VernacularTitle:儿童线粒体病心脏损害23例临床分析
- Author:
jian-guang, QI
;
ying, ZHANG
;
yu, QI
;
yan-ling, YANG
;
ye, WU
;
yu-wu, JIANG
;
jiong, QIN
;
jun-bao, DU
- Publication Type:Journal Article
- Keywords:
mitochondriopathies;
children;
cardiac involvement
- From:Journal of Applied Clinical Pediatrics
1986;0(01):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical characteristics of cardiac involvement in children with mitochondriopathies.Methods The clinical data of 23 children with mitochondriopathies were reviewed.The changes of electrocardiography,echocardiography and heart enzymes were analyzed.Results In 15 cases of mitochondrial encephalomyopathy,lactic acidosis,and stroke-like episode(MELAS syndrome),electrocardiography was performed on 9 cases,6 of them showed abnormal electrocardiographic findings,including right bundle branch block,ST-T change,Wolff-Parkinson-White syndrome,et al.On echocardiographic examination in 9 MELAS syndrome ca-ses,only 1 case showed hypertrophy cardiomyopathy.Six cases had increased plasma creatine kinaseMB(CK-MB) mass and only one of 12 MELAS syndrome cases had increased cardiac troponin I(cTnI) level.In 8 cases of subacute necrotizing encephalomyopathy(Leigh syndrome),electrocardiography was performed on 5 cases,4 of them showed abnormal electrocardiographic findings,including sinus tachycardia,ST-T change and low voltage.Two cases showed normal electrocardiography.Three out of 6 cases with Leigh syndrome showed increased plasma CK-MB mass.The molecular genetic examinations were performed in 13 cases of MELAS syndrome and 6 cases of Leigh syndrome.The mitochondrial DNA nt 3243 A→G mutation was found in white blood cells of 9 MELAS syndrome cases,the mutation rate being 37%-60%.The mitochondrial DNA nt 8993 T→C mutation was found in white blood cells of 2 Leigh syndrome cases.Conclusion In children with mitochondriopathies,myocardiac involvement is comparatively common,and even cardiomyopathy can occur.
