Mitochondrial cardiomyopathies

Rossijskij Vestnik Perinatologii i Pediatrii

E. A. Nikolaeva¹  I. V. Leontyeva¹ 

[1] Academician Yu.E. Veltishchev Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University;

关键词: children;    cardiomyopathy;    mitochondrial disease;    kearns–sayre syndrome;    melas syndrome;    merrf syndrome;    narp syndrome;    barth syndrome;    leigh syndrome;    ubidecarenone;    succinates;    cytochrome c;    levocarnitine;   

DOI  :  10.21508/1027-4065-2016-61-3-22-30

来源: DOAJ

【 摘 要 】

The paper considers the problem of diagnosing the heterogeneous forms of mitochondrial cardiomyopathies associated with impaired oxidative phosphorylation and decreased activity of the mitochondrial electron transport system due to mutations in mitochondrial or nuclear DNA genes. It is emphasized that mitochondrial cardiomyopathies rarely present as an isolated myocardial lesion and more frequently serve as a manifestation of multisystem disease. The paper provides the characteristics of cardiomyopathies in some mitochondrial syndromes and shows the possibilities of treating this disease.

【 授权许可】

Unknown