Defects in long chain fatty acid oxidation presenting as severe cardiomyopathy and cardiogenic shock in infancy

被引：5

作者：

Dereddy, Narendra R. ^{[1
]}

Kronn, David ^{[2
]}

Krishnan, Usha ^{[3
]}

机构：

[1] Maria Fareri Childrens Hosp, Westchester Med Ctr, Dept Pediat, Valhalla, NY USA

[2] Maria Fareri Childrens Hosp, Westchester Med Ctr, Dept Metab & Genet Disorders, Valhalla, NY USA

[3] Maria Fareri Childrens Hosp, Westchester Med Ctr, Dept Pediat Cardiol, Valhalla, NY USA

来源：

CARDIOLOGY IN THE YOUNG | 2009年 / 19卷 / 05期

关键词：

Heart failure; lipid metabolism; pericardial effusion; COA DEHYDROGENASE-DEFICIENCY;

D O I：

10.1017/S104795110999134X

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Inborn errors of fatty acid metabolism are important causes of reversible cardiomyopathy in infancy. Disorders in long chain fatty acid oxidation can lead to cardiomyopathy, as fatty acid beta oxidation is the major source of myocardial energy after birth. We present 2 cases of such disorders with cardiac manifestations during infancy, which responded well to a diet low in long chain fatty acids.

引用

页码：540 / 542

页数：3

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