Catalog

Dilated cardiomyopathy due to fatty acid oxidation disorder

No MMS code

Link to WHO Definition

Dilated cardiomyopathy due to fatty acid oxidation disorder – http://id.who.int/icd/entity/1234403849

Dilated cardiomyopathy due to fatty acid oxidation disorder is the presence of dilated cardiomyopathy in association with a disorder of the fatty acid beta oxidation pathway, including disorders of fatty acid uptake and transport (such as carnitine transport defects and malonyl-CoA decarboxylase deficiency (malonic aciduria)). Additional information. Characteristic is hypoglycaemia with low ketone levels due to an inability to convert fatty acids into ketones (hypoketotic hypoglycaemia), in association with low insulin levels.