Catalog

Very long chain acyl-CoA dehydrogenase deficiency

No MMS code

Link to WHO Definition

Very long chain acyl-CoA dehydrogenase deficiency – http://id.who.int/icd/entity/907810567

Acyl-CoA dehydrogenase very long chain (VLCAD) deficiency is an inborn error of mitochondrial long-chain fatty acid oxidation with variable presentation including a severe form with recurrent episodes of hypoketotic hypoglycaemia, often associated with hypertrophic cardiomyopathy with pericardial effusion, or arrythmia, which can lead to a cardiorespiratory arrest in neonates or infants.