" /> Very long chain acyl-CoA dehydrogenase deficiency - CISMeF





Preferred Label : Very long chain acyl-CoA dehydrogenase deficiency;

ICD-11 definition : 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 hypoglycemia, often associated with hypertrophic cardiomyopathy with pericardial effusion, or arrythmia, which can lead to a cardiorespiratory arrest in neonates or infants.;

ICD-11 synonym : VLCAD - [Very long chain acyl-CoA dehydrogenase] deficiency; VLCAD deficiency;

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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 hypoglycemia, often associated with hypertrophic cardiomyopathy with pericardial effusion, or arrythmia, which can lead to a cardiorespiratory arrest in neonates or infants.

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29/07/2025


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