Very long chain acyl-CoA dehydrogenase deficiency - CISMeF
Very long chain acyl-CoA dehydrogenase deficiencyICD-11 More detail
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.;
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.