'Department of Health and Human services, Victoria, Australia'

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Medium chain acyl-coa dehydrogenase deficiency (MCADD)

Publication Date: June 2020

Implementation Date: 1/07/2020

ICD 10 AM Edition: Eleventh Edition

Query Number: 3602

What is the appropriate code for Medium chain acyl-coa dehydrogenase deficiency (MCADD)?

MCADD is a rare genetic condition where a person has problems breaking down fat to use as an energy source. This means that someone with MCADD can become very ill if their body's energy demands exceed their energy intake, such as during infections or vomiting illnesses when they're unable to eat.

The best fit code I can find is:
-enzyme, circulating NEC E88.0
-fatty acid metabolism E71.3

E71.3 incorporates very long chain acyl-coa dehydrogenase deficiency (VLCAD) which I believe would be very similar to the MCADD.


VICC obtained clinical advice that indicates there are different types of disorders of fatty acid metabolism, such as Long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) deficiency; Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency; Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency; Short-chain acyl-CoA dehydrogenase (SCAD) deficiency.

As MCADD is a type of fatty acid metabolism disorder, VICC advises to follow the Index lead term Disorder/fatty acid metabolism to assign E71.3 Disorders of fatty-acid metabolism.