E71.3Disorders of fatty-acid metabolism

E71.3 is the ICD-10-CM diagnosis code for disorders of fatty-acid metabolism. It belongs to category E71 (disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is a header code, so it can't be reported by itself; one of its 4 subcodes must be used instead.

Within E71 (disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), E71.3 is specifically for disorders of fatty-acid metabolism. Related codes cover Maple-syrup-urine disease (E71.0), other disorders of branched-chain amino-acid metabolism (E71.1), disorder of branched-chain amino-acid metabolism, unspecified (E71.2), disorders of carnitine metabolism (E71.4) and peroxisomal disorders (E71.5).

To report this condition, pick the subcode that matches the documentation: E71.30 (unspecified), E71.31 (oxidation), E71.32 (metabolism) and E71.39 (metabolism).

Don't report E71.3 together with the conditions in its Excludes1 note: peroxisomal disorders (E71.5), Refsum's disease (G60.1) and Schilder's disease (G37.0).

The conditions in its Excludes2 note aren't part of E71.3, but may be coded alongside it if the patient has both: carnitine deficiency due to inborn error of metabolism (E71.42).

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.