E71.4Disorders of carnitine metabolism

E71.4 is the ICD-10-CM diagnosis code for disorders of carnitine 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 5 subcodes must be used instead.

Within E71 (disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), E71.4 is specifically for disorders of carnitine 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 fatty-acid metabolism (E71.3) and peroxisomal disorders (E71.5).

To report this condition, pick the subcode that matches the documentation: E71.40 (unspecified), E71.41 (deficiency), E71.42 (metabolism), E71.43 (deficiency) and E71.44 (deficiency).

Don't report E71.4 together with the conditions in its Excludes1 note: Muscle carnitine palmitoyltransferase deficiency (E71.314).

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