E88.4Mitochondrial metabolism disorders

E88.4 is the ICD-10-CM diagnosis code for mitochondrial metabolism disorders. It belongs to category E88 (other and unspecified metabolic disorders), 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 E88 (other and unspecified metabolic disorders), E88.4 is specifically for mitochondrial metabolism disorders. Related codes cover disorders of plasma-protein metabolism, not elsewhere classified (E88.0), lipodystrophy, not elsewhere classified (E88.1), Lipomatosis, not elsewhere classified (E88.2), tumor lysis syndrome (E88.3), other specified metabolic disorders (E88.8), metabolic disorder, unspecified (E88.9) and wasting disease (syndrome) due to underlying condition (E88.A).

To report this condition, pick the subcode that matches the documentation: E88.40 (unspecified), E88.41 (syndrome), E88.42 (syndrome), E88.43 (synthetases) and E88.49 (disorders).

Don't report E88.4 together with the conditions in its Excludes1 note: disorders of pyruvate metabolism (E74.4), Kearns-Sayre syndrome (H49.81), Leber's disease (H47.22), Leigh's encephalopathy (G31.82) and 2 more.

Use an additional code for: codes for associated conditions.

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