E71
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
E71 is a non-billable ICD-10-CM code category for disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. Report a more specific code from it instead. It is part of the FY2027 code set, effective October 1, 2026. The more specific codes under it are E71.0 Maple-syrup-urine disease; E71.1 Other disorders of branched-chain amino-acid metabolism; E71.2 Disorder of branched-chain amino-acid metabolism, unspecified; E71.3 Disorders of fatty-acid metabolism and 2 more. Its coding notes include 6 Excludes1 entries and 1 Excludes2 entry.
Excludes1
Not coded here. The listed conditions generally should not be reported together.
Also from chapter/block level (6 notes)
Excludes2
Not included here. Both codes may be reported if the patient has both conditions.
Notes
Also from chapter/block level (1 note)
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.