Alphabetic Index FY2027

E71.31

Disorders of fatty-acid oxidation

FY2027Chapter 4: Endocrine, nutritional and metabolic diseases/E70–E88 Metabolic disorders

Non-billable headerNot valid on its own. Use one of the more specific codes under it.

E71.31 is a non-billable ICD-10-CM code header for disorders of fatty-acid oxidation. 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 E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency, E71.311 Medium chain acyl CoA dehydrogenase deficiency, E71.312 Short chain acyl CoA dehydrogenase deficiency, E71.313 Glutaric aciduria type II and 2 more. Its coding notes include 9 Excludes1 entries and 2 Excludes2 entries.

Excludes1

Not coded here. The listed conditions generally should not be reported together.

Inherited from E71.3 Disorders of fatty-acid metabolism
  • peroxisomal disorders (E71.5)
  • Refsum's disease (G60.1)
  • Schilder's disease (G37.0)
Also from chapter/block level (6 notes)
Inherited from E70–E88 Metabolic disorders
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)
Inherited from Chapter 4 Endocrine, nutritional and metabolic diseases
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes2

Not included here. Both codes may be reported if the patient has both conditions.

Inherited from E71.3 Disorders of fatty-acid metabolism
  • carnitine deficiency due to inborn error of metabolism (E71.42)
Also from chapter/block level (1 note)
Inherited from E70–E88 Metabolic disorders
  • Ehlers-Danlos syndromes (Q79.6-)

Notes

Also from chapter/block level (1 note)
Inherited from Chapter 4 Endocrine, nutritional and metabolic diseases
  • 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.

More specific codes under E71.31 6