U.S. ICD-10-CM reference · FY2026 · April
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E71Category · further specificity requiredArchived

Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

HierarchyE70-E88 · E71

Code-level instructions

No additional notes.

Inherited instructions

Chapters 4 · Endocrine, nutritional and metabolic diseases (E00-E89)

Excludes 1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)

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.

E70-E88 · Metabolic disorders (E70-E88)

Excludes 1
  • 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)
Excludes 2

Ehlers-Danlos syndromes (Q79.6-)

More specific codes

E71.0Maple-syrup-urine diseaseBillable / specificE71.1Other disorders of branched-chain amino-acid metabolismCategory · further specificity requiredE71.2Disorder of branched-chain amino-acid metabolism, unspecifiedBillable / specificE71.3Disorders of fatty-acid metabolismCategory · further specificity requiredE71.4Disorders of carnitine metabolismCategory · further specificity requiredE71.5Peroxisomal disordersCategory · further specificity required
Code edition