ICD-10-CM category
E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
E71 is a category, not a billable code. Report one of the 38 complete codes below.
Codes in E71
- E71.0Maple-syrup-urine disease
- E71.110Isovaleric acidemia
- E71.1113-methylglutaconic aciduria
- E71.118Other branched-chain organic acidurias
- E71.120Methylmalonic acidemia
- E71.121Propionic acidemia
- E71.128Other disorders of propionate metabolism
- E71.19Other disorders of branched-chain amino-acid metabolism
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.30Disorder of fatty-acid metabolism, unspecified
- E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
- E71.311Medium chain acyl CoA dehydrogenase deficiency
- E71.312Short chain acyl CoA dehydrogenase deficiency
- E71.313Glutaric aciduria type II
- E71.314Muscle carnitine palmitoyltransferase deficiency
- E71.318Other disorders of fatty-acid oxidation
- E71.32Disorders of ketone metabolism
- E71.39Other disorders of fatty-acid metabolism
- E71.40Disorder of carnitine metabolism, unspecified
- E71.41Primary carnitine deficiency
- E71.42Carnitine deficiency due to inborn errors of metabolism
- E71.43Iatrogenic carnitine deficiency
- E71.440Ruvalcaba-Myhre-Smith syndrome
- E71.448Other secondary carnitine deficiency
- E71.50Peroxisomal disorder, unspecified
- E71.510Zellweger syndrome
- E71.511Neonatal adrenoleukodystrophy
- E71.518Other disorders of peroxisome biogenesis
- E71.520Childhood cerebral X-linked adrenoleukodystrophy
- E71.521Adolescent X-linked adrenoleukodystrophy
- E71.522Adrenomyeloneuropathy
- E71.528Other X-linked adrenoleukodystrophy
- E71.529X-linked adrenoleukodystrophy, unspecified type
- E71.53Other group 2 peroxisomal disorders
- E71.540Rhizomelic chondrodysplasia punctata
- E71.541Zellweger-like syndrome
- E71.542Other group 3 peroxisomal disorders
- E71.548Other peroxisomal disorders
Outside the US? E71 in the international WHO ICD-10 lists its inclusion and exclusion notes.
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ICD-10-CM is developed by the National Center for Health Statistics (NCHS), Centers for Disease Control and Prevention, U.S. Department of Health and Human Services, and is based on the WHO ICD-10. Codes and titles are reproduced unchanged from the FY2026 code list (effective 1 October 2025). Their use here does not imply endorsement by CDC, HHS or the U.S. Government. Changes effective on or after 1 October 2026 may not be reflected yet; check the current release at cdc.gov or cms.gov before submitting claims.
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