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ICD-10-CM · FY 2026 APRIL UPDATE

E71

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

Diagnosis reference

Nonbillable category

This entry is a category or subcategory. Choose the appropriate billable descendant based on the clinical documentation.

Effective periodOctober 1, 2026–September 30, 2027
SourceCMS ICD-10-CM order file
ClassificationDiagnosis · clinical modification

Related entries

Up to 30 descendants in this category

CODEDESCRIPTIONTYPE
E71.0Maple-syrup-urine diseaseBillable
E71.1Other disorders of branched-chain amino-acid metabolismCategory
E71.11Branched-chain organic aciduriasCategory
E71.110Isovaleric acidemiaBillable
E71.1113-methylglutaconic aciduriaBillable
E71.118Other branched-chain organic aciduriasBillable
E71.12Disorders of propionate metabolismCategory
E71.120Methylmalonic acidemiaBillable
E71.121Propionic acidemiaBillable
E71.128Other disorders of propionate metabolismBillable
E71.19Other disorders of branched-chain amino-acid metabolismBillable
E71.2Disorder of branched-chain amino-acid metabolism, unspecifiedBillable
E71.3Disorders of fatty-acid metabolismCategory
E71.30Disorder of fatty-acid metabolism, unspecifiedBillable
E71.31Disorders of fatty-acid oxidationCategory
E71.310Long chain/very long chain acyl CoA dehydrogenase deficiencyBillable
E71.311Medium chain acyl CoA dehydrogenase deficiencyBillable
E71.312Short chain acyl CoA dehydrogenase deficiencyBillable
E71.313Glutaric aciduria type IIBillable
E71.314Muscle carnitine palmitoyltransferase deficiencyBillable
E71.318Other disorders of fatty-acid oxidationBillable
E71.32Disorders of ketone metabolismBillable
E71.39Other disorders of fatty-acid metabolismBillable
E71.4Disorders of carnitine metabolismCategory
E71.40Disorder of carnitine metabolism, unspecifiedBillable
E71.41Primary carnitine deficiencyBillable
E71.42Carnitine deficiency due to inborn errors of metabolismBillable
E71.43Iatrogenic carnitine deficiencyBillable
E71.44Other secondary carnitine deficiencyCategory
E71.440Ruvalcaba-Myhre-Smith syndromeBillable

Use the complete reference

This reference does not reproduce the full tabular hierarchy, inclusion notes, Excludes1/2 notes, sequencing instructions, or seventh-character rules. Review the official tables and coding guidelines.

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ICD-10-CM codes describe diagnoses. They do not have an independent Medicare PFS reimbursement amount, and billability alone does not establish coverage.