E71
Non-Billable

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

Non-Billable / Non-Specific Code

What is Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism?

ICD-10-CM E71 is the diagnosis code for Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. This code falls under the section "Metabolic disorders" within Chapter 4 — Endocrine, nutritional and metabolic diseases (E00-E89). It is a non-billable/non-specific ICD-10-CM code that should not be used for reimbursement. A more specific sub-code is required for billing. Medical coders and healthcare providers use this code to document and classify diagnoses in electronic health records, insurance claims, and clinical databases.

Coding Guidelines for E71

Always refer to the official ICD-10-CM Tabular List for complete coding guidelines. Ensure documentation supports the specificity of the code selected. When in doubt, consult a certified medical coder or the latest CMS guidelines.

ICD-10-CM Code Hierarchy

Understanding where E71 sits in the ICD-10-CM classification helps ensure proper coding:

Current CodeE71 — Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Sub-Codes 6 entries
E71.0Maple-syrup-urine diseaseBillableE71.1Other disorders of branched-chain amino-acid metabolismNon-BillableE71.11Branched-chain organic aciduriasNon-BillableE71.110Isovaleric acidemiaBillableE71.1113-methylglutaconic aciduriaBillableE71.118Other branched-chain organic aciduriasBillableE71.12Disorders of propionate metabolismNon-BillableE71.120Methylmalonic acidemiaBillableE71.121Propionic acidemiaBillableE71.128Other disorders of propionate metabolismBillableE71.19Other disorders of branched-chain amino-acid metabolismBillableE71.2Disorder of branched-chain amino-acid metabolism, unspecifiedBillableE71.3Disorders of fatty-acid metabolismNon-BillableE71.30Disorder of fatty-acid metabolism, unspecifiedBillableE71.31Disorders of fatty-acid oxidationNon-BillableE71.310Long chain/very long chain acyl CoA dehydrogenase deficiencyBillableE71.311Medium chain acyl CoA dehydrogenase deficiencyBillableE71.312Short chain acyl CoA dehydrogenase deficiencyBillableE71.313Glutaric aciduria type IIBillableE71.314Muscle carnitine palmitoyltransferase deficiencyBillableE71.318Other disorders of fatty-acid oxidationBillableE71.32Disorders of ketone metabolismBillableE71.39Other disorders of fatty-acid metabolismBillableE71.4Disorders of carnitine metabolismNon-BillableE71.40Disorder of carnitine metabolism, unspecifiedBillableE71.41Primary carnitine deficiencyBillableE71.42Carnitine deficiency due to inborn errors of metabolismBillableE71.43Iatrogenic carnitine deficiencyBillableE71.44Other secondary carnitine deficiencyNon-BillableE71.440Ruvalcaba-Myhre-Smith syndromeBillableE71.448Other secondary carnitine deficiencyBillableE71.5Peroxisomal disordersNon-BillableE71.50Peroxisomal disorder, unspecifiedBillableE71.51Disorders of peroxisome biogenesisNon-BillableE71.510Zellweger syndromeBillableE71.511Neonatal adrenoleukodystrophyBillableE71.518Other disorders of peroxisome biogenesisBillableE71.52X-linked adrenoleukodystrophyNon-BillableE71.520Childhood cerebral X-linked adrenoleukodystrophyBillableE71.521Adolescent X-linked adrenoleukodystrophyBillableE71.522AdrenomyeloneuropathyBillableE71.528Other X-linked adrenoleukodystrophyBillableE71.529X-linked adrenoleukodystrophy, unspecified typeBillableE71.53Other group 2 peroxisomal disordersBillableE71.54Other peroxisomal disordersNon-BillableE71.540Rhizomelic chondrodysplasia punctataBillableE71.541Zellweger-like syndromeBillableE71.542Other group 3 peroxisomal disordersBillableE71.548Other peroxisomal disordersBillable
Related Codes

Frequently Asked Questions

Is E71 a billable ICD-10-CM code?

No, E71 is a non-billable/non-specific code. You should use a more specific sub-code for billing and reimbursement.

What does E71 mean?

E71 is the ICD-10-CM diagnosis code for "Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism". It is used by healthcare providers to classify and document this condition in medical records and insurance claims.

What section is E71 in?

E71 is located in Section E70-E88 — "Metabolic disorders" within Chapter 4 of the ICD-10-CM Tabular List.

What are the sub-codes of E71?

E71 has 6 sub-code(s) that provide more specific detail: E71.0, E71.1, E71.2, E71.3, E71.4 and more.

When should I use E71?

Use E71 when the patients documented diagnosis matches "Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism" and the clinical documentation supports this level of specificity. Always verify with the latest ICD-10-CM guidelines and payer requirements.

People Also Ask

What is the ICD-10 code for Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism?

The ICD-10-CM code for Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism is E71.

Can E71 be used for primary diagnosis?

No, E71 is non-billable. Use a more specific sub-code for primary diagnosis billing.

What chapter is E71 in?

E71 is in Chapter 4 of the ICD-10-CM Tabular List.

Is E71 valid for 2026?

Yes, E71 is a valid ICD-10-CM code for the 2026 fiscal year, subject to official CMS updates.

See Also

← PreviousE70.9Disorder of aromatic amino-acid metabolism, unspecifiedNext →E71.0Maple-syrup-urine disease
Code Details
E71
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Non-Billable / Non-Specific Code
4 — Endocrine, nutritional and metabolic diseases (E00-E89)
E70-E88 — Metabolic disorders
2026 ICD-10-CM