E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

⚠ Non-Billable ICD-10-CM 2026
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The ICD-10-CM code for Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism is E71 (FY2026). It is a non-billable category code — select a more specific code below for claims.

Classification

Section
E70-E88: Metabolic disorders (E70-E88)
Category E71
50 codes (38 billable)
FY2026 Status
Stable since FY2024

Official Coding Guidelines

Diabetes mellitus (TOC entry)

a. Diabetes mellitus ................................................................................................................... 39

— ICD-10-CM Official Guidelines for Coding and Reporting, FY2026, Section I.C.4.a
Obesity (TOC entry)

b. Obesity .................................................................................................................................. 42

— ICD-10-CM Official Guidelines for Coding and Reporting, FY2026, Section I.C.4.b

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

Cite This Page

Reference E71 with the canonical source and edition:

E71 — Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. ICD-10 Coding Pro (ICD-10-CM FY2026). https://icd10codingpro.com/codes/E71

Canonical URL: https://icd10codingpro.com/codes/E71

Related Codes

More Specific Codes

E71.0 Maple-syrup-urine disease E71.1 Other disorders of branched-chain amino-acid metabolism E71.2 Disorder of branched-chain amino-acid metabolism, unspecified E71.3 Disorders of fatty-acid metabolism E71.4 Disorders of carnitine metabolism E71.5 Peroxisomal disorders

All Codes in E71

Every most-specific code under E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism (38 codes):

Show the complete E71 code list
E71.0 Maple-syrup-urine disease E71.110 Isovaleric acidemia E71.111 3-methylglutaconic aciduria E71.118 Other branched-chain organic acidurias E71.120 Methylmalonic acidemia E71.121 Propionic acidemia E71.128 Other disorders of propionate metabolism E71.19 Other disorders of branched-chain amino-acid metabolism E71.2 Disorder of branched-chain amino-acid metabolism, unspecified E71.30 Disorder of fatty-acid metabolism, unspecified 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 E71.314 Muscle carnitine palmitoyltransferase deficiency E71.318 Other disorders of fatty-acid oxidation E71.32 Disorders of ketone metabolism E71.39 Other disorders of fatty-acid metabolism E71.40 Disorder of carnitine metabolism, unspecified E71.41 Primary carnitine deficiency E71.42 Carnitine deficiency due to inborn errors of metabolism E71.43 Iatrogenic carnitine deficiency E71.440 Ruvalcaba-Myhre-Smith syndrome E71.448 Other secondary carnitine deficiency E71.50 Peroxisomal disorder, unspecified E71.510 Zellweger syndrome E71.511 Neonatal adrenoleukodystrophy E71.518 Other disorders of peroxisome biogenesis E71.520 Childhood cerebral X-linked adrenoleukodystrophy E71.521 Adolescent X-linked adrenoleukodystrophy E71.522 Adrenomyeloneuropathy E71.528 Other X-linked adrenoleukodystrophy E71.529 X-linked adrenoleukodystrophy, unspecified type E71.53 Other group 2 peroxisomal disorders E71.540 Rhizomelic chondrodysplasia punctata E71.541 Zellweger-like syndrome E71.542 Other group 3 peroxisomal disorders E71.548 Other peroxisomal disorders
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Reviewed by Prajwal Shrestha, CPC, CRC
Certified Professional Coder (CPC) and Certified Risk Adjustment Coder (CRC) · AAPC Member ID 01997614 · About · Editorial policy · Content last reviewed: 2025-10-01

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