2026 ICD-10-CM Diagnosis Code E71.39Other disorders of fatty-acid metabolism

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71.39
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E71.39 is a billable ICD-10-CM diagnosis code for other disorders of fatty-acid metabolism. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E71.39
Billable Status
Yes — Valid for Submission
Code Describes
Other disorders of fatty-acid metabolism
Short Description
Other disorders of fatty-acid metabolism
Same as the full description in the CMS dataset.
Parent Code
E71.3
Disorders of fatty-acid metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71.39Other disorders of fatty-acid metabolism

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 3-hydroxy-3-methylglutaryl-coenzyme A synthase deficiency
  • Acetyl-CoA carboxylase deficiency
  • Carnitine acylcarnitine translocase deficiency
  • Deficiency of 2,4-dienoyl-CoA reductase
  • Deficiency of 2-hydroxyglutarate dehydrogenase
  • Deficiency of 3-hydroxybutyryl-CoA epimerase
  • Deficiency of 3-hydroxyisobutyryl CoA hydrolase
  • Deficiency of acetate-CoA ligase
  • Deficiency of acetoacetyl-CoA reductase
  • Deficiency of acetyl-CoA acylase
  • Deficiency of acetyl-CoA deacylase
  • Deficiency of acetyl-CoA hydrolase
  • Deficiency of carnitine acetyltransferase
  • Deficiency of coenzyme-A transferase
  • Deficiency of dehydrogenase
  • Deficiency of glutarate-CoA ligase
  • Deficiency of long-chain-fatty-acid-CoA ligase
  • Deficiency of malonyl-CoA decarboxylase
  • Deficiency of palmitoyl-CoA hydrolase
  • Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
  • Long-chain fatty acid transport deficiency
  • Malonic aciduria

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Disorder(of)
      • fatty acid
        • metabolism
          • specified NEC

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Lipid Metabolism Disorders

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E71.39 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.89 Metabolism disorder NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E71.39Overview

Is E71.39 (Disorders of fatty-acid metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other disorders of fatty-acid metabolism on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E71.39?

Under the General Equivalence Mappings, other disorders of fatty-acid metabolism converts to ICD-9-CM 277.89 (metabolism disorder NEC). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.