2026 ICD-10-CM Diagnosis Code E71.314Muscle carnitine palmitoyltransferase deficiency

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71.314
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E71.314 is a billable ICD-10-CM diagnosis code for muscle carnitine palmitoyltransferase deficiency. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as amino acid deficiency. 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
E71.314
Billable Status
Yes — Valid for Submission
Code Describes
Muscle carnitine palmitoyltransferase deficiency
Short Description
Muscle carnitine palmitoyltransferase deficiency
Same as the full description in the CMS dataset.
Parent Code
E71.31
Disorders of fatty-acid oxidation

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71.314Muscle carnitine palmitoyltransferase deficiency

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Amino acid deficiency
  • Carnitine deficiency
  • Carnitine palmitoyltransferase deficiency
  • Carnitine palmitoyltransferase I deficiency
  • Carnitine palmitoyltransferase II deficiency
  • Muscle carnitine deficiency
  • Myopathic form of carnitine palmitoyltransferase II deficiency

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.

    • Deficiency, deficient
      • carnitine
        • muscle palmityltransferase
    • Deficiency, deficient
      • muscle
        • carnitine (palmityltransferase)
    • Muscle, muscular
      • carnitine (palmityltransferase) deficiency

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

Clinical InformationClinical

  • Carnitine Palmitoyltransferase I Deficiency

    a rare autosomal recessive inherited disorder caused by mutations in the cpt1a gene. it is characterized by the presence of defective carnitine palmitoyltransferase 1a which is involved in fatty acid oxidation. signs and symptoms may be exacerbated during fasting and include hypoketotic hypoglycemia, increased levels of carnitine in the blood, hepatomegaly, seizures, and coma.

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 E71.314 to ICD-9-CMHistory

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

ICD-9-CM
277.85 Disorders acid oxidation
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 E71.314Overview

Is E71.314 (Disorders of fatty-acid oxidation) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report muscle carnitine palmitoyltransferase deficiency on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E71.314?

Under the General Equivalence Mappings, muscle carnitine palmitoyltransferase deficiency converts to ICD-9-CM 277.85 (disorders acid oxidation). 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.