2026 ICD-10-CM Diagnosis Code E88.40Mitochondrial metabolism disorder, unspecified

ICD-10-CM Codes›E00–E89›E70-E88›E88

ICD-10-CM E88.40
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E88.40 is a billable ICD-10-CM diagnosis code for mitochondrial metabolism disorder, unspecified. 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
E88.40
Billable Status
Yes — Valid for Submission
Code Describes
Mitochondrial metabolism disorder, unspecified
Short Description
Mitochondrial metabolism disorder, unspecified
Same as the full description in the CMS dataset.
Parent Code
E88.4
Mitochondrial metabolism disorders

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE88Other and unspecified metabolic disorders
This CodeE88.40Mitochondrial metabolism disorder, unspecified

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Chorea due to metabolic disorder
  • Chorea due to mitochondrial cytopathy
  • Demyelination of central nervous system co-occurrent and due to mitochondrial disease
  • Dilated cardiomyopathy due to mitochondrial disease
  • Dystonia due to mitochondrial disease
  • Glomerular disease due to mitochondrial cytopathy
  • Hypertrophic mitochondrial cardiomyopathy
  • Infantile hypertrophic cardiomyopathy due to MRPL44 deficiency
  • Liver disease co-occurrent and due to mitochondrial disorder
  • Mitochondrial cytopathy
  • Mitochondrial metabolism defect
  • Myoclonic disorder due to mitochondrial disorder
  • Non-syndromic mitochondrial sensorineural deafness

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)
      • metabolism NOS
        • mitochondrial
    • Disorder(of)
      • mitochondrial metabolism

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

Mitochondrial Diseases

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 E88.40 to ICD-9-CMHistory

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

ICD-9-CM
277.87 Dis mitochondrial metab
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 E88.40Overview

Is E88.40 (Mitochondrial metabolism disorders) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report mitochondrial metabolism disorder, unspecified on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E88.40?

Under the General Equivalence Mappings, mitochondrial metabolism disorder, unspecified converts to ICD-9-CM 277.87 (dis mitochondrial metab). 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.