2026 ICD-10-CM Diagnosis Code E88.49Other mitochondrial metabolism disorders

ICD-10-CM Codes›E00–E89›E70-E88›E88

ICD-10-CM E88.49
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E88.49 is a billable ICD-10-CM diagnosis code for other mitochondrial metabolism disorders. 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
E88.49
Billable Status
Yes — Valid for Submission
Code Describes
Other mitochondrial metabolism disorders
Short Description
Other mitochondrial metabolism disorders
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 CodeE88.49Other mitochondrial metabolism disorders

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acute hepatic failure
  • Acute infantile liver failure due to synthesis defect of mitochondrial deoxyribonucleic acid encoded protein
  • ATAD3A mitochondrial disease
  • Coenzyme Q10 deficiency
  • Combined complex deficiencies
  • Combined oxidative phosphorylation defect type 11
  • Combined oxidative phosphorylation defect type 13
  • Combined oxidative phosphorylation defect type 14
  • Combined oxidative phosphorylation defect type 15
  • Combined oxidative phosphorylation defect type 17
  • Combined oxidative phosphorylation defect type 2
  • Combined oxidative phosphorylation defect type 21
  • Combined oxidative phosphorylation defect type 23
  • Combined oxidative phosphorylation defect type 24
  • Combined oxidative phosphorylation defect type 25
  • Combined oxidative phosphorylation defect type 26
  • Combined oxidative phosphorylation defect type 27
  • Combined oxidative phosphorylation defect type 28
  • Combined oxidative phosphorylation defect type 29
  • Combined oxidative phosphorylation defect type 30
  • Combined oxidative phosphorylation defect type 4
  • Combined oxidative phosphorylation defect type 5
  • Combined oxidative phosphorylation defect type 7
  • Combined oxidative phosphorylation defect type 8
  • Combined oxidative phosphorylation defect type 9
  • Combined oxidative phosphorylation deficiency type 20
  • COQ2-gene related coenzyme Q10 deficiency
  • Cytochrome-c oxidase deficiency
  • Deficiency in enzyme complexes of mitochondrial respiratory chain
  • Deficiency of cytochrome-b>5< reductase
  • Deficiency of mitochondrial aspartyl-tRNA synthetase
  • Deficiency of mitochondrial complex III
  • Deficiency of NADH dehydrogenase
  • Deficiency of NADPH-ferrihemoprotein reductase
  • Deficiency of NAPH cytochrome-c>2< reductase
  • Deletion and duplication of mitochondrial DNA
  • Depletion of mitochondrial DNA
  • Developmental regression
  • Disorder of mitochondrial respiratory chain complexes
  • Disorder of pyruvate metabolism and mitochondrial respiratory chain
  • Encephalopathy due to mitochondrial and peroxisomal fission defect
  • Fatal infantile cytochrome C oxidase deficiency
  • Fatal mitochondrial disease due to combined oxidative phosphorylation deficiency 3
  • Generalized dystonia
  • Hepatoencephalopathy due to combined oxidative phosphorylation defect type 1
  • Hereditary cerebellar atrophy
  • Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial DNA mutation
  • Hypertrophic mitochondrial cardiomyopathy
  • Isolated ATP synthase deficiency
  • Isolated cytochrome C oxidase deficiency
  • Leigh syndrome due to cytochrome C oxidase deficiency
  • Leigh's disease
  • Leukoencephalopathy with brain stem and spinal cord involvement and high lactate syndrome
  • Leukoencephalopathy, thalamus and brainstem anomalies, high lactate syndrome
  • Lipoic acid synthetase deficiency
  • Lipoyl transferase 1 deficiency
  • Luft's hypermetabolic myopathy
  • MEPAN syndrome
  • Mitochondrial complex I deficiency nuclear type 10
  • Mitochondrial DNA depletion syndrome hepatocerebrorenal form
  • Mitochondrial DNA depletion syndrome, hepatocerebral form due to DGUOK deficiency
  • Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to MTO1 deficiency
  • Mitochondrial metabolism defect
  • Mitochondrial myopathy with reversible cytochrome C oxidase deficiency
  • Mitochondrial phosphate carrier deficiency
  • Mitochondrial respiratory chain complex I assembly gene defect
  • Mitochondrial respiratory chain complex I structural subunit gene defect
  • Mitochondrial respiratory chain complex II assembly gene defect
  • Mitochondrial respiratory chain complex II structural subunit gene defect
  • Mitochondrial respiratory chain complex III assembly gene defect
  • Mitochondrial respiratory chain complex III structural subunit gene defect
  • Mitochondrial respiratory chain complex IV assembly gene defect
  • Mitochondrial respiratory chain complex IV structural subunit gene defect
  • Mitochondrially encoded ATP synthase membrane subunit 6-related mitochondrial disease
  • Multiple mitochondrial dysfunctions syndrome
  • Multiple mitochondrial dysfunctions syndrome type 1
  • Multiple mitochondrial dysfunctions syndrome type 2
  • Multiple mitochondrial dysfunctions syndrome type 3
  • Multiple mitochondrial dysfunctions syndrome type 4
  • Multiple mitochondrial dysfunctions syndrome type 5
  • Multiple mitochondrial dysfunctions syndrome type 6
  • Nicotinamide adenine dinucleotide coenzyme Q reductase deficiency
  • Pearson's syndrome
  • QRSL1-related combined oxidative phosphorylation defect
  • Succinate-coenzyme Q reductase deficiency
  • Syndromic sensorineural deafness due to combined oxidative phosphorylation defect
  • Thymidine kinase 2 deficiency
  • Ubiquinone dehydrogenase deficiency
  • WARS2-related combined oxidative phosphorylation defect

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
          • other specified
    • MNGIE(Mitochondrial Neurogastrointestinal Encephalopathy) syndrome
    • NARP(Neuropathy, Ataxia and Retinitis pigmentosa) syndrome
    • Syndrome
      • Fukuhara
    • Syndrome
      • MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)
    • Syndrome
      • NARP (Neuropathy, Ataxia and Retinitis pigmentosa)

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

  • Coenzyme Q10 Deficiency

    a genetically heterogeneous condition, typically inherited in an autosomal recessive fashion, characterized by coenzyme q10 deficiency.

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 E88.49 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 E88.49Overview

Is E88.49 (Mitochondrial metabolism disorders) a billable code?

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

What is the ICD-9 equivalent of E88.49?

Under the General Equivalence Mappings, other mitochondrial metabolism disorders 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.