AutoICD API

E88.49

Billable

Other mitochondrial metabolism disorders

Other mitochondrial metabolism disorders

Status

Billable / Specific

Block

E70-E88

Parent Code

E88.4

Coding Notes

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Use Additional Code

Additional codes that should follow this code

  • codes for associated conditions
Related Codes(4)
E88.40

Mitochondrial metabolism disorder, unspecified

BillableE88.41

MELAS syndrome

BillableE88.42

MERRF syndrome

BillableE88.43

Disorders of mitochondrial tRNA synthetases

Billable
Also Known As / Clinical Terms(421)

SNOMED CT

UMLS

Clinical Terms

  • Deficiency of mitochondrial aspartyl-tRNA synthetase
  • Mitochondrial phosphate carrier deficiency
  • Mitochondrial complex I deficiency nuclear type 10
  • COXPD15 - combined oxidative phosphorylation defect type 15
  • Multiple mitochondrial dysfunctions syndrome type 5
  • Deficiency of ferrihaemoprotein P450 reductase
  • PMPCB (peptidase mitochondrial processing subunit beta) deficiency
  • COXPD2 - combined oxidative phosphorylation defect type 2
  • COXPD14 - combined oxidative phosphorylation defect type 14
  • COXPD29 - combined oxidative phosphorylation defect type 29
  • Deficiency of NADH dehydrogenase (ubiquinon)
  • Mitochondrial deoxyribonucleic acid depletion syndrome, hepatocerebral form due to deoxyguanosine kinase deficiency
  • IBA57 (iron-sulfur cluster assembly factor IBA57) deficiency
  • DYT29 - dystonia 29
  • Mitochondrial enoyl coenzyme A reductase protein-associated neurodegeneration syndrome
  • COXPD10 - combined oxidative phosphorylation defect type 10
  • Complex III deficiency
  • Deletion and duplication of mitochondrial DNA
  • Coenzyme Q10 deficiency
  • COXPD12 - combined oxidative phosphorylation defect type 12
  • Isolated mitochondrial respiratory chain complex V deficiency
  • Mitochondrial complex I deficiency
  • COXPD13 - combined oxidative phosphorylation defect type 13
  • Mitochondrial respiratory chain complex II assembly gene defect
  • Benign COX (cytochrome C oxidase) deficiency
  • Hepatoencephalopathy due to COXPD1 (combined oxidative phosphorylation defect type 1)
  • Acute infantile liver failure due to synthesis defect of mitochondrial deoxyribonucleic acid encoded protein
  • COX - Cytochrome C oxidase deficiency
  • Disorder of mitochondrial respiratory chain complexes
  • Mitochondrial myopathy with reversible cytochrome C oxidase deficiency
  • Isolated ATP synthase deficiency
  • Pearson syndrome
  • Combined oxidative phosphorylation defect type 9
  • Luft hypermetabolic myopathy
  • MMDS5 - multiple mitochondrial dysfunctions syndrome type 5
  • COXPD7 - combined oxidative phosphorylation defect type 7
  • Leigh disease
  • Mitochondrial respiratory chain complex IV structural subunit gene defect
  • COXPD23 - combined oxidative phosphorylation defect type 23
  • ATPase family, AAA Domain containing 3 mitochondrial cytopathy
  • MEPAN syndrome
  • COXPD8 - combined oxidative phosphorylation defect type 8
  • COXPD27 - combined oxidative phosphorylation defect type 27
  • Combined oxidative phosphorylation defect type 28
  • Deficiency of ubiquinone reductase
  • ISCA1 (iron-sulfur cluster assembly 1) deficiency
  • Reversible infantile respiratory chain deficiency
  • Multiple mitochondrial dysfunctions syndrome
  • Mitochondrial complex IV deficiency
  • SNEM - Subacute necrotizing encephalomyelopathy
  • BOLA3 (bolA family member 3) deficiency
  • Mitochondrial disorder, respiratory chain
  • QRSL1-related combined oxidative phosphorylation defect
  • SNEM - Subacute necrotising encephalomyelopathy
  • Succinate-coenzyme Q reductase deficiency
  • MMDS2 - multiple mitochondrial dysfunctions syndrome type 2
  • Hereditary cerebellar atrophy
  • Deficiency in enzyme complexes of mitochondrial respiratory chain
  • COXPD11 - combined oxidative phosphorylation defect type 11
  • Combined oxidative phosphorylation defect type 24
  • Childhood-onset generalized dystonia, optic atrophy syndrome
  • Multiple mitochondrial dysfunctions syndrome type 2
  • ATAD3A mitochondrial cytopathy
  • Complex I deficiency
  • Deficiency of ferrihemoprotein P450 reductase
  • Leigh syndrome
  • Combined oxidative phosphorylation defect type 7
  • Combined oxidative phosphorylation defect type 2
  • Hypertrophic mitochondrial cardiomyopathy
  • Neonatal severe cardiopulmonary failure due to mitochondrial methylation defect
  • Deficiency of NADPH-ferrihemoprotein reductase
  • Leucoencephalopathy with brain stem and spinal cord involvement and high lactate syndrome
  • Luft's hypermetabolic myopathy
  • Mitochondrial respiratory chain complex IV assembly gene defect
  • Combined oxidative phosphorylation defect type 13
  • Acute hepatic failure
  • Nicotinamide adenine dinucleotide coenzyme Q reductase deficiency
  • Fatal infantile cytochrome C oxidase deficiency
  • Mitochondrial respiratory chain complex I structural subunit gene defect
  • Subacute necrotising encephalomyelopathy
  • Combined oxidative phosphorylation defect type 10
  • COXPD20 - combined oxidative phosphorylation deficiency type 20
  • Lipoic acid synthetase deficiency
  • COXPD5 - combined oxidative phosphorylation defect 5
  • Mitochondrial tryptophanyl-tRNA synthetase deficiency
  • Combined oxidative phosphorylation defect type 14
  • Combined oxidative phosphorylation defect type 25
  • Deficiency of ubiquinone cytochrome c oxidoreductase
  • Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial deoxyribonucleic acid mutation
  • Syndromic sensorineural deafness due to COXPD (combined oxidative phosphorylation defect)
  • Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to mitochondrial transfer ribonucleic acid translation optimisation 1 deficiency
  • Hepatoencephalopathy due to combined oxidative phosphorylation defect type 1
  • COXPD25 - combined oxidative phosphorylation defect type 25
  • ATAD3A mitochondrial disease
  • Thymidine kinase 2 deficiency
  • COXPD26 - combined oxidative phosphorylation defect type 26
  • ISCA1 deficiency
  • Autosomal recessive childhood-onset dystonia DYT29 type
  • Infantile necrotising encephalomyelopathy
  • Deficiency of reduced nicotinamide adenine dinucleotide dehydrogenase (ubiquinon)
  • Isolated mitochondrial respiratory chain complex IV deficiency
  • Syndromic sensorineural deafness due to combined oxidative phosphorylation defect
  • Combined complex deficiencies
  • Acute liver failure
  • Mitochondrial respiratory chain complex III assembly gene defect
  • WARS2-related combined oxidative phosphorylation defect
  • Combined oxidative phosphorylation deficiency type 3
  • Isolated cytochrome C oxidase deficiency
  • Multiple mitochondrial dysfunctions syndrome type 6
  • Combined oxidative phosphorylation defect type 29
  • NDUFAF2-related mitochondrial respiratory chain complex I assembly gene defect
  • Mitochondrial respiratory chain complex II structural subunit gene defect
  • Combined oxidative phosphorylation defect type 21
  • Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to MTO1 deficiency
  • Deficiency of NADPH-ferrihaemoprotein reductase
  • Syndromic sensorineural hearing loss due to COXPD (combined oxidative phosphorylation defect)
  • Combined oxidative phosphorylation deficiency type 20
  • Subacute necrotising encephalopathy
  • Deficiency of mitochondrial complex III
  • Combined oxidative phosphorylation defect type 11
  • Lipoyl transferase 1 deficiency
  • ATPase family AAA domain containing 3A mitochondrial disease
  • Combined oxidative phosphorylation defect type 27
  • Deficiency of type I dehydrogenase
  • PMPCB deficiency
  • Depletion of mitochondrial DNA
  • Leigh's disease
  • Complex II deficiency
  • Leigh syndrome due to cytochrome C oxidase deficiency
  • COQ10D1- primary coenzyme Q10 deficiency-1
  • Combined oxidative phosphorylation defect type 5
  • Encephalopathy due to mitochondrial and peroxisomal fission defect
  • COXPD28 - combined oxidative phosphorylation defect type 28
  • Coenzyme Q2, polyprenyltransferase gene related-coenzyme Q10 deficiency
  • Combined oxidative phosphorylation defect type 12
  • Infantile necrotizing encephalomyelopathy
  • Deficiency of isolated CoQ cytochrome c reductase
  • MMDS3 - multiple mitochondrial dysfunctions syndrome type 3
  • Childhood-onset generalised dystonia, optic atrophy syndrome
  • Primary coenzyme Q10 deficiency-1
  • Combined oxidative phosphorylation defect type 17
  • MMDS1 - multiple mitochondrial dysfunctions syndrome type 1
  • Isolated COX (cytochrome C oxidase) deficiency
  • Fatal mitochondrial disease due to combined oxidative phosphorylation deficiency 3
  • MMDS - multiple mitochondrial dysfunctions syndrome
  • MMDS4 - multiple mitochondrial dysfunctions syndrome type 4
  • Depletion of mitochondrial deoxyribonucleic acid
  • Isolated adenosine triphosphate synthase deficiency
  • Complex IV deficiency
  • Pearson's syndrome
  • Mitochondrial respiratory chain complex III structural subunit gene defect
  • Mitochondrial DNA depletion syndrome, hepatocerebral form due to DGUOK deficiency
  • Deletion and duplication of mitochondrial deoxyribonucleic acid
  • Leucoencephalopathy with brain stem and spinal cord involvement and lactate elevation syndrome
  • Multiple mitochondrial dysfunctions syndrome type 1
  • COXPD9 - combined oxidative phosphorylation defect type 9
  • Cytochrome c oxidase deficiency
  • MC1DN10 - mitochondrial complex 1 deficiency nuclear type 10
  • Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to mitochondrial transfer ribonucleic acid translation optimization 1 deficiency
  • COXPD21 - combined oxidative phosphorylation defect type 21
  • Deficiency of reduced nicotinamide adenine dinucleotide phosphate-ferrihemoprotein reductase
  • COXPD4 - combined oxidative phosphorylation defect type 4
  • Deficiency of cytochrome-b>5< reductase
  • Generalised dystonia
  • Mitochondrial myopathy with reversible COX (cytochrome C oxidase) deficiency
  • Mitochondrial respiratory chain complex I assembly gene defect
  • Disorder of pyruvate metabolism and mitochondrial respiratory chain
  • Deficiency of mitochondrial aspartyl transfer ribonucleic acid synthetase
  • Mitochondrial DNA depletion syndrome hepatocerebrorenal form
  • Developmental regression
  • Deficiency of complex I dehydrogenase
  • Leighs disease
  • COQ2-gene related coenzyme Q10 deficiency
  • Infantile reversible cytochrome C oxidase deficiency myopathy
  • Leukoencephalopathy, thalamus and brainstem anomalies, high lactate syndrome
  • Deficiency of NADP-cytochrome reductase
  • MT-ATP6 mitochondrial disease
  • Combined oxidative phosphorylation defect type 8
  • Generalized dystonia
  • Hypotonia with lactic acidemia and hyperammonemia
  • Glutaminyl-transfer ribonucleic acid amidotransferase subunit-related combined oxidative phosphorylation defect
  • NFU1 (NFU1 iron-sulfur cluster scaffold) deficiency
  • Combined oxidative phosphorylation defect type 15
  • Leukoencephalopathy with brain stem and spinal cord involvement and high lactate syndrome
  • COXPD17 - combined oxidative phosphorylation defect type 17
  • Mitochondrial deoxyribonucleic acid depletion syndrome hepatocerebrorenal form
  • Deficiency of NAPH cytochrome-c>2< reductase
  • Mitochondrially encoded ATP synthase membrane subunit 6-related mitochondrial disease
  • Deficiency of reduced nicotinamide adenine dinucleotide phosphate-ferrihaemoprotein reductase
  • Reversible infantile cytochrome C oxidase deficiency
  • Combined oxidative phosphorylation defect type 30
  • COXPD30 - combined oxidative phosphorylation defect type 30
  • Hypotonia with lactic acidaemia and hyperammonaemia
  • Tryptophanyl tRNA synthetase 2, mitochondrial-related combined oxidative phosphorylation defect
  • NADH - Nicotinamide adenine dinucleotide coenzyme Q reductase deficiency
  • COXPD24 - combined oxidative phosphorylation defect type 24
  • Mitochondrial myopathy with reversible complex IV deficiency
  • Mitochondrial metabolism defect
  • Ubiquinone dehydrogenase deficiency
  • Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial DNA mutation
  • Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation syndrome
  • Subacute necrotizing encephalopathy
  • Cytochrome-c oxidase deficiency
  • Multiple mitochondrial dysfunctions syndrome type 3
  • Combined oxidative phosphorylation defect type 4
  • Combined oxidative phosphorylation defect type 23
  • Multiple mitochondrial dysfunctions syndrome type 4
  • Combined oxidative phosphorylation defect type 26
  • Subacute necrotizing encephalomyelopathy
  • ALF - Acute liver failure
Frequently Asked Questions
What is the ICD-10 code for other mitochondrial metabolism disorders?

The ICD-10-CM code for other mitochondrial metabolism disorders is E88.49. The full clinical description is "Other mitochondrial metabolism disorders". E88.49 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code E88.49 mean?

ICD-10-CM code E88.49 represents “Other mitochondrial metabolism disorders”. It is classified under Chapter 4: Endocrine, Nutritional and Metabolic Diseases and is a billable/specific code that can be used on a claim.

Is E88.49 a billable code?

Yes, E88.49 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is E88.49 in?

E88.49 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).

What codes cannot be used with E88.49?

E88.49 has Excludes1 notes indicating codes that cannot be used together with it, including: transitory endocrine and metabolic disorders specific to newborn (P70-P74); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); and 10 more.

Are additional codes required with E88.49?

Yes, when using E88.49, also report: codes for associated conditions.

What SNOMED CT codes does E88.49 map to?

E88.49 maps to 89 SNOMED CT concepts: 197270009, 1363370009, 720461006, 1236805005, 1208486005, and 84 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for E88.49?

E88.49 is linked to 1 UMLS Concept Unique Identifier: C2874313. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does E88.49 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like other mitochondrial metabolism disorders affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of E88.49?

There is no direct ICD-11 mapping available for E88.49 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.