E88.49
BillableOther mitochondrial metabolism disorders
Other mitochondrial metabolism disorders
Coding Notes
Excludes 1
Codes that cannot be used together with this code (mutual exclusion)
- transitory endocrine and metabolic disorders specific to newborn (P70-P74)
- androgen insensitivity syndrome (E34.5-)
- congenital adrenal hyperplasia (E25.0)
- hemolytic anemias attributable to enzyme disorders (D55.-)
- Marfan syndrome (Q87.4-)
- 5-alpha-reductase deficiency (E29.1)
- histiocytosis X (chronic) (C96.6)
- disorders of pyruvate metabolism (E74.4)
- Kearns-Sayre syndrome (H49.81)
- Leber's disease (H47.22)
- Leigh's encephalopathy (G31.82)
- Mitochondrial myopathy, NEC (G71.3)
- Reye's syndrome (G93.7)
Excludes 2
Conditions not included here, but the patient may have both
- Ehlers-Danlos syndromes (Q79.6-)
Use Additional Code
Additional codes that should follow this code
- codes for associated conditions
Also Known As / Clinical Terms
SNOMED CT
- Infantile necrotising encephalomyelopathy29570005
- Infantile necrotizing encephalomyelopathy29570005
- Leigh disease29570005
- Leigh syndrome29570005
- Leigh's disease29570005
- Leighs disease29570005
- SNEM - Subacute necrotising encephalomyelopathy29570005
- SNEM - Subacute necrotizing encephalomyelopathy29570005
- Subacute necrotising encephalomyelopathy29570005
- Subacute necrotising encephalopathy29570005
- Subacute necrotizing encephalomyelopathy29570005
- Subacute necrotizing encephalopathy29570005
- Luft hypermetabolic myopathy62266001
- Luft's hypermetabolic myopathy62266001
- COX - Cytochrome C oxidase deficiency67434000
- Complex IV deficiency67434000
- Cytochrome c oxidase deficiency67434000
- Cytochrome-c oxidase deficiency67434000
- Mitochondrial complex IV deficiency67434000
- Deficiency of cytochrome-b>5< reductase124184009
- Deficiency of NADP-cytochrome reductase124185005
- Deficiency of NADPH-ferrihaemoprotein reductase124185005
- Deficiency of NADPH-ferrihemoprotein reductase124185005
- Deficiency of ferrihaemoprotein P450 reductase124185005
- Deficiency of ferrihemoprotein P450 reductase124185005
- Deficiency of reduced nicotinamide adenine dinucleotide phosphate-ferrihaemoprotein reductase124185005
- Deficiency of reduced nicotinamide adenine dinucleotide phosphate-ferrihemoprotein reductase124185005
- Deficiency of NAPH cytochrome-c>2< reductase124186006
- Deficiency of NADH dehydrogenase (ubiquinon)124189004
- Deficiency of complex I dehydrogenase124189004
- Deficiency of reduced nicotinamide adenine dinucleotide dehydrogenase (ubiquinon)124189004
- Deficiency of type I dehydrogenase124189004
- Deficiency of ubiquinone reductase124189004
- ALF - Acute liver failure197270009
- Acute hepatic failure197270009
- Acute liver failure197270009
- Disorder of pyruvate metabolism and mitochondrial respiratory chain237981000
- Pearson syndrome237985009
- Pearson's syndrome237985009
- Disorder of mitochondrial respiratory chain complexes237986005
- Mitochondrial disorder, respiratory chain237986005
- Deficiency in enzyme complexes of mitochondrial respiratory chain237987001
- Complex I deficiency237988006
- Mitochondrial complex I deficiency237988006
- NADH - Nicotinamide adenine dinucleotide coenzyme Q reductase deficiency237988006
- Nicotinamide adenine dinucleotide coenzyme Q reductase deficiency237988006
- Complex II deficiency237989003
- Succinate-coenzyme Q reductase deficiency237989003
- Complex III deficiency237990007
- Ubiquinone dehydrogenase deficiency237990007
- Combined complex deficiencies237993009
- Deletion and duplication of mitochondrial DNA237994003
- Deletion and duplication of mitochondrial deoxyribonucleic acid237994003
- Depletion of mitochondrial DNA237995002
- Depletion of mitochondrial deoxyribonucleic acid237995002
- Generalised dystonia425492002
- Generalized dystonia425492002
- Hypertrophic mitochondrial cardiomyopathy472316006
- Developmental regression609225004
- Deficiency of isolated CoQ cytochrome c reductase709414007
- Deficiency of mitochondrial complex III709414007
- Deficiency of ubiquinone cytochrome c oxidoreductase709414007
- Fatal infantile cytochrome C oxidase deficiency718124006
- Acute infantile liver failure due to synthesis defect of mitochondrial deoxyribonucleic acid encoded protein720461006
- MMDS - multiple mitochondrial dysfunctions syndrome720827002
- Multiple mitochondrial dysfunctions syndrome720827002
- Combined oxidative phosphorylation deficiency type 3720951008
- Fatal mitochondrial disease due to combined oxidative phosphorylation deficiency 3720951008
- COXPD5 - combined oxidative phosphorylation defect 5724279004
- Combined oxidative phosphorylation defect type 5724279004
- Hypotonia with lactic acidaemia and hyperammonaemia724279004
- Hypotonia with lactic acidemia and hyperammonemia724279004
- Coenzyme Q10 deficiency724575009
- COXPD8 - combined oxidative phosphorylation defect type 8733600007
- Combined oxidative phosphorylation defect type 8733600007
- Leucoencephalopathy with brain stem and spinal cord involvement and high lactate syndrome735421004
- Leucoencephalopathy with brain stem and spinal cord involvement and lactate elevation syndrome735421004
- Leukoencephalopathy with brain stem and spinal cord involvement and high lactate syndrome735421004
- Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation syndrome735421004
- Deficiency of mitochondrial aspartyl transfer ribonucleic acid synthetase735422006
- Deficiency of mitochondrial aspartyl-tRNA synthetase735422006
- COXPD13 - combined oxidative phosphorylation defect type 13763110007
- Combined oxidative phosphorylation defect type 13763110007
- COXPD15 - combined oxidative phosphorylation defect type 15763203009
- Combined oxidative phosphorylation defect type 15763203009
- COXPD7 - combined oxidative phosphorylation defect type 7763204003
- Combined oxidative phosphorylation defect type 7763204003
- COXPD9 - combined oxidative phosphorylation defect type 9763209008
- Combined oxidative phosphorylation defect type 9763209008
- COXPD21 - combined oxidative phosphorylation defect type 21763211004
- Combined oxidative phosphorylation defect type 21763211004
- COXPD12 - combined oxidative phosphorylation defect type 12763366000
- Combined oxidative phosphorylation defect type 12763366000
- Leukoencephalopathy, thalamus and brainstem anomalies, high lactate syndrome763366000
- COXPD2 - combined oxidative phosphorylation defect type 2764943000
- Combined oxidative phosphorylation defect type 2764943000
- Hepatoencephalopathy due to COXPD1 (combined oxidative phosphorylation defect type 1)764962002
- Hepatoencephalopathy due to combined oxidative phosphorylation defect type 1764962002
- COXPD4 - combined oxidative phosphorylation defect type 4766876004
- Combined oxidative phosphorylation defect type 4766876004
- COXPD10 - combined oxidative phosphorylation defect type 10771478008
- Combined oxidative phosphorylation defect type 10771478008
- Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to MTO1 deficiency771478008
- Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to mitochondrial transfer ribonucleic acid translation optimisation 1 deficiency771478008
- Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to mitochondrial transfer ribonucleic acid translation optimization 1 deficiency771478008
- Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial DNA mutation771509001
- Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial deoxyribonucleic acid mutation771509001
- COXPD17 - combined oxidative phosphorylation defect type 17775908005
- Combined oxidative phosphorylation defect type 17775908005
- COXPD14 - combined oxidative phosphorylation defect type 14778065005
- Combined oxidative phosphorylation defect type 14778065005
- Isolated ATP synthase deficiency780820008
- Isolated adenosine triphosphate synthase deficiency780820008
- Isolated mitochondrial respiratory chain complex V deficiency780820008
- Lipoic acid synthetase deficiency782744007
- Lipoyl transferase 1 deficiency782745008
- Mitochondrial DNA depletion syndrome hepatocerebrorenal form782771007
- Mitochondrial deoxyribonucleic acid depletion syndrome hepatocerebrorenal form782771007
- COXPD20 - combined oxidative phosphorylation deficiency type 20783178001
- Combined oxidative phosphorylation deficiency type 20783178001
- COXPD11 - combined oxidative phosphorylation defect type 11783558004
- Combined oxidative phosphorylation defect type 11783558004
- Mitochondrial DNA depletion syndrome, hepatocerebral form due to DGUOK deficiency783734000
- Mitochondrial deoxyribonucleic acid depletion syndrome, hepatocerebral form due to deoxyguanosine kinase deficiency783734000
- Benign COX (cytochrome C oxidase) deficiency784370005
- Infantile reversible cytochrome C oxidase deficiency myopathy784370005
- Mitochondrial myopathy with reversible COX (cytochrome C oxidase) deficiency784370005
- Mitochondrial myopathy with reversible complex IV deficiency784370005
- Mitochondrial myopathy with reversible cytochrome C oxidase deficiency784370005
- Reversible infantile cytochrome C oxidase deficiency784370005
- Reversible infantile respiratory chain deficiency784370005
- COXPD30 - combined oxidative phosphorylation defect type 301172841001
- Combined oxidative phosphorylation defect type 301172841001
- COXPD29 - combined oxidative phosphorylation defect type 291172843003
- Combined oxidative phosphorylation defect type 291172843003
- COXPD27 - combined oxidative phosphorylation defect type 271172844009
- Combined oxidative phosphorylation defect type 271172844009
- COXPD26 - combined oxidative phosphorylation defect type 261173034002
- Combined oxidative phosphorylation defect type 261173034002
- COXPD25 - combined oxidative phosphorylation defect type 251173035001
- Combined oxidative phosphorylation defect type 251173035001
- COXPD23 - combined oxidative phosphorylation defect type 231173036000
- Combined oxidative phosphorylation defect type 231173036000
- Mitochondrial phosphate carrier deficiency1187515001
- Leigh syndrome due to cytochrome C oxidase deficiency1187520001
- Thymidine kinase 2 deficiency1187535000
- COXPD28 - combined oxidative phosphorylation defect type 281187640000
- Combined oxidative phosphorylation defect type 281187640000
- Neonatal severe cardiopulmonary failure due to mitochondrial methylation defect1187640000
- Mitochondrial respiratory chain complex IV assembly gene defect1196869004
- Mitochondrial respiratory chain complex II structural subunit gene defect1196870003
- Mitochondrial respiratory chain complex III assembly gene defect1196871004
- Mitochondrial respiratory chain complex I structural subunit gene defect1196872006
- Mitochondrial respiratory chain complex II assembly gene defect1196873001
- Mitochondrial respiratory chain complex I assembly gene defect1196948002
- Mitochondrial respiratory chain complex III structural subunit gene defect1196949005
- Mitochondrial respiratory chain complex IV structural subunit gene defect1196950005
- Glutaminyl-transfer ribonucleic acid amidotransferase subunit-related combined oxidative phosphorylation defect1197430005
- QRSL1-related combined oxidative phosphorylation defect1197430005
- MMDS1 - multiple mitochondrial dysfunctions syndrome type 11208485009
- Multiple mitochondrial dysfunctions syndrome type 11208485009
- NFU1 (NFU1 iron-sulfur cluster scaffold) deficiency1208485009
- BOLA3 (bolA family member 3) deficiency1208486005
- MMDS2 - multiple mitochondrial dysfunctions syndrome type 21208486005
- Multiple mitochondrial dysfunctions syndrome type 21208486005
- IBA57 (iron-sulfur cluster assembly factor IBA57) deficiency1208620009
- MMDS3 - multiple mitochondrial dysfunctions syndrome type 31208620009
- Multiple mitochondrial dysfunctions syndrome type 31208620009
- MMDS4 - multiple mitochondrial dysfunctions syndrome type 41208621008
- Multiple mitochondrial dysfunctions syndrome type 41208621008
- COXPD24 - combined oxidative phosphorylation defect type 241222680009
- Combined oxidative phosphorylation defect type 241222680009
- Autosomal recessive childhood-onset dystonia DYT29 type1236805005
- Childhood-onset generalised dystonia, optic atrophy syndrome1236805005
- Childhood-onset generalized dystonia, optic atrophy syndrome1236805005
- DYT29 - dystonia 291236805005
- MEPAN syndrome1236805005
- Mitochondrial enoyl coenzyme A reductase protein-associated neurodegeneration syndrome1236805005
- Encephalopathy due to mitochondrial and peroxisomal fission defect1236807002
- Mitochondrial tryptophanyl-tRNA synthetase deficiency1260128008
- Tryptophanyl tRNA synthetase 2, mitochondrial-related combined oxidative phosphorylation defect1260128008
- WARS2-related combined oxidative phosphorylation defect1260128008
- Syndromic sensorineural deafness due to COXPD (combined oxidative phosphorylation defect)1260133007
- Syndromic sensorineural deafness due to combined oxidative phosphorylation defect1260133007
- Syndromic sensorineural hearing loss due to COXPD (combined oxidative phosphorylation defect)1260133007
- Isolated COX (cytochrome C oxidase) deficiency1264009006
- Isolated cytochrome C oxidase deficiency1264009006
- Isolated mitochondrial respiratory chain complex IV deficiency1264009006
- ISCA1 (iron-sulfur cluster assembly 1) deficiency1279890001
- ISCA1 deficiency1279890001
- MMDS5 - multiple mitochondrial dysfunctions syndrome type 51279890001
- Multiple mitochondrial dysfunctions syndrome type 51279890001
- Multiple mitochondrial dysfunctions syndrome type 61279891002
- PMPCB (peptidase mitochondrial processing subunit beta) deficiency1279891002
- PMPCB deficiency1279891002
- ATAD3A mitochondrial cytopathy1363370009
- ATAD3A mitochondrial disease1363370009
- ATPase family AAA domain containing 3A mitochondrial disease1363370009
- ATPase family, AAA Domain containing 3 mitochondrial cytopathy1363370009
- MT-ATP6 mitochondrial disease1363509005
- Mitochondrially encoded ATP synthase membrane subunit 6-related mitochondrial disease1363509005
- MC1DN10 - mitochondrial complex 1 deficiency nuclear type 101365878007
- Mitochondrial complex I deficiency nuclear type 101365878007
- NDUFAF2-related mitochondrial respiratory chain complex I assembly gene defect1365878007
- COQ10D1- primary coenzyme Q10 deficiency-11366189009
- COQ2-gene related coenzyme Q10 deficiency1366189009
- Coenzyme Q2, polyprenyltransferase gene related-coenzyme Q10 deficiency1366189009
- Primary coenzyme Q10 deficiency-11366189009
- Hereditary cerebellar atrophy431641000124107
- Mitochondrial metabolism defect133791000119107
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 you should also code: 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.
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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.