2027 ICD-10-CM Diagnosis Code E88.49Other mitochondrial metabolism disorders
ICD-10-CM Codes›E00–E89›E70-E88›E88
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Chronic Condition
E88.49 is a billable ICD-10-CM diagnosis code for other mitochondrial metabolism disorders. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 42 closely related codes. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
E88.49 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 23 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23, ESRD (V21) category 23, ESRD (V24) category 23, and RxHCC Part D (V08) category 43 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E88.49 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
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
- Hypermetabolism
- Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial DNA mutation
- Hypertrophic mitochondrial cardiomyopathy
- Inborn error of amino acid metabolism
- 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
- Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency disorder
- 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
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E88.49: its own notes plus those printed at E88.4, E88, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- histiocytosis X (chronic) (C96.6)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Use Additional Code
Add a second code, after this one, to fully describe the condition.
- codes for associated conditions
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E88.49, its category, or a range that includes it.
Excludes1 1
These codes carry an Excludes1 note naming E88.49: they are not reported together with it, unless the two conditions are unrelated.
Excludes2 1
These codes carry an Excludes2 note naming E88.49: its condition is not part of those codes, and both may be reported when the patient has both.
- Block Q90-Q99 Chromosomal abnormalities, not elsewhere classifiedmitochondrial metabolic disorders (E88.4-) names E88.4-, which includes this code
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Disorder (of) See Also: Disease;
Syndrome See Also: Disease;
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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.
Code HistoryHistory
Questions About E88.49Overview
What is the ICD-10 code for other mitochondrial metabolism disorders?
The ICD-10-CM code for other mitochondrial metabolism disorders is E88.49 (sometimes written as E8849). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
What MS-DRG does E88.49 group to?
When other mitochondrial metabolism disorders is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Is E88.49 a CC or MCC?
CMS lists E88.49 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 42 closely related codes in its exclusion list.
Can E88.49 be reported with E74.4?
Not as a rule. The Excludes1 note at E88.4, which applies to E88.49, lists "disorders of pyruvate metabolism (E74.4)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
Does E88.49 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. E88.49 mapped to HCC 23 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the PACE (CMS-HCC V22) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V21) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V24) category 23 (Other Significant Endocrine and Metabolic Disorders), and RxHCC Part D (V08) category 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders).
