E88.49 is the authoritative medical code for Other mitochondrial metabolism disorders. This classification is used in medical billing and clinical recording to specify the clinical criteria for other mitochondrial metabolism disorders (ICD-10-CM E88.49), ensuring healthcare documentation aligns with 2026 federal coding standards.
Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.
Official Registry Overview & Definition
Other mitochondrial metabolism disorders is a billable ICD-10-CM diagnosis code E88.49. Excludes1 (not coded here): 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; histiocytosis X chronic C96.6; 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. Excludes2 (not included here): Ehlers-Danlos syndromes Q79.6-. Use additional code: codes for associated conditions.
Official Tabular Instructional Notes
Sequencing, inclusion, and exclusion notes published for E88.49 in the official ICD-10-CM tabular list.
Excludes1 — Not Coded Here
Conditions that can never be reported with this code; the two are mutually exclusive.
- 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
- histiocytosis X chronic C96.6
- 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
Excludes2 — Not Included Here
Conditions not covered by this code, but which may be reported alongside it when both are present.
- Ehlers-Danlos syndromes Q79.6-
Use Additional Code
Supplementary codes the tabular list directs you to add.
- codes for associated conditions
Frequently Asked Questions (FAQ) & Clinical Guidance
What can't be coded together with E88.49?
Per Excludes1 instructions, E88.49 must not be reported with: disorders of pyruvate metabolism (E74.4); Kearns-Sayre syndrome (H49.81); Leber's disease (H47.22); Leigh's encephalopathy (G31.82).
Can E88.49 be reported alongside related conditions?
Per Excludes2 instructions, E88.49 and the following may both be reported when both are present: Ehlers-Danlos syndromes (Q79.6-).
Is an additional code needed with E88.49?
Use additional code(s) to specify: codes for associated conditions.
Codes in This Family (29)
Official ICD-10-CM classifications in the same code family as E88.49, with their registry titles.
- E88 — Other and unspecified metabolic disorders
- E88.0 — Disorders of plasma-protein metabolism, not elsewhere classified
- E88.01 — Alpha-1-antitrypsin deficiency
- E88.02 — Plasminogen deficiency
- E88.09 — Other disorders of plasma-protein metabolism, not elsewhere classified
- E88.1 — Lipodystrophy, not elsewhere classified
- E88.10 — Lipodystrophy, unspecified
- E88.11 — Partial lipodystrophy
- E88.12 — Generalized lipodystrophy
- E88.13 — Localized lipodystrophy
- E88.14 — HIV-associated lipodystrophy
- E88.19 — Other lipodystrophy, not elsewhere classified
- E88.2 — Lipomatosis, not elsewhere classified
- E88.3 — Tumor lysis syndrome
- E88.4 — Mitochondrial metabolism disorders
- E88.40 — Mitochondrial metabolism disorder, unspecified
- E88.41 — MELAS syndrome
- E88.42 — MERRF syndrome
- E88.43 — Disorders of mitochondrial tRNA synthetases
- E88.8 — Other specified metabolic disorders
- E88.81 — Metabolic syndrome and other insulin resistance
- E88.810 — Metabolic syndrome
- E88.811 — Insulin resistance syndrome, Type A
- E88.818 — Other insulin resistance
- E88.819 — Insulin resistance, unspecified
- E88.82 — Obesity due to disruption of MC4R pathway
- E88.89 — Other specified metabolic disorders
- E88.9 — Metabolic disorder, unspecified
- E88.A — Wasting disease (syndrome) due to underlying condition
Indexed Clinical Terms (6)
Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code.
- Disorder (of), metabolism NOS, mitochondrial, other specified
- MNGIEsyndrome (Mitochondrial Neurogastrointestinal Encephalopathy)
- NARPsyndrome (Neuropathy, Ataxia and Retinitis pigmentosa)
- Syndrome, Fukuhara
- Syndrome, MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)
- Syndrome, NARP (Neuropathy, Ataxia and Retinitis pigmentosa)
Related Codes & Numerical Sequence (Crawl Map)
Search engines and indexers are advised to traverse adjacent medical codes in this sub-chapter range to find correlated diagnoses or therapeutic procedures: