E88.4
Non-Billable

Mitochondrial metabolism disorders

Non-Billable / Non-Specific Code

What is Mitochondrial metabolism disorders?

ICD-10-CM E88.4 is the diagnosis code for Mitochondrial metabolism disorders. This code falls under the section "Metabolic disorders" within Chapter 4 — Endocrine, nutritional and metabolic diseases (E00-E89). It is a non-billable/non-specific ICD-10-CM code that should not be used for reimbursement. A more specific sub-code is required for billing. Medical coders and healthcare providers use this code to document and classify diagnoses in electronic health records, insurance claims, and clinical databases.

⛔ Excludes1

Coding Guidelines for E88.4

⛔ Type 1 Excludes (Never code together)

The following conditions should never be coded at the same time as E88.4. They 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)

ICD-10-CM Code Hierarchy

Understanding where E88.4 sits in the ICD-10-CM classification helps ensure proper coding:

Sub-Codes 5 entries
E88.40Mitochondrial metabolism disorder, unspecifiedBillableE88.41MELAS syndromeBillableE88.42MERRF syndromeBillableE88.43Disorders of mitochondrial tRNA synthetasesBillableE88.49Other mitochondrial metabolism disordersBillable
Related Codes

Frequently Asked Questions

Is E88.4 a billable ICD-10-CM code?

No, E88.4 is a non-billable/non-specific code. You should use a more specific sub-code for billing and reimbursement.

What does E88.4 mean?

E88.4 is the ICD-10-CM diagnosis code for "Mitochondrial metabolism disorders". It is used by healthcare providers to classify and document this condition in medical records and insurance claims.

What is the parent code of E88.4?

The parent code of E88.4 is E88 ("Other and unspecified metabolic disorders"). E88.4 provides a more specific classification within this category.

What section is E88.4 in?

E88.4 is located in Section E70-E88 — "Metabolic disorders" within Chapter 4 of the ICD-10-CM Tabular List.

What are the sub-codes of E88.4?

E88.4 has 5 sub-code(s) that provide more specific detail: E88.40, E88.41, E88.42, E88.43, E88.49.

When should I use E88.4?

Use E88.4 when the patients documented diagnosis matches "Mitochondrial metabolism disorders" and the clinical documentation supports this level of specificity. Always verify with the latest ICD-10-CM guidelines and payer requirements.

People Also Ask

What is the ICD-10 code for Mitochondrial metabolism disorders?

The ICD-10-CM code for Mitochondrial metabolism disorders is E88.4.

Can E88.4 be used for primary diagnosis?

No, E88.4 is non-billable. Use a more specific sub-code for primary diagnosis billing.

What chapter is E88.4 in?

E88.4 is in Chapter 4 of the ICD-10-CM Tabular List.

What conditions are excluded from E88.4?

Type 1 Excludes for E88.4 include: disorders of pyruvate metabolism (E74.4); Kearns-Sayre syndrome (H49.81); Leber's disease (H47.22) and more.

Is E88.4 valid for 2026?

Yes, E88.4 is a valid ICD-10-CM code for the 2026 fiscal year, subject to official CMS updates.

See Also

← PreviousE88.3Tumor lysis syndromeNext →E88.40Mitochondrial metabolism disorder, unspecified
Code Details
E88.4
Mitochondrial metabolism disorders
Non-Billable / Non-Specific Code
4 — Endocrine, nutritional and metabolic diseases (E00-E89)
E70-E88 — Metabolic disorders
2026 ICD-10-CM