E88.40

✓ Billable

Mitochondrial metabolism disorder, unspecified

Endocrine, nutritional and metabolic diseases (E00-E89)

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❌ Excludes1

inherited

Rules inherited from parent codes in the hierarchy.

  • 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)

📋 Code Instructions

inherited
  • codes for associated conditions

Clinical Description

ICD-10-CM code E88.40 represents mitochondrial metabolism disorder, unspecified. This code falls under the Endocrine, nutritional and metabolic diseases (E00-E89) classification. It is a billable/specific code valid for HIPAA-covered transactions as of October 1, 2025 (FY2026). This code can be used to indicate a diagnosis for reimbursement purposes on insurance claims.

This is a 6-character code at the 5th level of specificity. This is the most specific code available in this subcategory. Coders should note that E88.40 has 7 Excludes1 restriction(s) — codes that cannot be reported together on the same claim.

Frequently Asked Questions

What is ICD-10 code E88.40?

ICD-10-CM code E88.40 is "Mitochondrial metabolism disorder, unspecified". It belongs to the category: Endocrine, nutritional and metabolic diseases (E00-E89).

Is E88.40 a billable ICD-10 code?

Yes, E88.40 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

What codes are excluded with E88.40?

E88.40 has 7 Excludes1 rule(s). These codes are mutually exclusive and cannot be coded together on the same claim.

Related Codes in E88.4

Other codes in the same category that may be relevant for differential coding:

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Code Information

CodeE88.40
BillableYes
ParentE88.4
CategoryEndocrine, nutritional and metabolic diseases (E00-E89)
Excludes17 rule(s)
Excludes20 note(s)

Data Source

CMS ICD-10-CM 2026 Tabular List

Updated October 1, 2025 (FY2026)