2026 ICD-10-CM Diagnosis Code E88.43Disorders of mitochondrial tRNA synthetases

ICD-10-CM Codes›E00–E89›E70-E88›E88

ICD-10-CM E88.43
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E88.43 is a billable ICD-10-CM diagnosis code for disorders of mitochondrial tRNA synthetases. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E88.43
Billable Status
Yes — Valid for Submission
Code Describes
Disorders of mitochondrial tRNA synthetases
Short Description
Disorders of mitochondrial tRNA synthetases
Same as the full description in the CMS dataset.
Parent Code
E88.4
Mitochondrial metabolism disorders

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE88Other and unspecified metabolic disorders
This CodeE88.43Disorders of mitochondrial tRNA synthetases

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • ARS2-related mitochondrial disorders
  • LBSL
  • Leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation
  • Leukoencephalopathy with thalamus - brainstem involvement - high lactate
  • LTBL
  • Mitochondrial aminoacyl-tRNA synthetase disorders

Code Also

  • , if applicable, associated condition such as:
  • leukoencephalopathy G93.49

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Disorder(of)
      • metabolism NOS
        • mitochondrial
          • aminoacyl-tRNA synthetase
    • Disorder(of)
      • metabolism NOS
        • mitochondrial
          • ARS2-related
    • Disorder(of)
      • metabolism NOS
        • mitochondrial
          • tRNA synthetases
    • LBSL(leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation)
    • LTBL(leukoencephalopathy with thalamus - brainstem involvement - high lactate)
    • Leukoencephalopathy
      • with
        • brainstem - spinal cord involvement - lactate elevation
    • Leukoencephalopathy
      • with
        • thalamus - brainstem involvement - high lactate

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Code History & ChangesHistory

Replacement E88.43 replaces the following previously assigned code(s):

  • E88.49 - Other mitochondrial metabolism disorders
FY 2024AddedAdded to the ICD-10-CM code setEffective October 1, 2023.
FY 2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E88.43Overview

Is E88.43 (Mitochondrial metabolism disorders) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report disorders of mitochondrial tRNA synthetases on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.