E88.43

Billable
ChapterE00–E89
Ranksubcategory
Official Description

Disorders of mitochondrial tRNA synthetases

Metadata & Insights
  • •2026 ICD-10-CM Edition - Effective October 1, 2025.
  • •Billable/specific code (valid for reimbursement).
  • •Chapter 4: Endocrine, Nutritional and Metabolic Diseases (E00-E89).

Instructional Notes

Includes
  • 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)
General instructions are available for the parent sections:
View CH4 NotesView E70-E88 NotesView E88 NotesView E88.4 Notes

Clinical Hierarchy

CH4Endocrine, nutritional and metabolic diseases (E00-E89)
E70-E88Metabolic disorders (E70-E88)
E88Other and unspecified metabolic disorders
E88.4Mitochondrial metabolism disorders
E88.43Disorders of mitochondrial tRNA synthetases

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to E88.43:

subcategoryE88.4
Mitochondrial metabolism disorders
View context instructions
categoryE88
Other and unspecified metabolic disorders
View context instructions
blockE70-E88
Metabolic disorders (E70-E88)
View context instructions
chapterCH4
Endocrine, nutritional and metabolic diseases (E00-E89)
View context instructions

Alphabetic Index Mentions

These are Diagnosis Index lookup paths that route to E88.43. Use them as entry terms for search, then confirm final coding instructions in the Tabular List and notes above.

7 raw paths
Disorder (of)3 unique routes
metabolism NOS(3 variants)
mitochondrial(3 variants)
aminoacyl-tRNA synthetase→E88.43
ARS2-related→E88.43
tRNA synthetases→E88.43
LBSL (leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation)1 unique routes
Direct index entry→E88.43
Leukoencephalopathy2 unique routes
with(2 variants)
brainstem - spinal cord involvement - lactate elevation→E88.43
thalamus - brainstem involvement - high lactate→E88.43
LTBL (leukoencephalopathy with thalamus - brainstem involvement - high lactate)1 unique routes
Direct index entry→E88.43

MS-DRG Classification

2026 v43.1

This code is grouped within the following Diagnosis Related Group(s) for clinical reimbursement:

DRG 642
Inborn and Other Disorders of Metabolism

Cross Reference

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