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ICD-10-CM 2027 diagnosis code

E88.43Disorders of mitochondrial tRNA synthetases

✓ Billable / specificCC — complication/comorbidity

E88.43 is a valid, billable ICD-10-CM code for disorders of mitochondrial trna synthetases. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.

Looking up by condition? See ICD-10 code for LBSL, ICD-10 code for LTBL.

Code last changed in FY2024 (effective October 1, 2023). Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About E88.43

E88.43 is the ICD-10-CM diagnosis code for disorders of mitochondrial tRNA synthetases. It belongs to category E88 (other and unspecified metabolic disorders), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E88.4 (mitochondrial metabolism disorders), E88.43 is specifically for disorders of mitochondrial tRNA synthetases. Related codes cover mitochondrial metabolism disorder, unspecified (E88.40), MELAS syndrome (E88.41), MERRF syndrome (E88.42) and other mitochondrial metabolism disorders (E88.49).

Use an additional code for: codes for associated conditions.

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It was added in FY2024, effective October 1, 2023.

Coding notes

Applicable toConditions and synonyms classified to this code
  • 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 alsoTwo codes may be needed; sequencing depends on circumstances
  • , if applicable, associated condition such as:
  • leukoencephalopathy (G93.49)

Notes that apply from higher levels

Instructions written at a parent level also apply to E88.43.

› From E88.4 Mitochondrial metabolism disorders
Excludes1
  • 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)
› From E88 Other and unspecified metabolic disorders
Excludes1
  • histiocytosis X (chronic) (C96.6)
Use additional code
  • codes for associated conditions

Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.

Alphabetic index entries

7 entries

Terms in the official ICD-10-CM index that lead to E88.43.

MS-DRG v44.0 grouping

All DRGs →

Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.

MDC 10 · Endocrine, Nutritional and Metabolic Diseases and Disorders

Code history

  1. 2016
  2. 2017
  3. 2018
  4. 2019
  5. 2020
  6. 2021
  7. 2022
  8. 2023
  9. 2024
  10. 2025
  11. 2026
  12. 2027
  • FY2024 (effective 10/1/2023): Added
  • No changes since FY2024.

Common questions about E88.43

Is E88.43 billable?

Yes. E88.43 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can E88.43 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict E88.43 as a principal diagnosis.

Is E88.43 a CC or MCC?

E88.43 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.

What DRG does E88.43 group to?

E88.43 is used in the MS-DRG v44.0 logic for MS-DRG 642 (Inborn and Other Disorders of Metabolism). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.