MCMedicalCodingSoftware
E88.43

E88.43: Disorders of mitochondrial tRNA synthetases

Billable / specific codeICD-10-CM

E88.43 is a billable, specific ICD-10-CM code for disorders of mitochondrial trna synthetases. It is valid for submission on a claim.

Check E88.43 with the rest of the claim: free scrubber

Code also

Both codes may be needed to describe the encounter fully. Sequencing depends on the reason for the visit.

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

Inclusion terms

Alternative wordings a physician may use in the chart for this same 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

Excludes1 inherited from the categories above E88.43

A category’s note binds every code beneath it.

Written on E88.4: Mitochondrial metabolism disorders

  • 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)

Written on E88: Other and unspecified metabolic disorders

  • histiocytosis X (chronic) (C96.6)

Risk adjustment

No CMS-HCC V28 category: this code does not change a RAF score.

Alphabetic index entries that lead here

  • 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
  • Disorder›metabolism NOS›mitochondrial›aminoacyl-tRNA synthetase
  • Disorder›metabolism NOS›mitochondrial›ARS2-related
  • Disorder›metabolism NOS›mitochondrial›tRNA synthetases

Code history

Unchanged in every release from FY2024 to FY2027.

Other codes under E88.4

ICD-10-CMFY2027· effective October 1, 2026

Loaded directly from the CMS/NCHS ICD-10-CM public-domain release: see data sources and our editorial policy. If this page disagrees with the CMS tabular list, this page is wrong. Reference information for professional coders; not medical or billing advice.