Alphabetic Index FY2027

E88.43

Disorders of mitochondrial tRNA synthetases

FY2027Chapter 4: Endocrine, nutritional and metabolic diseases/E70–E88 Metabolic disorders

BillableComplete code, valid to report
Care settingsHome Health Primary · MMTA - OtherHospital CC

E88.43 (also written E8843) is a billable ICD-10-CM diagnosis code for disorders of mitochondrial tRNA synthetases. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 24 codes in category E88 (Other and unspecified metabolic disorders). The Alphabetic Index lists it under 7 entries. Its coding notes include a Use additional code instruction, a Code also instruction, 13 Excludes1 entries and 1 Excludes2 entry.

Use additional code

Add the listed code as a secondary code, if known.

Inherited from E88 Other and unspecified metabolic disorders
  • codes for associated conditions

Code also

Two codes may be needed to describe the condition. This note gives no sequencing order.

This code
  • , if applicable, associated condition such as:

Excludes1

Not coded here. The listed conditions generally should not be reported together.

Inherited from 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)
Inherited from E88 Other and unspecified metabolic disorders
  • histiocytosis X (chronic) (C96.6)
Also from chapter/block level (6 notes)
Inherited from E70–E88 Metabolic disorders
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)
Inherited from Chapter 4 Endocrine, nutritional and metabolic diseases
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes2

Not included here. Both codes may be reported if the patient has both conditions.

Also from chapter/block level (1 note)
Inherited from E70–E88 Metabolic disorders
  • Ehlers-Danlos syndromes (Q79.6-)

Care settings

How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.

Home Health Primary

Accepted as the primary (principal) diagnosis on a home health claim. The Home Health Grouper assigns it to the MMTA - Other clinical group.

Source: CMS Home Health Grouper Software v07.2.26, effective October 1, 2026

Hospital CC

Counts as a complication or comorbidity (CC) when reported as a secondary diagnosis on an inpatient claim. It does not count when the principal diagnosis is one of the 39 codes in its exclusion list. Some MS-DRGs also ignore a CC or MCC that defines the DRG.

Source: CMS MS-DRG Definitions Manual v44, effective October 1, 2026, Appendix C

Inclusion terms

Conditions classified to this code.

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

Notes

Also from chapter/block level (1 note)
Inherited from Chapter 4 Endocrine, nutritional and metabolic diseases
  • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Alphabetic Index terms

Terms the official Alphabetic Index lists for this code. 7 entries