2027 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 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

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 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 39 closely related codes. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

E88.43 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 23 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23, ESRD (V21) category 23, ESRD (V24) category 23, and RxHCC Part D (V08) category 43 for payment year 2027.

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

Medicare Risk Adjustment (HCC)Billing

E88.43 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.

CMS-HCC V28 (Medicare Advantage Payment Model)
Not mapped
Dropped in V28 see all codes that no longer risk-adjust
Prior Model (CMS-HCC V24)
HCC 23
V24 retired last contributed to a Medicare Advantage risk score in payment year 2025
Other CMS Models
PACE (CMS-HCC V22): HCC 23 · ESRD (V21): HCC 23 · ESRD (V24): HCC 23
ESRD V21 weights: 0.013 dialysis, 0.234–0.359 functioning graft · ESRD V24 weights: 0.036 dialysis, 0.217–0.390 functioning graft
Part D (RxHCC)
RxHCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to E88.43: its own notes plus those printed at E88.4, E88, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • 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

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From E88.4 Mitochondrial metabolism disorders applies to 5 codes
  • 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 applies to 24 codes
  • histiocytosis X (chronic) (C96.6)
From Block E70-E88 Metabolic disorders applies to 286 codes
  • 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)
From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Block E70-E88 Metabolic disorders applies to 286 codes
  • Ehlers-Danlos syndromes (Q79.6-)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

From E88 Other and unspecified metabolic disorders applies to 24 codes
  • codes for associated conditions

Code Also

A second code may be needed to fully describe the condition; the note gives no sequencing direction.

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

Notes

General instructions on how these codes are used.

From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • 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.

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name E88.43, its category, or a range that includes it.

Excludes1 1

These codes carry an Excludes1 note naming E88.43: they are not reported together with it, unless the two conditions are unrelated.

  • G71.3 Mitochondrial myopathy, not elsewhere classified
    mitochondrial metabolism disorders (E88.4.-) names E88.4.-, which includes this code

Excludes2 1

These codes carry an Excludes2 note naming E88.43: its condition is not part of those codes, and both may be reported when the patient has both.

  • Block Q90-Q99 Chromosomal abnormalities, not elsewhere classified
    mitochondrial metabolic disorders (E88.4-) names E88.4-, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

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 2025–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About E88.43Overview

What is the ICD-10 code for disorders of mitochondrial tRNA synthetases?

The ICD-10-CM code for disorders of mitochondrial tRNA synthetases is E88.43 (sometimes written as E8843). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

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, 2026 through September 30, 2027.

What MS-DRG does E88.43 group to?

When disorders of mitochondrial tRNA synthetases is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.

Is E88.43 a CC or MCC?

CMS lists E88.43 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 39 closely related codes in its exclusion list.

Can E88.43 be reported with E74.4?

Not as a rule. The Excludes1 note at E88.4, which applies to E88.43, lists "disorders of pyruvate metabolism (E74.4)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

Does E88.43 risk-adjust for Medicare Advantage payment?

Not for Medicare Advantage. E88.43 mapped to HCC 23 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the PACE (CMS-HCC V22) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V21) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V24) category 23 (Other Significant Endocrine and Metabolic Disorders), and RxHCC Part D (V08) category 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders).