E88.43 Disorders of mitochondrial tRNA synthetases
Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.
Applicable to
- 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)
Applies from E88 Other and unspecified metabolic disorders
Excludes1 (never code together)
- histiocytosis X (chronic) (C96.6)
Use additional code
- codes for associated conditions
Applies from E88.4 Mitochondrial metabolism disorders
Excludes1 (never code together)
- 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)
Risk adjustment (HCC)
- CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
- RxHCC V08: HCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
- ESRD V24: HCC 23
MS-DRG index
Defines the logic of DRG(s) 642 in MDC 10.
Code annotations containing back-references to E88.43
Back-references to E88.4
- Type 1 Excludes:
G71.3 Mitochondrial myopathy, not elsewhere classified ("mitochondrial metabolism disorders (E88.4.-)")
Back-references to E88
- Type 2 Excludes:
G11 Hereditary ataxia ("metabolic disorders (E70-E88)") - Code First:
G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)") - Type 2 Excludes:
G71 Primary disorders of muscles ("metabolic disorders (E70-E88)") - Code First:
H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)") - Type 2 Excludes:
N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88") - Type 2 Excludes:
N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88") - Type 1 Excludes:
P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
Diagnosis Index entries for E88.43
- Disorder (of) › metabolism NOS › mitochondrial › aminoacyl-tRNA synthetase
- Disorder (of) › metabolism NOS › mitochondrial › ARS2-related
- Disorder (of) › metabolism NOS › mitochondrial › tRNA synthetases
- 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
Nearby codes
E88.3 Tumor lysis syndromeE88.4 Mitochondrial metabolism disordersE88.40 Mitochondrial metabolism disorder, unspecifiedE88.41 MELAS syndromeE88.42 MERRF syndromeE88.43 Disorders of mitochondrial tRNA synthetases (this code)E88.49 Other mitochondrial metabolism disordersE88.8 Other specified metabolic disordersE88.81 Metabolic syndrome and other insulin resistanceE88.810 Metabolic syndromeE88.811 Insulin resistance syndrome, Type A