Disorders of mitochondrial tRNA synthetases
Billable / specific
E88.43 is a billable, specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes in FY2027.
Inclusion terms
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
Includes (from E88)
- Note: 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.
Excludes1
Not coded here — never report together with this code.
- disorders of pyruvate metabolism (E74.4) (from E88.4)
- Kearns-Sayre syndrome (H49.81) (from E88.4)
- Leber's disease (H47.22) (from E88.4)
- Leigh's encephalopathy (G31.82) (from E88.4)
- Mitochondrial myopathy, NEC (G71.3) (from E88.4)
- Reye's syndrome (G93.7) (from E88.4)
- transitory endocrine and metabolic disorders specific to newborn (P70-P74) (from E88)
- androgen insensitivity syndrome (E34.5-) (from E88)
- congenital adrenal hyperplasia (E25.0) (from E88)
- hemolytic anemias attributable to enzyme disorders (D55.-) (from E88)
- Marfan syndrome (Q87.4-) (from E88)
- 5-alpha-reductase deficiency (E29.1) (from E88)
- histiocytosis X (chronic) (C96.6) (from E88)
Excludes2
Not included here — may be reported together when both conditions exist.
Use additional code
Add a code to identify the following, if applicable.
- codes for associated conditions (from E88)
Code also
An additional code may be needed.
- , if applicable, associated condition such as:
- leukoencephalopathy (G93.49)
Also known as
- 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
- Disorder, metabolism NOS, mitochondrial, aminoacyl-tRNA synthetase
- Disorder, metabolism NOS, mitochondrial, ARS2-related
- Disorder, metabolism NOS, mitochondrial, tRNA synthetases
- Leukoencephalopathy, with brainstem - spinal cord involvement - lactate elevation
- Leukoencephalopathy, with thalamus - brainstem involvement - high lactate
Parent code
- E88.4Mitochondrial metabolism disordersHeader