2017/18 ICD-10-CM Diagnosis Code E88.4

Mitochondrial metabolism disorders

Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as E88.4. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • disorders of pyruvate metabolism (
    ICD-10-CM Diagnosis Code E74.4

    Disorders of pyruvate metabolism and gluconeogenesis

      2016 2017 2018 Billable/Specific Code
    Applicable To
    • Deficiency of phosphoenolpyruvate carboxykinase
    • Deficiency of pyruvate carboxylase
    • Deficiency of pyruvate dehydrogenase
    Type 1 Excludes
    • disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
    • Leigh's syndrome (G31.82)
    E74.4
    )
  • Kearns-Sayre syndrome (
    ICD-10-CM Diagnosis Code H49.81

    Kearns-Sayre syndrome

      2016 2017 2018 Non-Billable/Non-Specific Code
    Applicable To
    • Progressive external ophthalmoplegia with pigmentary retinopathy
    Use Additional
    • code for other manifestation, such as:
    • heart block (I45.9)
    H49.81
    )
  • Leber's disease (
    ICD-10-CM Diagnosis Code H47.22

    Hereditary optic atrophy

      2016 2017 2018 Billable/Specific Code
    Applicable To
    • Leber's optic atrophy
    H47.22
    )
  • Leigh's encephalopathy (
    ICD-10-CM Diagnosis Code G31.82

    Leigh's disease

      2016 2017 2018 Billable/Specific Code
    Applicable To
    • Subacute necrotizing encephalopathy
    G31.82
    )
  • Mitochondrial myopathy, NEC (
    ICD-10-CM Diagnosis Code G71.3

    Mitochondrial myopathy, not elsewhere classified

      2016 2017 2018 Billable/Specific Code
    Type 1 Excludes
    • Kearns-Sayre syndrome (H49.81)
    • Leber's disease (H47.21)
    • Leigh's encephalopathy (G31.82)
    • mitochondrial metabolism disorders (E88.4.-)
    • Reye's syndrome (G93.7)
    G71.3
    )
  • Reye's syndrome (
    ICD-10-CM Diagnosis Code G93.7

    Reye's syndrome

      2016 2017 2018 Billable/Specific Code Pediatric Dx (0-17 years)
    Code First
    • poisoning due to salicylates, if applicable (T39.0-, with sixth character 1-4)
    Use Additional
    • code for adverse effect due to salicylates, if applicable (T39.0-, with sixth character 5)
    G93.7
    )
The following code(s) above E88.4 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E88.4:
  • E00-E89
    2018 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    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.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2018 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E88
    ICD-10-CM Diagnosis Code E88

    Other and unspecified metabolic disorders

      2016 2017 2018 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • histiocytosis X (chronic) (C96.6)
    Use Additional
    • codes for associated conditions
    Other and unspecified metabolic disorders
Code History
Code annotations containing back-references to E88.4:
ICD-10-CM Codes Adjacent To E88.4
E87.71 Transfusion associated circulatory overload
E87.79 Other fluid overload
E87.8 Other disorders of electrolyte and fluid balance, not elsewhere classified
E88 Other and unspecified metabolic disorders
E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
E88.01 Alpha-1-antitrypsin deficiency
E88.09 Other disorders of plasma-protein metabolism, not elsewhere classified
E88.1 Lipodystrophy, not elsewhere classified
E88.2 Lipomatosis, not elsewhere classified
E88.3 Tumor lysis syndrome
E88.4 Mitochondrial metabolism disorders
E88.40 Mitochondrial metabolism disorder, unspecified
E88.41 MELAS syndrome
E88.42 MERRF syndrome
E88.49 Other mitochondrial metabolism disorders
E88.8 Other specified metabolic disorders
E88.81 Metabolic syndrome
E88.89 Other specified metabolic disorders
E88.9 Metabolic disorder, unspecified
E89 Postprocedural endocrine and metabolic complications and disorders, not elsewhere classified
E89.0 Postprocedural hypothyroidism

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.