2021 ICD-10-CM Diagnosis Code E74.4

Disorders of pyruvate metabolism and gluconeogenesis

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • E74.4 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM E74.4 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of E74.4 - other international versions of ICD-10 E74.4 may differ.
Applicable To
  • Deficiency of phosphoenolpyruvate carboxykinase
  • Deficiency of pyruvate carboxylase
  • Deficiency of pyruvate dehydrogenase
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 E74.4. 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 and gluconeogenesis with anemia (
    ICD-10-CM Diagnosis Code D55
    • D55 Anemia due to enzyme disorders
      • D55.0 Anemia due to glucose-6-phosphate dehydrogena...
      • D55.1 Anemia due to other disorders of glutathione ...
      • D55.2 Anemia due to disorders of glycolytic enzymes...
      • D55.3 Anemia due to disorders of nucleotide metabol...
      • D55.8 Other anemias due to enzyme disorders
      • D55.9 Anemia due to enzyme disorder, unspecified
  • Leigh's syndrome (
    ICD-10-CM Diagnosis Code G31.82

    Leigh's disease

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Subacute necrotizing encephalopathy
The following code(s) above E74.4 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 E74.4:
  • E00-E89
    2021 ICD-10-CM Range E00-E89

    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.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2021 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndromes (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E74
    ICD-10-CM Diagnosis Code E74

    Other disorders of carbohydrate metabolism

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Other disorders of carbohydrate metabolism
Approximate Synonyms
  • Deficiency, pyruvate carboxylase
  • Deficiency, pyruvate deshydrogenase
  • Pyruvate carboxylase deficiency
  • Pyruvate dehydrogenase complex deficiency
  • Pyruvate metabolism and gluconeogenesis disorder
Clinical Information
  • A rare autosomal recessive inherited metabolic disorder caused by mutations in the pc gene. It results in the accumulation of lactic acid and other toxic substances in the blood. Signs and symptoms appear early in life and include failure to thrive, mental and growth retardation, motor disturbances, seizures, and lactic acidosis.
  • A very rare, autosomal recessive inherited disorder caused by deficiency of the enzyme phosphoenolpyruvate carboxykinase, which is involved in gluconeogenesis. It presents with hypoglycemia, failure to thrive, metabolic acidosis, muscle weakness, and hepatomegaly.
  • An autosomal recessive metabolic disorder caused by absent or decreased pyruvate carboxylase activity, the enzyme that regulates gluconeogenesis, lipogenesis, and neurotransmitter synthesis. Clinical manifestations include lactic acidosis, seizures, respiratory distress, marked psychomotor delay, periodic hypoglycemia, and hypotonia. The clinical course may be similar to leigh disease. (from am j hum genet 1998 jun;62(6):1312-9)
  • An inborn error of metabolism in which deficiency of pyruvate dehydrogenase complex is complicated by variable metabolic and neurological disorders. Neonatal lactic acidosis is usually associated with slow physical and mental development, microcephaly, dysgenesis and atrophic changes in the brain, cerebellar ataxia, seizures, and spasticity. Pyruvate dehydrogenase phosphatase (ec deficiency has been found to occur in leigh syndrome.
  • An inherited metabolic disorder caused by deficient enzyme activity in the pyruvate dehydrogenase complex, resulting in deficiency of acetyl coa and reduced synthesis of acetylcholine. Two clinical forms are recognized: neonatal and juvenile. The neonatal form is a relatively common cause of lactic acidosis in the first weeks of life and may also feature an erythematous rash. The juvenile form presents with lactic acidosis, alopecia, intermittent ataxia; seizures; and an erythematous rash. (from j inherit metab dis 1996;19(4):452-62) autosomal recessive and x-linked forms are caused by mutations in the genes for the three different enzyme components of this multisubunit pyruvate dehydrogenase complex. One of the mutations at xp22.2-p22.1 in the gene for the e1 alpha component of the complex leads to leigh disease.
ICD-10-CM E74.4 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 642 Inborn and other disorders of metabolism

Convert E74.4 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
Code annotations containing back-references to E74.4:
  • Type 1 Excludes: E88.4
    ICD-10-CM Diagnosis Code E88.4

    Mitochondrial metabolism disorders

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • 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)

Diagnosis Index entries containing back-references to E74.4:
  • Deficiency, deficient
    • pyruvate
      • carboxylase E74.4
      • dehydrogenase E74.4
    • phosphoenolpyruvate carboxykinase E74.4
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 2021 Billable/Specific Code
      • pyruvate E74.4
    • gluconeogenesis E74.4
    • pyruvate metabolism E74.4

ICD-10-CM Codes Adjacent To E74.4
E74.11 Essential fructosuria
E74.12 Hereditary fructose intolerance
E74.19 Other disorders of fructose metabolism
E74.2 Disorders of galactose metabolism
E74.20 …… unspecified
E74.21 Galactosemia
E74.29 Other disorders of galactose metabolism
E74.3 Other disorders of intestinal carbohydrate absorption
E74.31 Sucrase-isomaltase deficiency
E74.39 Other disorders of intestinal carbohydrate absorption
E74.4 Disorders of pyruvate metabolism and gluconeogenesis
E74.8 Other specified disorders of carbohydrate metabolism
E74.81 Disorders of glucose transport, not elsewhere classified
E74.810 Glucose transporter protein type 1 deficiency
E74.818 Other disorders of glucose transport
E74.819 Disorders of glucose transport, unspecified
E74.89 Other specified disorders of carbohydrate metabolism
E74.9 Disorder of carbohydrate metabolism, unspecified
E75 Disorders of sphingolipid metabolism and other lipid storage disorders
E75.0 GM2 gangliosidosis
E75.00 …… unspecified

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