2019 ICD-10-CM Diagnosis Code E72.4

Disorders of ornithine metabolism

    2016 2017 2018 2019 Billable/Specific Code
  • E72.4 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2019 edition of ICD-10-CM E72.4 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of E72.4 - other international versions of ICD-10 E72.4 may differ.
Applicable To
  • Hyperammonemia-Hyperornithinemia-Homocitrullinemia syndrome
  • Ornithinemia (types I, II)
  • Ornithine transcarbamylase deficiency
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 E72.4. 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.
  • hereditary choroidal dystrophy (
    ICD-10-CM Diagnosis Code H31.2

    Hereditary choroidal dystrophy

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 2 Excludes
    H31.2-
    )
The following code(s) above E72.4 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 E72.4:
  • E00-E89
    2019 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
    2019 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
  • E72
    ICD-10-CM Diagnosis Code E72

    Other disorders of amino-acid metabolism

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • disorders of:
    • aromatic amino-acid metabolism (E70.-)
    • branched-chain amino-acid metabolism (E71.0-E71.2)
    • fatty-acid metabolism (E71.3)
    • purine and pyrimidine metabolism (E79.-)
    • gout (M1A.-, M10.-)
    Other disorders of amino-acid metabolism
Approximate Synonyms
  • Deficiency, ornithine transcarbamylase
  • Disorder of ornithine metabolism
  • Hyperornithinemia hyperammonemia homocitrullinemia
  • Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
  • Ornithine carbamoyltransferase deficiency
  • Ornithine metabolism disorder
Clinical Information
  • An inherited urea cycle disorder associated with deficiency of the enzyme ornithine carbamoyltransferase, transmitted as an x-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (menkes, textbook of child neurology, 5th ed, pp49-50)
  • An x-linked urea cycle metabolic disorder characterized by deficiency of ornithine carbamoyltransferase, resulting in the accumulation of amino acids and ammonia in the serum. Signs and symptoms include seizures, delayed growth, behavioral changes, ataxia, lethargy, and coma.
  • An x-linked urea cycle metabolic disorder characterized by deficiency of ornithine transcarbamylase, resulting in the accumulation of ammonia in the serum. Symptoms include vomiting, lethargy, and coma.
  • Inborn error of metabolism of the urea cycle due to ornithine carbomoyltransferase (e.c. 2.1.3.3) deficiency associated with hyperammonemia and accumulation of ammonia in the brain and liver. In most cases the early symptoms appear within the first three days of life and include respiratory distress, feeding difficulty, hypotonia, lethargy, and death in untreated cases. Neonatal hyperammonemic coma lasting longer than 48 hours usually results in cortical atrophy and mental retardation. In late-onset otc deficiency the symptoms appear from 2 months to 44 years with normal appearance at birth, followed by irritability, vomiting, lethargy, seizures, delayed development, ataxia, and seizures. Accumulation of ammonium in the brain and blood usually follows a protein load or intermittent infection. Otc deficient patients are particularly sensitive to toxic effects of valproate.
ICD-10-CM E72.4 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 642 Inborn and other disorders of metabolism

Convert E72.4 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
Code annotations containing back-references to E72.4:
  • Type 1 Excludes: E72.2
    , E72.20
    ICD-10-CM Diagnosis Code E72.2

    Disorders of urea cycle metabolism

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • disorders of ornithine metabolism (E72.4)
    ICD-10-CM Diagnosis Code E72.20

    Disorder of urea cycle metabolism, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Hyperammonemia
    Type 1 Excludes
    • hyperammonemia-hyperornithinemia-homocitrullinemia syndrome E72.4
    • transient hyperammonemia of newborn (P74.6)
  • Type 2 Excludes: H31.2
    , H31.2
    ICD-10-CM Diagnosis Code H31.2

    Hereditary choroidal dystrophy

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 2 Excludes
    ICD-10-CM Diagnosis Code H31.2

    Hereditary choroidal dystrophy

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 2 Excludes

Diagnosis Index entries containing back-references to E72.4:
  • Deficiency, deficient
    • ornithine transcarbamylase E72.4
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 Billable/Specific Code
      • amino-acid E72.9
        ICD-10-CM Diagnosis Code E72.9

        Disorder of amino-acid metabolism, unspecified

          2016 2017 2018 2019 Billable/Specific Code
        • ornithine E72.4
      • ornithine E72.4
    • urea cycle metabolism E72.20
      ICD-10-CM Diagnosis Code E72.20

      Disorder of urea cycle metabolism, unspecified

        2016 2017 2018 2019 Billable/Specific Code
      Applicable To
      • Hyperammonemia
      Type 1 Excludes
      • hyperammonemia-hyperornithinemia-homocitrullinemia syndrome E72.4
      • transient hyperammonemia of newborn (P74.6)
      • ornithine transcarbamylase deficiency E72.4
    • ornithine metabolism E72.4
  • Disturbance(s) - see also Disease
    • metabolism E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 Billable/Specific Code
      • ornithine E72.4
  • Hyperaminoaciduria
    • ornithine E72.4
  • Hyperornithinemia E72.4
  • Ornithine metabolism disorder E72.4
  • Ornithinemia E72.4 (Type I) (Type II)
  • Syndrome - see also Disease
    • hyperammonemia-hyperornithinemia-homocitrullinemia E72.4

ICD-10-CM Codes Adjacent To E72.4
E72.11 Homocystinuria
E72.12 Methylenetetrahydrofolate reductase deficiency
E72.19 Other disorders of sulfur-bearing amino-acid metabolism
E72.2 Disorders of urea cycle metabolism
E72.20 Disorder of urea cycle metabolism, unspecified
E72.21 Argininemia
E72.22 Arginosuccinic aciduria
E72.23 Citrullinemia
E72.29 Other disorders of urea cycle metabolism
E72.3 Disorders of lysine and hydroxylysine metabolism
E72.4 Disorders of ornithine metabolism
E72.5 Disorders of glycine metabolism
E72.50 Disorder of glycine metabolism, unspecified
E72.51 Non-ketotic hyperglycinemia
E72.52 Trimethylaminuria
E72.53 Primary hyperoxaluria
E72.59 Other disorders of glycine metabolism
E72.8 Other specified disorders of amino-acid metabolism
E72.81 Disorders of gamma aminobutyric acid metabolism
E72.89 Other specified disorders of amino-acid metabolism
E72.9 Disorder of amino-acid metabolism, unspecified

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