2020 ICD-10-CM Diagnosis Code E72.3

Disorders of lysine and hydroxylysine metabolism

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E72.3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E72.3 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E72.3 - other international versions of ICD-10 E72.3 may differ.
Applicable To
  • Glutaric aciduria NOS
  • Glutaric aciduria (type I)
  • Hydroxylysinemia
  • Hyperlysinemia
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 E72.3. 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.
  • glutaric aciduria type II (
    ICD-10-CM Diagnosis Code E71.313

    Glutaric aciduria type II

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Glutaric aciduria type II A
    • Glutaric aciduria type II B
    • Glutaric aciduria type II C
    Type 1 Excludes
    • glutaric aciduria (type 1) NOS (E72.3)
    E71.313
    )
  • Refsum's disease (
    ICD-10-CM Diagnosis Code G60.1

    Refsum's disease

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Infantile Refsum disease
    G60.1
    )
  • Zellweger syndrome (
    ICD-10-CM Diagnosis Code E71.510

    Zellweger syndrome

      2016 2017 2018 2019 2020 Billable/Specific Code
    E71.510
    )
The following code(s) above E72.3 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 E72.3:
  • E00-E89
    2020 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
    2020 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 2020 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
  • 2-ketoadipic acidemia
  • Aciduria, ketoadipic
  • Disorder of lysine and hydroxylysine metabolism
  • Glutaric acidemia, type 1
  • Glutaric aciduria, type 1
  • Lysine and hydroxylysine metabolism disorder
Clinical Information
  • A group of inherited metabolic disorders which have in common elevations of serum lysine levels. Enzyme deficiencies of alpha-aminoadipic semialdehyde dehydrogenase and the saccharopine dehydrogenases have been associated with hyperlysinemia. Clinical manifestations include mental retardation, recurrent emesis, hypotonia, lethargy, diarrhea, and developmental delay. (from menkes, textbook of child neurology, 5th ed, p56)
  • A rare autosomal recessive inherited metabolic disorder caused by deficiency of the enzyme glutaryl-coa dehydrogenase. It is characterized by abnormalities in the metabolism of lysine, hydroxylysine, and tryptophan that result in the accumulation and urinary excretion of glutaric acid. Patients present with brain atrophy, microcephaly, and acute dystonia.
ICD-10-CM E72.3 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E72.3 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
Code annotations containing back-references to E72.3:
  • Type 1 Excludes: E71.313
    ICD-10-CM Diagnosis Code E71.313

    Glutaric aciduria type II

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Glutaric aciduria type II A
    • Glutaric aciduria type II B
    • Glutaric aciduria type II C
    Type 1 Excludes
    • glutaric aciduria (type 1) NOS (E72.3)

Diagnosis Index entries containing back-references to E72.3:
  • Acidemia E87.2
    ICD-10-CM Diagnosis Code E87.2

    Acidosis

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Acidosis NOS
    • Lactic acidosis
    • Metabolic acidosis
    • Respiratory acidosis
    Type 1 Excludes
    • diabetic acidosis - see categories E08-E10, E13 with ketoacidosis
    • pipecolic E72.3
  • Aciduria
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • amino-acid E72.9
        ICD-10-CM Diagnosis Code E72.9

        Disorder of amino-acid metabolism, unspecified

          2016 2017 2018 2019 2020 Billable/Specific Code
        • hydroxylysine E72.3
        • lysine E72.3
    • lysine and hydroxylysine metabolism E72.3
  • Disturbance(s) - see also Disease
    • metabolism E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • hydroxylysine E72.3
      • lysine E72.3
  • Glutaric aciduria E72.3
  • Hydroxylysinemia E72.3
  • Hyperaminoaciduria
    • lysine E72.3
  • Hyperlysinemia E72.3
  • Intolerance
    • lysine E72.3
  • Lysine and hydroxylysine metabolism disorder E72.3
  • Saccharopinuria E72.3

ICD-10-CM Codes Adjacent To E72.3
E72.10 …… unspecified
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

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