ICD-10-CM Code E72.3

Disorders of lysine and hydroxylysine metabolism

Version 2021 Billable Code

Valid for Submission

E72.3 is a billable code used to specify a medical diagnosis of disorders of lysine and hydroxylysine metabolism. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code E72.3 might also be used to specify conditions or terms like 2-aminoadipic 2-oxoadipic aciduria, 2-hydroxyglutaric aciduria, 2-hydroxyglutaric aciduria, 2-ketoadipic acidemia, d-2 glutaric aciduria, d-2 glutaric aciduria, etc

Short Description:Disorders of lysine and hydroxylysine metabolism
Long Description:Disorders of lysine and hydroxylysine metabolism

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code E72.3:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Glutaric aciduria NOS
  • Glutaric aciduria (type I)
  • Hydroxylysinemia
  • Hyperlysinemia

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E72.3 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • 2-aminoadipic 2-oxoadipic aciduria
  • 2-hydroxyglutaric aciduria
  • 2-hydroxyglutaric aciduria
  • 2-Ketoadipic acidemia
  • D-2 glutaric aciduria
  • D-2 glutaric aciduria
  • Deficiency of lysine racemase
  • Disorder of lysine and hydroxylysine metabolism
  • Disorder of lysine AND/OR hydroxylysine metabolism
  • Enchondromatosis
  • Glutaric aciduria
  • Glutaric aciduria, type 1
  • Glutaryl-CoA dehydrogenase deficiency
  • Hyperlysinemia
  • L-2 glutaric aciduria
  • Lysine intolerance
  • Lysinuric protein intolerance
  • Lysinuric protein intolerance, type 1
  • Lysinuric protein intolerance, type 2
  • Metaphyseal chondromatosis co-occurrent with D-2 hydroxyglutaric aciduria
  • Periodic hyperlysinemia
  • Periodic hyperlysinemia with hyperammonemia
  • Persistent hyperlysinemia
  • Saccharopinuria
  • Seizures and intellectual disability due to hydroxylysinuria

Convert E72.3 to ICD-9

  • 270.7 - Straig amin-acid met NEC (Approximate Flag)

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Other disorders of amino-acid metabolism (E72)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients

Amino Acid Metabolism Disorders

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Your digestive system breaks the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body. If you have a metabolic disorder, something goes wrong with this process.

One group of these disorders is amino acid metabolism disorders. They include phenylketonuria (PKU) and maple syrup urine disease. Amino acids are "building blocks" that join together to form proteins. If you have one of these disorders, your body may have trouble breaking down certain amino acids. Or there may be a problem getting the amino acids into your cells. These problems cause a buildup of harmful substances in your body. That can lead to serious, sometimes life-threatening, health problems.

These disorders are usually inherited. A baby who is born with one may not have any symptoms right away. Because the disorders can be so serious, early diagnosis and treatment are critical. Newborn babies get screened for many of them, using blood tests.

Treatments may include special diets, medicines, and supplements. Some babies may also need additional treatments if there are complications.

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Hyperlysinemia Hyperlysinemia is an inherited condition characterized by elevated blood levels of the amino acid lysine, a building block of most proteins. Hyperlysinemia is caused by the shortage (deficiency) of the enzyme that breaks down lysine. Hyperlysinemia typically causes no health problems, and most people with elevated lysine levels are unaware that they have this condition. Rarely, people with hyperlysinemia have intellectual disability or behavioral problems. It is not clear whether these problems are due to hyperlysinemia or another cause.
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