2026 ICD-10-CM Diagnosis Code E72.23Citrullinemia

ICD-10-CM Codes›E00–E89›E70-E88›E72

ICD-10-CM E72.23
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E72.23 is a billable ICD-10-CM diagnosis code for citrullinemia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as citrin deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E72.23
Billable Status
Yes — Valid for Submission
Code Describes
Citrullinemia
Short Description
Citrullinemia
Same as the full description in the CMS dataset.
Parent Code
E72.2
Disorders of urea cycle metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE72Other disorders of amino-acid metabolism
This CodeE72.23Citrullinemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Citrin deficiency
  • Citrullinemia
  • Citrullinemia type I
  • Citrullinemia type II
  • Citrullinemia, neonatal type
  • Citrullinemia, subacute type
  • Citrullinuria
  • Deficiency of argininosuccinate synthase
  • Late-onset citrullinemia type I
  • Neonatal metabolic acidemia

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Citrullinemia
    • Citrullinuria
    • Disorder(of)
      • urea cycle metabolism
        • citrullinemia
    • Disturbance(s)
      • metabolism
        • citrulline

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Citrullinemia

    a group of diseases related to a deficiency of the enzyme argininosuccinate synthase which causes an elevation of serum levels of citrulline. in neonates, clinical manifestations include lethargy, hypotonia, and seizures. milder forms also occur. childhood and adult forms may present with recurrent episodes of intermittent weakness, lethargy, ataxia, behavioral changes, and dysarthria. (from menkes, textbook of child neurology, 5th ed, p49)

  • Citrullinemia

    a rare autosomal recessive inherited disorder caused by mutations in the ass1 and slc25a13 genes. it is characterized by a defective urea cycle, resulting in the accumulation of ammonia and other toxic substances in the blood.

  • Citrullinemia Type I|Argininosuccinate Synthetase Deficiency|CTLN1

    an autosomal recessive sub-type of citrullinemia caused by mutation(s) in the ass1 gene, encoding argininosuccinate synthetase.

  • Citrullinemia Type II|CTLN2

    an autosomal recessive sub-type of citrullinemia caused by mutation(s) in the slc25a13 gene, encoding calcium-binding mitochondrial carrier protein aralar2.

  • SLC25A13 wt Allele|ARALAR-Related Gene 2|ARALAR2|CITRIN|CTLN2|Citrullinemia Type II Gene|NICCD|Solute Carrier Family 25 (Aspartate/Glutamate Carrier), Member 13 Gene|Solute Carrier Family 25 (Citrin), Member 13 Gene|Solute Carrier Family 25 Member 13 wt Allele|Solute Carrier Family 25, Member 13 (Citrin) Gene

    human slc25a13 wild-type allele is located in the vicinity of 7q21.3 and is approximately 202 kb in length. this allele, which encodes electrogenic aspartate/glutamate antiporter slc25a13, mitochondrial protein, plays a role in the malate-aspartate shuttle. loss of function mutations in the gene are associated with citrullinemia type 2.

Patient EducationClinical

Metabolic Disorders

Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E72.23 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
270.6 Dis urea cycle metabol
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E72.23Overview

Is E72.23 (Disorders of urea cycle metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report citrullinemia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E72.23?

Under the General Equivalence Mappings, citrullinemia converts to ICD-9-CM 270.6 (dis urea cycle metabol). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.