2026 ICD-10-CM Diagnosis Code E74.21Galactosemia

ICD-10-CM Codes›E00–E89›E70-E88›E74

ICD-10-CM E74.21
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E74.21 is a billable ICD-10-CM diagnosis code for galactosemia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). 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
E74.21
Billable Status
Yes — Valid for Submission
Code Describes
Galactosemia
Short Description
Galactosemia
Same as the full description in the CMS dataset.
Parent Code
E74.2
Disorders of galactose metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE74Other disorders of carbohydrate metabolism
This CodeE74.21Galactosemia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Classical galactosemia, heterozygous type
  • Classical galactosemia, homozygous Duarte-type
  • Classical galactosemia, homozygous Negro-type
  • Deficiency of galactose mutarotase
  • Deficiency of UDPglucose-hexose-1-phosphate uridylyltransferase
  • Deficiency of UTP-hexose-1-phosphate uridylyltransferase
  • Disorder of galactose metabolism
  • Early neonatal jaundice due to galactosemia
  • Erythrocyte uridine diphosphate galactose-4-epimerase deficiency
  • Galactose epimerase deficiency
  • Galactosemia
  • Generalized uridine diphosphate galactose-4-epimerase deficiency

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.

    • Cataract(cortical) (immature) (incipient)
      • associated with
        • galactosemia
    • Galactosemia(classic) (congenital)
    • Jaundice(yellow)
      • newborn
        • due to or associated with
          • galactosemia

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

  • Galactokinase Deficiency|GALK Deficiency|Galactosemia Type 2

    an autosomal recessive disorder caused by mutations in the galk1 gene. the disorder is characterized by an accumulation of galactose and galactitol secondary to the decreased conversion of galactose to galactose-1-phosphate by galactokinase. its major clinical symptom is the development of cataracts during the first weeks or months of life.

  • Galactosemia

    an autosomal recessive inherited metabolic disorder caused by mutations in the gale, galk1, and galt genes. it is characterized by deficiency of the enzymes responsible for the metabolism of galactose. signs and symptoms include intellectual disability, hepatomegaly, hepatic failure, and renal failure.

  • Transferase Deficiency Galactosemia|Transferase Deficient Galactosemia|Transferase-deficient Galactosemia

    a disorder characterized by the body's inability to metabolize galactose. this type of galactosemia is caused by germline mutations in the galt gene which leads to the inhibition in the activity of the enzyme galactose-1-phosphate uridyl transferase.

Patient EducationClinical

Carbohydrate 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. Chemicals in your digestive system (enzymes) break 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 tissues.

Read the full article at MedlinePlus

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

Convert E74.21 to ICD-9-CMHistory

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

ICD-9-CM
271.1 Galactosemia
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 E74.21Overview

Is E74.21 (Disorders of galactose metabolism) a billable code?

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

What is the ICD-9 equivalent of E74.21?

Under the General Equivalence Mappings, galactosemia converts to ICD-9-CM 271.1 (galactosemia). 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.