2026 ICD-10-CM Diagnosis Code E74.4Disorders of pyruvate metabolism and gluconeogenesis

ICD-10-CM Codes›E00–E89›E70-E88›E74

ICD-10-CM E74.4
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E74.4 is a billable ICD-10-CM diagnosis code for disorders of pyruvate metabolism and gluconeogenesis. 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
E74.4
Billable Status
Yes — Valid for Submission
Code Describes
Disorders of pyruvate metabolism and gluconeogenesis
Short Description
Disorders of pyruvate metabolism and gluconeogenesis
Same as the full description in the CMS dataset.
Parent Code
E74
Other disorders of carbohydrate metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE74Other disorders of carbohydrate metabolism
This CodeE74.4Disorders of pyruvate metabolism and gluconeogenesis

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Deficiency of acylphosphatase
  • Deficiency of dihydrolipoamide acetyltransferase
  • Deficiency of D-lactate dehydrogenase
  • Deficiency of glucose-1-phosphate phosphodismutase
  • Deficiency of lactate racemase
  • Deficiency of L-lactate dehydrogenase
  • Deficiency of pyruvate decarboxylase
  • Disorder of pyruvate metabolism and mitochondrial respiratory chain
  • Disorders of pyruvate metabolism and gluconeogenesis
  • Inborn error of pyruvate metabolism
  • Lactate dehydrogenase deficiency
  • Mitochondrial pyruvate carrier deficiency
  • Muscle L-lactate dehydrogenase deficiency
  • Phosphoenolpyruvate carboxykinase deficiency
  • Pyruvate carboxylase deficiency
  • Pyruvate dehydrogenase complex deficiency
  • Pyruvate dehydrogenase complex E1 beta subunit deficiency
  • Pyruvate dehydrogenase complex E1-alpha subunit deficiency
  • Pyruvate dehydrogenase complex E2 subunit deficiency
  • Pyruvate dehydrogenase phosphatase deficiency

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Deficiency of phosphoenolpyruvate carboxykinase
  • Deficiency of pyruvate carboxylase
  • Deficiency of pyruvate dehydrogenase

Type 1 Excludes

  • disorders of pyruvate metabolism and gluconeogenesis with anemia D55
  • Leigh's syndrome G31.82

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.

    • Deficiency, deficient
      • phosphoenolpyruvate carboxykinase
    • Deficiency, deficient
      • pyruvate
        • carboxylase
    • Deficiency, deficient
      • pyruvate
        • dehydrogenase
    • Disorder(of)
      • gluconeogenesis
    • Disorder(of)
      • metabolism NOS
        • pyruvate
    • Disorder(of)
      • pyruvate metabolism

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

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 E74.4 to ICD-9-CMHistory

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

ICD-9-CM
271.0 Glycogenosis
Approximate The match is approximate rather than exact.
ICD-9-CM
271.8 Dis carbohydr metab NEC
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 E74.4Overview

Is E74.4 (Other disorders of carbohydrate metabolism) a billable code?

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

What is the ICD-9 equivalent of E74.4?

Under the General Equivalence Mappings, disorders of pyruvate metabolism and gluconeogenesis converts to ICD-9-CM 271.0 (glycogenosis) and 271.8 (dis carbohydr metab NEC). 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.