2026 ICD-10-CM Diagnosis Code E74.03Cori disease

ICD-10-CM Codes›E00–E89›E70-E88›E74

ICD-10-CM E74.03
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E74.03 is a billable ICD-10-CM diagnosis code for cori disease. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis. 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
E74.03
Billable Status
Yes — Valid for Submission
Code Describes
Cori disease
Short Description
Cori disease
Same as the full description in the CMS dataset.
Parent Code
E74.0
Glycogen storage disease

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE74Other disorders of carbohydrate metabolism
This CodeE74.03Cori disease

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to HOIP deficiency
  • Glycogen storage disease type III
  • Glycogen storage disease, hepatic form
  • Glycogen storage disease, muscular form

Tabular List NotesGuidance

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

Inclusion Terms

  • Forbes disease
  • Type III glycogen storage disease

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.

    • Amylopectinosis(brancher enzyme deficiency)
    • Cori's disease(glycogen storage)
    • Deficiency, deficient
      • brancher enzyme (amylopectinosis)
    • Deficiency, deficient
      • debrancher enzyme (limit dextrinosis)
    • Dextrinosis, limit(debrancher enzyme deficiency)
    • Disease, diseased
      • Cori's (glycogenosis III)
    • Disease, diseased
      • Forbes' (glycogenosis III)
    • Disease, diseased
      • glycogen storage
        • Cori's
    • Disease, diseased
      • glycogen storage
        • Forbes'
    • Disease, diseased
      • glycogen storage
        • type III
    • Forbes' glycogen storage disease
    • Infiltrate, infiltration
      • liver
        • glycogen

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

  • Glycogen Storage Disease Type III

    an autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). the clinical course of the disease is similar to that of glycogen storage disease type i, but milder. massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. six subgroups have been identified, with subgroups type iiia and type iiib being the most prevalent.

  • AGL wt Allele|Amylo-1, 6-Glucosidase, 4-Alpha-Glucanotransferase Gene|Amylo-Alpha-1, 6-Glucosidase, 4-Alpha-Glucanotransferase Gene|Amylo-Alpha-1,6-Glucosidase and 4-Alpha-Glucanotransferase wt Allele|GDE|Glycogen Debrancher Enzyme Gene|Glycogen Debranching Protein Gene|Glycogen Storage Disease Type III Gene

    human agl wild-type allele is located in the vicinity of 1p21.2 and is approximately 75 kb in length. this allele, which encodes glycogen debranching enzyme protein, plays a role in glycogen degradation. mutations in the gene are associated with glycogen storage disease 3.

  • Glycogen Storage Disease Type III

    an autosomal recessive inherited type of glycogen storage disease caused by deficiency of the glycogen debranching enzyme. it results in the accumulation of structurally abnormal glycogen in the heart, skeletal muscles, and/or liver.

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 E74.03 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.

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 E74.03Overview

Is E74.03 (Glycogen storage disease) a billable code?

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

What is the ICD-9 equivalent of E74.03?

Under the General Equivalence Mappings, cori disease converts to ICD-9-CM 271.0 (glycogenosis). 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.