2026 ICD-10-CM Diagnosis Code E74.00Glycogen storage disease, unspecified

ICD-10-CM Codes›E00–E89›E70-E88›E74

ICD-10-CM E74.00
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E74.00 is a billable ICD-10-CM diagnosis code for glycogen storage disease, unspecified. 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
E74.00
Billable Status
Yes — Valid for Submission
Code Describes
Glycogen storage disease, unspecified
Short Description
Glycogen storage disease, unspecified
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 CodeE74.00Glycogen storage disease, unspecified

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Cardiomyopathy due to storage disease
  • Dilated cardiomyopathy due to glycogen storage disease
  • Dilated cardiomyopathy due to metabolic disorder
  • Fatal congenital hypertrophic cardiomyopathy due to glycogen storage disease
  • Generalized glycogenosis
  • Glycogen storage disease
  • Hypertrophic cardiomyopathy due to glycogen storage disease
  • Periodontitis exacerbated by glycogen storage disease
  • Restrictive cardiomyopathy secondary to glycogen storage disease
  • Secondary restrictive cardiomyopathy

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.

    • Aglycogenosis
    • Disease, diseased
      • glycogen storage
    • Disease, diseased
      • glycogen storage
        • generalized
    • Disorder(of)
      • tubulo-interstitial (in)
        • glycogen storage disease
    • Myopathy
      • in (due to)
        • glycogen storage disease
    • Nephritis, nephritic(albuminuric) (azotemic) (congenital) (disseminated) (epithelial) (familial) (focal) (granulomatous) (hemorrhagic) (infantile) (nonsuppurative, excretory) (uremic)
      • tubulo-interstitial (in)
        • due to
          • glycogen storage disease

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

  • Fanconi Syndrome

    a hereditary or acquired form of generalized dysfunction of the proximal kidney tubule without primary involvement of the kidney glomerulus. it is usually characterized by the tubular wasting of nutrients and salts (glucose; amino acids; phosphates; and bicarbonates) resulting in hypokalemia; acidosis; hypercalciuria; and proteinuria.

  • Glycogen Storage Disease

    a group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. in some patients, prominent liver involvement is presented. in others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement.

  • Glycogen Storage Disease Type I

    an autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. increased concentrations of lactic acid and hyperlipidemia appear in the plasma. clinical gout often appears in early childhood.

  • Glycogen Storage Disease Type II

    an autosomal recessively inherited glycogen storage disease caused by glucan 1,4-alpha-glucosidase deficiency. large amounts of glycogen accumulate in the lysosomes of skeletal muscle (muscle, skeletal); heart; liver; spinal cord; and brain. three forms have been described: infantile, childhood, and adult. the infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy (cardiomyopathy, hypertrophic). the childhood form usually presents in the second year of life with proximal weakness and respiratory symptoms. the adult form consists of a slowly progressive proximal myopathy. (from muscle nerve 1995;3:s61-9; menkes, textbook of child neurology, 5th ed, pp73-4)

  • Glycogen Storage Disease Type IIb

    an x-linked dominant multisystem disorder resulting in cardiomyopathy, myopathy and intellectual disability. it is caused by mutation in the gene encoding lysosomal-associated membrane protein 2.

  • 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.

  • Glycogen Storage Disease Type IV

    an autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal glycogen with long outer branches. clinical features are muscle hypotonia and cirrhosis. death from liver disease usually occurs before age 2.

  • Glycogen Storage Disease Type V

    glycogenosis due to muscle phosphorylase deficiency. characterized by painful cramps following sustained exercise.

  • Glycogen Storage Disease Type VI

    a hepatic glycogen storage disease in which there is an apparent deficiency of hepatic phosphorylase (glycogen phosphorylase, liver form) activity.

  • Glycogen Storage Disease Type VII

    an autosomal recessive glycogen storage disease in which there is deficient expression of 6-phosphofructose 1-kinase in muscle (phosphofructokinase-1, muscle type) resulting in abnormal deposition of glycogen in muscle tissue. these patients have severe congenital muscular dystrophy and are exercise intolerant.

  • Glycogen Storage Disease Type VIII

    an x-linked recessive hepatic glycogen storage disease resulting from lack of expression of phosphorylase-b-kinase activity. symptoms are relatively mild; hepatomegaly, increased liver glycogen, and decreased leukocyte phosphorylase are present. liver shrinkage occurs in response to glucagon.

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 E74.00 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 E74.00Overview

Is E74.00 (Glycogen storage disease) a billable code?

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

What is the ICD-9 equivalent of E74.00?

Under the General Equivalence Mappings, glycogen storage disease, unspecified 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.