ICD-10-CM Code E74.00

Glycogen storage disease, unspecified

Version 2020 Billable Code

Valid for Submission

E74.00 is a billable code used to specify a medical diagnosis of glycogen storage disease, unspecified. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code E74.00 might also be used to specify conditions or terms like dilated cardiomyopathy secondary to glycogen storage disease, generalized glycogenosis, glycogen storage disease, periodontitis co-occurrent with genetic disorder, periodontitis co-occurrent with glycogen storage disease, restrictive cardiomyopathy secondary to glycogen storage disease, etc

Short Description:Glycogen storage disease, unspecified
Long Description:Glycogen storage disease, unspecified

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E74.00 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Dilated cardiomyopathy secondary to glycogen storage disease
  • Generalized glycogenosis
  • Glycogen storage disease
  • Periodontitis co-occurrent with genetic disorder
  • Periodontitis co-occurrent with glycogen storage disease
  • Restrictive cardiomyopathy secondary to glycogen storage disease
  • Secondary restrictive cardiomyopathy
  • Splenomegaly co-occurrent and due to storage disease

Clinical Information

  • 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 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-. 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 II-. an autosomal recessively inherited glycogen storage disease caused by glucan 14 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 III-. an autosomal recessive metabolic disorder due to deficient expression of amylo 16 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 14 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.
  • 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.

Convert E74.00 to ICD-9

  • 271.0 - Glycogenosis (Approximate Flag)

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Other disorders of carbohydrate metabolism (E74)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients

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. If you have a metabolic disorder, something goes wrong with this process.

Carbohydrate metabolism disorders are a group of metabolic disorders. Normally your enzymes break carbohydrates down into glucose (a type of sugar). If you have one of these disorders, you may not have enough enzymes to break down the carbohydrates. Or the enzymes may not work properly. This causes a harmful amount of sugar to build up in your body. That can lead to health problems, some of which can be serious. Some of the disorders are fatal.

These disorders are inherited. Newborn babies get screened for many of them, using blood tests. If there is a family history of one of these disorders, parents can get genetic testing to see whether they carry the gene. Other genetic tests can tell whether the fetus has the disorder or carries the gene for the disorder.

Treatments may include special diets, supplements, and medicines. Some babies may also need additional treatments, if there are complications. For some disorders, there is no cure, but treatments may help with symptoms.

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